Abstract
Pulmonary atresia with ventricular septum defect and major aorto-pulmonary collateral arteries (PA / VSD / MAPCAs) is a rare congenital heart disease, occurring at a frequency of 10 per 100,000 live births. One-stage repair is associated with an extremely high risk because of pulmonary artery system underdevelopment, and surgeons choose a multi-stage strategy correction. Its goal is to provide a minimum sufficient pulmonary blood flow, and pulmonary blood circulation preparation for radical correction, by phased growth – “rehabilitation” of the pulmonary artery system. There are various approaches to surgical correction of PA / VSD / MAPCAs and in each case they are unique. This article narrates the case of staged surgical treatment in patient with PA / VSD / MAPCAs, accompanied by a reduced ejection fraction of the right ventricle.
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About the authors
- Anastasiya V. Shcherbak, Cardiovascular Surgeon, ORCID
- Mikhail M. Zelenikin, Dr. Med. Sc., Professor, Head of Department, ORCID
- Andrey A. Svobodov, Dr. Med. Sc., Cardiovascular Surgeon, ORCID
- Denis A. Dibin, Anesthesiologist-Resuscitator, ORCID
