Management of Pulmonary Arterial Supply Dependent on a Coronary Arterial Fistula in a Patient With Tetralogy of Fallot With Pulmonary Atresia

Abstract

In this report, we describe the surgical management of a patient with tetralogy of Fallot and pulmonary atresia, in whom a fistula from the anterior interventricular coronary artery was the predominant source of arterial supply to both the lungs.

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

“…[1][2][3][15][16][17] We are aware of less than 40 patients reported in the English medical literature in whom fistulous communication from the coronary arteries have provided the predominant or a significant source of pulmonary arterial supply. [7][8][9]12,18 In physiological and hemodynamic terms, fistulous communications from the coronary arteries are more likely to supply through the arterial duct than the more usual systemic-to-pulmonary collateral arteries. The fistulous communications typically supply the entirety of intrapericardial pulmonary arterial bed, feeding the arteries in antegrade fashion, as does the arterial duct.…”

“…Among the cases we reviewed, three-quarters had the fistula arising from the left coronary artery. [7][8][9]12,18 In a few cases, the fistula was described as arising from a solitary coronary artery, most commonly the left, which then supplied both coronary arterial systems. 7,9 In most cases involving the left coronary arterial system, the fistula arose from the anterior interventricular artery, followed by the main stem of the left coronary artery, and less frequently from the circumflex artery.…”

“…Subsequently, there have been several accounts of fistulous communications as the predominant source of pulmonary arterial supply. [7][8][9][10][11][12][13] Dabizzi and colleagues, 4 perhaps surprisingly, argued that one-tenth of their large series of patients studied angiocardiographically had such fistulous communications from the coronary arteries, but all the identified fistulas were small. The presence of such collateral channels nonetheless has important clinical and surgical implications.…”

Tetralogy of Fallot with coronary artery to pulmonary artery fistula

“…The prenatal diagnosis of ventriculo‐coronary artery fistula in the setting of pulmonary atresia with intact ventricular septum has also been made previously . In the literature, coronary–pulmonary artery fistulas have been reported in association with TOF and pulmonary atresia in 10%, and the majority of them have a fistula originating from the left coronary artery with >50% also having systemic‐to‐pulmonary collateral arteries (major aortopulmonary collateral arteries) …”

“…5 In the literature, coronary-pulmonary artery fistulas have been reported in association with TOF and pulmonary atresia in 10%, and the majority of them have a fistula originating from the left coronary artery with >50% also having systemic-to-pulmonary collateral arteries (major aortopulmonary collateral arteries). 6 Only one report described the prenatal diagnosis of a large fistula from the left coronary artery to the left atrial appendage in TOF, with congestive cardiac failure postnatally and death after surgical correction of TOF with ligation of the fistula at 3 months of age. 7 We did not find the association of TOF, coronary-pulmonary artery fistulas, and agenesis of the ductus arteriosus as described in the present case.…”