Concomitant Repair of Annuloaortic Ectasia, Aortic Regurgitation, and Pectus Excavatum in Marfan Syndrome / 日本心臓血管外科学会雑誌

Marfan syndrome may include cardiovascular disease co-exising with thoracic deformities. A 24 year-old man given a diagnosis of Marfan syndrome and annuloaortic ectasia (AAE), aortic regurgitation (Ar) and pectus excavatum, was referred to our hospital due to the rapid dilatation of a root aneurysm. Chest computed tomography showed a root aneurysm measuring about 60×55 mm in diameter with mild Ar. Moreover, the sternum, which had been displaced in a posterior direction, contacted with the root aneurysm and heart. The heart was deviated to the left, because of compression from the sternum. We performed a concomitant repair of AAE and Ar and pectus excavatum with partial sternal turnover and elevation, and Bentall procedure. First, median skin incision was made, and dissected to the sternum. The ribs and cartilage below the third rib were cut, and the sternum was transected at the two-thirds point. The root aneurysm and heart were visible so it was easier to operate than a post median sternotomy. A cardiopulmonary bypass was established by ascending aortic perfusion, right atrial drainage and pulmonary arterial venting. The Bentall procedure was done using a Carrel patch methods. The removed sternum was formed flat and turned over, and sternum elevation was perfomed using sternal wire, after cutting and removing the excess ribs and costal cartilage. The postoperative course was uneventful with good hemodynamic and respiratory function. Concomitant surgery provides good operative exposure, which can avoid accidental aneurysm laceration, although operation time is longer and operative invasion and bleeding are greater than in staged operations.

A Case of Total Anomalous Pulmonary Venous Connection with Two Vertical Veins Draining to the Infracardiac Level / 日本心臓血管外科学会雑誌

Infracardiac type total anomalous pulmonary venous connection (TAPVC) was diagnosed in a 1-day-old boy. We performed emergency total correction on day 1 and found 2 vertical veins draining to the infracardiac level separately. Each vertical vein was rerouted to the left atrium. On the first postoperative day, an extracorporeal membrane oxygenation was required because of respiratory failure. He died due to cerebral hemorrhage on the 5th day after the operation. Macroscopic findings showed the right sided vertical vein draining to the IVC, and the left sided one to the confluence of the hepatic vein and ductus venosus. Microscopic findings of the lung revealed markedly dilated lymphatics which was suspected as the cause of respiratory failure. Although cases with 2 separate vertical veins are very rare, the precise anatomy of PV return has to be checked intraoperatively when the preoperative identification has not been established.

Two Cases of Adventitial Inversion Technique for Stanford Type A Acute Dissecting Aortic Aneurysm / 日本心臓血管外科学会雑誌

We treated two cases of Stanford type A acute dissecting aortic aneurysm with the adventitial inversion technique. Both case 1) a 65-year-old woman and 2) a 74-year-old woman underwent emergency operation. After cardiopulmonary bypass was established as usual, the diseased aorta was resected, and the intima was trimmed about 10mm shorter than the transected adventitial line in both proximal and distal ends. After GRF glue was employed, the adventitia was inverted inward over the false-lumen, and then tacked with horizontal continuous mattress sutures using 5-0 polypropylene. The graft was then anastomosed with continuous sutures using 3-0 polypropylene. No bleeding occurred from the anastomosis site in both cases. This method was completed without the use of artificial reinforcement, nevertheless patent anastomosis was possible. This simple method was easily performed and proved to be safe and useful.