Elsevier

The Annals of Thoracic Surgery

Volume 78, Issue 6, December 2004, Pages 1994-1998
The Annals of Thoracic Surgery

Original Article
Cardiovascular
Interim Mortality in Pulmonary Atresia With Intact Ventricular Septum

https://doi.org/10.1016/j.athoracsur.2004.06.015Get rights and content

Background

Recent work has focused attention on interim mortality (death after hospital discharge and before second-stage surgery) in hypoplastic left heart syndrome. This study investigates interim mortality in infants undergoing systemic-to-pulmonary artery shunts for pulmonary atresia with intact ventricular septum.

Methods

At two centers in 11 years (January 1991 through December 2001), 35 infants underwent placement of shunts for palliation of pulmonary atresia with intact septum. Patients were identified from the cardiology database at each institution, and data were collected retrospectively. The infants were classified into two groups, with and without severe right ventricular hypoplasia, based on the initial surgical plan (Fontan or two-ventricle repair).

Results

The mean age and weight of the infants were 9 days and 3.1 kg. The right ventricle was severely hypoplastic in 22 of 35 infants. Hospital death occurred in 2 patients (9.1%), 1 with severe right ventricular hypoplasia. The remaining 33 patients form the study population. There were a total of 5 deaths (15%) after discharge and before second-stage operation, all in patients with severe right ventricular hypoplasia. Two patients, 1 with hypoplastic right ventricle, died after second-stage operation.

Conclusions

These data confirm a significant incidence of interim death in infants with pulmonary atresia and hypoplastic right ventricle. The interim mortality rate in the current two-institution study of infants with pulmonary atresia with intact ventricular septum is similar to that in hypoplastic left heart syndrome if all patients are considered (15%), and is somewhat higher (24%) than that for hypoplastic left heart syndrome if only patients with severe right ventricular hypoplasia are considered. This rate of interim death must be considered when different treatment options (such as shunt or transplant) are contemplated.

Section snippets

Patients and Methods

Between January 1991 and December 2001, at two centers, a total of 35 infants 60 days of age or younger underwent placement of systemic-to-pulmonary artery shunts as a source of pulmonary blood flow in patients undergoing palliative management of PAIVS. The period for the study was selected to allow evaluation of at least midterm outcome in all patients. Patients with PAIVS who had pulmonary valvotomy alone (no shunt), either in the cardiac catheterization laboratory or in the operating room,

Results

There were 22 patients with severe RV hypoplasia (61%), and 14 with two ventricles. One of the patients with two ventricles ultimately underwent a one-and-one-half ventricle repair. Twenty-two patients (including 6 without severe RV hypoplasia) had identifiable sinusoids or right-ventricle-to-coronary-artery fistulas. Only 1 patient was identified with RV-dependent coronary circulation. Two patients had Ebstein's malformation of the tricuspid valve. Four infants were premature (<35 weeks), 2

Comment

Neonatal cardiac surgery has become almost commonplace throughout the developed world, and excellent early results of both reparative and palliative operations are now essentially expected. Hospital mortality rates are in the single digits for repair or palliation of most cardiac defects, and 30-day survival even for infants with the most complex defects such as HLHS is 70% to 90% in many centers [1, 3, 5]. As a result, our attention has recently been redirected to improving intermediate-term

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