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Pulmonary atresia with intact ventricular septum: Is it possible to improve survival?

Published online by Cambridge University Press:  19 August 2008

Carlo Vosa*
Affiliation:
From the Medical and Surgical Institute of Cardiology, 1st Medical School Pediatric Cardiac Surgery, Hospital “V. Monaldi”, Naples
Paolo Arciprete
Affiliation:
From the Medical and Surgical Institute of Cardiology, 1st Medical School Pediatric Cardiac Surgery, Hospital “V. Monaldi”, Naples
Giuseppe Caianiello
Affiliation:
From the Medical and Surgical Institute of Cardiology, 1st Medical School Pediatric Cardiac Surgery, Hospital “V. Monaldi”, Naples
Gaetano Palma
Affiliation:
From the Medical and Surgical Institute of Cardiology, 1st Medical School Pediatric Cardiac Surgery, Hospital “V. Monaldi”, Naples
*
Dr Carlo Vosa, Hospital “V. Monaldi”, Naples, Italy. Tel. 35-81-5462091.

Summary

Between February 1986 and December 1991, 41 patients with pulmonary atresia and intact ventricular septum were treated in our institution following a multistage protocol of management. In all cases, the first step was to construct a right modified Blalock-Taussig shunt during the neonatal period regardless of the right ventricular anatomy. Then, in patients with well-developed right ventricles possessing all three components, we proceeded to early surgical repair. In contrast, in patients with right ventricles having obliteration of some components, yet deemed to be recoverable, the next step was to provide palliative relief of obstruction in the right ventricular outflow tract followed, if possible, by subsequent repair. Fontan's operation was performed in patients with right ventricles considered unsuitable from the outset to support the pulmonary circulation. Only one patient died following the initial shunt procedure (mortality of 2.43%). The subsequent program of treatment has now been concluded in 22 patients. In all those deemed to have favorable native anatomy (10 cases), the subsequent complete repair was successful. Among the 24 patients who required palliation of the outflow tract, five died while total repair was subsequently performed in eight. Fontan's operation was performed without mortality in five patients with small right ventricles, although one patient died while waiting for surgery. In all, 89 procedures were performed with an overall mortality of 14%.

Type
World Forum for Pediatric Cardiology Symposium on Pulmonary Atresia
Copyright
Copyright © Cambridge University Press 1992

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References

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