A 3.48-kg male infant was delivered by way of planned repeat Cesarean section at 39 2/7 weeks’ gestation to a 36 year-old gravida 3, para 2 mother. The pregnancy was complicated by a diagnosis of fetal aortic stenosis made at 22 3/7 weeks’ gestation (Fig. ). At 23 4/7 weeks’ gestation, fetal balloon aortic valvuloplasty was performed. After the procedure, as demonstrated by color Doppler, aortic arch flow had gone from retrograde to antegrade across the aortic valve and in the ascending and transverse aortic arch; there was mild aortic insufficiency. Postnatally the left ventricle and mitral valve were of normal size; the aortic valve was mildly hypoplastic at 5 mm with a peak Doppler-derived gradient of 60 mmHg; and there was significant left-ventricular hypertrophy (diastolic left ventricular posterior wall dimension, LVPWd 6 mm) with poor systolic function (LVEF% < 20%). Doppler flow in the transverse arch and isthmus was antegrade. Cardiac catheterization showed increased left atrial pressure of 25 mm Hg. The aortic valve was dilated by way of a retrograde approach with a 5-mm Slalom balloon catheter (Cordis Corporation, Miami, FL, USA) with a decrease in peak-to-peak gradient from 60 to 45 mm Hg. After valvuloplasty, a 5-mm stent was placed in the interatrial septum. The neonate was maintained on prostaglandin infusion.
Fig. 1 Fetal echocardiogram at 22 4/7 weeks’ gestation. The left ventricle is dilated with echo-bright endocardium suggestive of endocardial fibroelastosis (a), and there is left-to-right flow across the atrial septum (b). Sagittal two-dimensional (more ...)
On day of life 14, with no change in clinical status or LVEF%, the child underwent stage I hybrid palliation with placement of an 18-mm ductal stent and bilateral pulmonary artery banding. Repeat dilation of the aortic valve was performed at that time, resulting in a decrease in peak-to-peak gradient to 11 mm Hg and mild to moderate aortic insufficiency.
The LVEF% improved gradually to 60% during the subsequent months. Cardiac catheterization at 9 months of age showed a 38 mm Hg peak-to-peak gradient across the aortic valve and mild to moderate aortic insufficiency. Left atrial pressure was 11 mm Hg; left ventricular end diastolic pressure was 14 mm Hg; and estimated pulmonary vascular resistance was 4 Woods units. After recovery from a severe intercurrent pulmonary illness, the child underwent a Ross-Konno procedure, placement of a 16-mm right ventricle-to-pulmonary artery homograft, ductus arteriosus and atrial stent removal, aortic arch reconstruction, pulmonary artery debanding, resection of extensive EFE (Fig. ), and closure of an atrial septal defect. He was successfully extubated on postoperative day 7 and weaned from inotropic and vasodilatory support. Follow-up echocardiography showed no evidence of mitral inflow obstruction or regurgitation, normal left-ventricular systolic function, and a well-functioning autograft with no aortic arch obstruction. At catheterization 1 month later, the cardiac index was 3.3 ml/min/M2 by thermodilution; wedge pressure mean was 10 mm Hg; and pulmonary vascular resistance was 6 Woods units (decreasing to 2.6 Woods units in response to inhaled nitric oxide).
Fig. 2 Surgical specimens of the explanted aortic valve (inset, left) and left-ventricular endocardium. The aortic valve was severely dysplastic, doming, and unicuspid. At the time of biventricular repair, the valve was detached from the aortic wall. The endocardium (more ...)
At 1 year of life, left heart dimensions are normal (mitral valve 13.7 mm [Z score +0.7], LV end diastolic diameter 24.9 mm [Z score +0.3], and LVPWd 6 mm [Z score +2.4]), and his systolic function is normal with an LVEF% of 69% by Simpson’s biplane method (Fig. ).
Echocardiogram 2 months after biventricular repair demonstrates an apex-forming left ventricle and mild residual left-ventricular hypertrophy