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

The Fontan procedure is predominantly performed in children with a functionally univentricular heart. It remains unclear as to whether the Fontan procedure can be applied for adult patients. Furthermore, the optimal conduit route remains unknown for adults with apicocaval juxtaposition. Adult Fontan candidates are likely to have dilated ventricles with poor cardiac function caused by long-term hypoxia and ventricular volume overload. Consequently, an ideal course from the inferior vena cava to the pulmonary artery should be created with caution to avoid the formation of kink or collapse of the conduit. A 48-year-old man with a double outlet right ventricle, single right ventricle, along with valvular and supravalvular pulmonary stenosis, presented with exertional dyspnea and palpitation. The patient showed situs inversus with the cardiac apex and inferior vena cava on the left side. Preoperative catheterization data showed low pulmonary vascular resistance and pulmonary artery pressure because of valvular, supravalvular, and bilateral pulmonary artery stenosis. Primary fenestrated total cavopulmonary connection (TCPC), concomitant with pulmonary artery plasty, was performed. The conduit was constructed via an intra-atrial route to avoid being compressed posteriorly by the dilated ventricle. The postoperative course was uneventful, and the patient was discharged 32 days after surgery. His exercise tolerance improved gradually thereafter. He returned to his previous job 3 months after surgery. Our case demonstrates that adult Fontan candidates whose preoperative conditions satisfy appropriate criteria are capable of undergoing primary TCPC. However, surgical indications for adult TCPC should be carefully determined. In our present case, the intra-atrial conduit route was useful for an adult patient with apicocaval juxtaposition.