Resp/Cardio Flashcards
What is the cardiac lesion represented in this CXR?
TAPVD
What is the cardiac lesion represented in this cxr?
Tetralogy of fallot
What are the characteristics of Marfan syndrome?
Fibrillin gene defect
AUTOSOMINAL DOMINANT
Sx: thumb sign, pectus excavatum, scoliosis, long arms
Dilatation of the ascending Ao and MVP (mid systolic click)
What does an ASD murmur sound like?
Low pitched systolic ehection murmur at base
Fixed split S2
What does an AS murmur sound like?
SEM radiating to neck
What are the characteristics of cardiac syncope?
- Little or no prodrome
- Prolonged LOC
- Exercise induced
- Fright/startle induced
- Associated chest apin or palpitations
- History of cardiac disease: AS, PHTN
- FHx for: Long QTc, arrhythmia syndromes, devices, sudden death, cardiomyopathy
When does Torsades des Pointes occur?
Long QT and hypomagnesemia
What are the clues on history to long QT syndrome?
Unusual sz, palpitations while swimming
Deafness
FHx of sudden death, unexplained MVA, drownings, deafness
What are the causes of long QT interval?
Low Ca
Low Mg
Low K
Drugs (TCAs)
What is WPW?
Wolff Parkinson White
Accessory AV pathway allows for “early” depolarization of ventricles
Ass with ebsteins and CCTGA
Can cause SVT and sudden death
What is the first step of the SV palliation?
Glenn or BT shunt at 4-6 months
- SVC to RPA
- Expected sats 75-85%
Fontan at 2-y4 years
- IVC to RPA
- Expected sats usually >90%
What are the recommendations for antiplatelet therapy in Kawasaki disease?
If no aneurysms or ectasia or resolve within 6-8 weeks, discontinued in 6-8 weeks
If 1 large coronary artery aneurysm or complex aneurysms, long term antiplatelet therapy is recommended
What are the general guidelines for SBE PPx?
High risk lesion + high risk procedure
What qualifies as a high risk lesion?
- Cyanotic CHD, not repairs, shunts
- CHD repaired with prosthetic material
- CHD with residual defects
What is considered a high risk procedure?
Dental procedure where the gums or lining of mouth likely to be injured
Gut or GU surgery through infected area
Things that do not need antibiotics
Injections of anaesthetic to mouth
Loss of baby teeth
Accidental injury to lips/gums
Nosebleeds
Routine placements or adjustment of braces
Deliveries
Most surgeries and procedures
What are the cynaotic CHD lesions?
6Ts: TGA, TOF, TA, TAPVR, TA, “TINGLE” ventricle
2As: PA, Ebsteins anomaly
What are the lesions that cause CHF in the first weeks of life?
OBSTRUCTION
- HLHS
- Severe AS
- Coarctation
- Asphyxia
- Severe MR, TR
- Uncontrolled tachycardias
What are the lesions that cause CHF in weeks 2-6?
Left to right shunts:
VSD
AVSD
PDA
What are the lesions that cause CHF inolder children?
Pump failure
Dilated cardiomyopathy
Myocarditis
Tachycardias
What are the three cardinal signs of CHF in infants?
Tachycardia
Tachypnea
Hepatomegaly
What are the signs of infective endocarditis?
Janeway lesion
Embolus
Splinter hemorrhages
Oslers node: PAINFUL
What is rheumatic fever?
Occurs after GAS infection
Pancarditis: aschoff bodies (collection of immunological cells and fibrinoid material around a central necrosis)
What are the diagnostic criteria for rheumatic fever?
Major: (2)
- Carditis
- Polyarthritis
- Chorea
- Subcutaneous nodules
- EM
Minor:
- Fever
- Arthralgia
- Prolonged PR
- Elevated ESR, CRP
What are the complications of foreign body?
Recurrent pneumonia
Bronchiectasis
Cardiac arrest and death
In what findings MUST you r/o cystic fibrosis?
Meconium ileus
HypoNa, hypoCl metabolic alkalosis
Recurrent rectal prolapse
Clubbing
Bronciectasis
Nasal polyps
Pseudomonas colonization
What is the test positive in a sweat chloride?
>60 meQ/L
What are the prognostic factors in CF?
Male?female
Lung disease:FEV1, Burkohderia, PTX
Nutritional status: Wt/Ht, DM
How does primary ciliary dyskinesia?
Year round daily wet cough
Year round nasal congestion
Recurrent otitis media
Neonatal respiratory distress
Laterality defects
What qualifies as asthma control?
Daytime Sx < 4 days/week
Nighttime Sx <1 night/week
Physical activity normal
Exacerbation: mild, infrequency
Absence: none
SABA use < 4 dose/week
FEV1 >90% personal best
How do you diagnose asthma?
Clinical symptoms
+
Objective evidence: spirometry, peakflow, methacholine challenge
What are the sequalae of OSA?
Neurocognitive: behavioural, attention, schoolperformance, developement
Cardiovascular: HTN, cor pulmonale
FTT
Inflammatory
Qualityof life
How do diagnose OSA?
Polysomnography
What are the treatment modalities for OSA?
Non-pharmacologic: Nasal and sleep hygiene
Pharmacologic: intranasal corticosteriods, montelukast
Surgical: T&A
Weight loss
CPAP
What is the most common CHD?
VSD 25-30%, ASD +PDA 6-8%
What is the hyperoxia test?
If PaO2 rise above 150 mm during 100% O2 administration then a shunt lesion is excluded
What % of small VSD will close?
75% in the first 2 years of life
What are the potential complications of a VSD?
Endocarditis
Aortic regurg
Subaortic stenosis
RVOTO
LV TO RA shunting: Gerbode defect
What are the indications for surgery with a VSD?
< 6 months with uncontrolled CHF despite max medical RX
Children with significatn shunt and increased PA pressures
Children with significant shunt and N pressures
Subpulmonic and membranous defects
What are the three types of ASD?
Primum (15%)
Secundum (75%)
Sinus venosus (10%)
What are the features of an ASD?
Grade 3 SEM at ULSB with widely split S2
Age 3-4 years
What are the four features of tetralogy of fallot?
PS
Overriding aorta
RVH
VSD
What syndromes are associated with TOF?
T21, DiGeorge, Alagille
If you find long Qt, what should you check
Ca, Mg, K, Drugs (TCAs)
How do you approach SVT?
Stable: Vagal, Adenosine
Unstable: Syn cardio 0.5-1 J/kg
What is the description of a stills murmur?
VIbratory LLSB SEM, changes with position
DDx: SubAo stenosis, Small VSD
What is the description of a venous hum?
Infraclavicular hum, continuous R>L
DDx: PDA
What is the differential for a widely split S2?
AS, PS, Ebstein, TAPVR, RBBB
DDX for a wide pulse pressure?
PDA
Ao insuff
AVM
Vasodilation
What defect is associated with alpha 1 AT deficiency?
PiZZ
Will present as liver disease in children
What are the characteristics of CCAM?
Cystic adenomatoid malformation CCAM
Hamartomatous or dysplastic lung tissue mixed with normal lung
Presents in infancy
What are the characteristics of congenital lobar emphysema?
Presents at 6 months: resp distress
HYperinflation of affect lobe with atelectasis of other
What is the acronym to remember the compotents of CF?
CF PANCREAS
Chronic cough and wheezing
FTT
Pancreatic insufficiency
Alkalosis and HypoNa
Neonatal intestinal obstruction Nasal polyps
Clubbing
Rectal prolapse
Electrolytes elevation in sweat
Absence vas derens
Sputum with stap a or pseud
What is the progression of bugs in CF?
S aureus, H flu, pseudo aeroginosa, B cepacia
What are compotents of routine care for CF?
Chest physio + bronchodilatory BID
Balanced high calorie diet
Pancreatic supplemnts
ADEK replacement
Osteoporos monitoring
GERD monitoring
Whatis kartagener syndrome?
- Situs inversus (25% will have PCD)
- Chronic sinusitis and otitis
- Airway d/po leading to bronchiectasis
What is the most common cause of chest pain?
MSK
Cardiac <1%
What are the common MSK causes of CP?
Strained msucles
Costochondritis
Tietze syndrome
Precordial catch syndrome
What is tietze syndrome?
Localized form of costochondritis usually involving junst one costochondral junction, tender swollen mass palpable at the site
Costochrondritis is usually multiple sites without swelling
What is precordial catch syndrome?
Texidor twinge
Sudden onset CP in children, very localized, occurs most commonly over the left sternal border
At rest without trigger
Usuallys 30s to 3 minutes
What is the difference between a vascular ring and sling?
Ring: trachea and/or esophagus is encircled by aberrant vascular strctures
Sling: compressions by nonencircling vessels
What are the two types of complete vascular rings?
- Double Ao arch
- Right Ao arch with left ligamentum arteriosum
What are the cardiac causes of sudden death?
- HOCM
- Anomalies of cornoary arteries
- Margan
- Arrhythmogenic right ventricular dysplasia (ARVD)
- Brugada
- Prolonged QTC
What is commotio cordis?
Arrhythmia as a result of blunt, nonpenetrating direct blow to the chest
VF occurs during vulnerable phase of repolarization
CPR +Defib
What are the signs of a Coarct in an older children?
Differential BP
Systolic murmur in the back
SystolicHTN in the upper extremities
Diminished femorals
What are two neuromuscular conditions in which cardio consult is recommended?
DMD (Dystrophin)-Cardiomyopathy
Friedrich ataxia (Frataxin)-Cardiomyopathy, A fib/fliutter
What CHD gives you decreased lung markings?
TOF
Pulmonary Atresia
Tricuspid atresia
Ebstein
What lesions given with increased pulmonary markings?
Transposition
TAPVR
Truncus arteriosis
What are the classic chest xray findings in CHD?
Boot: TOF
Egg: TGA
Snowman: TAPVR
Rib notching: Coarctation
In what two conditions is a diminished murmur bad?
TOF: worsening RVOTO
VSD: eisenmenger syndrome
At what age do you need to investigate an PPS mrumur?
6 months or older
What is unique about a baby ECG?
RV dominance
T waves upright in chest leads, invert at 7 days until 12-13 years
What are the two most common mechanisms of SVT?
- WPW
- AV nodal reentry
When should you suspect myocarditis?
Unexplained CHF
Tachycardia out of proportion to fever, tachypnea, quiet precordium, muffled HS, gallop rhythm without murmur, HM
What are the clinical signs of pericarditis?
CP, fever, cough, palpitations
Friction rub, pallor, pulsus paradoxus
Pt sitting up and leaning forward
What are the side effects of indomethacin in the neonate?
- Decreased renal function
- Hyponatremia
- Platelet dysfunction
- GI blood loss
What are the side effects of PGE?
Apnea, fever, cutaneous flushing, seizures, hypotension, bradycardia
What do alpha receptors do?
Vascular smooth muscle, vasoconstriction
What do beta 1 receptors do?
Myocardial smooth msucle, increase myocardial contractility (inotropic), cardiac rate (chronotrophic) and AV conduction (dromotophic)
What do B2 receptors do?
Vascular smooth muscle, vasodilation
What do doapminergic receptors do?
Renal and mesenteric vascular smooth muscle
Vasodilation
What is the effect of sitting up on common innocent murmurs?
Venous hum= increased
Vinratory=decreases
What is a BT shunt?
Anastomosis between subclavian artery and pulmonary artery
What are the common rhythm issues post Fontan?
Loss of sinus
INtra-atrial reentrant tachycardia
Which lesion does protein losing enteropathy occur after correction?
Fontan
Abnormal flow to mesenteric vasculature
How common is allergic rhinitis?
40% of children experience
If both parents are asthma, what is the risk that their child will have asthma?
60%
What is the most common cause of infantile stridor?
Laryngomalacia
What is the basic defect in CF?
Defect in the CF transmembrane conductance regulator (CFTR) protein
Regulates chloride and sodium transfer across the apical membrane of the epithetial cells
What are Kussmaul respirations?
Deep, slow resps with prolonged exhalation
What are Cheyne-stokes resps?
Crescendo-descendo respira with alternating apnea
What are biot resps?
AKA ataxic breathing
Unpredictable irregularity in respirations
Most common CHD?
VSD
What is the medical management of a VSD prior to surgery?
- Nutrition
- Diuretics
- ACEi
What is the most common type of ASD?
Secundum 75%
What are the risks of non closure of an ASD?
- Right heart failure
- Embolism
- Arrhythmia
- PVD
What is the consequence of outflow tract obstruction?
Concentric ventricular hypertrophy from pressure overload
Initially adapative to maintain CO however becomes maladaptive increasing the stiffness of the chambers and resulting in diastolic dysfunction
What is william syndrome?
Hypercalcemia
Elfin features
Supravalvular AS
PA Branch stenosis
MR
What is situs solitus?
Normal viscera, lungs and atrial position
What is situs inversus
Abdo, atria and lungs and reversed
When does perfusion of the coronary arteries occur?
DIASTOLE
What are the strategies to maximize CO in CHF?
- Afterload reduction
- Preload reudction
- Sympathetic inhibition
- Cardiac remodelling prevention
- Inotrophy
What are the common viruses that cause percarditis?
Coxsackie
Echovirus
Adenovirus
EBV
Influenza
HIV
What is the benefit of FRC?
Functional residual capacity
Volume of air left in the lungs after tidal expiration
Buffer, minimizing PaO2 and PaCO2 changes during inspiration and expiration
Improved with PEEP
What is the major determinant of FRC?
Chest wall compliance
FRC= Outward elastic recoil= inward lung recoil
Infants, more compliant chest wall therefore lower FRC
Why are infants at a disadvantage?
- CNS depression =chest wall retraction
- Poor lung compliance= lung recoil, loss of FRC
- Compliant chest wall= loss of FRC
What is elastance?
Property of a substance to oppose deformation or stretching
Change in pressure over change in volume
Enbales return to original state
What is compliance
repicrocal of elastance
Distensibility
Change in volume/change in pressure
What is resistance?
Amount of pressure required to generate flow of gas across airways
R= 8 x length x viscosity/R4
How do you classify size of pleural effusions on CXR?
1/2 height if hemithorax = large
What are the signs of a pulmonary exacerbation in CF?
- Increased cough/sputum
- Hemoptysis
- Anorexia and weight loss
How do you interpret a PFT?
- Look at flow-volume curve: is it reproducible (aka straight exp line vs squiggly)?
- Look at effort curve: kids 12 should exhale for at least 10 seconds
- Look at FEV1: mild (70-79), moderate (60-69), severe (50-59), very severe (12% change is significant, CHEO uses > 10%)
- Compare FEV1 to last PFT: has it changed?
What are complications of Cystic Fibrosis from head to toe?
- Nasal polyps
- Chronic sinusitis
- Chronic cough
- Bronchiectasis
- Chronic colonization with staph, hemophilus, pseudomonas, burkholderia seppacia
- GERD secondary to obstructive lung disease, hyperinflation and diaphragms pushing on stomach
- Meconium ileus
- Failure to thrive
- Pancreatic insufficiency (with delta F508 mutation) –> Fat soluble vitamin deficiency, steatorrhea, eventual diabetes
- Liver cirrhosis - from thickened bile
- Congenital absence of vas deferens in males
- Infertility in both males and females - in females, thick cervical secretions or secondary amenorrhea from malnutrition
- Rectal prolapse - from chronic diarrhea, malnutrition and weak musculature
- Clubbing
- Cor pulmonary from pulmonary hypertension
What is the pattern of colonization/chronic infection in CF patients?
Babies - staph aureus
Toddlers - hemophilus b influenza
School age children - pseudomonas
Teenagers - Burkholderia seppacia, stenotrophomonas maltophilia
This is prognosticator important: if a baby is already infected with pseudomonas, this means their CF is advanced; if a teenager is only colonized with h.flu, doing well
What is the differential diagnosis of wheeze? (10)
- Asthma
- Aspiration penumonitis: ask about coughing/choking with feeds, reflux symptoms, rule out TEF
- BPD
- Bronchiolitis obliterans: chronic wheezing after adenovirus infection
- Congenital cardiac lesion: left to right shunts causing increased pulmonary blood flow
- Cystic fibrosis: ask about recurrent diarrhea, failure to thrive
- Foreign body aspiration
- Vascular rings/slings
- Airway malformations: tracheomalacia, lung cysts, mediastinal mass
- Primary ciliary dyskinesia: ask about recurrent sinusitis, ear infections
What are the classic ECG findings in a patient with pulmonary embolism?
Most common: sinus tachycardia
Most characteristic: S1-Q3-T3
-large S wave in lead 1, large Q wave in lead 3, inverted T wave in lead 3
Also can have RBBB and right axis deviation since right heart is pumping heart against a blocked PA
What is the most important cause of a false negative on a sweat chloride test?
Edema! (ie. hypoalbuminemia)
-other causes: malnutrition, hyponatremia (not enough sweat to collect)
What is ABPA?
- diagnosis?
- treatment?
Allergic bronchopulmonary aspergillosis: allergic hypersensitivity reaction to aspergillus in lungs
- occurs most commonly in CF pts, NOT responsive to antibiotics
- diagnosis: PERIPHERAL EOSINOPHILIA, sputum culture with branching hyphae, RUST-COLORED SPUTUM, proximal bronchiectasis on CXR
- Treatment: steroids and anti-fungals (voriconazole) x 6 wks
What is the inheritance pattern of cystic fibrosis?
Autosomal recessive
Which chromosome has the gene for cystic fibrosis?
-most common mutation?
Chromosome 7 (encodes CFTR protein) = deltaF508 mutation
- chloride channel on surface of epithelial cells responsible for movement of salt and water across cell membranes
- essentially have overactive sodium pumps and impermeable chloride channels = thus, Na can get out of tubule structures and draw water out with it, leaving thick secretions left behind
- most common mutation delta F508 mutation
What is considered a positive sweat chloride test?
CF test positive if > 60 mEQ/L
-negative if
What are the 3 most important interventions in CF that affects prognosis?
- Nutrition
- Chest physiotherapy
- Antibiotics
What are the 6 causes of hypoxia?
- V/Q mismatch
- Shunt
- Hypoventilation
- Low FiO2
- Diffusion barrier
- Abnormal Hgb saturation curve
What are 3 complications of bronchiectasis?
- Increased risk of infection secondary to trapping of secretions
- Increased risk of pneumothorax
- Increased risk of pulmonary hemorrhage (and thus hemoptysis)
What is the differential for:
- diffuse wheezing
- localized wheezing
Diffuse wheezing:
- CF
- Asthma
- Bronchiolitis
- Anaphylaxis with resp involvement
- GERD
Localized wheezing:
- Foreign body
- Extrinsic compression by mass
- Bronchomalacia
What are the complications of chronic recurrent aspiration? (3)
- Granuloma formation
- Interstitial fibrosis
- Lipoid pneumonia = through oral or nasal administration of animal or vegetable oils to treat childhood illnesses as a folk remedy
What is the most common underlying problem associated with recurrent pneumonias in hospitalized children?
-defn of recurrent pneumonia?
Oropharyngal incoordination/dysphagia leading to chronic recurrent aspiration
- 2nd most common is probably GERD
- defn of recurrent pneumonia: 2 or more pneumonias in 1 year OR 3 or more in a lifetime
What are the differences between a barium swallow, swallowing study with video fluoroscopy, and a gastroesophageal scintigraphy (milk scan)?
***All look for causes of recurrent aspiration
Barium swallow: Pt drinks barium and you take xrays to look at ANATOMIC ABNORMALITIES (vascular ring, stricture, hiatal hernia, TEF). Short viewing time so insensitive and nonspecific for GERD
-Swallowing study with video fluoroscopy: conducted with OT feeding different consistencies and is the gold standard for evaluating swallowing mechanism. More sensitive for demonstrating aspiration.
-Milk scan: pt swallows milk with radiolabel and multiple xrays are taken up to 60 minutes later to check gastric emptying
What are the early childhood risk factors for persistent asthma? (10)
- Parental asthma
- Atopy
- Reduced lung function at birth
- Possible use of acetaminophen
- Exposure to chlorinated swimming pools
- Severe lower resp tract infection (pneumonia, bronchiolitis requiring hospitalization)
- Male gender
- Wheezing apart from colds
- Environmental tobacco smoke exposure
- Low birthweight
What are the 2 main types of childhood asthma?
- Recurrent wheezing in early childhood (ie. transient early wheezing): triggered by viruses, tends to resolve during preschool years without increased risk for asthma in later life
- Chronic asthma (persistent atopy-associated asthma): associated with allergy (usually persists into later childhood and often adulthood)
What is the treatment for acute vocal cord dysfunction exacerbations?
- Relaxation breathing techniques
- Inhalation of heliox to relieve vocal cord spasm
What are the abnormalities seen on spirometry in asthma?
- Airflow limitation
- low FEV1
- FEV1/FVC ratio 12% - Exercise challenge: worsening in FEV1 > 15%
What is the broad differential for bronchiectasis?
- Cystic fibrosis
- Aspergillus (proximal bronchiectasis)
- Ciliary dyskinesia
- Immunodeficiencies
What are the criteria for determining if a patient’s asthma is well-controlled? (7)
Daytime
Night
What is the gene involved in congenital central hypoventilation syndrome?
PHOX2B gene: essential to embryologic development of the autonomic nervous system from the neural crest
- 90-95% are de novo mutations and the rest inherit the mutation from asymptomatic mosaic parent (autosomal dominant)
- an individual with CCHS has 50% chance of passing on the mutation to their children
In a child with congenital central hypoventilation syndrome presenting with constipation, what condition should be ruled out?
Hirschsprung disease: 20% of CCHS children
-should undergo rectal biopsy to screen for absence of ganglion cells
What are the clinical features of congenital central hypoventilation syndrome?
Deficient hypercarbia and hypoxia sensitivity during wakefulness and sleep and thus do not respond with increased ventilation or arousal during sleep (or awake for some)
- shallow respirations and apneas presenting in first few hours after birth
- can also present with autonomic nervous system abnormalities: cardiac asystole, tumors of neural crest origin, Hirschsprung disease
What investigations should be ordered for a baby suspected to have central sleep apnea?
- Congenital central hypoventilation: PHOX2B genetic testing
- Airway obstruction: consider bronchoscopy/CT chest
- Diaphragm dysfunction: diaphragm fluoroscopy
- Congenital cardiac disease: consider ECHO
- Structural hindbrain or brainstem abnormality: MRI head
- Metabolic disorders
What are the long term complications of obstructive sleep apnea?
- Metabolic: insulin resistance, dyslipidemia
- Cardiovascular disease
- Pulmonary hypertension with cor pulmonale
- Neurocognitive disturbances
What are the long term complications of obstructive sleep apnea?
- Metabolic: insulin resistance, dyslipidemia
- Cardiovascular disease
- Pulmonary hypertension with cor pulmonale
- Neurocognitive disturbances
What are the clinical manifestations of OSA?
-daytime vs nighttime
Daytime: mouth breathing and dry mouth, chronic nasal congestion, hyponasal speech, morning headaches, difficulty swallowing, poor appetite/FTT, secondary enuresis (due to disruption of normal nocturnal pattern of ADH), mood/behaviour/learning difficulties, daytime sleepiness, frequent napping
Nighttime: snoring, breathing pauses, choking or gasping arousals, restless sleep, adopt unusual sleeping positions to keep their necks hyperextended to open up airway
How well did you know this?
What is the gold standard diagnostic test for obstructive sleep apnea?
- What is the first line treatment for obstructive sleep apnea?
- 2nd line?
Gold standard test: polysomnography (sleep study)
- 1st line treatment: Adenotonsillectomy
- 2nd line: CPAP if surgery fails or pts who are not candidates for surgery
What is the polysomnographic parameter most commonly used in evaluating sleep disordered breathing?
-normal values for children 12 yo?
Apnea/Hyponea index (AHI): tells us number of apneic and hypopneic events per hr of sleep
-Normal cutoff AHI value for OSA in kids 1.5
-normal cutoff AHI value for OSA in kids > 12: >5
***consensus is that any child with an apnea index > 5 should be treated
What is the treatment of a pneumothorax that is small
No treatment needed: will self-resolve usually in 1 wk
-recurrent pneumothoraces: may need to induce formation of strong adhesions between the lung and chest wall (sclerosing procedure with talc or iodopovidone into pleural space, VATS with stripping of the pleura to leave inflamed pleura to heal with sealing adhesions)
What is the mechanism of action of theophylline?
- why is it not a first-line agent for young children?
- adverse effects?
Bronchodilation and anti-inflammatory properties (phosphodiesterase inhibitor)
- not first line agent because of differences in absorption and metabolism of theophylline in different patients –> narrow therapeutic window and need to closely monitor serum theophylline levels
- adverse effects with overdose: headaches, vomiting, cardiac arrhythmias, seizures and death
What are the biologic risk factors for asthma morbidity and mortality? (8)
- Previous hx of ICU admission or intubation due to severe asthma exacerbation
- Sudden respiratory failure or arrest
- Two or more hospitalizations for asthma in past year
- 3 or more ED visits for asthma in past year
- Use of > 2 cannisters of SABA per month
- Poor response to systemic corticosteroid therapy
- Low birthweight
- Nonwhite ethnicity
What are the 3 stages of symptoms from aspiration of an object into the airway?
- Initial event: Violent paroxysms of coughing, choking, gagging, and airway obstruction
- Asymptomatic interval: The foreign body becomes lodged, reflexes fatigue, and the immediate irritating symptoms subside. This stage is most treacherous and accounts for a large percentage of delayed diagnoses and overlooked foreign bodies. It is during this 2nd stage, when the child is first seen, that the possibility of a foreign body aspiration is minimized, the physician being reassured by the absence of symptoms that no foreign body is present.
- Complications: Obstruction, erosion, or infection develops to direct attention again to the presence of a foreign body. In this 3rd stage, complications include fever, cough, hemoptysis, pneumonia, and atelectasis.
What is the difference between a primary and secondary lung abscess?
Primary: occurs in previously healthy patient with no underlying medical conditions
-more commonly found on right side
Secondary: occurs in patient with underlying medical conditions
-more commonly on the left side
Which conditions predispose children to the development of pulmonary abscesses? (6)
- Aspiration pneumonia
- Cystic fibrosis
- GERD
- TEF
- Immunodeficiencies
- Neurological conditions
**anything that can lead to aspiration of infected materials with oral organisms
**can also occur secondary to a pneumonia
What is the classic finding on CXR for a lung abscess?
Parenchymal inflammation with a cavity containing an air fluid level
What is the treatment for a pulmonary abscess?
- empiric abx
- treatment duration
- option for patient who does not improve with IV abx
2-3 wk course of IV abx followed by PO abx to complete 4-6 wk course
- choice of abx should be guided by results of gram stain and culture but should initially include agents with both aerobic and anaerobic coverage
- Ceftriaxone + clindamycin, add aminoglycoside if gram negative bacteria are isolated
- for severely ill patients who do not improve after 7-10 d IV abx, consider surgical intervention (CT guided percutaneous drainage or thoractomy)
What is the most common cause of recurrent or persistent cough in children?
Asthma
What is the characteristic features of a habit (tic) cough?
-treatment?
- Cough occurs only during the day and never during sleep
- Cough is absent if physician listens outside the exam room but appears immediatly on direct attention to the child and the symptom
Treatment: reassurance that pathologic lung condition is absent, recommend that child resume school immediately, speech therapy to allow child to increase awareness of the sensations that trigger cough, self hypnosis
A child presents to your clinic with a cough that has persisted for > 6 weeks. What is your next step in management?
Test for cystic fibrosis regardless of race or ethnicity
What is the most common foreign body aspirated by children?
Nuts!
-must ask parents specifically about nuts; if there is any history of eating nuts, bronchoscopy should be carried out immediately
What percentage of foreign bodies lodge in the right main bronchus in children?
-what is the most important factor in determining the need for bronchoscopy in possible foreign body aspiration?
60%
-most important factor: history! (imaging may be negative in up to 30% of cases)
What is the first step in evaluation for foreign body aspiration?
-what might you see if there is a foreign body obstructing a bronchus?
CXR! Expiratory PA chest film is most helpful
-will see obstructive emphysema and air trapping with persistent inflation of the obstructed lung and shift of the mediastium towards the opposite side during expiration
What is the treatment for ARDS?
Overall: supportive management
- Nitric oxide
- Oxygen
- Prone position (improves V/Q mismatch)
- Surfactant (does not change mortality however)
- Sedation (decrease pulm htn)
- Limit tidal volumes to 6-8 ml/kg)
- HFO (gentle ventilation)
- Neuromuscular blockade
- Permissive hypercapnea
- Steroids (start within 72 hrs, can be used up to 30 d)
***If all else fails, ECMO
What are the causes of ARDS?
-two broad categories
Direct lung injury
- Pneumonia (most common)
- Aspiration
- Pulmonary contusion
- Submersion injury
- Inhalational injury
Indirect lung injury:
- Sepsis
- Shock
- Burns
- Transfusion related
- Trauma
What are the 3 phases of ARDS?
- Exudative phase: decreased pulmonary compliance, increased hypoxia, increased tachypnea
- see inflammation, diffuse alveolar infiltrates, pulmonary edema - Fibroproliferative phase: increased alveolar dead space, pulmonary hypertension
- scarring of lung, epithelial damage, surfactant deactivation - Recovery phase: restoration of pulmonary epithelial barrier
What is the definition of ARDS? (4)
- Acute onset
- PaO2/FiO2
What are the benefits of using a spacer with an MDI? (4)
-optimal technique for MDI + spacer?
- Decreases the coordination required to use MDIs
- Improves delivery of drug to lower airways
- Minimizes risk of thrush
- Decreases risk of oral deposition of medicine
**Optimal technique: for each puff, take 5 sec inhalation with 5-10 sec breath hold
-for young children, use spacer and mask and after each puff, hold mask on face for 5-10 breaths (or 30 seconds)
How does the presentation of alpha-1-antitrypsin deficiency differ between children and adults?
In children: has little or no detectable pulmonary disease during childhood; instead, causes liver disease
In adults: causes early-onset pulmonary emphysema in 30s and 40s
-alpha-1-antitrypsin is a protein that deactivates proteolytic enzymes released from dead bacteria or leukocytes in the lung: without this protein, the enyzmes destroy the pulmonary tissue and cause emphysema
What is the treatment for alpha-1-antitrypsin deficiency?
IV enzyme replacement from pooled human plasma
What are common risk factors for thromboembolic disease in children? (7)
-what thrombophilia disorders cause hypercoagulability? (5)
- Malignancy (solid tumors > non solid)
- Presence of a central venous catheter (induces endothelial damage)
- Thrombophilia (deficiency of antithrombin 3, protein C or S; factor 5 leiden, hyperhomocysteinemia, antiphospholipid antibodies)
- Recent surgery/immobilization
- Nephrotic syndrome (losing protein C & S)
- SLE (systemic inflammation)
- OCP
***Remember virschow’s triad: hypercoagulability, endothelial injury, stasis
What is the gold standard for diagnosis of PE?
- what is the diagnostic test of choice?
- what is the utility of a V/Q scan?
Gold standard: pulmonary angiography
- not necessary except in unusual cases since spiral CT is almost always available
- diagnostic test of choice: spiral CT with IV contrast (specificity 90%)
- V/Q scans are noninvasive and sensitive: use if pre-test probability is low
What is the treatment for a PE?
-what is the mortality rate in children from PE?
Heparin infusion (aim for aPTT to be 1.5-2x normal) x several days, then begin PO warfarin x 3-6 mo
- LMWH can also be used
- thrombolytic agents should only be used in combo with anticoagulants in the sickest patients
- mortality rate: 2.2%
In children with prolonged or high inhaled corticosteroid therapy, what are two things you should monitor for?
-does the use of inhaled corticosteroids affect adult height?
- Height velocity
- Cataracts
***Does not affect adult height
What are the two most common adverse effects of inhaled corticosteroids?
- Thrush
- Hoarse voice (dysphonia)
What population do you most commonly see idiopathic pneumothorax in?
Tall, thin male with subpleural bleb
What recreational drug is associated with pneumothorax?
Ecstasy!
What are the 5 Ts of anterior mediastinal mass?
- Thymoma
- T cell leukemia
- Terrible lymphoma
- Thyroid tumors
- Teratoma
What is the differential diagnosis for middle mediastinal mass?
A+B
- Adenopathy: infectious (histoplasmosis most common), neoplastic, metastatic, sarcoidosis
- Bronchogenic cyst
What is the differential diagnosis for posterior mediastinal mass?
The Ns
- Neurogenic tumor: neuroblastoma, benign ganglioneuroma, ganglioneuroblastoma
- Neurofibroma
- Esophageal duplication cysts
- Pulmonary sequestration
In which infection does CXR look worse than clinical picture?
Mycoplasma chlamydia and mycoplasma pneumoniae
What radiological signs can be seen on a CXR in a patient with ABPA?
- Proximal bronchiectasis
- Bronchial wall thickening
- Pulmonary infiltrates
What controls respiratory drive?
- Central chemoreceptors in the medulla = sense hypercarbia (major role in respirations)
- Peripheral chemoreceptors in the carotid arteries/arch of aorta = sense hypoxia (minor role in respirations)
What is a clue on exam that can help you differentiate between laryngomalacia vs. tracheomalacia?
- Laryngomalacia = inspiratory stridor (above thoracic inlet)
- tracheomalacia (weak tracheal rings collapse with expiration)= expiratory or biphasic stridor (below thoracic inlet
What is the differential diagnosis for chronic cough? (9)
-initial work-up?
Chronic cough > 3 wks:
- Asthma
- Recurrent infections
- Cystic fibrosis
- GERD
- Immunodeficiency: IgA deficiency, hypogammaglobulinemia
- Primary ciliary dyskinesia (Kartagener syndrome, immotile cilia syndrome)
- Anatomic lesions: foreign body, mediastinal tumor, congenital heart disease), TEF
- Allergy/irritants: tobacco smoke, pollution, etc.
- Psychogenic cough
- initial work up: guided by clues in history and physical; if no clues, then start with: - CXR
- PFTs if possible
- Sweat chloride
What can give falsely high O2 sat readings?
-what can give falsely low O2 sat readings?
Pulse oximeter measures differential absorption of light and calculates ratio of oxyhemoglobin to deoxyhemoglobin
- Falsely high O2 sat readings (ie. monitor reads 100% sat but patient is actually hypoxic) = carbon monoxide poisoning due to high infinity of CO to hemoblogin and thus, looks like hemoglobin is saturated
- Falsely low O2 sat readings (ie. monitor reads 70% but patient is not hypoxic) = methemoglobin
A patient with a pleural effusion receives a chest tube for drainage. The pleural fluid is chocolate colored. What is your diagnosis?
Amebiasis (entamoeba histolytica)
What causes chylothorax? (3)
- aggravating factor?
- treatment? (2)
Chylothorax = disruption of thoracic duct leading to accumulation of chyle (high lipid and protein content)
- Thoracic surgery complication
- Birth injury: thoracic duct leaks with stretch
- Obstruction of central veins by thrombosis or malignancy
- worsened by ingestion of fat
- treatment: - low fat diet (portagen formula = medium-chain fatty acids, or TPN without lipids)
- If severe, can ligate thoracic duct
What are the main differences between exudative and transudative effusions? (ie. modified lights criteria)
Exudative:
- pH 0.5
- pleural:serum LDH > 0.6
- high polymorphonuclear leukocytes
Transudative:
- pH > 7.3
- glucose > 50% of serum
- pleural:serum protein
What is a possible resp complication of empyema?
Bronchopleural fistula
Causes of transudative pleural effusions? (5)
- Cirrhosis
- Congestive heart failure
- Nephrotic syndrome
- Malignancies
- Infections (viral, Klebsiella, GAS, etc.)
What age group do you commonly see the peak incidence of the following:
- croup
- epiglottitis
- bacterial tracheitis
-Croup: 3 mo-4 yo
-bacterial tracheitis: 3 mo - 4 yo
-epiglottitis: 2 yo - 10 yo
***overall, these can occur in any age really
Where is the acute airway obstruction seen in croup?
Subglottic narrowing at the cricoid cartilage!!
What are the 3 most common causes of epiglottis in the post-HIB vaccine era?
- Strep pneumo
- GAS
- Staph aureus
What is the management of epiglottitis?
This is a MEDICAL EMERGENCY! Child looks toxic and can progress to complete airway obstruction within 24 hrs of presentation
- clinical diagnosis = do NOT need xray to confirm if this will only agitate child (thumbprint sign on xray)
- Send CBC and blood culture since bacteremia occurs before epiglottitis and can help identify the organism
- don’t agitate the child (no IV, no bloodwork until airway secured)
- start immediate antibiotics once airway is secured (3rd generation cephalosporin IV +/- vancomycin)
How can you differentiate between epiglottitis vs. croup vs. bacterial tracheitis?
***All have inspiratory stridor
- Epiglottitis: drooling, tripod position, no cough, toxic appearance, high fever
- Croup: non toxic appearance, responds to epi, hoarse barking cough
- bacterial tracheitis: toxic appearance, prominent brassy cough (usually no drooling or dysphagia), do not respond to epi, high fever, usually a secondary complication of croup
What is the most common bacterial infection associated with bronchiolitis?
AOM
What are the 2 most sensitive physical findings in pneumonia?
- Fever
- Tachypnea
***unfortunately neither are very specific
What are the components of pulmonary toileting? (3)
Overall goal: improve mucous clearance
- Chest physio
- Mucolytics
- Bronchodilators
What is the role of azithromycin therapy in the treatment of CF?
Azithromycin = shown to reduce lung inflammation if given 3x weekly, thus decreasing pulmonary exacerbations
What are risk factors for persistent asthma into adulthood? (5)
- Maternal history of asthma
- Asthma onset prior to 3 years of age
- Eosinophilia
- Elevated IgE levels
- Allergic rhinitis
What are clinical features of Kartagener syndrome? (7)
- diagnosis?
- treatment?
Primary ciliary dyskinesia!
- Chronic sinusitis with nasal polyps
- Situs inversus in 50% of patients
- Chronic otitis media with hearing loss
- Bronchitis and bronchiectasis
- Pneumonia
- Clubbing
- Male and female infertility/decreased fertility
- abnormal sperm movement
- increased ectopic pregnancy
Diagnosis:
- CXR = dextrocardia, bronchiectasis
- Gold standard = look at cilia from nasal/bronchial brush by electron microscopy
Treatment:
- Pulmonary toilet to maximize mucous clearance
- Antibiotics to treat infection
- May need lobectomy if severe bronchiectasis
- What do you see on PFTs of someone with restrictive lung disease?
- What is the differential diagnosis of restrictive lung disease?
- On PFT: see normal FEV1/FVC, decreased FEV1, decreased FVC, decreased TLC, decreased RV
- Differential diagnosis: think from lungs and work outwards
- Lung:
1. Resection
2. Atelectasis
3. Interstitial lung disease (fibrosis) = ex. sarcoidosis, fibrosis from radiation, infections (adenovirus, CMV, PCP, aspergillus, mycoplasma), langerhans histiocytosis, hemosiderosis
4. Pulmonary vascular congestion secnodary to CHF
5. Tumor
6. Pneumonia - Pleural cavity:
7. Effusion/empyema - Chest wall:
8. Scoliosis
9. Splinting due to pain
10. Ascites - Muscle:
11. Neuromuscular disease
12. Diaphragmatic paralysis
What is the gold standard for diagnosis for interstitial lung disease?
-additional workup?
Lung biopsy
- workup needs to rule out conditions on the huge ddx:
1. Infectious work-up: mycoplasma PCR, CMV serologies, chlamydia culture, sputum samples
2. Rheumatic disease markers
3. ACE level (sarcoidosis)
4. UA to rule out associated renal disease
A patient presents with chronic cough and shortness of breath. After extensive investigations, you decide to do a bronchoscopy. On sputum sample, you see siderophages. What is your differential diagnosis? (6)
Hemosiderosis!
-siderophages = macrophages with iron inside
Differential diagnosis:
- Heiner’s syndrome: CMPA with pulmonary hemorrhage
- Idiopathic pulmonary hemosiderosis
- Pulmonary capillary hemangiomatosis (proliferation of capillaries with alveolar hemorrhage)
- Goodpasture syndrome
- Wegner’s granulomatosis
What medication class should be avoided in individuals with history of obstructive or central sleep apnea?
OPIOIDS!!!! They are very sensitive to these medications and have worsening of apnea
What is the differential diagnosis for obstructive sleep apnea? (5)
- Tonsilly/adenoid hypertrophy (most common)
- Obesity
- Craniofacial anomalies
- Neuromuscular disorders (ie. muscular dystrophy)
- Neurologic disorders involving incoordination of upper airway musculature
Why should you never put O2 on a child with history of OSA and walk away?
Children with OSA are chronic CO2 retainers and thus their respiratory drive is dependent on hypoxia! If you give them O2, then there will be no more respiratory drive and they will become apneic
A baby born vaginally to a poor controlled gestatioal diabetic mom had cyanosis, irregular work of breathing and decreased breath sounds on the right side. There is also decreased tone in the right arm. A CXR is normal. What is your next best test to figure out diagnosis?
Chest ultrasound to rule out phrenic nerve paralysis
What are the treatment modalities of CF? (10)
- Nutrtional support
- Inhalation therapy
- Chest PT
- Bronchodilators
- Oxygen
- Antibiotics
- alpha-1-antitrypsin (inhibits neutrophil elastase which breaks down lung tissue)
- amiloride (diuretic inhibiting sodium and water reabsorption from secretions)
- DNAse = reacts with DNA released by dead leukocytes to reduce sputum viscosity
- Gene therapy: alters CF gene
What is considered delayed passage of meconium?
>48 hrs after birth with no passage
What are 5 causes of a false positive sweat chloride test?
- Untreated adrenal insufficiency (Addisons)
- Untreated hypothyroidism
- Ectodermal dysplasia
- Atopic dermatitis
- Age
A 16 yo patient with CF complains of chest pain and a cough that produced a rust-colored sputum. What is the most likely diagnosis?
ABPA
What is the second line monotherapy for mild asthma?
Leukotriene receptor antagonists! (Montelukast)
What are poor prognostic indicators in CF? (4)
- Poor nutritional status
- Pneumothorax
- Burkholderia cepacia (very deadly bug in CF)
- Poor FEV1
What is the CLASSIC clinical feature on history for primary ciliary dyskinesia?
YEAR ROUND DAILY WET COUGH AND NASAL CONGESTION!!!!
What is the best test to rule out laryngeal cleft?
-clinical feature of laryngeal cleft?
Clinical feature: coughing/choking occurs DURING swallowing
-best test: rigid bronchoscopy is REQUIRED to rule it out!
How do you diagnose TEF? Name 2 tests.
UGI series to start, rigid bronchoscopy is gold standard
UGI series to start, rigid bronchoscopy is gold standard
- Confirm diagnosis
- Environmental controls, education and written action plan: eliminate smoking, second hand smoke, allergens, need to improve adherence
- Fast-acting bronchodilators prn
- Inhaled corticosteroids:
(-low dose: 6-11 yo: 12 yo: 12 yo: 251-500 mcg/d)
- for age 6-11:
a. start with low dose ICS
b. Then increase to medium dose ICS
c. Then add a LABA (for child with more exercise s/s) OR LTRA (for more atopic child)
d. Then use medium dose ICS + LABA + LTRA - for 12 and older:
a. start with low dose ICS
b. then ADD LABA
c. THEN increase ICV to medium dose OR add a LTRA
d. THEN use medium dose ICS + LTRA + LABA
At what age should you start using the yellow aerochamber? Blue?
Yellow = pediatric = 1-5 yo Blue = adult = \>5 yo
A 10 yo girl with asthma is prescribed fluticasone 50 mcg 1 puff BID. She has no night time symptoms, has daytime symptoms of cough/wheezing 4x/wk while playing hockey. Most appropriate next step?
a. Take salbutamol before exercise
b. Increase fluticasone to 125 mcg 1 puff BID
c. add montelukast
d. add LABA
B! This is a child 6-11 yo with poorly controlled asthma since she is needing beta agonist > 4x/wk!
-she is currently on low dose ICS THUS next step is to increase to medium dose ICS!
What is the differential diagnosis for vocal cord paralysis?
-unilateral vs. bilateral?
Bilateral vocal cord paralysis (hear inspiratory stridor)
-congenital CNS lesion (myelomeningocele, arnold-chiari malformation, hydrocephalus) = think of things that compress the brainstem (CN X)
Unilateral vocal cord paralysis (see choking, aspiration, coughing, weak cry)
-usually damaged by surgery/intubation to the recurrent laryngeal nerve
Dx: flexible awake laryngoscopy, need MRI head if bilateral vocal cord palsy, consider Neuro and Cardio consult
Tx: most spontaneously resolve within 6-12 mo but some bilateral paralysis require trach temporarily
What is the differential diagnosis of prolonged QT?
- Congenital:
- Romano-Ward syndrome (most common genetic cause of prolonged QT syndrome, autosomal dominent)
- Jervell-Lange-Nielsen syndrome (associated with deafness) - Acquired
- Drugs (antiarrhythmics, dexmedetomidate, TCAs, antipsychotics, antibiotics such as macrolides)
- Electrolytes (hypocalcemia, hypokalemia, hypomagnesemia)
- Intracranial lesion (encephalitis, head trauma, stroke)
- Cardiac disease
What is the treatment for myocarditis?
SUPPORTIVE for mild cases
- Bed rest
- Digoxin
- Cautious diuresis since they’re preload dependent (will need some volume)
- Afterload reduction - ACE inhibitor
- Anti arrhythmic
For severe cases: may need inotropes/ICU support
What is the rule of 1/3s for myocarditis?
1/3 complete recover
1/3 develop chronic dilated cardiomyopathy and require lifelong failure management
1/3 require transplant or have sudden death from arrhythmia
What is the differential diagnosis for left axis deviation on ECG (5)?
Left axis deviation is always abnormal
- AVSD
- Tricuspid atresia
- Pulmonary atresia
- Pulmonary stenosis
- Single LV
What is the cause of biventricular hypertrophy on ECG?
VSD
What 3 congenital heart diseases is most likely to present with severe cyanosis within the first few hours of life?
- Transposition of the great arteries (MOST COMMON PRESENTING CYANOTIC LESION TO PRESENT IN NEWBORN PERIOD)
- only mixing is through an open PDA and PFO
- need PGE1 to keep PDA open in order to maintain oxygenation to systemic circulation - Pulmonary atresia
- Ebstein’s anomaly
What are the clinical signs and symptoms of a “tet spell” (3)?
What occurs physiologically during a “tet spell”?
(aka hypercyanotic spell)
- Acute onset of intense cyanosis
- Sudden softening of murmur (due to decreased blood flow through the obstructed right outflow tract)
- Deep rapid respiratory pattern (no work of breathing should be seen theoretically)
“Tet spell” - acute, sudden onset of increased right ventricular outflow tract obstruction and resultant decreased pulmonary blood flow (can be precipitated by agitation, fever, exercise, etc) –> obstruction of deoxygenated blood flow into the pulmonary artery –> deoxygenated blood shunts through the large VSD into the left ventricle –> deoxygenated blood flows into systemic circulation causing cyanosis
- typically present starting at around 2 months of age as right ventricular outflow tract obstruction worsens
- indication for urgent surgical repair
What are the 4 characteristic heart defects in Tetralogy of Fallot?
- Overriding aorta (empties from both the LV and RV)
- RVH
- Right ventricular outflow tract obstruction (ie. pulmonary stenosis)
- VSD
In Tetralogy of Fallot, what does the severity of clinical signs and symptoms depend on?
The degree of right ventricular outflow tract obstruction
What 3 genetic conditions can be commonly be associated with Tetrology of Fallot?
- DiGeorge syndrome (22q11 deletion)
- Down syndrome
- Alagille syndrome
What is the immediate management of a “tet spell”?
Step-wise approach: if one step fails, move onto the next
- Put patient in knee-chest position
- as with squatting, knee-chest position increases systemic vascular resistance, thus increasing left ventricular pressure and decreasing the right to left shunt of deoxygenated blood, promoting flow instead back into the lungs
- also increases venous return to right side of heart to promote flow to lungs - Administer O2
- O2 is pulmonary vasodilator (decreasing pulmonary vascular resistance and thus increases blood flow to lungs) and systemic vasoconstrictor - Administer morphine (0.1 mg/kg) and give fluid bolus
- morphine theoretically decreases agitation and thus RVOT obstruction
- fluids improves venous return - Administer IV beta blocker (propanolol 0.1 mg/kg)
- causes relaxation of RVOT obstruction - Administer IV phenylephrine (selective alpha agonist)
- increases afterload (systemic vascular resistance)
What is a PVC?
Premature ventricular contraction - heartbeat is initiated by Purkinje fibers in the ventricles instead of the SA node
- wide QRS
- ventricles contract before atria has had time to fill them with blood
What are the 4 types of PVCs?
- Bigeminy
- PVC occurs after every normal beat = non concerning - Trigeminy
- two normal beats to one PVC = non concerning - Quadrigeminy
- three normal beats to one PVC = non concerning - Ventricular tachycardia
- 3 or more PVCs occuring in a row = concerning obviously
What noninvasive tests can be ordered to investigate a history of arrhythmia (4)?
- ECG
- Holter monitor
- Event recorders
- loop recorders: children always wear the device and press a button when they experience symptoms
- pacer tracers: connected to telephone for recording - ECHO
What are the two treatment modalities for chronic arrhythmias?
- Medical therapy with antiarrhythmics
- Catheter ablation therapy
When would catheter ablation be the treatment of choice for a child with a tachyarrthymia over medical therapy?
If tachyarrhythmia has caused a life-threatening event
What is the treatment of choice for children with bradyarrythmias?
Cardiac pacing
What can be seen on ECG of a patient with Wolff-Parkinson-White syndrome (2)?
- Delta waves - short PR interval with upsloping, reflects pre-excitation (electrical conduction from atria to ventricle via pathway other than AV node) since the normal delay between the P and the R is missing (ie. the normal delay of electrical conduction by the AV node)
- Wide QRS
What are the 2 types of SVT (and 2 subtypes within each type)?
- Re-entry
a) AVRT (atrioventricular reentrant tachycardia)
b) AVNRT (atrioventricular nodal reentrant tachycardia) - Automaticity: MAY see variability, warm up and slow down period, usually a bit lower rate, most will NOT respond to adenosine
a) Ectopic atrial tachycardia
b) Junctional ectopic tachycardia (JET)
What is Wolff-Parkinson-White Syndrome?
Pre-excitation syndrome: presence of abnormal accessory electrical conduction pathway (Bundle of Kent) between the atria and ventricles
- pathway conducts electrical activity at higher rate than AV node and thus, if pt had atrial flutter, would conduct all the beats to the ventricles (bypassing the AV node’s natural ability to slow down atrial impulses to protect the ventricles from beating too fast)
- risk of ventricular fibrillation and sudden death
Describe the pathophysiology of AVRT SVT.
- presence of accessory pathway outside of AV node
- normal SA antegrade conduction goes through the AV node and then retrograde up accessory pathway to reactivate the ventricles
- in WPW, normal SA goes antegrade through the accessory pathway bypassing AV node (and thus no normal delay in conduction)
Describe the pathophysiology of AVNRT SVT.
- presence of two conducting pathways WITHIN the AV node (one fast and slow) creating a reentrant loop using one pathway in antegrade direction and one in retrograde
- more likely in > 6 yo
Adenosine is only effective at terminating which type of SVT (2)?
Re-entry
- AVRT
- AVNRT
-Adenosine blocks AV node conduction
Why do vagal maneuvers assist in terminating SVTs?
Vagal maneuvers activate parasympathetic nervous system conducted to the heart by the vagus nerve
-increase AV nodal block to eliminate conduction to the ventricles
Which antiarrhythmics should be avoided in children with WPW (2)?
- Digoxin
- Calcium channel blockers (ie. verapamil)
Both have potential proarrhythmia effects and can enhance conduction properties of accessory pathway (by blocking AV node so the impulse is forced to go to the accessory pathway) resulting in more rapid ventricular rate during atrial flutter and increased risk for sudden death
In which clinical circumstances do you worry that PVCs may lead to increased risk of sudden cardiac death/
- Post-op CHD
- Poor cardiac function (ie. cardiomyopathy)
What 4 features of syncope may be suggestive of a cardiogenic etiology?
- Sudden onset (“drop”) without prodromal symptoms or awareness
- Occurred during exercise
- Limp or lifeless during syncope leading to injury
- Palpitations or abnormal heart rate noted before event
- Family history of sudden death
What are 5 causes of cardiogenic syncope with risk of sudden cardiac arrest?
- HOCM
- WPW
- Prolonged QT syndrome
- Coronary artery anomalies (ie. coronaries comes between pulmonary artery and aorta; during exercise, increases stroke volume and thus great vessel size and squishes coronary arteries and get MI)
- Arrhythmogenic RV dysplasia
What 3 congenital heart defects are seen with maternal diabetes?
- VSD
- HOCM
- PDA
Which cyanotic heart defect is most likely to present in:
- newborn period
- infancy period
- childhood period
- Newborn period: transposition of great arteries
- Infancy period: tricuspid atresia
- Childhood period: TOF
What is Ebstein’s anomaly?
Abnormal tricuspid valve leaflets (either abnormal shape, too large or adhered to the septum)
- get constant connection between RA and RV with tricuspid regurgitation due to inadequate valve closure
- wall to wall heart on CXR
When interpreting an ECG in a patient with a pacemaker, what does it mean to have “good capture”?
Should always check for good capture on an ECG in a patient with a pacemaker:
-means that every time the pacemaker discharges (“spike” on ECG), there should be a subsequent QRS following each “spike”
What is the sequence of reading an ECG?
- Heart rate
- Rhythm
- Sinus: P before every QRS, upright P in leads II, III, and AVF - Axis
- Look at lead I and AVL: upright?
- Look at II, III, and AVF: upright?
(overall: leads II, III and AVF are stickers on legs so if you see upright QRS, means that electrical impulse is moving towards the stickers on legs from above ie. the SA node so this means this is atrial impulse. If you see inverted QRS, means that the electrical impulse is coming from the ventricles moving away from the legs) - Look at PR and QRS intervals and compare to normal for age values
- Look at precordial leads (V1-V6) for normal R wave progression
- QRS should get bigger as you move from V1-V6 (since in older children, left ventricle is thicker than right ventricle so higher voltage) - Look at T wave in all leads
- should always be upright in limb leads
- in children 10 yo, should start to have upright T waves starting in V6 and moving towards V1 (can take up to 18 yo for T wave to become upright in all V1-V6 leads) - QTc calculation and compare to normal for age
What clinical features are seen in DiGeorge Syndrome?
CATCH-22
Deletion of chromosome 22q11
- Congenital heart defect (usually TOF)
- Abnormal facies
- Thymic aplasia
- Cleft palate
- Hypocalcemia
What are 2 things you MUST do prior to cardioverting an infant presenting with new SVT?
Since we don’t know how long infant has been in SVT for, may have developed heart failure or poor cardiac function; thus, may be rate dependent for cardiac output. With cardioversion and return to normal HR, may significantly decrease cardiac output and thus cause them to go into cardiogenic shock.
- Need to call Cardiology and PICU and be prepared for this circumstance in order to do in controlled setting
- Need to put a 12 lead ECG or rhythm monitor on the patient
What is Beck’s triad?
Clinical features of cardiac tamponade:
- Hypotension
- Muffled heart sounds
- Distended neck veins
What is the underlying mutation in prolonged QT syndrome?
Mutations in cardiac potassium and sodium channels = ion channelopathies
What is the most common presenting symptom of LQTS in children?
Syncopal episode brought on by exercise, fright, or sudden startle
-some occur during sleep (LQT type 3)
What is QTc?
-What QTc is considered prolonged?
- QTc = QT interval (ventricular depolarization and repolarization) that is adjusted for heart rate differences
- Prolonged = > 0. 440
What is the significance of genetic testing in identifying prolonged QT syndrome?
Genotyping can identify mutation in ~75% of patients known to have LQTS by clinical criteria.
-if negative, it does NOT mean that a patient does not have LQTS if they fit clinical criteria BUT it does help identify asymptomatic affected relatives of the index case
What is the treatment for prolonged QT syndrome?
- Beta blockers
- if beta blockers cause significant bradycardia, may need pacemaker - For patients who have had cardiac arrest, need implantable cardiac defibrillator (ICD)
What other ECG features can be seen in prolonged QT syndrome? (3)
-what are patients with prolonged QT syndrome at risk for? (2)
- Bradycardia
- Notched T waves
- T wave alternans (alternating upright and downgoing T waves)
-At risk for torsades de pointes and ventricular fibrillation
What is the underlying pathophysiology of Hypoplastic Left Heart Syndrome?
- how does circulation occur?
- main problems resulting from abnormal circulation?
- Hypoplasia of left ventricle (secondary to atresia of the mitral orifice)
- Hypoplasia of ascending aorta (secondary to atresia of the aortic ortifice)
**Remember: no flow, no grow
*Circulation: red blood (100% O2) from lungs –> LA cannot pass to LV and aorta –> passes through PFO or ASD –> RA and mixes with blue blood (60%) from SVC/IVC = 85% O2 –> RV –> pulmonary artery –> lungs and systemic circulation via PDA to descending aorta with blood flow retrograde to ascending aorta (to supply cerebral and upper extremity perfusion) and coronary arteries
- main problems:
1. Increased pulmonary flow (from moderate or large ASD and all blood flowing into RA –> RV –> lungs)
2. Pulmonary vein hypertension (from small ASD or restrictive PFO leading to backup of blood in LA and pulmonary vein)
3. Inadequate maintenance of systemic circulation
What are the clinical features of hypoplastic left heart syndrome? (3)
- Increased pulmonary flow: can have pulmonary edema (crackles, wet lungs on CXR)
- Decreased systemic circulation: weak or absent pulses, cyanosis, shock once PDA starts closing
- Right ventricular volume overload: right ventricular parasternal lift
**typically present within 1st few hours or days of life
What 3 genetic syndromes can HLHS be seen in?
- Turners
- Trisomy 13
- Trisomy 18
What is seen on ECG of patients with HLHS?
- Right ventricular hypertrophy (due to volume overload)
- Prominent P waves (right atrial dilatation from volume overload)
- Reduced left sided signals
What is the association of central nervous system abnormalities with HLHS?
-association of dysmorphic features?
30% of HLHS have major or minor central nervous system abnormalities
- 40% of HLHS have dysmorphic features
- thus, need genetics, neuro, ophtho assessment before surgery
What is the treatment for HLHS?
- Prostaglandin E1 to maintain PDA
- Norwood procedure
Describe the Norwood procedure.
- stage 1 (commonly called the Norwood)
- stage 2
- stage 3
Stage 1 (neonatal period): commonly referred to as the Norwood
- atrial septectomy
- attach the proximal pulmonary artery to the hypoplastic aortic arch with a patch to form a neoaorta to supply systemic circulation
- Blalock-Taussig shunt connects the subclavian artery to the main pulmonary artery to increase total pulmonary blood flow and thus increases oxygen saturation to systemic circulation (decreases cyanosis)
Stage 2 (2-6 mo of age): Glenn anastomosis -disconnect the SVC from the RA and connect it directly to the pulmonary arteries
Stage 3 (2-3 yr of age: modified Fontan procedure -disconnect the IVC from the RA and connect it directly to the pulmonary arteries
Describe the circulation in HLHS post Norwood procedure.
Blue blood from SVC/IVC enters pulmonary artery directly –> passive flow to lungs –> LA –> ASD –> RA –> RV –> pulmonary artery –> neoaorta to systemic circulation
What are the common ductal-dependent cardiac lesions?
- ductal dependent pulmonary blood flow (4)
- ductal dependent systemic blood flow (3)
Ductal dependent pulmonary blood flow:
- Critical pulmonary valve stenosis
- Pulmonary atresia
- TOF with severe pulmonary stenosis
- Tricuspid atresia with pulmonary stenosis or pulmonary atresia
Ductal dependent systemic blood flow:
- Coarctation of the aorta
- Hypoplastic left heart syndrome
- Interrupted aortic arch
What is the distribution of aortic stenosis in males vs. females?
More common in males (3:1)
What is the most common form of aortic stenosis and what is the pathophysiology?
Most common form: valvular aortic stenosis
- thickened aortic leaflets and commissures are fused
- obstruction to LV blood flow = increased LV systolic pressure = LVH
What is the triad of Shone syndrome?
- Subvalvular aortic stenosis
- Mitral stenosis
- Coarctation of aorta
What are clinical features of Williams syndrome? (7)
-what is the underlying genetic mutation?
- Supravalvular aortic stenosis
- Developmental delay
- Hypercalcemia
- Elf face (prominent lips, abnormal teeth)
- Hypothyroidism
- Cocktail party personality
- Joint hypermobility
Genetic mutation: chromosome 7q11.23 (codes for elastin protein)
Williams loves going to 7/11 to drink slurpees and socialize with his big lips
What are the clinical features of aortic stenosis?
-murmur?
Think decreased left ventricular outtract flow and back up of blood into LA and lungs!
- decreased cardiac output = decreased pulses, decreased urine output
- left ventricular failure and pulmonary edema
- murmur: early systolic ejection click at apex and left sternal edge with RUSB murmur radiating to neck (aortic arch)
What are findings on ECG of aortic stenosis?
- LVH
- left heart strain (inverted T waves in left precordial leads)
What is the treatment for aortic stenosis?
-what is a complication of untreated severe aortic stenosis?
Balloon valvuloplasty or surgery
-untreated severe AS: sudden cardiac death especially during exercise
What are patients with bicuspid aortic valves at increased risk for developing?
Dilated ascending aorta
-dissection and rupture are described in adult populations due to severe aortic root dilation but there is insufficient data to determine the risk in children (only isolated cases reported)
What is the triad of Holt-Oram syndrome?
- Hypoplastic or absent radii
- 1st degree heart block
- ASD
What is the difference between an ostium primum and ostium secundum?
Both are types of ASDs
- ostium primum: defect is at center of the septum
- ostium secundum: defect is inferior and is part of an AV canal defect and can involve tricuspid or mitral valve abnormalities
How do children with VSDs typically present?
-large vs. small
Large VSD: signs of CHF usually appear at 4-8 wks of age when the pulmonary vascular resistance drops and pulmonary blood flow increases from L-R shunting
Small VSD: spontaneous closure often occurs
What is the indication for closure of ASD?
-when does it occur?
Secundum ASD associated with RV dilation (from volume overload) and increased pulmonary blood flow
-repair occurs electively at 3-5 years of age
What are the clinical features of ASD?
Most children are asymptomatic
-when they do have symptoms, you hear a fixed split S2
Why do you hear a fixed split S2 in ASD?
From RV volume overload and prolonged ejection of blood during systole leading to delayed pulmonary valve closure
What is the result of persistent increased pulmonary blood flow?
Pulmonary HTN
What is the pathophysiology behind congestive heart failure due to left to right shunting?
-Eisenmenger
- Resp symptoms due to increased pulmonary blood flow and pulmonary edema
- Increased left ventricular output since cardiac output to systemic circulation is decreased due to loss through the L to R shunt –> to maintain this output, have increased heart rate and stroke volume –> increased sympathetic nervous system activity –> increased catecholamines –> increased total body oxygen consumption, sweating, irritability and FTT –> remodeling of heart with dilatation and hypertrophy –> Eisenmenger (pulmonary hypertension with reversal of shunt from R-L)
What are characteristics of Still murmur?
- location & quality
- age group
Classic vibratory murmur
- LLSB and apex
- low-frequency vibratory “twanging string”
- 3-6 yo
- turbulent flow out of LV
- decreases in intensity with sitting
What are the characteristics of a pulmonary ejection murmur?
- location & quality
- age group
- ULSB
- early to midsystolic murmur
- crescendo-decrescendo
- 8-14 yo
- from turbulence of blood flow into the pulmonary vein
What are the characteristics of a pulmonary flow murmur of newborn? (PPS)
- location & quality
- age group
- ULSB
- transmits to axilla and back
- premature and full-term newborns
- disappears by 3-6 months of age
- angulation or narrowing of peripheral pulmonary stenosis
- heard usually during resolving URTI
What are the characteristics of a venous hum murmur?
- location & quality
- age group
Continuous, supraclavicular murmur
-3-6 years old
-from turbulence of flow in jugular venous system HENCE why you hear it best in the supraclavicular area
-can decrease it by turning neck or compressing the jugular venous system in the neck
*****ONLY INNOCENT MURMUR THAT WILL INCREASE WITH SITTING
What are the characteristics of a carotid bruit murmur?
Right supraclavicular areas and over carotids
-any age
What does sitting up do to:
- venous hum murmur
- Still’s murmur
Venous hum: increases with sitting up
Still’s: decreases with sitting up
What is the most common innocent murmur?
Still’s murmur = medium pitched, vibratory or musical systolic ejection murmur heard at the LLSB
What is pulsus alternans?
Pulsus alternans: beat to beat variability of pulse strength due to decreased left ventricular performance
-seen in severe CHF
Describe the pathophysiology of pulsus paradoxus.
Fall in SBP > 10 mm Hg during inspiration
- NORMALLY: when you take a breath, you increase intrathoracic negative pressure and increase venous return to the right side of the heart and lungs = this causes blood pooling in the lungs and thus decreased pulmonary venous blood flow = decreased blood return to LA = decreased cardiac output = SBP drops by no more than 10 mm Hg. Also more right heart filling impairs left heart filling and decreased stroke volume
- IN PULSUS PARADOXUS: pressure is equalized between right heart and left heart = when right heart fills, pushes septum into left side of heart impairing filling since left heart cannot expand to accommodate blood flow with the constriction) = decreased stroke volume = drop in SBP > 10 mm Hg. Also can have increased negative thoracic pressure exerting a transmural load onto the LV and thus increases afterload, decreasing stroke volume
What are causes of pulsus paradoxus? (4)
- Pericardial tamponade
- Constrictive pericarditis
- Severe respiratory disease (status asthmaticus, pneumonia)
- Myocardium diseases that affects wall compliance (amyloidosis)
What is the antidote for digoxin toxicity?
-what if digibind is not available?
Digibind = digoxin-specific Fab antibody fragments = binds free digoxin in intravascular and interstitial space that forms inactive complex and then you pee it out
- if not available, can use phenytoin or lidocaine to stabilize the myocardium and decrease ventricular irritability
- atropine is helpful too
- also use activated charcoal
How does Digoxin work?
What are the signs and symptoms of digoxin toxicity? (5)
- Digoxin blocks the Na-K-ATPase pump = leads to movement of K from intracellular to extracellular and movement of Na and Ca into cells
- increase of Ca in cardiac myocardial cells improves inotropy. Also decreases nodal conduction
Digoxin toxicity:
- bradycardia & heart block
- nausea and vomiting
- lethargy, confusion and weakness
- hyperkalemia
- pulsus bigeminus (2 heart beats close together followed by long pause)
Why should digoxin NEVER be given in WPW patient?
Digoxin decreases AV nodal conduction BUT can enhance cnoduction through a bypass tract THUS increasing chance of ventricular fibrillation
What connective tissue diseases may be associated with mitral valve prolapse? (3)
- Marfan syndrome
- Ehlers-Danlos syndrome
- Osteogenesis imperfecta
What is mitral valve prolapse?
- physical exam finding?
- treatment?
Abnormal mitral valve mechanism that causes billowing of one or both mitral valve leaflets into the LA at the end of systole
- physical exam finding: apical murmur with a click
- Minimal mitral valve prolapse can be a normal variant
- Treatment: nothing!
What conditions are associated with the development of myocarditis?
(3 broad classes)
- Infectious
- bacterial: diphtheria, strep species, mycoplasma
- viral: Coxsackie B (most common), HIV, echoviruses, rubella, adenovirus
- fungal
- protozoal: trypanosomiasis (Chagas disease), toxoplasmosis - Inflammatory
- Kawasaki
- SLE
- RA
- IBD - Chemical and physical agents
- doxorubicin
- radiation injury
- lead
- alcohol
What is Chagas disease?
- clinical features (2)
- what is Romana sign?
American trypanosomiasis infection
-Romana sign (unilateral, painless, violaceous, palpebral edema accompanied by conjunctivitis) + CHF
What are the causes of a single S2? (6)
- Physical presence of only one semilunar valve:
1. Aortic atresia
2. Pulmonary atresia
3. Truncus arteriosus - P2 not audible:
4. Tetralogy of fallot (pulmonary stenosis)
5. Transposition of great arteries - A2 delayed:
6. Severe aortic stenosis
What is the cause of a loud P2?
Pulmonary hypertension since pulmonary valve is closing against pressure
Name the 4 cyanotic CHD with increased pulmonary blood flow?
- Single ventricle pathology
1. HLHS
2. Truncus arteriosus
- Transposition of the great vessels
- TAPVR without obstruction
***These cause cyanosis because of systemic and venous blood mixing
Name the 4 cyanotic CHD with decreased pulmonary blood flow?
- TOF
- Pulmonary atresia
- Tricuspid atresia
- TAPVR
***These cause cyanosis because of obstruction to pulmonary blood flow
What syndrome is associated with pulmonary stenosis and hypercholesterolemia?
Alagille syndrome (hypercholesterolemia is secondary to liver disease)
What evaluations can be done if a vascular ring is suspected? (5)
- CXR: to identify right sided aortic arch
- Barium swallow: was the gold standard; confirms external indentation of esophagus in 95% of cases
- MRI: noninvasive and is the primary diagnostic modality
- Arteriogram: rarely used
- Echocardiogram: cannot identify the ring itself but can rule out congenital heart lesions which can occur simultaneously
What is commotio cordis?
Ventricular fibrillation due to a blunt, non-penetrating direct blow to the chest during the vulnerable phase of repolarization
What percentage of HOCM is genetically inherited?
-what is the inheritance pattern?
60% of HOCM is genetic
-AD
What are the 4 common causes of AV block?
- Myocarditis
- Maternal history of lupus
- Lyme disease
- Acute rheumatic fever
What cardiac defect is associated with maternal lithium use?
Ebstein’s anomaly
What are the features of VACTERL syndrome?
Vertebral anomalies
Anal atresia
Cardiac defects
TEF
Renal agenesis/dysplasia
Limb defects
What cardiac condition is associated with bilateral rib notching?
Coarctation of the aorta = due to collateral vessels forming around the ribs impairing bone growth
Name two cardiac conditions in which a decrease or disappearance of a murmur is BAD NEWS BEARS.
- Tetralogy of fallot: indicates worsening RV outflow tract obstruction
- VSD: indicates that Eisenmenger syndrome has developed with increased pulmonary vascular resistance and thus reversal of shunt from R to L
What are the ECG findings of hypokalemia? (3)
- Prominent U wave (small deflection immediately following the T wave
- Diphasic T wave
- Depressed ST segment
What are the ECG findings of hyperkalemia? (4)
- Prolonged PR interval
- Wide QRS
- Tall, peaked T waves
- Sinusoidal wave
What is the definition of torsades de pointes?
Polymorphic ventricular tachycardia with a prolonged QT interval
What vagal maneuvers can be used to treat SVT in infants? (2)
- Ice in bag over face
- Gagging with tongue depressor
***Do NOT use ocular pressure as it has been associated with retinal damage
What are the two types of familial hypercholesterolemia?
- pathophysiology?
- features of each?
- bloodwork findings?
Lack of LDL receptors on cell membrane so no negative feedback loop to stop cholesterol production
Who should be checked for elevated cholesterol levels?
2-10 year old:
- Family history (parent or grandparent) with age 95%)
- Diabetes
- Hypertension > 95th percentile
- Smokers
- nephrotic syndrome
What is the management of hypercholesterolemia?
- Lifestyle + diet modification x 6 months, then recheck
- not a lot of research on pharmaceutical therapy on treating hypercholesterolemia in children - Statins
What bloodwork should you monitor while patients are on statins? (2)
- Liver enzymes
- CK
What are cardiac causes of SVT? (4)
- Post op cardiac
- Ebstein’s anomaly
- Cardiomyopathy
- Myocarditis
What is the lethal arrhythmia of WPW syndrome?
Atrial fibrillation which then travels down the bundle of kent accessory pathway with a rapid ventricular response leading to ventricular fibrillation
What is the most common cause of SVT in children?
WPW syndrome
What are Osler nodes?
What are Janeway lesions?
Osler nodes: PAINFUL nodules on pads of fingers and toes in IE due to immune complex deposition
Janeway lesions: PAINLESS, hemorrhagic nodular lesions on palms and soles in IE due to septic emboli
Osler is a dude so he’s whiney when it comes to pain
What are the signs of pericarditis on exam?
Friction rub, distended neck veins, hepatomegaly, pulsus paradoxus, muffled heart sounds
What is the most important complication of untreated Kawasaki disease?
Coronary artery aneurysms –> heal with stenosis –> MI
What is the typical age of children with Kawasaki disease?
6 mo - 5 yr
-however, can also happen in infants and teenagers (higher risk of developing coronary artery aneurysms, possible because of delayed diagnosis)
What condition should be suspected if you see a child with fever for 5 days and marked erythema/induration around BCG scar?
Kawasaki = early, specific marker
What is the medical management of Kawasaki disease?
- IVIG: decreases incidence of coronary artery aneurysms
- single infusion over 8-12 hrs of 2 g/kg
- if still febrile > 36 hr after first infusion, can give second infusion of 2 g/kg - High dose aspirin (80 mg/kg div q6h) (decreases fever and discomfort, unclear whether it decreases coronary artery aneurysms) until fever free x 48 hrs, then switch to low dose aspirin (3 mg/kg/d) x 6-8 wks until f/u ECHO (decreases thrombotic complications).
- If ECHO normal, d/c. If ECHO abnormal, then continue indefinitely.
What is the likelihood of developing coronary artery aneurysms with untreated vs. treated Kawasaki disease?
Untreated: 25%
Treated: Lower
What are the indications for prostaglandin E1 in the neonate?
- Inadequate pulmonary blood flow:
- Pulmonary atresia with intact ventricular septum
- Tricuspid atresia with intact ventricular septum
- critical pulmonary stenosis - Inadequate systemic blood flow:
- HLHS
- interrupted aortic arch
- Critical coarc of aorta - Inadequate mixing
- TGA
What are the actions of the following receptors:
- alpha receptors
- beta 1 receptors
- beta 2 receptors
- dopaminergic receptors
- alpha receptors: vascular smooth muscle vasoconstriction
- beta 1 receptors: myocardial smooth muscle inotropy and chronotropy
- beta 2 receptors: vascular smooth muscle vasodilation (bronchodilation)
- dopaminergic: renal and mesenteric vascular smooth muscle vasodilation
What are 2 common rhythm disturbances after the Fontan procedure?
Fontan procedure involves manipulation of the RA
- Loss of sinus rhythm –> nonsinus atrial rhythm or junctional rhythm
- Intra-atrial reentrant tachycardia
What is the most common form of ASD?
Ostium secundum
What are the findings on ECG of ASD?
- Right axis deviation
- RVH
- RSR prime
What are the indications for indomethacin in treating PDA? (3)
-dose?
What are the contraindications for indomethacin in treating PDA? (7)
Indications:
- Deteriorating respiratory status
- Poor cardiac output
- Evidence of CHF
- dose: indomethacin 0.1 mg/kg/day x 6 days
Contraindications:
- Thrombocytopenia
- Renal impairment
- Active bleeding (GI or intracranial)
- Proven or suspected untreated infection
- NEC or suspected NEC
- Evidence of bleeding diathesis
- CHD in which PDA is needed for pulmonary or systemic blood flow
What are the side effects of indomethacin?
- Renal impairment
- Platelet dysfunction
- Pulmonary hemorrhage
- GI hemorrhage
- Hyponatremia
- Hypoglycemia
- Hyperkalemia (due to decreased renal function)
What are 3 CXR findings of coarctation of the aorta?
- Rib notching
- Intrapulmonary vascular markings increased
- 3-sign on left para mediastinal shadow
- prominent aortic bulge, indentation of descending aorta, post stenotic dilation of aorta
What is the most common cardiac defect associated with Noonan syndrome and Alagille syndrome?
Pulmonary stenosis
What congenital heart defect is most commonly seen with congenital rubella syndrome?
Peripheral pulmonary stenosis (of the pulmonary arteries leading to restricted bloodflow to lungs)
What is the differential diagnosis of intracranial bruit? (9)
- Fever
- Cardiac murmur
- Intracerebral tumor
- Thyrotoxicosis
- Cerebral aneurysm
- Increased ICP
- Anemia
- AV malformation (ie. vein of galen)
- Meningitis
What is the most common cardiac finding in IDM?
-treatment
Septal hypertrophy with possible left ventricular outflow obstruction
- resolves spontaneously within 1 year
- usually requires no treatment
- may need beta blockers if severe left ventricular outflow obstruction
What is the vein of galen AVM?
-associated with? (2)
Blood is shunted from arterial vasculature into venous system so you get high output heart failure
-associated with heart failure & hydrocephalus
What cardiac defect is a common cause of infective endocarditis in the Western world?
Mitral valve prolapse
-but do NOT need prophylaxis because incidence of infective endocarditis is so low and mitral valve prolapse incidence is low
What is the most common type of cardiomyopathy caused by doxorubin?
-mortality risk?
Dilated cardiomyopathy
- dose dependent
- more likely to have cardiac damage if they have radiation treatment at the same time
- mortality risk if heart failure develops: 30-50%
- develops chronically (over months-years)
What are ECG findings of anorexia nervosa?
- Bradycardia
- Low voltages
- Prolonged QT
- Wide QRS
Where is the most common location of coarctation of aorta?
Juxtaductal (right beside PDA, below the origin of the left subclavian artery)
What are the Duke’s Criteria for infective endocarditis?
Definite diagnosis:
1. Intracardiac or embolized vegetations on histology
Possible diagnosis:
Need 2 major, 1 major + 3 minor or 5 minor
Major:
- 2 positive blood cultures
- Evidence of endocardial involvement: positive echo for vegetations OR new regurg murmur
Minor:
- predisposition:
1. Predisposition: predisposing heart condition OR IV drug use
2. Fever
3. Vascular phenomena: septic emboli to lungs, intracranial hemorrhage, conjunctival hemorrhages, janeway lesions
4. Immunologic phenomena: glomerulonephritis, Roth spots, Osler’s nodes, positive RF
5. Microbiological evidence: positive blood culture x 1 or of organism not known to cause IE
6. Echocardiographic findings: consistent with IE but does not meet major criterion as noted above
What organisms most commonly cause infective endocarditis? (4)
- what empiric abx would you start in a patient with suspected IE?
- treatment duration
- Strep viridans
- Staph aureus
- Staph epi
- H flu
- start Amp + Gent
- treatment duration 4-6 wks
What are three causes of congenital heart block?
- Maternal lupus
- Sjogren’s syndrome
- Myocardial tumors
What is Pompe’s disease?
- how is it inherited?
- clinical features?
- lab findings?
Glycogen storage disease
- glycogen accumulates in cardiac, smooth muscle, and skeletal muscle
- autosomal recessive
- Presents with severe hypotonia, cardiomegaly & heart failure, feeding difficulties, constipation, hepatomegaly
- lab findings: elevated CK, LDH, AST
What ECG findings are found in Pompe’s disease?
-CXR finding?
- High voltage QRS due to ventricular hypertrophy
- Shortened PR
CXR: ECG finding
What are anesthesia considerations in pre-op assessment of Down Syndrome patient? (6)
- Atlantoaxial instability: consider in intubation, order flex-ex views before intubation
- Large tongue: difficult airway
- Risk of post op resp infections
- Congenital cardiac lesions: if repaired, increased risk of arrhythmias post-op
- Pulmonary hypertension
- OSA
What is a cardiac condition that needs to be ruled out in every patient who has hypertension?
Coarctation of aorta
In what condition do you see radio-femoral delay?
-what is the cause?
Radio-femoral delay: seen in coarctation of aorta
-normally, femoral pulse comes before radial pulse; however, it is reversed in coarc of aorta since femoral pulse is generated by collaterals which takes longer than the generation of radial pulse by large vessels
In a patient with coarc of aorta, what does the presence of a systolic ejection click or thrill in the suprasternal notch suggest?
Bicuspid aortic valve (present in 70% of coarc cases)
What is the underlying primary defect in tetralogy of fallot leading to the classic 4 findings?
Anterior deviation of the infundibular septum (conus)
- allows for the VSD to happen since septum can’t grow up towards the conus
- anterior deviation pulls the aorta forward giving the overarching aorta
- spasm of the excess muscle causing the anterior deviation leads to tet spell
Why might a baby with TOF suddenly deteriorate at 7 days of life?
-what treatment should be started immediately?
If their right ventricular outflow tract obstruction is severe, their PDA may be their only means of pulmonary blood flow
- at 7 days, the PDA closes, thus causing them to go into cardiogenic shock
- treatment: PGE1
What is the corrective surgical therapy for tetralogy of fallot?
- Relief of RV outflow tract obstruction - resection of obstructive muscle bundles
- Patch closure of VSD
- Pulmonary valvotomy (if pulmonary valve is stenotic) or valvectomy (if pulmonary valve is thickened or too small = add in a transannular patch)
- valvotomy = leads to pulmonary insufficiency
- now we aim to leave as much of the pulmonary stenosis as we can to prevent backflow but add in a patch to allow good forward flow
2nd option (less preferred): BT shunt
What is an alternate way of obtaining a BP if you are getting an inaccurate one from the monitor?
Doppler BP! (can only get SBP from it however since the doppler is so sensitive, you will still hear the pulse down to pressure of 0 so won’t be able to obtain DBP)
How do you check for RV enlargement in a neonate?
Put your thumb under the xiphoid process - should not feel any pulse if RV is normal. If RV enlarged, will feel a pulse
What is considered a pre/pos ducal sat differential?
>10% is abnormal
What is the hyperoxic test?
- Draw ABG on RA, expect PAO2 = 80-100 mm Hg; if PAO2 150 then it is likely not cardiac disease.
****Really, it is the lack of change with 100% O2 that indicates cardiac disease
You have an unwell 2 mo baby with cyanosis and a CXR showing small heart and extremely wet lungs. What is your diagnosis?
Obstructed TAPVD
-blood is trying to get back to the heart through a small vessel and into RA. If PFO is open, bloodflow will go from RA to LA to LV to body. If PFO closes, then you’re screwed
Difference in CXR findings between obstructed TAPVR vs. unobstructed TAPVR?
Obstructed (obstructed flow into the heart): small heart, very wet lungs (looks reticular, like RDS)
Unobstructed (L to R shunt): snowman sign, enlarged heart
What is the classic finding of coarctation of aorta in neonate on ECG?
-in children?
- in neonates: RVH on ECG since it is the right heart that’s been pumping through the PDA against the coarctation
- in children: LVH on ECG since it’s the left heart that’s pumping against the coarctation
What is the starting dose of PGE1?
-other management with starting PGE1?
Start high at 0.2 mcg/kg/minute IV
- Give a fluid bolus 20 cc/kg prior to prevent hypotension, run TFI at 130-150 cc/kg/d
- Best to leave baby free breathing if stablized from above. Intubate if severely acidotic
What is the most common type of VSD?
Perimembranous (70%)
When is surgical correction indicated in VSD?
When there is no weight gain
What are the V4R and V3R for on ECG?
V4R, V3R = look at right sided pathology
What is the fast way to access rate on ECG?
300, 150, 100, 75, 60, 50 for each big box (5 little boxes)
What are the criteria for sinus rhythm? (2)
- P for every QRS and QRS for ever P
- Normal P axis
At what age does RAD disappear on ECG?
After 6 months
What does tall P wave on ECG signify? What about wide P wave or bifid?
Tall = right atrial enlargement
Wide or bifid = left atrial enlargement
What is the treatment for first degree AV block?
Nothing - usually asymptomatic, seen in athletes, anorexia nervosa
What are 4 potential causes of third degree (complete) AV block?
Myocarditis, lyme disease, congenital, cardiac surgery
Which leads should you see Q waves?
Q waves = septal depolarizaion
- should be in the leads that look left (V5-V6) and leads that look down (II, III)
- abnormal if absent in the expected leads or presence in other leads
- common in African American population
What is the difference between complete vs. incomplete RBBB?
-what is the significance of the LBBB?
Complete = > 3 small squares = common in post op cardiac paients
What is the normal QTC in males vs. females?
QTC 500 msec, the risk of arrhythmias increases dramatically
What are 2 signs on ECG of acute pericarditis?
- Depression of PR interval
- Diffuse ST elevation
What does it mean if you have normal V1 and V6 but deep or tall V2-V4?
Nothing. This is normal in children
What are ECG findings of LVH?
- ST depression or T wave inversion in V5-7
- S wave in V1 > 95h percentile for age
- R wave in V6 > 95th percentile for age
- Deep Q waves in V5-7
What is a possible cause of myocardial ischemia in a young child?
Anomalous left coronary artery from the pulmonary artery = see biventricular hypertropy, Q wave in V6, ST elevation
Which CHD are essentially normal children post-op (and thus we do not need to call cardio if these children present in ED)? (5)
No exercise restrictions, no limitations, they are essentially a normal child
- VSD
- ASD
- AVSD
- TAPVD
- Coarctation
***Only care if there are residual lesions
Which post-op CHD should we worry about if they present to ED (and thus call cardio)?
Degree of worry is based on residual defect:
- TOF
- TGA
- HLHS
Essentially, worry MOST about Norwood, Glenn, Fontan, PA/VSD/MAPCAs (multiple aortopulmonary collaterals) = call cardio always if these patients come in for anything
Why do we worry about children with a transannular patch for CHD presenting with gastroenteritis?
May have stiff RV = very dependent on preload for cardiac output to the lungs for oxygenation
What are post-op complications of tetralogy of fallot?
-do we worry if they get a URTI/LRTI?
- Severe PI –> dilated RV –> decreased RV function
- Branch PA stenoses
- Arrhythmias (RBBB) due to RV dilatation/transannular patch
- since we expect them to be normal hemodynamically post-op, don’t worry about resp infections
What is the surgical repair for TGA?
- what are post-op complications? (3)
- a patient post op TGA presents with chest pain. Worried or not worried?
Arterial switch operation at 2nd week of life (Jatene operation)
- coronary buttons are reimplanted
- LPA and RPA are draped in front of the aorta = pulling the PA anterior and can cause PA stenosis
Post-op complications:
- Branch PA stenosis
- Proximal coronary button stenosis
- Dilatation of ascending aorta
Chest pain? = WORRY!
Which CHD undergo norwood/glenn/fontan repair?
Overall: these have a single usuable ventricle and an unusable ventricle:
No pulmonary output: TA, PA, PS, Ebstein’s
OR
No systemic output: HLHS, MS
OR
Unobstructed pulmonary flow: single ventricle with no PS, DORV with PS
What is the Norwood 3 stage palliation?
-ages for each?
Stage 1: norwood @ 7 d old
Stage 2: Glenn @ 4-6 mo
Stage 3: Fontan @ 3 yo
What is the norwood operation?
-what happens if you give these children O2?
Overall: want the heart to be one big bag that pumps to the body and not at all to the lungs
- Atrial septectomy (take out the atrial septum completely)
- Cut the MPA off the heart
- Take the outflow tracts (aorta and MPA) and merge them with a patch (pericardium or gortex) = sew together = DKS = side to side anastamosis
- BT shunt = systemic to pulmonary shunt = allows just enough blood to go to the PA from the systemic circulation
- it does siphon away blood from the coronaries during diastole (blood can choose to go through the aorta to the coronary OR through the BTS to the PA…will usually choose BTS because pulmonary pressures are lower than systemic = this is why 30% of children die from this = coronary steal)
- new procedure now = hybrid procedure: only put in a PDA stent with bilateral PA bands so that there is still pulmonary flow from the descending aorta without compromising coronary artery flow, then postpone the rest of the Atrial septectomy, MPA removal, etc. in Glenn procedure
- normal stas: 75-85%
- O2 causes pulmonary vasodilation and increases pulmonary blood flow; this is BAD because it STEALS the blood from their systemic circulation and can cause arrest so do NOT target 100% sats!
What are the expected O2 sats for post-Glenn?
-what happens if you give O2 to Glenn?
Normal sats = 75-85%
- O2 causes pulmonary vasodilation and increases pulmonary blood flow and increase cardiac output since the blood will then go back to the body
- if high PVR = blood can’t flow to lungs and thus blood can’t flow to the system since they have a SERIES circuit instead of parallel
What are 3 questions to ask the patient who has gone through the Norwood 3 stage procedure? (ie. fontan is completed)
- What are your baseline sats?
- Are you fenestrated?
- protective mechanism against pulmonary hypertension
- if the pressures in the lung are so high that blood can’t flow there, then at least the blood will flow from the extracardiac conduit (tube that connects IVC to PA) to the right atrium (that isn’t being used), through the ASD, into the LA –> LV to be pumped to the body = at least you’ll get some O2 to systemic circulation
- this is why they are on anticoagulation = if they have a thrombus somewhere, can flow from right side to left side and get a stroke!!! Bad!
- if yes: sats can be low 85%
- if no, sats should be normal - Is your warfarin therapeutic?
What is the Fontan procedure?
-complications? (4)
Essentially, you cut the IVC off the heart and connect it to an extracardiac conduit which then connects to the PA
- you do get sluggish flow
1. Fontan circuit obstruction (thrombosis, vascular stenosis)
2. Outflow obstruction
3. Ventricular dysfunction
4. Arrhythmias
5. Strokes
What does 80% sat on a finger mean in a kid with a single ventricle?
Tells you there is equal bloodflow to the lungs and the body
Ao sat% - SVC sat% / PV Sat % - PA sat%
-Ao and PA sats should be the same
-SVC sat % = expected to be 60%
-PV sat% = expected to be 100%
= 80-60/100-80 = 20/20 = 1:1 = this is why we target 80% sats!
If sats = 90%, this tells you there is too much PBF and not enough to body
If sats = 70%, there is too little PBF
-if you have wet lungs, then this is BAD, means overcirculation BUT your sats will be high so don’t let this trick you
What are the effects of the following on children with single ventricle pathophysiology?
- URTI
- Gastroenteritis
Treatment?
Colds increase PVR thus decreasing PBF
V&D decreases SBF from hypovolemia
Treatment: GIVE VOLUME!!! Don’t be worried about giving them too much fluid!
What is the cause of sinus arrhythmia?
Increased vagal tone
-HR increases with inspiration, decreases with expiration
What are 5 causes of first degree AV block?
- High vagal tone
- Acute rheumatic fever
- Hyper/hypokalemia, hypermagnesemia
- Drug effect/toxicity (digoxin)
- Lyme disease
- CHD: ASD, AVSD, ebstein’s anomaly
What are the 2 types of second degree AV block?
Mobitz I (Wenckebach): progressive PR lengthening prior to block -usually due to high vagal tone
Mobitz II: no change in PR interval before block
- THIS is the one that should worry us!
1. Exposure to maternal autoantibodies (ie. SLE)
2. Drugs
3. CHD
What are 2 common causes of 3rd degree block?
- Postop CHD
- Lyme disease
What are PACs?
-treatment?
Premature P waves differing in axis and morphology from normal sinus P waves
-AV conduction may be normal, aberrant (BBB), or blocked
-Rarely require treatment
(50% newborns, 14-64% infants & children)
How can you tell the difference between AVRT and AVNRT on ECG? (2)
AVRT = retrograde P waves following QRS, with RP interval > 0.07 sec
-more common in infants/children
AVNRT: retrograde P waves within QRS or at terminal end, RP internal
How does the valsalva work for SVT?
Increases intra-thoracic pressure and affects baro-receptors (pressure sensors) within the arch of the aorta = increases vagal tone = needs to be in the supine position x 15 seconds
What 5 medications can be used for acute treatment of SVT?
-what 4 medications can be used for chronic treatment of SVT?
Acute treatment:
- Adenosine
- Verapamil
- Procainamide
- Esmolol
- Amiodarone
Chronic treatment:
- Propranolol
- Sotalol
- Flecainide
- Propafenone
What 2 populations will you see atrial flutter in?
Atrial rates 250-480 bpm
- Neonate with structurally normal heart
- Older patient s/p CHD surgery
What 2 populations will you see atrial fibrillation in?
-treatment?
Atrial rates > 350 bpm
“Irregularly irregular” ventricular response
1. CHD
2. Thyrotoxicosis
-if no prior CHADS (congestive heart failure, hypertension, diabetes, stroke), no oral anticoagulation needed. Just cardiovert
Why do we not worry about accelerated idioventricular tachycardia?
Not worrisome because starts and stops by itself, rate is not that much faster than sinus
-still important to make sure heart is structurally normal with an ECHO
How much deoxygenated hemoglobin must be present before you can clinically see cyanosis?
- how does this change in a patient with anemia?
- how does this change in a patient with polycythemia?
5 g/100 ml of deoxygenated hemoglobin must be present before cyanosis can be appreciated
- in anemic patient: have much less hemoglobin so need higher percentage of reduced hemoglobin before cyanosis will be appreciated. Thus they might be hypoxemic without showing any cyanosis
- in polycythemic patient: have much more hemoglobin so need lower percentage of reduced hemoglobin before cyanosis will be appreciated. Thus they might be cyanotic without actually being hypoxemic
What is the treatment for hypertrophic cardiomyopathy?
- No competitive sports and strenuous physical activity = risk of sudden death
- Beta blockers or calcium channel blockers = diminish ventricular outflow tract obstruction, modifies ventricular hypertrophy, improves ventricular filling
- Anti arrhythmics or implantable cardioverter defibrillator = if patients have history of arrhythmias, strong family histories of arrhythmias or sudden death, patients with syncope
- Screen first degree relatives with ECG and ECHO, genetic testing
What connective tissue diseases may be associated with MVP?
Marfan syndrome, Ehlers-Danlos syndrome, pseudoxanthoma elasticum, osteogenesis imperfecta, and Hurler syndrome may be associated with MVP.
Name five disorders in which a screening ECG might identify a subject at risk for sudden death
- Wolf-Parkinson-White syndrome: short PR, delta wave, T wave abnormalities leading to supraventricular tachycardia and ventricular fibrillation
- Prolonged QT syndrome: Secondary to congenital channelopathy, electrolyte or drug-induced abnormality leading to ventricular tachycardia and torsades de pointes
- Brugada syndrome: Right ventricular conduction delay with profound ST elevation in V1-V3 leading to ventricular fibrillation
- Hypertrophic cardiomyopathy
- AV block
What are two of the more common neuromuscular diseases in which a cardiac consultation is routinely recommended?
•
Duchenne muscular dystrophy is an X-recessive disease with an abnormality in the dystrophin gene, which leads to muscle necrosis and fibrosis. Although the majority of deaths are due to respiratory insufficiency, death from cardiomyopathy can occur in up to 25% of patients. Symptoms of heart disease are typically hidden by the skeletal myopathy that masks any exercise-induced complaints such as shortness of breath with exertion. Therefore, screening echocardiograms and ECGs are recommended for long-term follow-up.
•
Friedrich ataxia is an autosomal recessive disorder involving a gene encoding frataxin, a mitochondrial protein. Symptoms include ataxia and muscle weakness, typically manifesting by 9 years of age. Cardiac abnormalities include both dilated and concentric cardiomyopathies. Atrial fibrillation and atrial flutter are commonly reported arrhythmias. Because muscle weakness and ataxia will prevent prolonged exertion, periodic echocardiograms and ECGs are recommended.
Decreased pulmonary markings (diminished pulmonary blood flow)
Pulmonary atresia or severe stenosis
•
Tetralogy of Fallot
•
Tricuspid atresia
•
Ebstein anomaly
Increased pulmonary markings (increased pulmonary blood flow)
•
Transposition of great arteries
•
Total anomalous pulmonary venous return
•
Truncus arteriosus
What are the common ductal-dependent cardiac lesions?
Ductal-dependent pulmonary blood flow
•
Critical pulmonary valve stenosis
•
Pulmonary atresia
•
Tetralogy of Fallot with severe pulmonary stenosis
•
Tricuspid atresia with pulmonary stenosis or pulmonary atresia
Ductal-dependent systemic blood flow
•
Coarctation of the aorta
•
HLHS
•
Interrupted aortic arch
What should parents be told about the risk for recurrence of common heart defects?
The risk for CHD in pregnancies after the birth of one affected child is about 1% to 4%. With two affected first-degree relatives, the risk is about 10%. With three affected children, the family may be considered at even higher risk.
Common Cardiac Causes of Congestive Heart Failure in a 6-Week-Old Infant
1.
Ventricular septal defect
2.
Atrioventricular canal
3.
Patent ductus arteriosus
4.
Coarctation of the aorta
the murmurs
If both parents are asthmatic, what is the risk that their child will have asthma?
The risk is 60%. For a child with only one parent with asthma, the risk is estimated to be about 20%. If neither parent has asthma, the risk is 6% to 7%.
A patient with chronic sinusitis and recurrent pulmonary infections has a chest radiograph that demonstrates a right-sided cardiac silhouette. What diagnostic test should be considered next?
Bronchial or nasal turbinate mucosal biopsy for electron microscopic evaluation of cilia should be performed. Kartagener syndrome is one of the ciliary dyskinesia (or immotile cilia) syndromes
A 2-day-old infant presents in congestive heart failure. He has hydrocephalus. He has a seizure 1 hour after admission. Most likely cause:
a) vein of Galen aneurysm
b) intraventricular hemorrhage
c) hypoxic ischemic encephalopathy d) cerebral abscess
e) meningitis
a) vein of Galen aneurysm
- Which of the following is associated with an increased risk of necrotizing fasciitis?
a) parvo virus
b) roseola
c) Kawasaki disease d) varicella
e) rosacea
d) varicella
PGE SE
- apnea/respiratory depression
- cutaneous vasodilation/flushing (10%)
- fevers
- hypotension
PDA NSAID SE
- renal failure
- decreased u/o
- bleeding (platelet adhesion defect)
- IVH
- GI bleeding/NEC -CNS bleeding -hyponatremia
List four clinical signs of endocarditis in a patient with fever and a new murmur.
- splenomegaly, petichiae
- osler nodes, janeway lesions, roth spots, splinter hemorrhages
-tachycardia -evidence of CHF -athritis
4 risk factors for atherosclerosis.
- smoking
- DM2
- hyperlipidemia
-early MI in family
Neonate with complete heart block. ECHO normal. List 2 causes.
-congenital: maternal lupus or Sjogren syndrome other AI disease
- genetic: long QT
- Congenital malformation of conduction system – Kearn-Sayre syndrome
2 year old with signs of CHF. EKG looks like SVT. Give 3 things that would suggest SVT on EKG
Narrow complex
Tachycardia >200
No P waves visible, abnormal P waves
WhatisthemostsensitivePFTforsmallairwaysdisease:
a) FEV1
b) FEV1/FVC
c) FEF25-75
c) FEF25-75
A patient with recurrent pneumonia, sinusitis, bronchiectasis in RML. One should investigate for all of the following except:
- CF
- Alpha-1-antitrypsin deficiency
- Ciliary dyskinesia
- Hypogammaglobulinemia
- CGD
- Alpha-1-antitrypsin deficiency
4 causes of a false postive sweat chloride test
- eczema
- ectodermal dysplasia
- malnutrition/FTT
- anorexia nervosa
- CAH
- adrenal insufficiency
- glucose-6-phosphatase deficiency -nephrogenic DI
- hypothryoidism
Teenager with history of asthma. She is currently on Fluticasone 125 mcg bid. She has been needing to use her Ventolin puffers, two to three times a week in the day time over the past while. What four suggestions could you make for her management?
- ensure correct technique with MDIs, ensure compliance -evaluate for triggers: ?environmental, ?allergies
- could increase dose of fluticasone switch to ciclesonide -could add singular or LABA
- CXR, PFTs
- evaluate for possible VC dysfunction, anxiety component to symptoms -teach how to do peak flow monitoring at bedside
You are unable to obtain a sweat sample in a neonate. List 3 other ways to confirm the diagnosis of CF.
- nasal potential difference
- genetic test for common mutations
- newborn screen (serum immunoreactive trypsinogen)
