A Double Connection of Mixed Total Anomalous Pulmonary Venous Connection : Successful Patch Closure of Residual Shunt / 日本心臓血管外科学会雑誌

Double drainage sites from a common venous confluence of the pulmonary veins of a mixed total anomalous pulmonary venous connection is a rare condition that is called a “double connection.” There have been very few reports of reoperation for minor drainage of a double connection. A 28-year-old male with double connection type (Ia: major drainage + IIa: minor drainage) mixed total anomalous pulmonary venous connection (TAPVC) was referred to our institution. He had undergone TAPVC type Ia (major drainage) repair at the age of 1 year. Postoperative enhanced computed tomography showed residual TAPVC IIa (minor drainage) at the age of 15 years. Therefore, the definitive diagnosis was double connection-type mixed TAPVC. Residual shunt gradually increased, and cardiac catheterization revealed an increased pulmonary blood flow/systemic blood flow ratio. Echocardiography showed enlarged shunt vessel and mild tricuspid regurgitation. Catheter intervention was considered too risky and, therefore, we performed patch closure of the residual shunt through a right atriotomy. The postoperative course was uneventful, and follow-up computed tomography showed shrinkage of the shunt vessel without any thromboembolic events.

A Case of Surgically Corrected-Combined form of Total Anomalous Pulmonary Venous Return

Total anomalous pulmonary venous return(connection) is a congenital heart disease. Especially, the reports of the patients with double connections of all pulmonary veins were rare. An infant who underwent operation for total anomalous pulmonary venous return had double drainge of all pulmonary veins to both left vertical vein and anomalous descending vein. This second lesion was not diagnosed preoperatively, but in operative field. Operative treatments were performed by ligation of left vertical vein and central pulmonary vein-to-left atrium anastomosis without ligation of the descending anomalous vein. For these less common variants fo total anomalous pulmonary venous return,a more detailed and precise understanding of morphology can facilitate accurate surgical repair. And also, early detection of the disease is an another issue for a good prognosis.