Resp/Cardio Flashcards

Question

What are the complications of foreign body?

Answer 1

Recurrent pneumonia Bronchiectasis Cardiac arrest and death

Answer 2

Meconium ileus HypoNa, hypoCl metabolic alkalosis Recurrent rectal prolapse Clubbing Bronciectasis Nasal polyps Pseudomonas colonization

Answer 3

\>60 meQ/L

Answer 4

Male?female Lung disease:FEV1, Burkohderia, PTX Nutritional status: Wt/Ht, DM

Answer 5

Year round daily wet cough Year round nasal congestion Recurrent otitis media Neonatal respiratory distress Laterality defects

Answer 6

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

Answer 7

Clinical symptoms + Objective evidence: spirometry, peakflow, methacholine challenge

Answer 8

Neurocognitive: behavioural, attention, schoolperformance, developement Cardiovascular: HTN, cor pulmonale FTT Inflammatory Qualityof life

Answer 9

Polysomnography

Answer 10

Non-pharmacologic: Nasal and sleep hygiene Pharmacologic: intranasal corticosteriods, montelukast Surgical: T&A Weight loss CPAP

Answer 11

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

Answer 12

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

Answer 13

75% in the first 2 years of life

Answer 14

Endocarditis Aortic regurg Subaortic stenosis RVOTO LV TO RA shunting: Gerbode defect

Answer 15

\< 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

Answer 16

Primum (15%) Secundum (75%) Sinus venosus (10%)

Answer 17

Grade 3 SEM at ULSB with widely split S2 Age 3-4 years

Answer 18

PS Overriding aorta RVH VSD

Answer 19

T21, DiGeorge, Alagille

Answer 20

Ca, Mg, K, Drugs (TCAs)

Answer 21

Stable: Vagal, Adenosine Unstable: Syn cardio 0.5-1 J/kg

Answer 22

VIbratory LLSB SEM, changes with position DDx: SubAo stenosis, Small VSD

Answer 23

Infraclavicular hum, continuous R\>L DDx: PDA

Answer 24

AS, PS, Ebstein, TAPVR, RBBB

Answer 25

PDA Ao insuff AVM Vasodilation

Answer 26

PiZZ Will present as liver disease in children

Answer 27

Cystic adenomatoid malformation CCAM Hamartomatous or dysplastic lung tissue mixed with normal lung Presents in infancy

Answer 28

Presents at 6 months: resp distress HYperinflation of affect lobe with atelectasis of other

Answer 29

CF PANCREAS ## Footnote 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

Answer 30

S aureus, H flu, pseudo aeroginosa, B cepacia

Answer 31

Chest physio + bronchodilatory BID Balanced high calorie diet Pancreatic supplemnts ADEK replacement Osteoporos monitoring GERD monitoring

Answer 32

1. Situs inversus (25% will have PCD) 2. Chronic sinusitis and otitis 3. Airway d/po leading to bronchiectasis

Answer 33

MSK Cardiac \<1%

Answer 34

Strained msucles Costochondritis Tietze syndrome Precordial catch syndrome

Answer 35

Localized form of costochondritis usually involving junst one costochondral junction, tender swollen mass palpable at the site Costochrondritis is usually multiple sites without swelling

Answer 36

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

Answer 37

Ring: trachea and/or esophagus is encircled by aberrant vascular strctures Sling: compressions by nonencircling vessels

Answer 38

1. Double Ao arch 2. Right Ao arch with left ligamentum arteriosum

Answer 39

1. HOCM 2. Anomalies of cornoary arteries 3. Margan 4. Arrhythmogenic right ventricular dysplasia (ARVD) 5. Brugada 6. Prolonged QTC

Answer 40

Arrhythmia as a result of blunt, nonpenetrating direct blow to the chest VF occurs during vulnerable phase of repolarization CPR +Defib

Answer 41

Differential BP Systolic murmur in the back SystolicHTN in the upper extremities Diminished femorals

Answer 42

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

Answer 43

TOF Pulmonary Atresia Tricuspid atresia Ebstein

Answer 44

Transposition TAPVR Truncus arteriosis

Answer 45

Boot: TOF Egg: TGA Snowman: TAPVR Rib notching: Coarctation

Answer 46

TOF: worsening RVOTO VSD: eisenmenger syndrome

Answer 47

6 months or older

Answer 48

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

Answer 49

1. WPW 2. AV nodal reentry

Answer 50

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

Answer 51

CP, fever, cough, palpitations Friction rub, pallor, pulsus paradoxus Pt sitting up and leaning forward

Answer 52

1. Decreased renal function 2. Hyponatremia 3. Platelet dysfunction 4. GI blood loss

Answer 53

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

Answer 54

Vascular smooth muscle, vasoconstriction

Answer 55

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

Answer 56

Vascular smooth muscle, vasodilation

Answer 57

Renal and mesenteric vascular smooth muscle Vasodilation

Answer 58

Venous hum= increased Vinratory=decreases

Answer 59

Anastomosis between subclavian artery and pulmonary artery

Answer 60

Loss of sinus INtra-atrial reentrant tachycardia

Answer 61

Fontan Abnormal flow to mesenteric vasculature

Answer 62

40% of children experience

Answer 63

Laryngomalacia

Answer 64

Defect in the CF transmembrane conductance regulator (CFTR) protein Regulates chloride and sodium transfer across the apical membrane of the epithetial cells

Answer 65

Deep, slow resps with prolonged exhalation

Answer 66

Crescendo-descendo respira with alternating apnea

Answer 67

AKA ataxic breathing Unpredictable irregularity in respirations

Answer 68

1. Nutrition 2. Diuretics 3. ACEi

Answer 69

Secundum 75%

Answer 70

1. Right heart failure 2. Embolism 3. Arrhythmia 4. PVD

Answer 71

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 72

Hypercalcemia Elfin features Supravalvular AS PA Branch stenosis MR

Answer 73

Normal viscera, lungs and atrial position

Answer 74

Abdo, atria and lungs and reversed

Answer 75

1. Afterload reduction 2. Preload reudction 3. Sympathetic inhibition 4. Cardiac remodelling prevention 5. Inotrophy

Answer 76

Coxsackie Echovirus Adenovirus EBV Influenza HIV

Answer 77

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 78

Chest wall compliance FRC= Outward elastic recoil= inward lung recoil Infants, more compliant chest wall therefore lower FRC

Answer 79

1. CNS depression =chest wall retraction 2. Poor lung compliance= lung recoil, loss of FRC 3. Compliant chest wall= loss of FRC

Answer 80

Property of a substance to oppose deformation or stretching Change in pressure over change in volume Enbales return to original state

Answer 81

repicrocal of elastance Distensibility Change in volume/change in pressure

Answer 82

Amount of pressure required to generate flow of gas across airways R= 8 x length x viscosity/R⁴

Answer 83

1/2 height if hemithorax = large

Answer 84

1. Increased cough/sputum 2. Hemoptysis 3. Anorexia and weight loss

Answer 85

1. Look at flow-volume curve: is it reproducible (aka straight exp line vs squiggly)? 2. Look at effort curve: kids 12 should exhale for at least 10 seconds 3. Look at FEV1: mild (70-79), moderate (60-69), severe (50-59), very severe (12% change is significant, CHEO uses \> 10%) 8. Compare FEV1 to last PFT: has it changed?

Answer 86

1. Nasal polyps 2. Chronic sinusitis 3. Chronic cough 4. Bronchiectasis 5. Chronic colonization with staph, hemophilus, pseudomonas, burkholderia seppacia 6. GERD secondary to obstructive lung disease, hyperinflation and diaphragms pushing on stomach 7. Meconium ileus 8. Failure to thrive 9. Pancreatic insufficiency (with delta F508 mutation) --\> Fat soluble vitamin deficiency, steatorrhea, eventual diabetes 10. Liver cirrhosis - from thickened bile 11. Congenital absence of vas deferens in males 12. Infertility in both males and females - in females, thick cervical secretions or secondary amenorrhea from malnutrition 13. Rectal prolapse - from chronic diarrhea, malnutrition and weak musculature 14. Clubbing 15. Cor pulmonary from pulmonary hypertension

Answer 87

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 88

1. Asthma 2. Aspiration penumonitis: ask about coughing/choking with feeds, reflux symptoms, rule out TEF 3. BPD 4. Bronchiolitis obliterans: chronic wheezing after adenovirus infection 5. Congenital cardiac lesion: left to right shunts causing increased pulmonary blood flow 6. Cystic fibrosis: ask about recurrent diarrhea, failure to thrive 7. Foreign body aspiration 8. Vascular rings/slings 9. Airway malformations: tracheomalacia, lung cysts, mediastinal mass 10. Primary ciliary dyskinesia: ask about recurrent sinusitis, ear infections

Answer 89

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 90

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

Answer 91

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 92

Autosomal recessive

Answer 93

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 94

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

Answer 95

1. Nutrition 2. Chest physiotherapy 3. Antibiotics

Answer 96

1. V/Q mismatch 2. Shunt 3. Hypoventilation 4. Low FiO2 5. Diffusion barrier 6. Abnormal Hgb saturation curve

Answer 97

1. Increased risk of infection secondary to trapping of secretions 2. Increased risk of pneumothorax 3. Increased risk of pulmonary hemorrhage (and thus hemoptysis)

Answer 98

Diffuse wheezing: 1. CF 2. Asthma 3. Bronchiolitis 4. Anaphylaxis with resp involvement 5. GERD Localized wheezing: 1. Foreign body 2. Extrinsic compression by mass 3. Bronchomalacia

Answer 99

1. Granuloma formation 2. Interstitial fibrosis 3. Lipoid pneumonia = through oral or nasal administration of animal or vegetable oils to treat childhood illnesses as a folk remedy

Answer 100

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 101

\*\*\*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 102

1. Parental asthma 2. Atopy 3. Reduced lung function at birth 4. Possible use of acetaminophen 5. Exposure to chlorinated swimming pools 6. Severe lower resp tract infection (pneumonia, bronchiolitis requiring hospitalization) 7. Male gender 8. Wheezing apart from colds 9. Environmental tobacco smoke exposure 10. Low birthweight

Answer 103

1. 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 2. Chronic asthma (persistent atopy-associated asthma): associated with allergy (usually persists into later childhood and often adulthood)

Answer 104

1. Relaxation breathing techniques 2. Inhalation of heliox to relieve vocal cord spasm

Answer 105

1. Airflow limitation - low FEV1 - FEV1/FVC ratio 12% 3. Exercise challenge: worsening in FEV1 \> 15%

Answer 106

1. Cystic fibrosis 2. Aspergillus (proximal bronchiectasis) 3. Ciliary dyskinesia 4. Immunodeficiencies

Answer 107

Daytime ## Footnote Night

Answer 108

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 109

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

Answer 110

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 111

1. Congenital central hypoventilation: PHOX2B genetic testing 2. Airway obstruction: consider bronchoscopy/CT chest 3. Diaphragm dysfunction: diaphragm fluoroscopy 4. Congenital cardiac disease: consider ECHO 5. Structural hindbrain or brainstem abnormality: MRI head 6. Metabolic disorders

Answer 112

1. Metabolic: insulin resistance, dyslipidemia 2. Cardiovascular disease 3. Pulmonary hypertension with cor pulmonale 4. Neurocognitive disturbances

Answer 113

1. Metabolic: insulin resistance, dyslipidemia 2. Cardiovascular disease 3. Pulmonary hypertension with cor pulmonale 4. Neurocognitive disturbances

Answer 114

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 115

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 116

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 117

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 118

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 119

1. Previous hx of ICU admission or intubation due to severe asthma exacerbation 2. Sudden respiratory failure or arrest 3. Two or more hospitalizations for asthma in past year 4. 3 or more ED visits for asthma in past year 5. Use of \> 2 cannisters of SABA per month 6. Poor response to systemic corticosteroid therapy 7. Low birthweight 8. Nonwhite ethnicity

Answer 120

1. Initial event: Violent paroxysms of coughing, choking, gagging, and airway obstruction 2. 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. 3. 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 121

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 122

1. Aspiration pneumonia 2. Cystic fibrosis 3. GERD 4. TEF 5. Immunodeficiencies 6. Neurological conditions \*\*anything that can lead to aspiration of infected materials with oral organisms \*\*can also occur secondary to a pneumonia

Answer 123

Parenchymal inflammation with a cavity containing an air fluid level

Answer 124

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 125

1. Cough occurs only during the day and never during sleep 2. 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 126

Test for cystic fibrosis regardless of race or ethnicity

Answer 127

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

Answer 128

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

Answer 129

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 130

Overall: supportive management 1. Nitric oxide 2. Oxygen 3. Prone position (improves V/Q mismatch) 4. Surfactant (does not change mortality however) 5. Sedation (decrease pulm htn) 6. Limit tidal volumes to 6-8 ml/kg) 7. HFO (gentle ventilation) 8. Neuromuscular blockade 9. Permissive hypercapnea 10. Steroids (start within 72 hrs, can be used up to 30 d) \*\*\*If all else fails, ECMO

Answer 131

Direct lung injury 1. Pneumonia (most common) 2. Aspiration 3. Pulmonary contusion 4. Submersion injury 5. Inhalational injury Indirect lung injury: 1. Sepsis 2. Shock 3. Burns 4. Transfusion related 5. Trauma

Answer 132

1. Exudative phase: decreased pulmonary compliance, increased hypoxia, increased tachypnea - see inflammation, diffuse alveolar infiltrates, pulmonary edema 2. Fibroproliferative phase: increased alveolar dead space, pulmonary hypertension - scarring of lung, epithelial damage, surfactant deactivation 3. Recovery phase: restoration of pulmonary epithelial barrier

Answer 133

1. Acute onset 2. PaO2/FiO2

Answer 134

1. Decreases the coordination required to use MDIs 2. Improves delivery of drug to lower airways 3. Minimizes risk of thrush 4. 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 135

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 136

IV enzyme replacement from pooled human plasma

Answer 137

1. Malignancy (solid tumors \> non solid) 2. Presence of a central venous catheter (induces endothelial damage) 3. Thrombophilia (deficiency of antithrombin 3, protein C or S; factor 5 leiden, hyperhomocysteinemia, antiphospholipid antibodies) 4. Recent surgery/immobilization 5. Nephrotic syndrome (losing protein C & S) 6. SLE (systemic inflammation) 7. OCP \*\*\*Remember virschow's triad: hypercoagulability, endothelial injury, stasis

Answer 138

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 139

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 140

1. Height velocity 2. Cataracts \*\*\*Does not affect adult height

Answer 141

1. Thrush 2. Hoarse voice (dysphonia)

Answer 142

Tall, thin male with subpleural bleb

Answer 143

1. Thymoma 2. T cell leukemia 3. Terrible lymphoma 4. Thyroid tumors 5. Teratoma

Answer 144

A+B 1. Adenopathy: infectious (histoplasmosis most common), neoplastic, metastatic, sarcoidosis 2. Bronchogenic cyst

Answer 145

The Ns 1. Neurogenic tumor: neuroblastoma, benign ganglioneuroma, ganglioneuroblastoma 2. Neurofibroma 3. Esophageal duplication cysts 4. Pulmonary sequestration

Answer 146

Mycoplasma chlamydia and mycoplasma pneumoniae

Answer 147

1. Proximal bronchiectasis 2. Bronchial wall thickening 3. Pulmonary infiltrates

Answer 148

1. Central chemoreceptors in the medulla = sense hypercarbia (major role in respirations) 2. Peripheral chemoreceptors in the carotid arteries/arch of aorta = sense hypoxia (minor role in respirations)

Answer 149

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

Answer 150

Chronic cough \> 3 wks: 1. Asthma 2. Recurrent infections 3. Cystic fibrosis 4. GERD 5. Immunodeficiency: IgA deficiency, hypogammaglobulinemia 6. Primary ciliary dyskinesia (Kartagener syndrome, immotile cilia syndrome) 7. Anatomic lesions: foreign body, mediastinal tumor, congenital heart disease), TEF 8. Allergy/irritants: tobacco smoke, pollution, etc. 9. Psychogenic cough - initial work up: guided by clues in history and physical; if no clues, then start with: 1. CXR 2. PFTs if possible 3. Sweat chloride

Answer 151

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 152

Amebiasis (entamoeba histolytica)

Answer 153

Chylothorax = disruption of thoracic duct leading to accumulation of chyle (high lipid and protein content) 1. Thoracic surgery complication 2. Birth injury: thoracic duct leaks with stretch 3. Obstruction of central veins by thrombosis or malignancy - worsened by ingestion of fat - treatment: 1. low fat diet (portagen formula = medium-chain fatty acids, or TPN without lipids) 2. If severe, can ligate thoracic duct

Answer 154

Exudative: - pH 0.5 - pleural:serum LDH \> 0.6 - high polymorphonuclear leukocytes Transudative: - pH \> 7.3 - glucose \> 50% of serum - pleural:serum protein

Answer 155

Bronchopleural fistula

Answer 156

1. Cirrhosis 2. Congestive heart failure 3. Nephrotic syndrome 4. Malignancies 5. Infections (viral, Klebsiella, GAS, etc.)

Answer 157

-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 158

Subglottic narrowing at the cricoid cartilage!!

Answer 159

1. Strep pneumo 2. GAS 3. Staph aureus

Answer 160

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 161

\*\*\*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 162

1. Fever 2. Tachypnea \*\*\*unfortunately neither are very specific

Answer 163

Overall goal: improve mucous clearance 1. Chest physio 2. Mucolytics 3. Bronchodilators

Answer 164

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

Answer 165

1. Maternal history of asthma 2. Asthma onset prior to 3 years of age 3. Eosinophilia 4. Elevated IgE levels 5. Allergic rhinitis

Answer 166

Primary ciliary dyskinesia! 1. Chronic sinusitis with nasal polyps 2. Situs inversus in 50% of patients 3. Chronic otitis media with hearing loss 4. Bronchitis and bronchiectasis 5. Pneumonia 6. Clubbing 7. Male and female infertility/decreased fertility - abnormal sperm movement - increased ectopic pregnancy Diagnosis: 1. CXR = dextrocardia, bronchiectasis 2. Gold standard = look at cilia from nasal/bronchial brush by electron microscopy Treatment: 1. Pulmonary toilet to maximize mucous clearance 2. Antibiotics to treat infection 3. May need lobectomy if severe bronchiectasis

Answer 167

- 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 168

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 169

Hemosiderosis! -siderophages = macrophages with iron inside Differential diagnosis: 1. Heiner's syndrome: CMPA with pulmonary hemorrhage 2. Idiopathic pulmonary hemosiderosis 3. Pulmonary capillary hemangiomatosis (proliferation of capillaries with alveolar hemorrhage) 4. Goodpasture syndrome 5. Wegner's granulomatosis

Answer 170

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

Answer 171

1. Tonsilly/adenoid hypertrophy (most common) 2. Obesity 3. Craniofacial anomalies 4. Neuromuscular disorders (ie. muscular dystrophy) 5. Neurologic disorders involving incoordination of upper airway musculature

Answer 172

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 173

Chest ultrasound to rule out phrenic nerve paralysis

Answer 174

1. Nutrtional support 2. Inhalation therapy 3. Chest PT 4. Bronchodilators 5. Oxygen 6. Antibiotics 7. alpha-1-antitrypsin (inhibits neutrophil elastase which breaks down lung tissue) 8. amiloride (diuretic inhibiting sodium and water reabsorption from secretions) 9. DNAse = reacts with DNA released by dead leukocytes to reduce sputum viscosity 10. Gene therapy: alters CF gene

Answer 175

\>48 hrs after birth with no passage

Answer 176

1. Untreated adrenal insufficiency (Addisons) 2. Untreated hypothyroidism 3. Ectodermal dysplasia 4. Atopic dermatitis 5. Age

Answer 177

Leukotriene receptor antagonists! (Montelukast)

Answer 178

1. Poor nutritional status 2. Pneumothorax 3. Burkholderia cepacia (very deadly bug in CF) 4. Poor FEV1

Answer 179

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

Answer 180

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

Answer 181

UGI series to start, rigid bronchoscopy is gold standard

Answer 182

1. Confirm diagnosis 2. Environmental controls, education and written action plan: eliminate smoking, second hand smoke, allergens, need to improve adherence 3. Fast-acting bronchodilators prn 4. 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 183

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

Answer 184

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 185

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 186

1. Congenital: - Romano-Ward syndrome (most common genetic cause of prolonged QT syndrome, autosomal dominent) - Jervell-Lange-Nielsen syndrome (associated with deafness) 2. Acquired - Drugs (antiarrhythmics, dexmedetomidate, TCAs, antipsychotics, antibiotics such as macrolides) - Electrolytes (hypocalcemia, hypokalemia, hypomagnesemia) - Intracranial lesion (encephalitis, head trauma, stroke) - Cardiac disease

Answer 187

SUPPORTIVE for mild cases 1. Bed rest 2. Digoxin 3. Cautious diuresis since they're preload dependent (will need some volume) 4. Afterload reduction - ACE inhibitor 5. Anti arrhythmic For severe cases: may need inotropes/ICU support

Answer 188

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 189

Left axis deviation is always abnormal 1. AVSD 2. Tricuspid atresia 3. Pulmonary atresia 4. Pulmonary stenosis 5. Single LV

Answer 190

1. 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 2. Pulmonary atresia 3. Ebstein's anomaly

Answer 191

(aka hypercyanotic spell) 1. Acute onset of intense cyanosis 2. Sudden softening of murmur (due to decreased blood flow through the obstructed right outflow tract) 3. 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 192

1. Overriding aorta (empties from both the LV and RV) 2. RVH 3. Right ventricular outflow tract obstruction (ie. pulmonary stenosis) 4. VSD

Answer 193

The degree of right ventricular outflow tract obstruction

Answer 194

1. DiGeorge syndrome (22q11 deletion) 2. Down syndrome 3. Alagille syndrome

Answer 195

Step-wise approach: if one step fails, move onto the next 1. 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 2. Administer O2 - O2 is pulmonary vasodilator (decreasing pulmonary vascular resistance and thus increases blood flow to lungs) and systemic vasoconstrictor 3. Administer morphine (0.1 mg/kg) and give fluid bolus - morphine theoretically decreases agitation and thus RVOT obstruction - fluids improves venous return 4. Administer IV beta blocker (propanolol 0.1 mg/kg) - causes relaxation of RVOT obstruction 5. Administer IV phenylephrine (selective alpha agonist) - increases afterload (systemic vascular resistance)

Answer 196

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 197

1. Bigeminy - PVC occurs after every normal beat = non concerning 2. Trigeminy - two normal beats to one PVC = non concerning 3. Quadrigeminy - three normal beats to one PVC = non concerning 4. Ventricular tachycardia - 3 or more PVCs occuring in a row = concerning obviously

Answer 198

1. ECG 2. Holter monitor 3. Event recorders - loop recorders: children always wear the device and press a button when they experience symptoms - pacer tracers: connected to telephone for recording 4. ECHO

Answer 199

1. Medical therapy with antiarrhythmics 2. Catheter ablation therapy

Answer 200

If tachyarrhythmia has caused a life-threatening event

Answer 201

Cardiac pacing

Answer 202

1. 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) 2. Wide QRS

Answer 203

1. Re-entry a) AVRT (atrioventricular reentrant tachycardia) b) AVNRT (atrioventricular nodal reentrant tachycardia) 2. 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 204

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 205

- 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 206

- 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 207

Re-entry 1. AVRT 2. AVNRT -Adenosine blocks AV node conduction

Answer 208

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 209

1. Digoxin 2. 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 210

1. Post-op CHD 2. Poor cardiac function (ie. cardiomyopathy)

Answer 211

1. Sudden onset ("drop") without prodromal symptoms or awareness 2. Occurred during exercise 3. Limp or lifeless during syncope leading to injury 4. Palpitations or abnormal heart rate noted before event 5. Family history of sudden death

Answer 212

1. HOCM 2. WPW 3. Prolonged QT syndrome 4. 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) 5. Arrhythmogenic RV dysplasia

Answer 213

1. VSD 2. HOCM 3. PDA

Answer 214

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

Answer 215

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 216

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 217

1. Heart rate 2. Rhythm - Sinus: P before every QRS, upright P in leads II, III, and AVF 3. 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) 4. Look at PR and QRS intervals and compare to normal for age values 5. 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) 6. 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) 7. QTc calculation and compare to normal for age

Answer 218

CATCH-22 Deletion of chromosome 22q11 1. Congenital heart defect (usually TOF) 2. Abnormal facies 3. Thymic aplasia 4. Cleft palate 5. Hypocalcemia

Answer 219

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. 1. Need to call Cardiology and PICU and be prepared for this circumstance in order to do in controlled setting 2. Need to put a 12 lead ECG or rhythm monitor on the patient

Answer 220

Clinical features of cardiac tamponade: 1. Hypotension 2. Muffled heart sounds 3. Distended neck veins

Answer 221

Mutations in cardiac potassium and sodium channels = ion channelopathies

Answer 222

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

Answer 223

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

Answer 224

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 225

1. Beta blockers - if beta blockers cause significant bradycardia, may need pacemaker 2. For patients who have had cardiac arrest, need implantable cardiac defibrillator (ICD)

Answer 226

1. Bradycardia 2. Notched T waves 3. T wave alternans (alternating upright and downgoing T waves) -At risk for torsades de pointes and ventricular fibrillation

Answer 227

1. Hypoplasia of left ventricle (secondary to atresia of the mitral orifice) 2. 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 228

1. Increased pulmonary flow: can have pulmonary edema (crackles, wet lungs on CXR) 2. Decreased systemic circulation: weak or absent pulses, cyanosis, shock once PDA starts closing 3. Right ventricular volume overload: right ventricular parasternal lift \*\*typically present within 1st few hours or days of life

Answer 229

1. Turners 2. Trisomy 13 3. Trisomy 18

Answer 230

1. Right ventricular hypertrophy (due to volume overload) 2. Prominent P waves (right atrial dilatation from volume overload) 3. Reduced left sided signals

Answer 231

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 232

1. Prostaglandin E1 to maintain PDA 2. Norwood procedure

Answer 233

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 234

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

Answer 235

Ductal dependent pulmonary blood flow: 1. Critical pulmonary valve stenosis 2. Pulmonary atresia 3. TOF with severe pulmonary stenosis 4. Tricuspid atresia with pulmonary stenosis or pulmonary atresia Ductal dependent systemic blood flow: 1. Coarctation of the aorta 2. Hypoplastic left heart syndrome 3. Interrupted aortic arch

Answer 236

More common in males (3:1)

Answer 237

Most common form: valvular aortic stenosis - thickened aortic leaflets and commissures are fused - obstruction to LV blood flow = increased LV systolic pressure = LVH

Answer 238

1. Subvalvular aortic stenosis 2. Mitral stenosis 3. Coarctation of aorta

Answer 239

1. Supravalvular aortic stenosis 2. Developmental delay 3. Hypercalcemia 4. Elf face (prominent lips, abnormal teeth) 5. Hypothyroidism 6. Cocktail party personality 7. 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 240

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 241

1. LVH 2. left heart strain (inverted T waves in left precordial leads)

Answer 242

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

Answer 243

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 244

1. Hypoplastic or absent radii 2. 1st degree heart block 3. ASD

Answer 245

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 246

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 247

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

Answer 248

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

Answer 249

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

Answer 250

Pulmonary HTN

Answer 251

1. Resp symptoms due to increased pulmonary blood flow and pulmonary edema 2. 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 252

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 253

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

Answer 254

- 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 255

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 256

Right supraclavicular areas and over carotids -any age

Answer 257

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

Answer 258

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

Answer 259

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

Answer 260

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 261

1. Pericardial tamponade 2. Constrictive pericarditis 3. Severe respiratory disease (status asthmaticus, pneumonia) 4. Myocardium diseases that affects wall compliance (amyloidosis)

Answer 262

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 263

- 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 264

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

Answer 265

1. Marfan syndrome 2. Ehlers-Danlos syndrome 3. Osteogenesis imperfecta

Answer 266

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 267

1. Infectious - bacterial: diphtheria, strep species, mycoplasma - viral: Coxsackie B (most common), HIV, echoviruses, rubella, adenovirus - fungal - protozoal: trypanosomiasis (Chagas disease), toxoplasmosis 2. Inflammatory - Kawasaki - SLE - RA - IBD 3. Chemical and physical agents - doxorubicin - radiation injury - lead - alcohol

Answer 268

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

Answer 269

- 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 270

Pulmonary hypertension since pulmonary valve is closing against pressure

Answer 271

- Single ventricle pathology 1. HLHS 2. Truncus arteriosus 3. Transposition of the great vessels 4. TAPVR without obstruction \*\*\*These cause cyanosis because of systemic and venous blood mixing

Answer 272

1. TOF 2. Pulmonary atresia 3. Tricuspid atresia 4. TAPVR \*\*\*These cause cyanosis because of obstruction to pulmonary blood flow

Answer 273

Alagille syndrome (hypercholesterolemia is secondary to liver disease)

Answer 274

1. CXR: to identify right sided aortic arch 2. Barium swallow: was the gold standard; confirms external indentation of esophagus in 95% of cases 3. MRI: noninvasive and is the primary diagnostic modality 4. Arteriogram: rarely used 5. Echocardiogram: cannot identify the ring itself but can rule out congenital heart lesions which can occur simultaneously

Answer 275

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

Answer 276

60% of HOCM is genetic -AD

Answer 277

1. Myocarditis 2. Maternal history of lupus 3. Lyme disease 4. Acute rheumatic fever

Answer 278

Ebstein's anomaly

Answer 279

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

Answer 280

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

Answer 281

1. Tetralogy of fallot: indicates worsening RV outflow tract obstruction 2. VSD: indicates that Eisenmenger syndrome has developed with increased pulmonary vascular resistance and thus reversal of shunt from R to L

Answer 282

1. Prominent U wave (small deflection immediately following the T wave 2. Diphasic T wave 3. Depressed ST segment

Answer 283

1. Prolonged PR interval 2. Wide QRS 3. Tall, peaked T waves 4. Sinusoidal wave

Answer 284

Polymorphic ventricular tachycardia with a prolonged QT interval

Answer 285

1. Ice in bag over face 2. Gagging with tongue depressor \*\*\*Do NOT use ocular pressure as it has been associated with retinal damage

Answer 286

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

Answer 287

2-10 year old: - Family history (parent or grandparent) with age 95%) - Diabetes - Hypertension \> 95th percentile - Smokers - nephrotic syndrome

Answer 288

1. Lifestyle + diet modification x 6 months, then recheck - not a lot of research on pharmaceutical therapy on treating hypercholesterolemia in children 2. Statins

Answer 289

1. Liver enzymes 2. CK

Answer 290

1. Post op cardiac 2. Ebstein's anomaly 3. Cardiomyopathy 4. Myocarditis

Answer 291

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

Answer 292

WPW syndrome

Answer 293

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 294

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

Answer 295

Coronary artery aneurysms --\> heal with stenosis --\> MI

Answer 296

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 297

Kawasaki = early, specific marker

Answer 298

1. 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 2. 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 299

Untreated: 25% Treated: Lower

Answer 300

1. Inadequate pulmonary blood flow: - Pulmonary atresia with intact ventricular septum - Tricuspid atresia with intact ventricular septum - critical pulmonary stenosis 2. Inadequate systemic blood flow: - HLHS - interrupted aortic arch - Critical coarc of aorta 3. Inadequate mixing - TGA

Answer 301

- 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 302

Fontan procedure involves manipulation of the RA 1. Loss of sinus rhythm --\> nonsinus atrial rhythm or junctional rhythm 2. Intra-atrial reentrant tachycardia

Answer 303

Ostium secundum

Answer 304

1. Right axis deviation 2. RVH 3. RSR prime

Answer 305

Indications: 1. Deteriorating respiratory status 2. Poor cardiac output 3. Evidence of CHF - dose: indomethacin 0.1 mg/kg/day x 6 days Contraindications: 1. Thrombocytopenia 2. Renal impairment 3. Active bleeding (GI or intracranial) 4. Proven or suspected untreated infection 5. NEC or suspected NEC 6. Evidence of bleeding diathesis 7. CHD in which PDA is needed for pulmonary or systemic blood flow

Answer 306

1. Renal impairment 2. Platelet dysfunction 3. Pulmonary hemorrhage 4. GI hemorrhage 5. Hyponatremia 6. Hypoglycemia 7. Hyperkalemia (due to decreased renal function)

Answer 307

1. Rib notching 2. Intrapulmonary vascular markings increased 3. 3-sign on left para mediastinal shadow - prominent aortic bulge, indentation of descending aorta, post stenotic dilation of aorta

Answer 308

Pulmonary stenosis

Answer 309

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

Answer 310

1. Fever 2. Cardiac murmur 3. Intracerebral tumor 4. Thyrotoxicosis 5. Cerebral aneurysm 6. Increased ICP 7. Anemia 8. AV malformation (ie. vein of galen) 9. Meningitis

Answer 311

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 312

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

Answer 313

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

Answer 314

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 315

1. Bradycardia 2. Low voltages 3. Prolonged QT 4. Wide QRS

Answer 316

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

Answer 317

Definite diagnosis: 1. Intracardiac or embolized vegetations on histology Possible diagnosis: Need 2 major, 1 major + 3 minor or 5 minor Major: 1. 2 positive blood cultures 2. 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 318

1. Strep viridans 2. Staph aureus 3. Staph epi 4. H flu - start Amp + Gent - treatment duration 4-6 wks

Answer 319

1. Maternal lupus 2. Sjogren's syndrome 3. Myocardial tumors

Answer 320

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 321

1. High voltage QRS due to ventricular hypertrophy 2. Shortened PR CXR: ECG finding

Answer 322

1. Atlantoaxial instability: consider in intubation, order flex-ex views before intubation 2. Large tongue: difficult airway 3. Risk of post op resp infections 4. Congenital cardiac lesions: if repaired, increased risk of arrhythmias post-op 5. Pulmonary hypertension 6. OSA

Answer 323

Coarctation of aorta

Answer 324

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 325

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

Answer 326

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 327

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 328

1. Relief of RV outflow tract obstruction - resection of obstructive muscle bundles 2. Patch closure of VSD 3. 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 329

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 330

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

Answer 331

\>10% is abnormal

Answer 332

1. 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 333

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 334

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 335

- 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 336

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 337

Perimembranous (70%)

Answer 338

When there is no weight gain

Answer 339

V4R, V3R = look at right sided pathology

Answer 340

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

Answer 341

1. P for every QRS and QRS for ever P 2. Normal P axis

Answer 342

After 6 months

Answer 343

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

Answer 344

Nothing - usually asymptomatic, seen in athletes, anorexia nervosa

Answer 345

Myocarditis, lyme disease, congenital, cardiac surgery

Answer 346

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 347

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

Answer 348

QTC 500 msec, the risk of arrhythmias increases dramatically

Answer 349

1. Depression of PR interval 2. Diffuse ST elevation

Answer 350

Nothing. This is normal in children

Answer 351

1. ST depression or T wave inversion in V5-7 2. S wave in V1 \> 95h percentile for age 3. R wave in V6 \> 95th percentile for age 4. Deep Q waves in V5-7

Answer 352

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

Answer 353

No exercise restrictions, no limitations, they are essentially a normal child 1. VSD 2. ASD 3. AVSD 4. TAPVD 5. Coarctation \*\*\*Only care if there are residual lesions

Answer 354

Degree of worry is based on residual defect: 1. TOF 2. TGA 3. 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 355

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

Answer 356

1. Severe PI --\> dilated RV --\> decreased RV function 2. Branch PA stenoses 3. 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 357

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: 1. Branch PA stenosis 2. Proximal coronary button stenosis 3. Dilatation of ascending aorta Chest pain? = WORRY!

Answer 358

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 359

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

Answer 360

Overall: want the heart to be one big bag that pumps to the body and not at all to the lungs 1. Atrial septectomy (take out the atrial septum completely) 2. Cut the MPA off the heart 3. Take the outflow tracts (aorta and MPA) and merge them with a patch (pericardium or gortex) = sew together = DKS = side to side anastamosis 4. 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 361

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 362

1. What are your baseline sats? 2. 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 3. Is your warfarin therapeutic?

Answer 363

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 364

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 365

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 366

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

Answer 367

1. High vagal tone 2. Acute rheumatic fever 3. Hyper/hypokalemia, hypermagnesemia 4. Drug effect/toxicity (digoxin) 5. Lyme disease 6. CHD: ASD, AVSD, ebstein's anomaly

Answer 368

``` 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 369

1. Postop CHD 2. Lyme disease

Answer 370

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 371

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 372

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 373

Acute treatment: 1. Adenosine 2. Verapamil 3. Procainamide 4. Esmolol 5. Amiodarone Chronic treatment: 1. Propranolol 2. Sotalol 3. Flecainide 4. Propafenone

Answer 374

Atrial rates 250-480 bpm 1. Neonate with structurally normal heart 2. Older patient s/p CHD surgery

Answer 375

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 376

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 377

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 378

1. No competitive sports and strenuous physical activity = risk of sudden death 2. Beta blockers or calcium channel blockers = diminish ventricular outflow tract obstruction, modifies ventricular hypertrophy, improves ventricular filling 3. Anti arrhythmics or implantable cardioverter defibrillator = if patients have history of arrhythmias, strong family histories of arrhythmias or sudden death, patients with syncope 4. Screen first degree relatives with ECG and ECHO, genetic testing

Answer 379

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

Answer 380

* 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 381

• 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 382

Pulmonary atresia or severe stenosis • Tetralogy of Fallot • Tricuspid atresia • Ebstein anomaly

Answer 383

• Transposition of great arteries • Total anomalous pulmonary venous return • Truncus arteriosus

Answer 384

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 385

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 386

1. Ventricular septal defect 2. Atrioventricular canal 3. Patent ductus arteriosus 4. Coarctation of the aorta

Answer 387

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 388

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 389

a) vein of Galen aneurysm

Answer 390

d) varicella

Answer 391

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

Answer 392

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

Answer 393

- splenomegaly, petichiae - osler nodes, janeway lesions, roth spots, splinter hemorrhages -tachycardia -evidence of CHF -athritis

Answer 394

- smoking - DM2 - hyperlipidemia -early MI in family

Answer 395

-congenital: maternal lupus or Sjogren syndrome other AI disease - genetic: long QT - Congenital malformation of conduction system – Kearn-Sayre syndrome

Answer 396

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

Answer 397

c) FEF25-75

Answer 398

2. Alpha-1-antitrypsin deficiency

Answer 399

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

Answer 400

- 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 401

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