Coronary-pulmonary artery fistula in tetralogy of Fallot with pulmonary atresia

Abstract

Surgical correction of patients with tetralogy of Fallot with pulmonary atresia is now one of the routine procedures performed by pediatric cardiac surgeons. In one variant, the pulmonary arterial supply is derived from a fistulous communication from the coronary arteries. This rare and interesting situation poses a diagnostic and therapeutic dilemma, as well as providing specific management challenges to the surgical team. Here, we discuss important aspects of this rare variant, specifically its morphology, presentation, evaluation and management.

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

“…Surgical repair for these patients varies according to the size of the PA branches, the size of the fistula, the presence or absence of MAPCAs, and their size. Repair may be single‐stage or 2‐stage repair . Amin et al .…”

“…Repair may be single-stage or 2-stage repair. 11 Amin et al 3 ORCID Wael Attia http://orcid.org/0000-0001-5822-6481…”

Pulmonary artery from the left main coronary artery

“…In contrast to MAPCAs which usually connect to branch pulmonary artery at the hilum or inside lung parenchyma, CPAF is intrapericardial and usually connected to the main pulmonary trunk through a tortuous channel. [1] To the best of our knowledge, only 52 cases of CPAF have been reported in the literature. However, in a case series of 87 patients with VSD with PA, Amin et al reported an incidence of 10% (9 patients) and suggested commoner occurrence and emphasized the importance of routine ascending aortogram to avoid missing the diagnosis.…”

“…[3] Typically, the fistulous connection is restrictive, and there is no significant elevation of the pulmonary pressures. [1] Surgical management entails clipping the fistula along with the usual intracardiac repair with or without a conduit.…”

Bilateral Coronary-Pulmonary Artery Fistulas in Pulmonary Atresia With Ventricular Septal Defect

“…2) A coronary-pulmonary artery fistula arises more commonly from the left coronary artery, especially the left circumflex artery, than from the right coronary artery, and occasionally arises from a solitary coronary system. 3) Coronarypulmonary artery fistulas associated with PA/VSD usually coexist with major aortopulmonary collaterals. Two reports have described the coexistence of a coronary-pulmonary artery fistula and a nonconfluent left pulmonary artery connected through a patent ductus arteriosus, and both cases were similar to the present case.…”