Successful management of severe life-threatening hypoxemia due to pulmonary arteriovenous malformation.

Abstract

AFTER cavopulmonary anastomosis, pulmonary arteriovenous malformations (PAVMs) may develop in nearly all pre-Fontan patients, but only about 20% are clinically important. 1 They are thought to be the persistence of arteriovenous communications of the fetal and neonatal pulmonary circulation. It is speculated that the lack of hepatic venous blood flowing through the lungs and, hence, the absence of some stimulating or suppressive factor, may contribute to this condition. 2 The PAVMs may lead to progressive desaturation (>5%) in the years after undergoing Glenn anastomosis. However, normal arterial oxygenation does not exclude the presence of persisting PAVMs. 1 The diagnosis is made by the detection of a rapid appearance of contrast dye in the pulmonary veins after injection into the pulmonary arteries or by contrast echocardiography. This is a report of successful administration of nitric oxide (NO) in a patient after Fontan repair with severe life-threatening hypoxemia due to preoperatively undetected PAVMs, thus avoiding extracorporeal membrane oxygenation (ECMO) support.