Coronary‐pulmonary arterial fistula in a neonate with pulmonary atresia—ventricular septal defect and single coronary artery

Abstract

In cases of pulmonary atresia with ventricular septal defect (PA-VSD), coronary-pulmonary arterial fistula (CPAF) as the main source of pulmonary blood supply is extremely rare. These fistulae may arise from the left coronary artery, right coronary artery, or a single coronary artery. Fistulae from a single coronary artery are unusual. We are reporting a case of PA-VSD with single coronary artery and CPAF as the main source of pulmonary supply in addition to two major aortopulmonary collateral arteries (MAPCAS). Successful surgical correction with VSD closure and right ventricle (RV) to the pulmonary artery (PA) conduit was made.

Collateral or fistula? Coronary artery as the primary source of pulmonary blood flow in a patient with pulmonary atresia and ventricular septal defect

“…The CPAF involved the LMCA in 34 cases, RCA in 12 cases, a single coronary artery in 5 cases, and both coronary arteries in 2 cases. [8]…”

“…In this setting, whether such a communication should be referred to as a CAPA or fistula can be put up for debate. CAPA nomenclature is supported by the fact that no clinical evidence of coronary steal is found in the majority of patients with PA/VSD and coronary–pulmonary “fistula.”[89]…”

Utilisation of a three-dimensional printed model for the management of coronary-pulmonary artery fistula from left main coronary artery

“…The origin of the coronarypulmonary artery fistula was the left coronary artery in the majority of reported cases; however, they can also arise from either the right or from a single coronary artery. 1 The presence of a coronary-pulmonary artery fistula is often suspected by transthoracic echocardiography. This diagnosis usually needs to be confirmed by cardiac catheterisation and cross-sectional imaging, which helps to better delineate the complex anatomy for surgical planning purposes.…”

“…The incidence of coronary-pulmonary fistulas in pulmonary atresia with ventricular septal defect is reported to be as high as 10%. 1 Patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries can have very intricate anatomy. Unifocalisation of major aortopulmonary collateral arteries is often required as part of the initial surgical palliation and necessitates a complete understanding of the pulmonary blood supply.…”

“…Unifocalisation of major aortopulmonary collateral arteries is often required as part of the initial surgical palliation and necessitates a complete understanding of the pulmonary blood supply. 1 Pre-surgical planning often depends upon cardiac catheterisation and cross-sectional imaging in these cases. Recently, threedimensional printed models were reported to be useful for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.…”