Original article
Cardiovascular
Natural History of Pulmonary Atresia With Intact Ventricular Septum and Right-Ventricle–Dependent Coronary Circulation Managed by the Single-Ventricle Approach

Presented at the Forty-first Annual Meeting of The Society of Thoracic Surgeons, Tampa, FL, Jan 24–26, 2005.
https://doi.org/10.1016/j.athoracsur.2005.11.041Get rights and content

Background

Long-term outcome of patients with pulmonary valvar atresia and intact ventricular septum with right-ventricle–dependent coronary circulation (PA/IVS-RVDCC) managed by staged palliation directed toward Fontan circulation is unknown, but should serve as a basis for comparison with management protocols that include initial systemic-to-pulmonary artery shunting followed by listing for cardiac transplantation.

Methods

Retrospective review of patients admitted to our institution with the diagnosis of PA/IVS-RVDCC from 1989 to 2004. All angiographic imaging studies, operative reports, and follow-up information were reviewed. Right-ventricle–dependent coronary circulation was defined as situations in which ventriculocoronary fistulae with proximal coronary stenosis or atresia were present, putting significant left ventricle myocardium at risk for ischemia with right ventricle decompression.

Results

Thirty-two patients were identified with PA/IVS-RVDCC. All underwent initial palliation with modified Blalock-Taussig shunt (BTS). Median tricuspid valve z-score was -3.62 (-2.42 to -5.15), and all had moderate (n = 13) or severe (n = 19) right ventricular hypoplasia. Median follow-up was 5.1 years (9 months to 14.8 years). Overall mortality was 18.8% (6 of 32), with all deaths occurring within 3 months of BTS. Aortocoronary atresia was associated with 100% mortality (3 of 3). Of the survivors (n = 26), 19 have undergone Fontan operation whereas 7, having undergone bidirectional Glenn shunt, currently await Fontan. Actuarial survival by the Kaplan-Meier method for all patients was 81.3% at 5, 10, and 15 years, whereas mean survival was 12.1 years (95% confidence interval: 10.04 to 14.05). No late mortality occurred among those surviving beyond 3 months of age.

Conclusions

In patients with PA/IVS-RVDCC, early mortality appears related to coronary ischemia at the time of BTS. Single-ventricle palliation yields excellent long-term survival and should be the preferred management strategy for these patients. Those with aortocoronary atresia have a particularly poor prognosis and should undergo cardiac transplantation.

Section snippets

Patient Selection

Using the Cardiovascular Program’s database at Children’s Hospital Boston, after approval of this study by the Institutional Review Board, we identified all patients who were admitted to our institution from January 1, 1989, until December 31, 2004, with either a new diagnosis of PA/IVS-RVDCC or who were referred from an outside institution with an established diagnosis of PA/IVS-RVDCC having already undergone modified Blalock-Taussig shunt (BTS) or bidirectional Glenn (BDG) shunt following

Results

There were 32 patients admitted to our institution with PA/IVS-RVDCC, including 10 patients referred from outside institutions. Five patients were referred for BDG after BTS, 3 had already completed BDG and were referred for Fontan completion, and 2 patients who had new-onset LV dysfunction after BTS (at 11 and 82 days, respectively) were referred for transplant evaluation.

Patient characteristics are depicted in Table 1. In this cohort, median age at BTS was 2 days (range, 0 to 38), and median

Comment

Pulmonary atresia and intact ventricular septum is an uncommon congenital cardiac lesion that accounts for 3% of serious congenital heart defects, and the subset with RVDCC is even less common [22]. Whether the inciting developmental event occurs early or late in fetal life is unknown; however, it has been postulated that right ventricular outflow tract obstruction later during fetal life results in underdevelopment of the pulmonary valve, leading to a spectrum of associated abnormalities

References (36)

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