Diagnosis and Management of Diagnosis and Management of the Neonate With Critical the Neonate With Critical Congenital Heart DiseaseCongenital Heart Disease

Department of Pediatrics

National Naval Medical Center

15 April 03

Neonate With Critical CHDNeonate 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 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 ViewNormal Fetal Echocardiogram: Four Chamber View

Ebstein’s AnomalyEbstein’s Anomaly

Critical CHD: Initial Evaluation and Critical CHD: Initial Evaluation and

ManagementManagement 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 Suspected CHD: Initial Evaluation

and Managementand 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 TransportStabilization 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 TransportStabilization 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 PGESide effects of PGE11

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 DiagnosisConfirmation 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 EvaluationGenetic 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 Evaluation of Other Organ SystemsSystems

CNS: CNS anomalies and ischemic injuryGI: risk for NECRenal: 3-6% incidence of urinary tract

anomalies

Timing and Type of SurgeryTiming and Type of Surgery

Cardiac catheterization procedures– Balloon atrial septostomy– Balloon valvuloplasty– Balloon angioplasty

Open versus ClosedPalliative versus Corrective

– Trend towards early, corrective surgery, even in preterm or low birth weight infants

Critical CHD: Lesion Specific Critical CHD: Lesion Specific ManagementManagement

Ductal dependent for systemic blood flow– HLHS management

Ductal dependent for pulmonary blood flowD-transposition of the great arteriesTotal anomalous pulmonary venous

connection with obstruction

Hypoplastic Left Heart SyndromeHypoplastic Left Heart Syndrome

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: Hypoplastic Left Heart Syndrome: Clinical PresentationClinical 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: Hypoplastic Left Heart Syndrome: Initial Medical ManagementInitial 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: Hypoplastic Left Heart Syndrome: Stage One NorwoodStage 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 Hypoplastic Left Heart Syndrome: S/P Stage One NorwoodNorwood

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: Hypoplastic Left Heart Syndrome: HemiFontan ProcedureHemiFontan 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: Hypoplastic Left Heart Syndrome: Fontan ProcedureFontan Procedure

Performed around 18-24 months

Venous and systemic circulations are separated

Survival: excellent Long term issues: RV

function, arrhythmias

Hypoplastic Left Heart Syndrome: Hypoplastic Left Heart Syndrome: Fenestrated Fontan ProcedureFenestrated Fontan Procedure

Transplant in Hypoplastic Left Heart Transplant in Hypoplastic Left Heart SyndromeSyndrome

Issues of waiting for donor heartExcellent operative resultsLimited donor availabilityIssues of life long immunosuppresion

Coarctation of the AortaCoarctation of the Aorta

Critical Pulmonary Valve StenosisCritical Pulmonary Valve Stenosis

Critical Pulmonary Valve Stenosis: Critical Pulmonary Valve Stenosis: Tricuspid RegurgitationTricuspid Regurgitation

Ebstein AnomalyEbstein Anomaly

D-transposition of the Great ArteriesD-transposition of the Great Arteries

Arterial Switch Procedure for Arterial Switch Procedure for D-TGAD-TGA

Total Anomalous Pulmonary Venous Total Anomalous Pulmonary Venous ConnectionConnection

Total Anomalous Pulmonary Venous Total Anomalous Pulmonary Venous Connection With ObstructionConnection With Obstruction

Total Anomalous Pulmonary Venous Total Anomalous Pulmonary Venous Connection With ObstructionConnection With Obstruction