Cardiology Board Review

1. Cardiology Board Review August 30th, 2011

2. Test Question • The Saints will win the Superbowl this year. • True • False

3. Cardiac history and Exam

4. Cardiac History • Infants • Gestation • FHx • Neonatal status • Growth and development • Feeding patterns • Children • Presence of palpitations • Chest pain • Lightheadedness or syncope • Activity level

5. Cardiac Exam • Observation • Respiratory distress? • Central cyanosis? • Clubbing? • Syndromic appearance? • Palpation • Hyperdynamicprecordium? • Displaced PMI? • HSM? • Distal pulses equal? Bounding? • Ausculatation

6. Heart Sounds S1: AV valve closure S2: Aortic and pulmonary valve closure (split) A2 P2 (“hangout” time) S3: rapid filling of the ventricles Normal in kids! S4: pathologic, heard with stiff ventricles

7. Murmurs Intensity Placement in cardiac cycle Systolic murmurs Pansystolic: begin with the first heart sound Ejection: begin after the first heart sound, have crescendo- decrescendo quality Diastolic murmurs Immediate Early or medium Late • Grade 1: Barely audible • Grade 2: Audible and constant • Grade 3: Loud without thrill • Grade 4: Loud with thrill • Grade 5: Heard with stethoscope just touching the chest • Grade 6: Heard with stethoscope off the chest

8. Question #1 • Which of the following accurately describes the Still murmur? • A: Mid-systolic murmur of low intensity heard at the base of the heart, in the axillae, and over the back • B: Systolic or diastolic murmur heard in the infraclavicular region (usually right sided); disappears when the patient is lying down • C: Systolic murmur best heard above the clavicles, due to turbulence in the carotid artery • D: Musical or vibratory quality at the mid-to-lower sternal border and toward the apex • E: Loud continuous “machine-like” murmur

9. Innocent Murmurs • Uniform characteristics • Early systolic ejection • Short duration • Low intensity (grade 1&2) • Vibrating or musical quality • Examples • Still murmur • Venous hum • Supraclavicular bruit • PPS (peripheral pulmonary artery stenosis)

10. Pathologic Murmurs • Longer and louder • All diastolic (except venous hum) and pansystolic murmurs are pathologic • Systolic ejection murmur • Ejection click? • Is S2 widely split? • Does the S2 split move? Normally? • Hyperdynamicprecordium? • Distended neck veins? • Peripheral pulses normal?

11. Congenital Heart Disease in neonate and young infant

12. Clinical Presentations • Recognize dysmorphic features as increased risk for CHD • Trisomy 21→ endocardial cushion defect • Trisomy 18→ VSD • Fetal alcohol syndrome→ ASD, VSD • 22q11 microdeltion→ interrupted aortic arch, Tetralogy of Fallot, truncus arteriosus, atrial or ventricular septal defects, vascular rings • 45 XO→ bicuspid aortic valve (16%), coarctation of aorta (11%)

13. Clinical Presentations • Bicuspid aortic valve • Evaluated every 2 to 3 years • 70% develop some degree of stenosis or insufficiency by age 30 • Will require catheter-based or surgical intervention at some point

14. Clinical Presentations • Most have 1 of 4 presentations: • 1) Asymptomatic murmur • 2) Cyanosis (often without murmur) • 3) Gradually progressing symptoms of heart failure • 4) Catastrophic heart failure and shock

15. Asymptomatic with a Murmur • Caused by regurgitant valves (mitral or tricuspid) or by lesions producing turbulence in a great artery (PS, AS, nonphysiologic peripheral pulmonary stenosis, supravalvular AS) • Systolic murmur that obscures S1 is probably VSD or AV valve regurg

16. Asymptomatic with a Murmur • ASD = fixed split S2 (increased flow across the pulmonary valve) • Pulmonary stenosis = systolic click, heard best at left sternal border, radiates to back and axilla • Aortic stenosis = ejection click (does not change with position), heard best at RUSB with radiation to neck • PDA = continuous “machinery” murmur, loudest at left infraclavicular area • VSD = holosystolic (if small, high-pitched and heard along sternal border)

17. Asymptomatic with murmur • PDA • For preterm, can cause volume overload of right heart • Hyperdynamic precordium and wide pulse pressure • Dx by echo • Treat with indomethacin x 3 • If fails to close, surgical ligation

18. Asymptomatic with murmur • VSD • For moderate-to-large, systemic pressure in right ventricle leads to systemic pulmonary pressure • CHF evolves over first 1 to 2 postnatal months • Most surgical repair is at 4 to 6 months • If the normal decrease in pulmonary vascular resistance does not occur→ no left-to-right shunt→ could develop Eisenmenger’s and irreversible pulmonary vascular changes→ now right-to-left shunt (right heart failure, valve dysfunction, arrhythmias)

19. Question #2 • A 6-hour-old term male infant develops severe cyanosis, but has no murmur on cardiac ausculatation. He is in no apparent distress, and CXR reveals prominent pulmonary vascular markings. • The next MOST appropriate step is: • Start oxygen via nasal canula • Prostaglandin infusion • Atrial septostomy in cath lab • Echocardiogram • IV Furosemide

20. Cyanotic CHD • Transposition of the Great Arteries (TGA) • Early severe cyanosis (hours after birth) • Oxygenated pulmonary venous blood is unable to reach systemic circulation • Prostaglandin E1 given to keep ductus open • Balloon atrial septostomy to ensure atrial-level communication prior to anatomic correction

21. Cyanotic CHD • Pulmonary valve atresia • Presents with early severe cyanosis once the ductus starts to close • Start prostaglandin! • Ebstein malformation • Apical displacement of the tricuspid valve • Right to left shunting (through PFO) results in ↓pulmonary blood flow

22. TGA Ebstein’s Pulm atresia

23. Cyanotic CHD • Most commonly, the presence of serious CHD is heralded by the identification of cyanosis in an infant who is not in respiratory distress (“happy cyanosis”)

24. Cyanotic CHD • Tricuspid atresia • Sytemic venous return passes through PFO to LA and LV where it mixes with oxygenated blood entering the LA • The large single LV conducts blood into both great arteries • VSD • Large = minimal cyanosis • Small = a loud murmur • Absent = clinical cyanosis

25. Cyanotic CHD • Total Anomalous Pulmonary Venous Connection • PFO/ASD allows blood to enter the left heart • If pulm. vein connection is above the diaphragm, there is significant volume load on the right side of the heart, ↑ pulm blood flow, and minimal cyanosis

26. Cyanotic CHD • TAPV can be difficult to distinguish from ASD • A palpable sternal lift, wide and fixed splitting of S2 (due to RV overload), a pulmonary flow murmur, a tricuspid valve diastolic flow rumble, and tachypnea caused by pulm edema • Only difference is presence of cyanosis • When connection is below the diaphragm • Infants are ill in newborn nursery with severe pulm edema, pulm HTN, and cyanosis

27. Cyanotic CHD • Truncus arteriosus • Dilated aorta overrides a large VSD • Well developed PA arises from aorta • Mixing occurs in the great artery • Minimal cyanosis and no significant murmur

28. Cyanotic CHD • S2 is loud due to anterior position of truncal aortic valve • Ventricular lift is always palapable • Ejection click (or multiple clicks) caused by a valve that can have as many as 6 leaflets • Commonly present with failure to thrive several months after birth

29. Cyanotic CHD • Review: Admixture lesions • Tricuspid atresia, TAPV, truncus arteriosus • Absence of pulmonary stenosis leads to minimal cyanosis • Sometimes transient cyanosis on first postnatal day • Pulse ox below 95% are never normal in otherwise healthy-appearing infants after the first 6 postnatal hours

30. Cyanotic CHD • Hyperoxia test • Cyanosis typically present with sats ≤ 85% • Place the patient in a high-oxygen atmosphere (FiO2 near 1.0) • No increase in sats or PaO2 (≥150), cyanotic congenital heart disease should be considered

31. Question #3 • A 4-month-old infant with unrepaired congenital heart disease has had fever and URI symptoms since last evening. Her mother brings her to clinic, and on physical exam the patient is crying and unconsolable, has marked cyanosis, a decrease in the loudness of her murmur, and deep, rapid breathing. • Of the following, the next MOST appropriate step is: A. Echocardiogram B. IVFs C. Sedation with Morphine D. Acetaminophen E. IV Furosemide

32. Cyanotic CHD • Tetrology of Fallot • Usually are acyanotic at birth, but often have systolic murmurs • If pulm atresia or severe pulm stenosis, murmur is less obvious but cyanosis is severe • Exception is Tet with pulm atresia and multiple aortopulmonary collateral vessels→ prevent cyanosis, continuous murmur heard throughout the back VSD, pulm stenosis, overriding aorta, RVH

33. Cyanotic CHD • Hypercyanotic spells (“tet spells”) • Unusual in newborn period, but can happen any time in an unrepaired tet • Intense cyanosis develops rapidly • Sudden decrease in loudness of murmur caused by ↓ systolic flow through the RV outflow tract • Deep, rapid respiratory pattern • Calming the infant (with or without sedation) may resolve the spell

34. Cyanotic CHD • Prognosis for TOF • Once infant develops progressive cyanosis, surgical correction is indicated • Elective repair in first postnatal year • Surgical survival of 95% • However, 10% of neonates requiring bypass surgery have some element of neurodevelopmental delay or cognitive/school performance abnormality • Speech and behavior disorders are common among those who undergo neonatal heart surgery

35. Cyanotic CHD • Polycythemia • Desaturated arterial blood can cause increased erythropoetin secretion • Increased blood viscosity • Risk for thrombosis to lungs, kidney, or brain • Risk for cerebrovascular accident • Anemia • Chronic cyanotic heart disease pts. Are at risk for cerebrovascular accident due to paradoxic emboli and a relative anemia • Iron deficiency in the presence of polycythemia poses a greater risk for stroke

36. CHD with progressive heart failure • Too much pulmonary blood flow or too little systemic blood flow • When pulm vascular resistance falls, signs and symptoms start • Tachypnea, sweating, difficulty feeding, FTT, gallop rhythm, hepatomegaly • Left-to-right shunt via VSD, atrioventricular septal defect, or PDA causes pulm overcirculation • Most diseases causing heart failure in infants can be treated surgically as soon as signs develop

37. Question #4 • A 3-week-old infant presents to ER with tachypnea, mottled gray skin, poor perfusion, decreased peripheral and central pulses, a gallop rhythm and hepatomegaly. CXR shows cardiomegaly. You suspect left heart obstructive disease and order prostaglandin. • The MOST appropriate next step is: • NS bolus 20ml/kg • Electrocardiogram • Start oxygen via nasal canula • Captopril 0.01mg/kg • Place patient in knee-to-chest position

38. CHD presenting as shock or catastrophic heart failure • Critical coarctation of the aorta, interrupted aortic arch, critical aortic valve stenosis, hypoplastic left heart syndrome • Inadequate left heart development compromises cardiac output

39. CHD presenting as shock or catastrophic heart failure • Potential for catastrophic deterioration due to inadequate systemic flow is greater in these lesions as the ductus undergoes spontaneous closure • Oxygenated blood from the lungs is diverted across an atrial-level communication into right ventricle • Mixing in right atrium→ increased sats of right heart blood→ minimizes the appearance of cyanosis

40. CHD presenting as shock or catastrophic heart failure • In newborn nursery • A single and loud S2 • Increase in RV activity on precordial palpation • Minimally abnormal postductal pulse ox • Decreased femoral pulses not present because ductus is still large

41. CHD presenting as shock or catastrophic heart failure • Check pre- and post-ductal sats • A postductal sat above 96-97% rule out a completely ductal-dependent left heart obstruction • High sats can occur in the setting of coarctation of the aorta

42. CHD presenting as shock or catastrophic heart failure • Often present after discharge due to ductal patency • As ductus closes, decreased systemic blood flow, oliguria, acidosis, pulmonary edema, and heart failure • May mimic sepsis (tachypnea, mottled skin, poor perfusion) • Critical clues: a gallop rhythm and marked hepatomegaly or cardiomegaly

43. CHD presenting as shock or catastrophic heart failure • If suspect critical coarc or HLH, prostaglandin should be started immediately • EKG and echo are part of evaluation for patient with possible cardiogenic shock • Fluid resuscitation as well as respiratory and inotropic support are essential treatments

44. Follow-up for Coarc • Recoarctation can occur as child grows • Higher incidence of hypertension • Frequent BP measurements by pediatrician

45. Question #5 • A 4 week old infant born at term without complications presents with poor feeding. He ate well for the first 3 weeks after birth and gained weight appropriately. For the past week, however, Mom reports that he appears hungry but fatigues with feeding (now taking twice as long to feed). He breathes fast during his feedings and stops frequently to “catch his breath.” Of the following, what is the most likely additional finding in this infant? • A. Lobar consolidation on CXR • B. Lipid laden macrophages on BAL • C. Elevated BNP • D. Positive RSV • E. Posterior esophageal indentation on barium swallow

46. Heart Failure

47. Definition • HF results when cardiac output is insufficient to meet the metabolic demands of the body • Triggers of HF in children: • Excessive preload • Excessive afterload • Abnormal rhythm • Decreased contractility

48. Pathophysiology

49. Clinical Manifestations Infants Older Children Exercise intolerance Somnolence Anorexia Cough/wheezing Crackles Gallop rhythm Hepatomegaly JVD Peripheral edema • Feeding difficulties • Volume and duration • Increased fatigability • Mild to severe retractions • Tachypnea/ dsypnea • Grunting • Tachycardia • Gallop rhythm (S3,S4) • Hepatomegaly

50. Pulmonary Edema