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 Childrens Hospital of Wisconsin were prenatally diagnosed

Normal Fetal Echocardiogram: Four Chamber View

Ebsteins Anomaly

Critical CHD: Initial Evaluation and Management

ABCs Oxygen (judicial) to saturations of 80-85% Place umbilical lines PGE (0.025-0.1 micrograms/Kg/min) History Complete physical with four extremity BPs 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

ABCs Place lines (UVC, UAC) Check and administer

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 ABGs Sepsis evaluation. Antibiotics

glucose and calcium as

Stabilization and Transport

PGE1

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 PGE1

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 (Turners syndrome): Coarctation 22q11 Deletion (DiGeorge syndrome): Conotruncal

abnormalities 7q11 Deletion (Williams syndrome) Single gene defects (Noonans, 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 Ductal

dependent for systemic blood flow

HLHS management

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