Diagnosis and Management of the Neonate With Critical Congenital Heart Disease

Department of Pediatrics National Naval Medical Center 15 April 03

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

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

Normal Fetal Echocardiogram: Four Chamber View

Ebstein’s Anomaly

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

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

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

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

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

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)

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

Evaluation of Other Organ Systems

 CNS: CNS anomalies and ischemic injury  GI: risk for NEC  Renal: 3-6% incidence of urinary tract anomalies

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

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

Hypoplastic Left Heart Syndrome

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

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.

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

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.

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%

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

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

Hypoplastic Left Heart Syndrome: Fenestrated Fontan Procedure

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

Coarctation of the Aorta

Critical Pulmonary Valve Stenosis

Critical Pulmonary Valve Stenosis: Tricuspid Regurgitation

Ebstein Anomaly

D-transposition of the Great Arteries

Arterial Switch Procedure for D-TGA

Total Anomalous Pulmonary Venous Connection

Total Anomalous Pulmonary Venous Connection With Obstruction

Total Anomalous Pulmonary Venous Connection With Obstruction