Original Articles
Biventricular repair in neonates with hypoplastic left heart complex

Presented at the Thirty-fourth Annual Meeting of The Society of Thoracic Surgeons, New Orleans, LA, Jan 26–28, 1998.
https://doi.org/10.1016/S0003-4975(98)00803-0Get rights and content

Abstract

Background. Multiple obstructions in the left heart–aorta complex have been associated with poor survival. No consensus exists as to whether these patients will have a favorable outcome with biventricular repair where most advocate a univentricular approach.

Methods. Since late 1988, all 11 neonates seen with hypoplastic left heart complex, which includes aortic arch obstruction, underwent biventricular repair. All patients had antegrade aortic flow and no intrinsic aortic or mitral stenosis. Elimination of the extracardiac afterload was achieved by extensive ascending aorta and aortic arch reconstruction with a pulmonary homograft patch. All intracardiac shunts were eliminated to fully preload the left heart. The median age at first operation was 7 days and the mean weight, 3.59 ± 0.49 kg. The echocardiographic variables used to evaluate the left heart–aorta complex were reviewed, and the preoperative and postoperative measurements were compared.

Results. There were two early deaths. Four patients had six reoperations for left ventricular outflow tract obstruction, 2 of whom have required prosthetic valve replacement (1, aortic and mitral; 1, aortic), and 2 patients had three reoperations for recurrent coarctation. There was one late death at 3 years from pulmonary hypertension. Mean follow-up was 44 ± 35 months. The 8 current survivors are all in New York Heart Association class I or II. The actuarial survival rate at 8 years is 63%, and the freedom from reoperation at 3 years is 25%.

Conclusions. We have successfully achieved biventricular repair in most of the patients with hypoplastic left heart complex, a subset of patients with hypoplastic left heart syndrome. Some growth of the left ventricular structures was already observed at the time of hospital discharge. However, reoperation, particularly for left ventricular outflow tract obstruction, appears likely. Increasing experience will more accurately define predictive criteria for the feasibility of biventricular repair.

Section snippets

Material and methods

Between November 1988 and June 1997, all 11 neonates seen with HLHC at The Montreal Children’s Hospital underwent biventricular repair. There were 5 boys and 6 girls. The median age was 7 days (range, 5 to 45 days). The mean weight was 3.59 ± 0.49 kg, and the mean body surface area (BSA) was 0.24 ± 0.04 m2.

Hypoplastic left heart complex consists of multiple hypoplastic structures of the left heart–aorta complex including the mitral valve (MV), the left ventricle, the left ventricular outflow

Postoperative hemodynamics

Most remarkable in the immediate postoperative period was the high LAP. Arterial blood pressure was usually stable with high-dose inotropic support. As early as 6 hours postoperatively, a marked decrease in LAP occurred and by 24 hours postoperatively, LAPs were almost normal (Fig 2).

Structural measurements

The postoperative data were the measurements made at the end of the hospital stay prior to discharge (see Table 1). They did not include measurements on the aortic arch and the ascending aorta, which were

Comment

Hypoplastic left heart syndrome is a term describing a tremendously variable set of structural defects in the left heart–aorta complex. The literature usually reserves this term for extreme underdevelopment of the left ventricle secondary to aortic atresia or stenosis, mitral atresia or stenosis, or both, which makes it incapable of supporting the systemic circulation [1]. Some patients in whom the left heart is unable to support the systemic circulation are probably also found in the

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