Abstract
Pulmonary atresia with ventricular septal defect (severe form of tetralogy of Fallot) and major aortopulmonary collateral arteries is a rare (10 out of 100,000 live births) complex congenital heart disease with variable segmental pulmonary blood flow. The natural history of this defect is prognostically unfavorable: survival during the first year of life is no more than 20%. The ultimate goal of surgical treatment for this defect is to create completely separate, sequential pulmonary and systemic blood flows.
This paper presents a case of one-stage bilateral procedure of unifocalization of the major aortopulmonary collaterals with the creation of a central systemic-to-pulmonary anastomosis in a patient with conotruncal defect with pulmonary blood flow obstruction in the neonatal period. The article discusses the features of management of this defect, and substantiates the feasibility of an early unifocalization procedure.
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About the authors
- Elizaveta S. Dorokhina, Cardiovascular Surgeon; ORCID
- Aleksey E. Chernogrivov, Dr. Med. Sci., Chief Researcher, Cardiovascular Surgeon; ORCID
- Inessa E. Nefedova, Dr. Med. Sci., Head of Cardiac Surgery Department (Children’s) No. 1; ORCID
- Alina S. Sarkisyan, Pediatric Cardiologist; ORCID
