diagnosis and management of the neonate with critical congenital heart disease department of...
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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