Total correction for a chronic aortic dissection, producing progressive enlargement of the false lumen of the aorta involving wide range of aorta and aortic manifestation of Marfan's syndrome is a very difficult procedure. However, with the recent development in surgical techniques and management, it became possible to replace total or subtotal aorta with the prosthetic graft. Recently, we treated a 24 y/o male patient with annuloaortic ectasia, DeBakey type II+IIIb aortic dissection, and obstruction of right common iliac artery, associated with Marfan's syndrome with a two-staged operation. For the first stage, we performed Cabrol's procedure on his lesions in ascending aorta. About 2 years after that, for the second stage, replacement of total descending and abdominal aorta was pertformed.

A 67-year-old man was hospitalized due to congestive heart failure. The blood pressure was 180/72mmHg in the right arm and 100/70mmHg in the right leg. Aortography revealed that the aortic arch was completely interrupted just distal to the origin of the left subclavian artery and the descending aorta was clearly outlined by contrast medium coming from well-developed collateral vessels. There was no congenital anomaly such as PDA or VSD except for the interruption of the aortic arch. The final diagnosis was solitary interruption of the aortic arch (type A). An extra-anatomical bypass using a 16mm woven Dacron graft was placed from the ascending aorta to the descending aorta at the level of the diaphragm with cardiopulmonary bypass. The pressure gradient across the interruption disappeared immediately after opening the graft. Although he had perioperative myocardial infarction in the area of the right coronary artery, he recovered fully and when discharged he was in NYHA class 1 condition.

Sixteen patients with congenitally corrected transposition of the great arteries (CTGA) underwent operative closure of ventricular septal defects (VSD). Ages of the patients ranged from 10 months to 25 years. Three different approaches were employed to access to the defect: through right ventriculotomy 3, through left ventriculotomy 5, and de Leval's maneuver 8. Here, right or left ventricle refers to its anatomic morphology. Early postoperative death occurred in a patient who concomitantly underwent extracardiac couduit repair between left ventricle and pulmonary trunk. Late death occourred in 5 (left ventriculotomy in 1 and righ ventriculotomy in 4), among whom 2 expired suddenly of unknown cause (one in each of the right and left ventriculotomy), and 1 expired of pneumonia. Two other deaths were related to their reoperations for replacement of the incompetent left atrioventricular (AV) valve. Another patient who had been repaired by de Leval's maneuver also underwent replacement of the left AV valve and survived. Two patients who had undergone left ventriculotomy developed com-plete heart block leading to implantation of permanent pacemaker. Postoperative complete heart block was temporarily noted in a patient who had been repaired by de Leval's maneuver but returned to sinus rhythm on the 10th postoperative day. Late postoperative function of the systemic ventricle was assessed in 8 by gated radionuclide ventriculography. Calculated ejection fractions in each of the methods were the followings. Left ventriculotomy: 0.38, 0.47. Right ventriculotomy: 0.13. de Leval's maneuver: 0.29, 0.54, 0.66, 0.47, 0.36. These results draw us to the following conclusions that either ventriculotomy holds its drawbacks, that is, left ventriculotomy is apt to develop complete heart block and right ventriculotomy can predispose incompetent left AV valve ultimately leading to the fatal congestive heart failure. de Leval's maneuver, however, is rare to be complicated by the above morbidity and is considered to be the best operative method currently available.