Resp/Cardio Flashcards

Question 1

Q

What is the cardiac lesion represented in this CXR?

Question 2

Q

What is the cardiac lesion represented in this cxr?

Answer

A

Tetralogy of fallot

Question 3

Q

What are the characteristics of Marfan syndrome?

Answer

A

Fibrillin gene defect

AUTOSOMINAL DOMINANT

Sx: thumb sign, pectus excavatum, scoliosis, long arms

Dilatation of the ascending Ao and MVP (mid systolic click)

Question 4

Q

What does an ASD murmur sound like?

Answer

A

Low pitched systolic ehection murmur at base

Fixed split S2

Question 5

Q

What does an AS murmur sound like?

Answer

A

SEM radiating to neck

Question 6

Q

What are the characteristics of cardiac syncope?

Answer

A

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

Question 7

Q

When does Torsades des Pointes occur?

Answer

A

Long QT and hypomagnesemia

Question 8

Q

What are the clues on history to long QT syndrome?

Answer

A

Unusual sz, palpitations while swimming

Deafness

FHx of sudden death, unexplained MVA, drownings, deafness

Question 9

Q

What are the causes of long QT interval?

Answer

A

Low Ca

Low Mg

Low K

Drugs (TCAs)

Question 10

Q

What is WPW?

Answer

A

Wolff Parkinson White

Accessory AV pathway allows for “early” depolarization of ventricles

Ass with ebsteins and CCTGA

Can cause SVT and sudden death

Question 11

Q

What is the first step of the SV palliation?

Answer

A

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%

Question 12

Q

What are the recommendations for antiplatelet therapy in Kawasaki disease?

Answer

A

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

Question 13

Q

What are the general guidelines for SBE PPx?

Answer

A

High risk lesion + high risk procedure

Question 14

Q

What qualifies as a high risk lesion?

Answer

A

Cyanotic CHD, not repairs, shunts
CHD repaired with prosthetic material
CHD with residual defects

Question 15

Q

What is considered a high risk procedure?

Answer

A

Dental procedure where the gums or lining of mouth likely to be injured

Gut or GU surgery through infected area

Question 16

Q

Things that do not need antibiotics

Answer

A

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

Question 17

Q

What are the cynaotic CHD lesions?

Answer

A

6Ts: TGA, TOF, TA, TAPVR, TA, “TINGLE” ventricle

2As: PA, Ebsteins anomaly

Question 18

Q

What are the lesions that cause CHF in the first weeks of life?

Answer

A

OBSTRUCTION

HLHS
Severe AS
Coarctation
Asphyxia
Severe MR, TR
Uncontrolled tachycardias

Question 19

Q

What are the lesions that cause CHF in weeks 2-6?

Answer

A

Left to right shunts:

VSD

AVSD

PDA

Question 20

Q

What are the lesions that cause CHF inolder children?

Answer

A

Pump failure

Dilated cardiomyopathy

Myocarditis

Tachycardias

Question 21

Q

What are the three cardinal signs of CHF in infants?

Answer

A

Tachycardia

Tachypnea

Hepatomegaly

Question 22

Q

What are the signs of infective endocarditis?

Answer

A

Janeway lesion

Embolus

Splinter hemorrhages

Oslers node: PAINFUL

Question 23

Q

What is rheumatic fever?

Answer

A

Occurs after GAS infection

Pancarditis: aschoff bodies (collection of immunological cells and fibrinoid material around a central necrosis)

Question 24

Q

What are the diagnostic criteria for rheumatic fever?

Answer

A

Major: (2)

Carditis
Polyarthritis
Chorea
Subcutaneous nodules
EM

Minor:

Fever
Arthralgia
Prolonged PR
Elevated ESR, CRP

Question 25

Q

What are the complications of foreign body?

Answer

A

Recurrent pneumonia

Bronchiectasis

Cardiac arrest and death

Question 26

Q

In what findings MUST you r/o cystic fibrosis?

Answer

A

Meconium ileus

HypoNa, hypoCl metabolic alkalosis

Recurrent rectal prolapse

Clubbing

Bronciectasis

Nasal polyps

Pseudomonas colonization

Question 27

Q

What is the test positive in a sweat chloride?

Answer

A

>60 meQ/L

Question 28

Q

What are the prognostic factors in CF?

Answer

A

Male?female

Lung disease:FEV1, Burkohderia, PTX

Nutritional status: Wt/Ht, DM

Question 29

Q

How does primary ciliary dyskinesia?

Answer

A

Year round daily wet cough

Year round nasal congestion

Recurrent otitis media

Neonatal respiratory distress

Laterality defects

Question 30

Q

What qualifies as asthma control?

Answer

A

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

Question 31

Q

How do you diagnose asthma?

Answer

A

Clinical symptoms

+

Objective evidence: spirometry, peakflow, methacholine challenge

Question 32

Q

What are the sequalae of OSA?

Answer

A

Neurocognitive: behavioural, attention, schoolperformance, developement

Cardiovascular: HTN, cor pulmonale

FTT

Inflammatory

Qualityof life

Question 33

Q

How do diagnose OSA?

Answer

A

Polysomnography

Question 34

Q

What are the treatment modalities for OSA?

Answer

A

Non-pharmacologic: Nasal and sleep hygiene

Pharmacologic: intranasal corticosteriods, montelukast

Surgical: T&A

Weight loss

CPAP

Question 35

Q

What is the most common CHD?

Answer

A

VSD 25-30%, ASD +PDA 6-8%

Question 36

Q

What is the hyperoxia test?

Answer

A

If PaO2 rise above 150 mm during 100% O2 administration then a shunt lesion is excluded

Question 37

Q

What % of small VSD will close?

Answer

A

75% in the first 2 years of life

Question 38

Q

What are the potential complications of a VSD?

Answer

A

Endocarditis

Aortic regurg

Subaortic stenosis

RVOTO

LV TO RA shunting: Gerbode defect

Question 39

Q

What are the indications for surgery with a VSD?

Answer

A

< 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

Question 40

Q

What are the three types of ASD?

Answer

A

Primum (15%)

Secundum (75%)

Sinus venosus (10%)

Question 41

Q

What are the features of an ASD?

Answer

A

Grade 3 SEM at ULSB with widely split S2

Age 3-4 years

Question 42

Q

What are the four features of tetralogy of fallot?

Answer

A

PS

Overriding aorta

RVH

VSD

Question 43

Q

What syndromes are associated with TOF?

Answer

A

T21, DiGeorge, Alagille

Question 44

Q

If you find long Qt, what should you check

Answer

A

Ca, Mg, K, Drugs (TCAs)

Question 45

Q

How do you approach SVT?

Answer

A

Stable: Vagal, Adenosine

Unstable: Syn cardio 0.5-1 J/kg

Question 46

Q

What is the description of a stills murmur?

Answer

A

VIbratory LLSB SEM, changes with position

DDx: SubAo stenosis, Small VSD

Question 47

Q

What is the description of a venous hum?

Answer

A

Infraclavicular hum, continuous R>L

DDx: PDA

Question 48

Q

What is the differential for a widely split S2?

Answer

A

AS, PS, Ebstein, TAPVR, RBBB

Question 49

Q

DDX for a wide pulse pressure?

Answer

A

PDA

Ao insuff

AVM

Vasodilation

Question 50

Q

What defect is associated with alpha 1 AT deficiency?

Answer

A

PiZZ

Will present as liver disease in children

Question 51

Q

What are the characteristics of CCAM?

Answer

A

Cystic adenomatoid malformation CCAM

Hamartomatous or dysplastic lung tissue mixed with normal lung

Presents in infancy

Question 52

Q

What are the characteristics of congenital lobar emphysema?

Answer

A

Presents at 6 months: resp distress

HYperinflation of affect lobe with atelectasis of other

Question 53

Q

What is the acronym to remember the compotents of CF?

Answer

A

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

Question 54

Q

What is the progression of bugs in CF?

Answer

A

S aureus, H flu, pseudo aeroginosa, B cepacia

Question 55

Q

What are compotents of routine care for CF?

Answer

A

Chest physio + bronchodilatory BID

Balanced high calorie diet

Pancreatic supplemnts

ADEK replacement

Osteoporos monitoring

GERD monitoring

Question 56

Q

Whatis kartagener syndrome?

Answer

A

Situs inversus (25% will have PCD)
Chronic sinusitis and otitis
Airway d/po leading to bronchiectasis

Question 57

Q

What is the most common cause of chest pain?

Answer

A

MSK

Cardiac <1%

Question 58

Q

What are the common MSK causes of CP?

Answer

A

Strained msucles

Costochondritis

Tietze syndrome

Precordial catch syndrome

Question 59

Q

What is tietze syndrome?

Answer

A

Localized form of costochondritis usually involving junst one costochondral junction, tender swollen mass palpable at the site

Costochrondritis is usually multiple sites without swelling

Question 60

Q

What is precordial catch syndrome?

Answer

A

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

Question 61

Q

What is the difference between a vascular ring and sling?

Answer

A

Ring: trachea and/or esophagus is encircled by aberrant vascular strctures

Sling: compressions by nonencircling vessels

Question 62

Q

What are the two types of complete vascular rings?

Answer

A

Double Ao arch
Right Ao arch with left ligamentum arteriosum

Question 63

Q

What are the cardiac causes of sudden death?

Answer

A

HOCM
Anomalies of cornoary arteries
Margan
Arrhythmogenic right ventricular dysplasia (ARVD)
Brugada
Prolonged QTC

Question 64

Q

What is commotio cordis?

Answer

A

Arrhythmia as a result of blunt, nonpenetrating direct blow to the chest

VF occurs during vulnerable phase of repolarization

CPR +Defib

Answer 64

A

Differential BP

Systolic murmur in the back

SystolicHTN in the upper extremities

Diminished femorals

Answer 65

A

DMD (Dystrophin)-Cardiomyopathy

Friedrich ataxia (Frataxin)-Cardiomyopathy, A fib/fliutter

Answer 66

A

TOF
Pulmonary Atresia

Tricuspid atresia

Ebstein

Answer 67

A

Transposition

TAPVR

Truncus arteriosis

Answer 68

A

Boot: TOF
Egg: TGA

Snowman: TAPVR

Rib notching: Coarctation

Answer 69

A

TOF: worsening RVOTO

VSD: eisenmenger syndrome

Answer 70

A

6 months or older

Answer 71

A

RV dominance

T waves upright in chest leads, invert at 7 days until 12-13 years

Answer 72

A

WPW
AV nodal reentry

Answer 73

A

Unexplained CHF

Tachycardia out of proportion to fever, tachypnea, quiet precordium, muffled HS, gallop rhythm without murmur, HM

Answer 74

A

CP, fever, cough, palpitations

Friction rub, pallor, pulsus paradoxus

Pt sitting up and leaning forward

Answer 75

A

Decreased renal function
Hyponatremia
Platelet dysfunction
GI blood loss

Answer 76

A

Apnea, fever, cutaneous flushing, seizures, hypotension, bradycardia

Answer 77

A

Vascular smooth muscle, vasoconstriction

Answer 78

A

Myocardial smooth msucle, increase myocardial contractility (inotropic), cardiac rate (chronotrophic) and AV conduction (dromotophic)

Answer 79

A

Vascular smooth muscle, vasodilation

Answer 80

A

Renal and mesenteric vascular smooth muscle

Vasodilation

Answer 81

A

Venous hum= increased

Vinratory=decreases

Answer 82

A

Anastomosis between subclavian artery and pulmonary artery

Answer 83

A

Loss of sinus

INtra-atrial reentrant tachycardia

Answer 84

A

Fontan

Abnormal flow to mesenteric vasculature

Answer 85

A

40% of children experience

Answer 86

A

Laryngomalacia

Answer 87

A

Defect in the CF transmembrane conductance regulator (CFTR) protein

Regulates chloride and sodium transfer across the apical membrane of the epithetial cells

Answer 88

A

Deep, slow resps with prolonged exhalation

Answer 89

A

Crescendo-descendo respira with alternating apnea

Answer 90

A

AKA ataxic breathing

Unpredictable irregularity in respirations

Answer 91

A

Nutrition
Diuretics
ACEi

Answer 92

A

Secundum 75%

Answer 93

A

Right heart failure
Embolism
Arrhythmia
PVD

Answer 94

A

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

Answer 95

A

Hypercalcemia

Elfin features

Supravalvular AS

PA Branch stenosis

MR

Answer 96

A

Normal viscera, lungs and atrial position

Answer 97

A

Abdo, atria and lungs and reversed

Answer 98

A

Afterload reduction
Preload reudction
Sympathetic inhibition
Cardiac remodelling prevention
Inotrophy

Answer 99

A

Coxsackie

Echovirus

Adenovirus

EBV

Influenza

HIV

Answer 100

A

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

Answer 101

A

Chest wall compliance

FRC= Outward elastic recoil= inward lung recoil

Infants, more compliant chest wall therefore lower FRC

Answer 102

A

CNS depression =chest wall retraction
Poor lung compliance= lung recoil, loss of FRC
Compliant chest wall= loss of FRC

Answer 103

A

Property of a substance to oppose deformation or stretching

Change in pressure over change in volume

Enbales return to original state

Answer 104

A

repicrocal of elastance

Distensibility

Change in volume/change in pressure

Answer 105

A

Amount of pressure required to generate flow of gas across airways

R= 8 x length x viscosity/R⁴

Answer 106

A

1/2 height if hemithorax = large

Answer 107

A

Increased cough/sputum
Hemoptysis
Anorexia and weight loss

Answer 108

A

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?

Answer 109

A

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

Answer 110

A

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

Answer 111

A

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

Answer 112

A

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

Answer 113

A

Edema! (ie. hypoalbuminemia)
-other causes: malnutrition, hyponatremia (not enough sweat to collect)

Answer 114

A

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

Answer 115

A

Autosomal recessive

Answer 116

A

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

Answer 117

A

CF test positive if > 60 mEQ/L
-negative if

Answer 118

A

Nutrition
Chest physiotherapy
Antibiotics

Answer 119

A

V/Q mismatch
Shunt
Hypoventilation
Low FiO2
Diffusion barrier
Abnormal Hgb saturation curve

Answer 120

A

Increased risk of infection secondary to trapping of secretions
Increased risk of pneumothorax
Increased risk of pulmonary hemorrhage (and thus hemoptysis)

Answer 121

A

Diffuse wheezing:

CF
Asthma
Bronchiolitis
Anaphylaxis with resp involvement
GERD

Localized wheezing:

Foreign body
Extrinsic compression by mass
Bronchomalacia

Answer 122

A

Granuloma formation
Interstitial fibrosis
Lipoid pneumonia = through oral or nasal administration of animal or vegetable oils to treat childhood illnesses as a folk remedy

Answer 123

A

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

Answer 124

A

***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

Answer 125

A

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

Answer 126

A

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)

Answer 127

A

Relaxation breathing techniques
Inhalation of heliox to relieve vocal cord spasm

Answer 128

A

Airflow limitation
- low FEV1
- FEV1/FVC ratio 12%
Exercise challenge: worsening in FEV1 > 15%

Answer 129

A

Cystic fibrosis
Aspergillus (proximal bronchiectasis)
Ciliary dyskinesia
Immunodeficiencies

Answer 130

A

Daytime

Night

Answer 131

A

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

Answer 132

A

Hirschsprung disease: 20% of CCHS children
-should undergo rectal biopsy to screen for absence of ganglion cells

Answer 133

A

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

Answer 134

A

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

Answer 135

A

Metabolic: insulin resistance, dyslipidemia
Cardiovascular disease
Pulmonary hypertension with cor pulmonale
Neurocognitive disturbances

Answer 136

A

Metabolic: insulin resistance, dyslipidemia
Cardiovascular disease
Pulmonary hypertension with cor pulmonale
Neurocognitive disturbances

Answer 137

A

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?

Answer 138

A

Gold standard test: polysomnography (sleep study)

1st line treatment: Adenotonsillectomy
2nd line: CPAP if surgery fails or pts who are not candidates for surgery

Answer 139

A

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

Answer 140

A

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)

Answer 141

A

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

Answer 142

A

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

Answer 143

A

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.

Answer 144

A

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

Answer 145

A

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

Answer 146

A

Parenchymal inflammation with a cavity containing an air fluid level

Answer 147

A

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)

Answer 148

A

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

Answer 149

A

Test for cystic fibrosis regardless of race or ethnicity

Answer 150

A

Nuts!
-must ask parents specifically about nuts; if there is any history of eating nuts, bronchoscopy should be carried out immediately

Answer 151

A

60%
-most important factor: history! (imaging may be negative in up to 30% of cases)

Answer 152

A

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

Answer 153

A

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

Answer 154

A

Direct lung injury

Pneumonia (most common)
Aspiration
Pulmonary contusion
Submersion injury
Inhalational injury

Indirect lung injury:

Sepsis
Shock
Burns
Transfusion related
Trauma

Answer 155

A

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

Answer 156

A

Acute onset
PaO2/FiO2

Answer 157

A

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)

Answer 158

A

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

Answer 159

A

IV enzyme replacement from pooled human plasma

Answer 160

A

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

Answer 161

A

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

Answer 162

A

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%

Answer 163

A

Height velocity
Cataracts

***Does not affect adult height

Answer 164

A

Thrush
Hoarse voice (dysphonia)

Answer 165

A

Tall, thin male with subpleural bleb

Answer 166

A

Thymoma
T cell leukemia
Terrible lymphoma
Thyroid tumors
Teratoma

Answer 167

A

A+B

Adenopathy: infectious (histoplasmosis most common), neoplastic, metastatic, sarcoidosis
Bronchogenic cyst

Answer 168

A

The Ns

Neurogenic tumor: neuroblastoma, benign ganglioneuroma, ganglioneuroblastoma
Neurofibroma
Esophageal duplication cysts
Pulmonary sequestration

Answer 169

A

Mycoplasma chlamydia and mycoplasma pneumoniae

Answer 170

A

Proximal bronchiectasis
Bronchial wall thickening
Pulmonary infiltrates

Answer 171

A

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)

Answer 172

A

Laryngomalacia = inspiratory stridor (above thoracic inlet)
tracheomalacia (weak tracheal rings collapse with expiration)= expiratory or biphasic stridor (below thoracic inlet

Answer 173

A

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

Answer 174

A

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

Answer 175

A

Amebiasis (entamoeba histolytica)

Answer 176

A

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

Answer 177

A

Exudative:

pH 0.5
pleural:serum LDH > 0.6
high polymorphonuclear leukocytes

Transudative:

pH > 7.3
glucose > 50% of serum
pleural:serum protein

Answer 178

A

Bronchopleural fistula

Answer 179

A

Cirrhosis
Congestive heart failure
Nephrotic syndrome
Malignancies
Infections (viral, Klebsiella, GAS, etc.)

Answer 180

A

-Croup: 3 mo-4 yo
-bacterial tracheitis: 3 mo - 4 yo
-epiglottitis: 2 yo - 10 yo
***overall, these can occur in any age really

Answer 181

A

Subglottic narrowing at the cricoid cartilage!!

Answer 182

A

Strep pneumo
GAS
Staph aureus

Answer 183

A

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)

Answer 184

A

***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

Answer 185

A

Fever
Tachypnea

***unfortunately neither are very specific

Answer 186

A

Overall goal: improve mucous clearance

Chest physio
Mucolytics
Bronchodilators

Answer 187

A

Azithromycin = shown to reduce lung inflammation if given 3x weekly, thus decreasing pulmonary exacerbations

Answer 188

A

Maternal history of asthma
Asthma onset prior to 3 years of age
Eosinophilia
Elevated IgE levels
Allergic rhinitis

Answer 189

A

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

Answer 190

A

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

Answer 191

A

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

Answer 192

A

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

Answer 193

A

OPIOIDS!!!! They are very sensitive to these medications and have worsening of apnea

Answer 194

A

Tonsilly/adenoid hypertrophy (most common)
Obesity
Craniofacial anomalies
Neuromuscular disorders (ie. muscular dystrophy)
Neurologic disorders involving incoordination of upper airway musculature

Answer 195

A

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

Answer 196

A

Chest ultrasound to rule out phrenic nerve paralysis

Answer 197

A

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

Answer 198

A

>48 hrs after birth with no passage

Answer 199

A

Untreated adrenal insufficiency (Addisons)
Untreated hypothyroidism
Ectodermal dysplasia
Atopic dermatitis
Age

Answer 200

A

Leukotriene receptor antagonists! (Montelukast)

Answer 201

A

Poor nutritional status
Pneumothorax
Burkholderia cepacia (very deadly bug in CF)
Poor FEV1

Answer 202

A

YEAR ROUND DAILY WET COUGH AND NASAL CONGESTION!!!!

Answer 203

A

Clinical feature: coughing/choking occurs DURING swallowing
-best test: rigid bronchoscopy is REQUIRED to rule it out!

Answer 204

A

UGI series to start, rigid bronchoscopy is gold standard

Answer 205

A

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

Answer 206

A

Yellow = pediatric = 1-5 yo
Blue = adult = \>5 yo

Answer 207

A

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!

Answer 208

A

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

Answer 209

A

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

Answer 210

A

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

Answer 211

A

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

Answer 212

A

Left axis deviation is always abnormal

AVSD
Tricuspid atresia
Pulmonary atresia
Pulmonary stenosis
Single LV

Answer 213

A

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

Answer 214

A

(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

Answer 215

A

Overriding aorta (empties from both the LV and RV)
RVH
Right ventricular outflow tract obstruction (ie. pulmonary stenosis)
VSD

Answer 216

A

The degree of right ventricular outflow tract obstruction

Answer 217

A

DiGeorge syndrome (22q11 deletion)
Down syndrome
Alagille syndrome

Answer 218

A

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)

Answer 219

A

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

Answer 220

A

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

Answer 221

A

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

Answer 222

A

Medical therapy with antiarrhythmics
Catheter ablation therapy

Answer 223

A

If tachyarrhythmia has caused a life-threatening event

Answer 224

A

Cardiac pacing

Answer 225

A

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

Answer 226

A

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)

Answer 227

A

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

Answer 228

A

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)

Answer 229

A

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

Answer 230

A

Re-entry

AVRT
AVNRT

-Adenosine blocks AV node conduction

Answer 231

A

Vagal maneuvers activate parasympathetic nervous system conducted to the heart by the vagus nerve
-increase AV nodal block to eliminate conduction to the ventricles

Answer 232

A

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

Answer 233

A

Post-op CHD
Poor cardiac function (ie. cardiomyopathy)

Answer 234

A

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

Answer 235

A

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

Answer 236

A

VSD
HOCM
PDA

Answer 237

A

Newborn period: transposition of great arteries
Infancy period: tricuspid atresia
Childhood period: TOF

Answer 238

A

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

Answer 239

A

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”

Answer 240

A

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

Answer 241

A

CATCH-22
Deletion of chromosome 22q11

Congenital heart defect (usually TOF)
Abnormal facies
Thymic aplasia
Cleft palate
Hypocalcemia

Answer 242

A

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

Answer 243

A

Clinical features of cardiac tamponade:

Hypotension
Muffled heart sounds
Distended neck veins

Answer 244

A

Mutations in cardiac potassium and sodium channels = ion channelopathies

Answer 245

A

Syncopal episode brought on by exercise, fright, or sudden startle
-some occur during sleep (LQT type 3)

Answer 246

A

QTc = QT interval (ventricular depolarization and repolarization) that is adjusted for heart rate differences
Prolonged = > 0. 440

Answer 247

A

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

Answer 248

A

Beta blockers
- if beta blockers cause significant bradycardia, may need pacemaker
For patients who have had cardiac arrest, need implantable cardiac defibrillator (ICD)

Answer 249

A

Bradycardia
Notched T waves
T wave alternans (alternating upright and downgoing T waves)

-At risk for torsades de pointes and ventricular fibrillation

Answer 250

A

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

Answer 251

A

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

Answer 252

A

Turners
Trisomy 13
Trisomy 18

Answer 253

A

Right ventricular hypertrophy (due to volume overload)
Prominent P waves (right atrial dilatation from volume overload)
Reduced left sided signals

Answer 254

A

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

Answer 255

A

Prostaglandin E1 to maintain PDA
Norwood procedure

Answer 256

A

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

Answer 257

A

Blue blood from SVC/IVC enters pulmonary artery directly –> passive flow to lungs –> LA –> ASD –> RA –> RV –> pulmonary artery –> neoaorta to systemic circulation

Answer 258

A

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

Answer 259

A

More common in males (3:1)

Answer 260

A

Most common form: valvular aortic stenosis

thickened aortic leaflets and commissures are fused
obstruction to LV blood flow = increased LV systolic pressure = LVH

Answer 261

A

Subvalvular aortic stenosis
Mitral stenosis
Coarctation of aorta

Answer 262

A

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

Answer 263

A

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)

Answer 264

A

LVH
left heart strain (inverted T waves in left precordial leads)

Answer 265

A

Balloon valvuloplasty or surgery
-untreated severe AS: sudden cardiac death especially during exercise

Answer 266

A

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)

Answer 267

A

Hypoplastic or absent radii
1st degree heart block
ASD

Answer 268

A

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

Answer 269

A

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

Answer 270

A

Secundum ASD associated with RV dilation (from volume overload) and increased pulmonary blood flow
-repair occurs electively at 3-5 years of age

Answer 271

A

Most children are asymptomatic
-when they do have symptoms, you hear a fixed split S2

Answer 272

A

From RV volume overload and prolonged ejection of blood during systole leading to delayed pulmonary valve closure

Answer 273

A

Pulmonary HTN

Answer 274

A

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)

Answer 275

A

Classic vibratory murmur

LLSB and apex
low-frequency vibratory “twanging string”
3-6 yo
turbulent flow out of LV
decreases in intensity with sitting

Answer 276

A

ULSB
early to midsystolic murmur
crescendo-decrescendo
8-14 yo
from turbulence of blood flow into the pulmonary vein

Answer 277

A

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

Answer 278

A

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

Answer 279

A

Right supraclavicular areas and over carotids
-any age

Answer 280

A

Venous hum: increases with sitting up
Still’s: decreases with sitting up

Answer 281

A

Still’s murmur = medium pitched, vibratory or musical systolic ejection murmur heard at the LLSB

Answer 282

A

Pulsus alternans: beat to beat variability of pulse strength due to decreased left ventricular performance
-seen in severe CHF

Answer 283

A

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

Answer 284

A

Pericardial tamponade
Constrictive pericarditis
Severe respiratory disease (status asthmaticus, pneumonia)
Myocardium diseases that affects wall compliance (amyloidosis)

Answer 285

A

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

Answer 286

A

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)

Answer 287

A

Digoxin decreases AV nodal conduction BUT can enhance cnoduction through a bypass tract THUS increasing chance of ventricular fibrillation

Answer 288

A

Marfan syndrome
Ehlers-Danlos syndrome
Osteogenesis imperfecta

Answer 289

A

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!

Answer 290

A

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

Answer 291

A

American trypanosomiasis infection
-Romana sign (unilateral, painless, violaceous, palpebral edema accompanied by conjunctivitis) + CHF

Answer 292

A

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

Answer 293

A

Pulmonary hypertension since pulmonary valve is closing against pressure

Answer 294

A

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

Answer 295

A

TOF
Pulmonary atresia
Tricuspid atresia
TAPVR

***These cause cyanosis because of obstruction to pulmonary blood flow

Answer 296

A

Alagille syndrome (hypercholesterolemia is secondary to liver disease)

Answer 297

A

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

Answer 298

A

Ventricular fibrillation due to a blunt, non-penetrating direct blow to the chest during the vulnerable phase of repolarization

Answer 299

A

60% of HOCM is genetic
-AD

Answer 300

A

Myocarditis
Maternal history of lupus
Lyme disease
Acute rheumatic fever

Answer 301

A

Ebstein’s anomaly

Answer 302

A

Vertebral anomalies
Anal atresia
Cardiac defects
TEF
Renal agenesis/dysplasia
Limb defects

Answer 303

A

Coarctation of the aorta = due to collateral vessels forming around the ribs impairing bone growth

Answer 304

A

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

Answer 305

A

Prominent U wave (small deflection immediately following the T wave
Diphasic T wave
Depressed ST segment

Answer 306

A

Prolonged PR interval
Wide QRS
Tall, peaked T waves
Sinusoidal wave

Answer 307

A

Polymorphic ventricular tachycardia with a prolonged QT interval

Answer 308

A

Ice in bag over face
Gagging with tongue depressor

***Do NOT use ocular pressure as it has been associated with retinal damage

Answer 309

A

Lack of LDL receptors on cell membrane so no negative feedback loop to stop cholesterol production

Answer 310

A

2-10 year old:

Family history (parent or grandparent) with age 95%)
Diabetes
Hypertension > 95th percentile
Smokers
nephrotic syndrome

Answer 311

A

Lifestyle + diet modification x 6 months, then recheck
- not a lot of research on pharmaceutical therapy on treating hypercholesterolemia in children
Statins

Answer 312

A

Liver enzymes
CK

Answer 313

A

Post op cardiac
Ebstein’s anomaly
Cardiomyopathy
Myocarditis

Answer 314

A

Atrial fibrillation which then travels down the bundle of kent accessory pathway with a rapid ventricular response leading to ventricular fibrillation

Answer 315

A

WPW syndrome

Answer 316

A

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

Answer 317

A

Friction rub, distended neck veins, hepatomegaly, pulsus paradoxus, muffled heart sounds

Answer 318

A

Coronary artery aneurysms –> heal with stenosis –> MI

Answer 319

A

6 mo - 5 yr
-however, can also happen in infants and teenagers (higher risk of developing coronary artery aneurysms, possible because of delayed diagnosis)

Answer 320

A

Kawasaki = early, specific marker

Answer 321

A

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.

Answer 322

A

Untreated: 25%
Treated: Lower

Answer 323

A

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

Answer 324

A

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

Answer 325

A

Fontan procedure involves manipulation of the RA

Loss of sinus rhythm –> nonsinus atrial rhythm or junctional rhythm
Intra-atrial reentrant tachycardia

Answer 326

A

Ostium secundum

Answer 327

A

Right axis deviation
RVH
RSR prime

Answer 328

A

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

Answer 329

A

Renal impairment
Platelet dysfunction
Pulmonary hemorrhage
GI hemorrhage
Hyponatremia
Hypoglycemia
Hyperkalemia (due to decreased renal function)

Answer 330

A

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

Answer 331

A

Pulmonary stenosis

Answer 332

A

Peripheral pulmonary stenosis (of the pulmonary arteries leading to restricted bloodflow to lungs)

Answer 333

A

Fever
Cardiac murmur
Intracerebral tumor
Thyrotoxicosis
Cerebral aneurysm
Increased ICP
Anemia
AV malformation (ie. vein of galen)
Meningitis

Answer 334

A

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

Answer 335

A

Blood is shunted from arterial vasculature into venous system so you get high output heart failure
-associated with heart failure & hydrocephalus

Answer 336

A

Mitral valve prolapse
-but do NOT need prophylaxis because incidence of infective endocarditis is so low and mitral valve prolapse incidence is low

Answer 337

A

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)

Answer 338

A

Bradycardia
Low voltages
Prolonged QT
Wide QRS

Answer 339

A

Juxtaductal (right beside PDA, below the origin of the left subclavian artery)

Answer 340

A

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

Answer 341

A

Strep viridans
Staph aureus
Staph epi
H flu

start Amp + Gent
treatment duration 4-6 wks

Answer 342

A

Maternal lupus
Sjogren’s syndrome
Myocardial tumors

Answer 343

A

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

Answer 344

A

High voltage QRS due to ventricular hypertrophy
Shortened PR

CXR: ECG finding

Answer 345

A

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

Answer 346

A

Coarctation of aorta

Answer 347

A

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

Answer 348

A

Bicuspid aortic valve (present in 70% of coarc cases)

Answer 349

A

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

Answer 350

A

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

Answer 351

A

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

Answer 352

A

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)

Answer 353

A

Put your thumb under the xiphoid process - should not feel any pulse if RV is normal. If RV enlarged, will feel a pulse

Answer 354

A

>10% is abnormal

Answer 355

A

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

Answer 356

A

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

Answer 357

A

Obstructed (obstructed flow into the heart): small heart, very wet lungs (looks reticular, like RDS)
Unobstructed (L to R shunt): snowman sign, enlarged heart

Answer 358

A

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

Answer 359

A

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

Answer 360

A

Perimembranous (70%)

Answer 361

A

When there is no weight gain

Answer 362

A

V4R, V3R = look at right sided pathology

Answer 363

A

300, 150, 100, 75, 60, 50 for each big box (5 little boxes)

Answer 364

A

P for every QRS and QRS for ever P
Normal P axis

Answer 365

A

After 6 months

Answer 366

A

Tall = right atrial enlargement
Wide or bifid = left atrial enlargement

Answer 367

A

Nothing - usually asymptomatic, seen in athletes, anorexia nervosa

Answer 368

A

Myocarditis, lyme disease, congenital, cardiac surgery

Answer 369

A

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

Answer 370

A

Complete = > 3 small squares = common in post op cardiac paients

Answer 371

A

QTC 500 msec, the risk of arrhythmias increases dramatically

Answer 372

A

Depression of PR interval
Diffuse ST elevation

Answer 373

A

Nothing. This is normal in children

Answer 374

A

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

Answer 375

A

Anomalous left coronary artery from the pulmonary artery = see biventricular hypertropy, Q wave in V6, ST elevation

Answer 376

A

No exercise restrictions, no limitations, they are essentially a normal child

VSD
ASD
AVSD
TAPVD
Coarctation

***Only care if there are residual lesions

Answer 377

A

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

Answer 378

A

May have stiff RV = very dependent on preload for cardiac output to the lungs for oxygenation

Answer 379

A

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

Answer 380

A

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!

Answer 381

A

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

Answer 382

A

Stage 1: norwood @ 7 d old
Stage 2: Glenn @ 4-6 mo
Stage 3: Fontan @ 3 yo

Answer 383

A

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!

Answer 384

A

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

Answer 385

A

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?

Answer 386

A

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

Answer 387

A

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

Answer 388

A

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!

Answer 389

A

Increased vagal tone
-HR increases with inspiration, decreases with expiration

Answer 390

A

High vagal tone
Acute rheumatic fever
Hyper/hypokalemia, hypermagnesemia
Drug effect/toxicity (digoxin)
Lyme disease
CHD: ASD, AVSD, ebstein’s anomaly

Answer 391

A

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

Answer 392

A

Postop CHD
Lyme disease

Answer 393

A

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)

Answer 394

A

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

Answer 395

A

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

Answer 396

A

Acute treatment:

Adenosine
Verapamil
Procainamide
Esmolol
Amiodarone

Chronic treatment:

Propranolol
Sotalol
Flecainide
Propafenone

Answer 397

A

Atrial rates 250-480 bpm

Neonate with structurally normal heart
Older patient s/p CHD surgery

Answer 398

A

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

Answer 399

A

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

Answer 400

A

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

Answer 401

A

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

Answer 402

A

Marfan syndrome, Ehlers-Danlos syndrome, pseudoxanthoma elasticum, osteogenesis imperfecta, and Hurler syndrome may be associated with MVP.

Answer 403

A

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

Answer 404

A

•

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.

Answer 405

A

Pulmonary atresia or severe stenosis

•

Tetralogy of Fallot

•

Tricuspid atresia

•

Ebstein anomaly

Answer 406

A

•

Transposition of great arteries

•

Total anomalous pulmonary venous return

•

Truncus arteriosus

Answer 407

A

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

Answer 408

A

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.

Answer 409

A

1.

Ventricular septal defect

2.

Atrioventricular canal

3.

Patent ductus arteriosus

4.

Coarctation of the aorta

Answer 410

A

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%.

Answer 411

A

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

Answer 412

A

a) vein of Galen aneurysm

Answer 413

A

d) varicella

Answer 414

A

apnea/respiratory depression
cutaneous vasodilation/flushing (10%)
fevers
hypotension

Answer 415

A

renal failure
decreased u/o
bleeding (platelet adhesion defect)
IVH
GI bleeding/NEC -CNS bleeding -hyponatremia

Answer 416

A

splenomegaly, petichiae
osler nodes, janeway lesions, roth spots, splinter hemorrhages

-tachycardia -evidence of CHF -athritis

Answer 417

A

smoking
DM2
hyperlipidemia

-early MI in family

Answer 418

A

-congenital: maternal lupus or Sjogren syndrome other AI disease

genetic: long QT
Congenital malformation of conduction system – Kearn-Sayre syndrome

Answer 419

A

Narrow complex
Tachycardia >200
No P waves visible, abnormal P waves

Answer 420

A

c) FEF25-75

Answer 421

A

Alpha-1-antitrypsin deficiency

Answer 422

A

eczema
ectodermal dysplasia
malnutrition/FTT
anorexia nervosa
CAH
adrenal insufficiency
glucose-6-phosphatase deficiency -nephrogenic DI
hypothryoidism

Answer 423

A

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

Answer 424

A

nasal potential difference
genetic test for common mutations
newborn screen (serum immunoreactive trypsinogen)

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