Pediatric Cardiology Snippets and Board Review

1. Pediatric Cardiology Snippets and Board Review Thomas Burklow, MD National Capital Military Children’s Center

2. Attempting the impossible… • Ch. 23: Recognition of cardiovascular disorders • Ch. 24: Congenital heart disease • Ch. 25: Acquired heart disease • Ch. 26: Arrhythmias and conduction disorders

3. Ground rules • You already know this stuff (at least it seems vaguely familiar • Material reflects materials covered by ABP examination (and not necessarily clinical practice)

4. Recognition of Cardiovascular Disorders National Capital Military Children’s Center

5. Physiology

6. Vena Cavae (VC) Pulmonary Veins (PV) Right Atrium (RA) Left Atrium (LA) The Heart Right Ventricle (RV) Left Ventricle (LV) Pulmonary Artery (PA) Aorta (Ao)

7. Normal newborn cardiopulmonary physiology • Anatomically… • Ductus arteriosus • Foramen ovale • Physiologically… • Normal physiology • Left-right intra-atrial shunting • Left-right ductal shunting VC PV RA RV LV PA Ao

8. Initial physiology: high pulmonary vascular resistance At birth VC PV RA RV LV PA Ao

9. Several hours after birth… Pulmonary vascular resistance typically falls VC PV RA RV LV PA Ao

10. Quiz: Murmurs, the benign ones • Healthy one month old with a systolic murmur noted in both axillae and back • Bilateral pulmonary artery branch stenosis • 4 year old with a newly noted murmur, best heard along LSB, louder supine, attenuates when stands. “Vibratory” in character • Still’s murmur, or ‘normal flow murmur’ • 3 year old with a continuous murmur noted primarily at the RUSB • Venous hum • Neonate with mild persistent tachypnea with a murmur best heard at the lower sternal border, systolic regurgitant (“holosystolic”) which attentuates over the next few days • Tricuspid regurgitation

11. Newborn murmurs • In the newborn, systolic murmurs can be caused by all but: • Patent ductus arteriosus • Peripheral pulmonary stenosis • Pulmonary outflow tract murmur • Pulmonary insufficiency from congenital dysplastic pulmonary valve • Benign left ventricular outflow tract murmur (pulmonary insufficiency is a diastolic murmur)

12. Murmur referrals? • Which of the following is cause of appropriate referral to cardiology? • Holosystolic murmur • Late systolic murmur • Murmur with a thrill • Continuous murmur (that is not a venous hum) • Diastolic murmur All are appropriate referrals...

13. Syndromes and heart disease What is the incidence and typical heart disease associated with the following? • Down syndrome • 50%; Ventricular septal defect • Noonan syndrome • 50%; Pulmonary stenosis, HCM • Turner syndrome • 33%; Coarctation, aortic valve disease, PAPVR • Williams syndrome • 33%; supravalvar aortic stenosis • DiGeorge syndrome • 35%; conotruncal abnormalities (iAA type B, TA, ToF)

14. Chest pain in Children • Identify the false statement • Most chest pain is musculoskeletal or idiopathic in origin • An echocardiogram is a reasonable tool in the assessment of most chest pain • A history of structural heart disease or previous Kawasaki disease should raise concern • EKG abnormalities are common in children with chest pain

15. Neonatal cyanosis • What is the hyperoxia challenge test? • Below what value of paO2 suggests congenital heart disease as cause for cyanosis? • 150 mmHg • Which parameter on the ABG is strongly suggestive of pulmonary disease? • Elevation in paCO2

16. Congestive heart failure • Which of the following are the best tests for determining if CHF is present? • Echocardiogram • Electrocardiogram • History • Serum sodium and creatinine • Physical examination • Chest roentgenogram

17. CHF and shock • A 4 day infant presents to the emergency room tachypnea, tachycardia, perfusing poorly, and showing signs of obtundation. • Physical examination shows a quiet precordium except for tachycardia, clear lungs, poor distal pulses and capillary refill, and marked hepatomegaly. • What type of structural heart diseases could this presentation be consistent with?

18. Initial physiology Ductal dependent left-sided obstruction VC PV RA Hypoplastic Left Heart Syndrome RV LV PA Ao

19. The ductus closes…. Critical Aortic Stenosis Critical Coarctation Ductal dependent left-sided obstruction VC PV RA RV LV PA Ao

20. CHF and shock • A 1 month infant presents to your clinic with parental concerns of poor feeding, diaphoresis and tachypnea. • Physical examination shows a grade III harsh systolic ejection murmur, tachypnea to 70 bpm, normal distal pulses and capillary refill, and marked hepatomegaly. • What structural heart diseases could this presentation be consistent with?

21. Left to right shunt with falling PVR: eg. VSD at birth VC PV RA RV LV PA Ao

22. VSD physiology at one month of age VC PV RA RV LV PA Ao

23. CHF: treatment with digoxin • 4 month old on digoxin whose father can’t remember how many doses of digoxin he administered. Which is the earliest sign of digoxin toxicity? • Atrioventricular block • Sinus bradycardia • Feeding intolerance • Supraventricular arrhythmias

24. Congenital Heart Disease National Capital Military Children’s Center

25. Ventricular Septal Defect • Most common congenital heart defect • Most common in chromosomal abnormalities • Identified by location • Membranous septum • Inlet septum • Muscular septum • Supracristal

26. Board question • 6 week old with a known VSD and worsening failure. Which of the following statements is MOST appropriate?: • Oxygen therapy can be deleterious • Caloric needs are reduced • Rales often accompany congestive heart failure in infants • Afterload reduction is contraindicated • IV furosemide is deleterious

27. Patent ductus arteriosus • Presentation depends upon degree of left-to-right shunting • Can cause significant volume overload of the heart, like a VSD VC PV RA RV LV PA Ao

28. Patent ductus arteriosus • Presentation depends upon degree of left-to-right shunting • Can cause significant volume overload of the heart, like a VSD VC PV RA RV LV PA Ao

29. The physical finding that is the hallmark of the PDA is… • Fixed S2 split • S3 gallop • Continuous murmur at LUSB • Diastolic murmur at the LLSB • S4

30. Atrial septal defect • This includes any defect in the atrial septum to include: • Secundum ASD • Primum ASD • Sinus venosus ASD • More common in females • Occasionally inherited as in Holt-Oram and Klippel-Feil syndromes

31. A 14 year female is found to have a loud murmur on examination. Which of the following would NOT suggest a ASD? • A fixed S2 split • Right ventricular enlargement on EKG • Increased pulmonary vascular markings on CXR • A diastolic murmur over the right lower sternal border All of these findings can be seen with ASDs

32. ASD physiology VC PV RA RV LV PA Ao

33. Coarctation of the aorta • Obstruction of the aorta just distal to the left subclavian artery in proximity of the PDA insertion site • More common in males • Most common lesion found in Turner syndrome • Presentation may range from an asymptomatic murmur to cardiogenic shock

34. A 5 year girl is being evaluated for short stature. Which of the following would NOT suggest the presence of a coarctation of the aorta? • Systolic hypertension in the arms • A absent or markedly diminished femoral pulse • Left ventricular hypertrophy on EKG • Right axis deviation on EKG • Rib notching on CXR

35. What does rib notching look like?

36. Tetralogy of Fallot • The most common cyanotic heart disease diagnosed outside of the neonatal period • Features • Components of valvar, subvalvar and supravalvar pulmonary stenosis • Large VSD • Right ventricular hypertrophy • Overriding aorta • CXR classically describes the boot-shaped heart

37. Physiology

39. Hypercyanotic spells can be induced by all of the following except: • Pain • Induction of anesthesia • Dehydration • Iron Deficiency • Squatting

40. d-Transposition of the Great Arteries • The most common cyanotic heart disease identified in neonatal period • Profoundly cyanotic and acidotic • Physical finding often lack specific cardiac findings such as a murmur

41. Physiology

42. A 3 hour old infant is found profoundly cyanotic and in shock. Prostaglandin E1 is begun. The side effects of PGE1 include… • Fever • Apnea • Hypotension • Flushing • Seizures • Male pattern baldness

43. Acquired Heart Disease National Capital Military Children’s Center

44. An 4-year-old girl is seen 10 days following an upper respiratory infection. Her knees and ankles were swollen and painful, and her temperature is 104F degrees. The cardiac examination is significant for grade 6/6 systolic murmur at the apex. These findings are consistent with which of the following: • Acute rheumatic fever • Septic arthritis • Juvenile rheumatoid arthritis • Pericarditis • Kawasaki disease

45. A 2-year-old boy presents with fever of 5 days duration, swollen hands and feet, strawberry tongue, maculopapular rash, and conjunctivitis. The most likely diagnosis is: • Lyme disease • Kawasaki disease • Stevens-Johnson syndrome • Mononucleosis • Acute rheumatic fever

46. A 12-year-old boy presents with a 24 hour history of sharp, pleuritic chest pain that is worse in the supine position. His fever is 101.3F and pericardial friction rub. The most likely diagnosis is: • Musculoskeletal chest pain • Pericarditis • Bacterial endocarditis • Mycoplasma pneumonia • Pulmonary embolus

47. Infective endocarditis • Causative bugs • Streptococcal viridans • Staphylococcal aureus • Staphylococcal epidermidis • Fungal pathogens • HACEK group

48. Classic physical findings • Janeway lesions • Osler nodes • Roth spots

49. Acute Rheumatic Fever • Delayed sequelae of Grp A streptococcal infection of the pharynx • Streptococcal infections of the skin and other sites do NOT lead to ARF • Not on boards: belongs to Grp A type 18 • Typically follows 1 to 5 weeks after pharyngitis

50. Jones Criteria • J • O • N • E • S oints…polyarticular migratory arthritis bvious…carditis odules…subcutaneous, painless rythema marginatum yndenham chorea Two major; one major and two minor PLUS evidence of an antecedent Streptococcal infection…ASO or throat culture