TABLE 1

Cyanotic Congenital Heart Disease or Aortic Arch Obstruction Diagnosed in Newborns at BWH or Referred From Other Hospitals to the CICU at BCH in the First 10 Weeks After Birth During the Study Period

DiagnosisBorn at BWHBorn at Other Hospitals
PrenatalPostnatal% PrenatalPrenatalPostnatal% Prenatal
Lesions selected to be screened by AAP
 Hypoplastic left heart16023
 Pulmonary atresia-intact ventricular septum4004a
 Tetralogy of Fallotb17096
 dextro-transposition of the great arteriesc180210
 Tricuspid atresia4020
 Total anomalous pulmonary venous connection0d007
 Truncus arteriosus4000
 Total630100153033
Aortic arch obstruction
 Coarctation of the aorta200714
 Interrupted aortic arch, ventricular septal defect6102
 Total2619671630
Other cyanotic congenital heart disease22e01006f7g46
Suspected aortic arch obstruction not confirmed by postnatal echocardiographyh151
  • —, not applicable.

  • a Two patients had critical pulmonary stenosis with near atresia.

  • b Eighteen with pulmonary stenosis, 11 with pulmonary atresia, 2 with absent pulmonary valve, 1 with complete atrioventricular canal.

  • c Twenty-one with intact ventricular septum, 3 with ventricular septal defect, 6 with ventricular septal defect and pulmonary stenosis.

  • d Total anomalous pulmonary venous connection did occur in 3 patients with heterotaxy and atrioventricular canal defect that was detected prenatally, and are listed in the “Other cyanotic congenital heart disease” section.

  • e Heterotaxy (total 7) with atrioventricular canal and total anomalous pulmonary venous connection (3), unbalanced atrioventricular canal and hypoplastic left ventricle (2), or atrioventricular canal, double outlet right ventricle, pulmonary stenosis (2); complex congenital heart disease with single ventricle physiology (5); critical aortic stenosis with fetal intervention (4); unbalanced complete atrioventricular canal (2); Ebstein anomaly of the tricuspid valve (1); pulmonary atresia with large apical muscular ventricular septal defect (1); hypoplastic right ventricle, ventricular septal defect, pulmonary stenosis (1); double outlet right ventricle, superior-inferior ventricles, crisscross atrioventricular valves (1).

  • f Heterotaxy (total 2) with double outlet right ventricle, ventricular septal defect, crisscross atrioventricular valves, pulmonary stenosis (1), or tetralogy of Fallot and malposed atrial septum (1); Ebstein anomaly of the tricuspid valve (1); dysplastic tricuspid valve (1); right dominant unbalanced atrioventricular canal, small left ventricle, coarctation of the aorta (1); double outlet right ventricle, transposition of the great arteries, pulmonary stenosis (1).

  • g Critical aortic stenosis (1); arterial tortuosity syndrome and midaortic syndrome (1); pericardial teratoma compressing airway (1); pulmonary glycogenosis and pulmonary vein stenosis (1); vascular ring (right aortic arch with aberrant left subclavian artery compressing airway and multiple ventricular septal defects) (1); Ebstein anomaly of the tricuspid valve (1); arcade mitral valve associated with severe mitral regurgitation (1).

  • h Left-ventricular–right-ventricular size discrepancy or aortic arch hypoplasia noted on fetal echocardiography.