日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Patients with asplenia syndrome usually have various cardiac malformations. Although most of these patients are Fontan candidates, some cannot achieve Fontan completion because of pulmonary artery and vein problems. We report a case of asplenia syndrome with Fontan completion achieved after pulmonary venous obstruction (PVO) was repaired twice. The patient underwent her first operation (repair of total anomalous venous connection, modified Blalock–Taussig [BT] shunt, patent ductus arteriosus division, and pulmonary artery plasty) at 8 days of age (body weight: 2,097 g). As the pulmonary venous orifice was obstructed postoperatively, we performed surgical PVO release, but left PVO occurred again. We created a left modified BT shunt to increase pulmonary flow to the left pulmonary artery and preserve pulmonary vein flow, and performed consecutive PVO release . Subsequently, Fontan completion was achieved through a bidirectional Glenn operation with additional flow from the systemic to the pulmonary artery. Planned adjustment of pulmonary blood flow can be an effective way to prevent progression of PVO and promote pulmonary artery growth.