Kawashima Procedure After Staged Unifocalizations in Asplenia With Major Aortopulmonary Collateral Arteries

Abstract

We report a Kawashima procedure (total cavopulmonary shunt) successfully carried out for asplenia syndrome, pulmonary atresia, and major aortopulmonary collateral arteries. At the age of 8, the patient underwent staged bilateral unifocalizations using confluent central pulmonary arteries concomitant with bilateral modified Blalock-Taussig shunts. As the result of an interrupted inferior vena cava with azygous continuation, the patient required a Kawashima procedure with augmentation of the central pulmonary arteries for definitive palliation 1 year later. Cyanosis, respiratory distress, and ventricular function improved.

Major Aortopulmonary Collateral Arteries With Anatomy Other Than Pulmonary Atresia/Ventricular Septal Defect

“…It is not surprising that there is a limited amount of literature on this subject, because atypical anatomy represents a small fraction of an already rare condition of MAPCAs. The few reports on patients with MAPCAs and atypical anatomy would suggest that they may have a wide array of intracardiac anatomy, including many patients with complex single ventricle [9][10][11][12][13] or total anomalous pulmonary venous connection (TAPVC) [14]. These prior reports have focused on specific subsets of patients with atypical anatomy and MAPCAs; therefore, this subset is certainly not representative of the group as a whole.…”