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.

Marfan's syndrome associated with multiple arterial aneurysms of the abodominal aortic branches in a pair of identical twins is described. The first case, a 21-year-old male, visited our affiliated hospital because of severe upper abdominal pain. Computed tomography (CT) and aortography (AOG) demonstrated multiple abdominal aneurysms, i.e., celiac artery, bilateral renal arteries and superior mesenteric artery, but there were no findings of dissecting aneurysm of the aorta. The aneurysm of the superior mesenteric artery, which had ruptured, was replaced with a greater saphenous vein graft. The postoperative course was uneventful until sudden cardiac arrest occurred on the 5th POD. An autopsy revealed a dissecting aortic aneurysm (DeBakey type I), massive intrathoracic hemorrhage, and subarachnoidal hemorrhage. Two years later, the second case, the 23-year-old twin brother of the prior case, visited our hospital because of upper abdominal pain similar to that of his brother. The clinical course bore a close resemblance to his brother's. CT and AOG revealed the intact aorta and aneurysms of the celiac artery and right renal artery. The ruptured aneurysm of the celiac artery was ligated. Immediately after his returning to the ICU, he developed sudden massive intrathoracic bleeding and cardiac arrest. This is the fourth report of identical twins with Marfan's syndrome in Japan. It is also noteworthy that in both cases rupture of the extra-aortic aneurysm occurred prior to the aortic dissection or aneurysmal formation.

The patient is a 47-year-old male who presented with abnormal shadows in his chest X-ray. On the third intercostal space, diastolic regurgitant murmur and systolic ejection murmur were heard. X-ray of the chest showed a projection of the right second costal arch in addition to the right atrium shadow. Cardiac catheterization showed no abnormalities except for a rise in the left ventricular end diastolic pressure which was 18mmHg. The patient was found to have Grade II aortic regurgitation. All there findings diagnosis of the case as extra-cardiac right Valsalva sinus aneurysm with aortic regurgitation. Incision of the aneurysm, showed a Valsalva sinus aneurysm having an opening of approximately 3cm just above the right aortic valve ring with the orifice of the right coronary artery occluded. Complete patch closure was performed with elevation of the aortic valve ring. No reconstruction for the right coronary artery was made.

Between February 1999 and November 1999, 33 patients (age 67.0±7.6 years old) underwent off-pump CABG using coronary shunt tubes. The number of graft anastomoses per patient was 2.8±0.8. The operative mortality was 0%. There was no incidence of on-pump conversion, low cardiac output syndrome, IABP insertion, mediastinitis or stroke. The maximum CPK-MB during the perioperative period was 25.9±18.8IU/l. One patient had perioperative myocardial infarction probably due to native coronary artery spasm. In patients with off-pump CABG, the intubation time, the ICU stay and the hospital stay were shorter. The number of patients who were extubated in the operating room was higher and the cost was lower than those with on-pump CABG. An early phase study revealed patency ratios of 85% (the previous term) and 97% (the latter term). Off-pump CABG is a safe and effective means of revascularization with no mortality, minimal morbidity and good short-term patency.

A 2-month-old boy developed Methicillin-resistant Staphylococcus aureus (MRSA) mediastinitis after bidirectional Glenn anastomosis for hypoplastic left heart syndrome. After reexploration, only the skin was closed but the sternum left open, and continuous mediastinal irrigation using saline containing isodine was commenced at an infusion rate of 20-40ml/h. The sternum was closed on day 7 and irrigation was stopped on day 21. The patient was weaned from the ventilator 4 days later, and is currently in a good condition awaiting a Fontan operation.