Neonatal Cardiology

1. Neonatal Cardiology

2. Goals Review both fetal and neonatal cardiac physiology Understand what murmurs are, how they occur, and how to describe them Discuss several types of congenital heart disease and how to distinguish them Review common genetic syndromes and their associated heart defects

3. Normal Cardiac Anatomy

4. Differences in the Fetus Foramen Ovale Ductus Arteriosus Right heart is the dominant ventricle – pumps 2/3 of cardiac output Relative RVH in utero

5. Differences in the Fetus

6. Differences in the Fetus

7. Changes after Birth Pulmonary Vascular Resistance begins to fall immediately Foramen Ovale closes – within the first hour Ductus Arteriosus closes – up to 48 hours can be normal Left heart now the dominant side Process continues for up to 6-8 weeks

8. Cardiac Evaluation History Exam: Inspection Palpation Auscultation

9. History Prematurity Maternal pregnancy complications (DM, PIH, infections, teratogen exposure) Abnormal ultrasounds Family history of congenital heart disease

10. History Infants symptoms: Tachypnea Diaphoresis Fatigue Cyanosis Especially if any symptoms with feeding (exercise for babies)

11. Physical exam – inspection and palpation Inspection Chest symmetric, normal shape Other systems (dysmorphic, edema, cyanosis, clubbing) Palpation PMI Thrills (palpable murmurs) Pulses (brachial and femoral)

12. Cardiac Exam – Auscultation Rate and rhythm Heart sounds Extra sounds Murmurs

13. What is a heart murmur? Results from turbulent blood flow, typically from the pressure difference between adjacent cardiac structures Can be normal (physiologic, benign, flow, transitional, etc) vs abnormal (pathologic) Most (80%) children will have soft murmurs in the perinatal period

14. Murmurs Location Radiation Timing (systolic, diastolic, continuous) Intensity (1-6 systolic, 1-4 diastolic) Pitch (high frequency [diaphragm better] vs low frequency [bell better]) Quality

15. Benign Murmurs Pulmonary flow (LUSB, soft) Peripheral pulmonary branch stenosis (axillae, back) Neonatal Still’s murmur (LLSB, “vibratory” or “musical”) Venous hum (continuous, under either clavicle but R more often than L)

18. RED FLAGS Diastolic murmurs (only venous hum is OK) Continuous murmurs (PDA should be gone by 48 hours) Loud murmurs + thrills SYMPTOMS, especially cyanosis

19. Pathologic murmurs Caused by abnormal anatomy or communications and the turbulent blood flow through them Typically from problems with valves (pulmonic stenosis, aortic stenosis), narrowings (coarctation) or holes where they shouldn’t be (VSD, ASD, PDA)

20. Acyanotic Heart Disease VSD May not hear at birth until PVR drops Typically holosystolic (engulfs S1 and S2) Typically loudest LLSB May have a thrill Louder murmurs are typically smaller holes (greater pressure difference)

23. Acyanotic Heart Disease Patent Ductus Arteriosus Continuous, “machine like” murmur Best under L clavicle Should disappear by 48 hours

25. Acyanotic Heart Disease Coarctation of the aorta Often can’t appreciate until ductus arteriosus closes, then rapid detioration Systolic ejection murmur best LUSB and over back Decreased femoral pulses

27. Cyanotic Heart Disease Most infants with cyanotic heart disease are cyanotic at birth, so shouldn’t be in Level II Check mucous membranes, nailbeds, etc (all infants can get perioral vascular congestion which isn’t real cyanosis) Caused by shunting of blood from the right to the left (deoxygenated blood) The 5 “Terrible T’s”

28. Truncus Arteriosus Only one vessel coming off the ventricles

31. Transposition of the Great Arteries Aorta off the RV, Pulmonary artery off the LV Must have mixing (ASD, VSD, PDA) or incompatible with life “Egg on a string” x-ray Often no murmur

35. Tricuspid Atresia (and Ebstein’s) Tricuspid valve is closed (atresia) or displaced and dysfunctional (Ebstein’s) HUGE heart on x-ray (mainly right atrium)

38. Tetralogy of Fallot VSD Overriding aorta Right ventricular hypertrophy Pulmonary stenosis “Boot shaped heart” on x-ray Murmur is from pulmonic stenosis, not from VSD

41. Total Anomalous Pulmonary Venous Return (TAPVR) Pulmonary veins come back somewhere besides the left atrium If obstructed, is the only pediatric cardiac surgical EMERGENCY CXR is “snowman in a snowstorm”

44. “Terrible” hypoplastic left heart syndrome Spectrum of disease, extreme form has almost no left ventricle, mitral atresia, aortic atresia, coarctation of the aorta As PDA closes, no blood to body – incompatible with life Often very non-specific physical exam, CXR

46. And now for some EKG reading!

48. Genetic syndromes associated with CHD Trisomy 13 PDA, septal defects, pulmonic and aortic stenosis Trisomy 18 VSD, polyvalvular disease, coronary abnormalities Trisomy 21 – 45% have heart defect AV canal, VSD, PDA, anomalous subclavian artery All need echo

49. More Syndromes Turner (XO) 30% bicuspid aortic valve; 10% coarctation Noonan pulmonary valve stenosis, ASD Hypertrophic cardiomyopathy in 20% DiGeorge/ VCF/ 22q11 Interrupted aortic arch, right aortic arch truncus arteriosus, tetrology of Fallot, pulmonary atresia with VSD

50. And more syndromes! Marfan: dilatation of ascending aorta/ aortic sinus, aortic and mitral insufficiency VACTERL: VSD in majority of cases Williams: supravalvular aortic stenosis, pulmonary artery stenosis

51. More syndromes again… Ellis-van Creveld: ASD or single Fetal Alcohol Syndrome: VSD Holt-Oram: atrial and ventricular septal defects, arrhythmias

52. Last page of syndromes! Pompe disease: (glycogen storage) cardiomyopathy MPS: storage of MPS in arteries, valves w/ insufficiency and stenosis Hyperlipoproteinemia: premature atherosclerosis Freidrich ataxia: cardiomyopathy Muscular dystrophy: myocardial degeneration and fibrosis

53. Key Points Neonatal period, and particularly the first few days, are a time of great change Most murmurs are benign, but if its loud, harsh, diastolic, or the infant has symptoms, be concerned 1-2-3-4-5 cyanotic heart diseases Genetic syndromes have commonly associated heart defects

54. Any Questions ?