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Staged Correction of Pulmonary Atresia, Ventricular Septal Defect and Collateral Arteries
  • +3
  • Pieter van de Woestijne,
  • M. Mokhles,
  • Ingrid van Beynum,
  • Peter de Jong,
  • Jeroen Wilschut,
  • Ad Bogers
Pieter van de Woestijne
Erasmus MC

Corresponding Author:[email protected]

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M. Mokhles
Erasmus University Medical Center
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Ingrid van Beynum
Erasmus MC
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Peter de Jong
Erasmus MC
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Jeroen Wilschut
Erasmus MC
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Ad Bogers
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Objectives Pulmonary atresia (PA) with ventricular septal defect (VSD) and systemic-pulmonary collateral arteries (SPCA’s) has a variable anatomy with regard to the pulmonary vasculature, asking for an individualized surgical treatment. A protocol was applied consisting of staged unifocalization and correction. Methods Since 1989 39 consecutive patients were included (median age at first operation 13 months). In selected cases a central aorto-pulmonary shunt was performed as first procedure. Unifocalization procedures were performed through a lateral thoracotomy. Correction consisted of shunt takedown, VSD closure and interposition of an allograft between the right ventricle and the reconstructed pulmonary artery. Postoperatively and at follow up echocardiographic data were obtained. Results In 39 patients 66 unifocalization procedures were performed. Early mortality was 5%. Seven patients were considered not suitable for correction, four of them died. One patient is awaiting further correction. Correction was done successfully in 28 patients. Operative mortality was 3% and late mortality 11%. Median follow-up after correction was 19 years. Eleven patients needed homograft replacement. Freedom from conduit replacement was 88%, 73% and 60% at 5, 10 and 15 years respectively. Right ventricular function was reasonable or good in 75 % of the patients. Conclusions After complete unifocalization 30/37 patients (81%) were considered correctable. The main reasons for palliative treatment without correction were pulmonary hypertension and/or inadequate outgrowth of pulmonary arteries. Staged approach of PA, VSD and SPCA’s results in adequate correction and good functional capacity. RV function after correction remains reasonable or good in the majority of patients.
26 Sep 2021Submitted to Journal of Cardiac Surgery
27 Sep 2021Submission Checks Completed
27 Sep 2021Assigned to Editor
27 Sep 2021Reviewer(s) Assigned
11 Oct 2021Review(s) Completed, Editorial Evaluation Pending
11 Oct 2021Editorial Decision: Revise Major
25 Nov 20211st Revision Received
26 Nov 2021Submission Checks Completed
26 Nov 2021Assigned to Editor
26 Nov 2021Reviewer(s) Assigned
06 Dec 2021Review(s) Completed, Editorial Evaluation Pending
07 Dec 2021Editorial Decision: Revise Major
21 Dec 20212nd Revision Received
22 Dec 2021Submission Checks Completed
22 Dec 2021Assigned to Editor
22 Dec 2021Reviewer(s) Assigned
27 Dec 2021Review(s) Completed, Editorial Evaluation Pending
28 Dec 2021Editorial Decision: Accept
09 Feb 2022Published in Journal of Cardiac Surgery. 10.1111/jocs.16299