This document summarizes the diagnosis and management of critical congenital heart disease in neonates. It discusses prenatal evaluation, initial evaluation and stabilization, confirmation of diagnosis, preoperative evaluation of other organ systems, and lesion-specific surgical management. Key lesions discussed in detail include hypoplastic left heart syndrome, transposition of the great arteries, and total anomalous pulmonary venous connection.

1. Diagnosis and Management of the Neonate With Critical Congenital Heart Disease Department of Pediatrics National Naval Medical Center 15 April 03

2. Neonate With Critical CHD Prenatal evaluation Initial neonatal evaluation and management Stabilization and transport Confirmation of the diagnosis Preoperative evaluation of non-cardiac organ systems Timing and type of surgery Lesion specific management

3. Prenatal Assessment Obstetric history Genetic evaluations Prenatal ultrasound Fetal echocardiography 60% of cardiology admissions at CHOP prenatally diagnosed 49% of HLHS admissions at Children’s Hospital of Wisconsin were prenatally diagnosed

4. Normal Fetal Echocardiogram: Four Chamber View

5. Ebstein’s Anomaly

6. Critical CHD: Initial Evaluation and Management ABC’s Oxygen (judicial) to saturations of 80-85% Place umbilical lines PGE (0.025-0.1 micrograms/Kg/min) History Complete physical with four extremity BP’s Pre and post-ductal oxygen saturations Hyperoxia test CXR EKG Echocardiogram

7. Suspected CHD: Initial Evaluation and Management Pre and post-ductal oxygen saturations If pre-ductal sat higher than post-ductal sat (differential cyanosis) Left heart abnormalities (such as aortic arch hypoplasia, critical aortic stenosis, interrupted aortic arch) Persistent pulmonary hypertension If post-ductal sat higher than pre-ductal (reverse differential cyanosis) TGA with CoA or TGA with IAA TGA with supersystemic pulmonary vascular resistance

8. Stabilization and Transport ABC’s Place lines (UVC, UAC) Check and administer glucose and calcium as needed If severe respiratory distress, shock, or severe cyanosis: sedate, paralyze, intubate, and mechanically ventilate to oxygen sats of 80-85%. Place NG tube. Check ABG’s Sepsis evaluation. Antibiotics

9. Stabilization and Transport PGE 1 lowest dose possible (0.025 micrograms/kg/min) Judicious use of pressors Dopamine and Dobutamine Milrinone Consider use of 2-3% CO2 in ventilated patients with left sided obstructive lesions

10. Side effects of PGE 1 More common in premature infants Clinical deterioration if pulmonary venous obstruction present HLHS with restrictive/intact atrial septum TGA with intact ventricular septum and a restrictive/intact atrial septum TAPVR with obstruction Apnea Hypotension

11. Confirmation of the Diagnosis Echocardiography primary diagnostic modality for anatomic definition Not “non-invasive” in sick newborn Cardiac catheterization Rarely indicated to confirm diagnosis Therapeutic (interventional procedures)

12. Genetic Evaluation Genetic Trisomies 13, 18, 21 Monosomy X (Turner’s syndrome): Coarctation 22q11 Deletion (DiGeorge syndrome): Conotruncal abnormalities 7q11 Deletion (Williams syndrome) Single gene defects (Noonan’s, Holt-Oram, Ellis-van Crevald, Alagille) Unknown cause Vacterl Charge

13. Evaluation of Other Organ Systems CNS: CNS anomalies and ischemic injury GI: risk for NEC Renal: 3-6% incidence of urinary tract anomalies

14. Timing and Type of Surgery Cardiac catheterization procedures Balloon atrial septostomy Balloon valvuloplasty Balloon angioplasty Open versus Closed Palliative versus Corrective Trend towards early, corrective surgery, even in preterm or low birth weight infants

15. Critical CHD: Lesion Specific Management Ductal dependent for systemic blood flow HLHS management Ductal dependent for pulmonary blood flow D-transposition of the great arteries Total anomalous pulmonary venous connection with obstruction

16. Hypoplastic Left Heart Syndrome

17. Hypoplastic Left Heart Syndrome: Pathology: aortic atresia/severe stenosis, mitral atresia/severe stenosis, hypoplastic left ventricle and aortic arch. 1.5% of congenital heart defects. Most common cause of cardiac related neonatal mortality. Ductal dependent for systemic blood flow at birth Patients may have associated chromosomal or developmental abnormalities

18. Hypoplastic Left Heart Syndrome: Clinical Presentation May be diagnosed by fetal ultrasound. Prognostic issues: atrial septal position, size of foramen ovale (if restrictive, pulmonary venous obstruction) Classic presentation: cardiogenic shock, poor perfusion, decreased pulses, profound metabolic acidosis. May have systolic murmur. Diagnosis: echocardiogram . CXR and EKG are non-specific.

19. Hypoplastic Left Heart Syndrome: Initial Medical Management Prostaglandin E1 0.025 to 0.2 micrograms/kg/min- watch for side effects Room air ventilation: ideal ABG ph 7.4/ pco2 40/ po2 40 Inhaled CO2 to manipulate pulmonary vascular resistance? Watch the use of pressors- may be harmful

20. Hypoplastic Left Heart Syndrome: Stage One Norwood Performed in neonatal period Procedure: MPA divided; distally MPA closed with patch; hypoplastic aortic arch reconstructed and anastomosed to the proximal MPA with homograft augmentation; atrial septosomy; systemic shunt placed.

21. Hypoplastic Left Heart Syndrome: S/P Stage One Norwood Surgical issues: Unobstructed aortic arch Adequate atrial septectomy Balanced pulmonary and systemic blood flow (Qp:Qs 1:1) Survival at major centers: 80%

22. Hypoplastic Left Heart Syndrome: HemiFontan Procedure Shunt ligated, superior vena cava anastomosis to pulmonary artery, pulmonary arteries augmented, flap of tissue closes SVC-RA junction Performed around 6 months of age following Norwood Volume load on right ventricle removed Excellent survival statistics

23. Hypoplastic Left Heart Syndrome: Fontan Procedure Performed around 18-24 months Venous and systemic circulations are separated Survival: excellent Long term issues: RV function, arrhythmias

24. Hypoplastic Left Heart Syndrome: Fenestrated Fontan Procedure

25. Transplant in Hypoplastic Left Heart Syndrome Issues of waiting for donor heart Excellent operative results Limited donor availability Issues of life long immunosuppresion

26. Coarctation of the Aorta

27. Critical Pulmonary Valve Stenosis

28. Critical Pulmonary Valve Stenosis: Tricuspid Regurgitation

30. Ebstein Anomaly

31. D-transposition of the Great Arteries

32. Arterial Switch Procedure for D-TGA

33. Total Anomalous Pulmonary Venous Connection

34. Total Anomalous Pulmonary Venous Connection With Obstruction

35. Total Anomalous Pulmonary Venous Connection With Obstruction