Multiple Aneurysms of Abdominal Branches in Twins with Marfan's Syndrome / 日本心臓血管外科学会雑誌

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.

Extracardiac aneurysm of the right sinus of valsalva / 日本心臓血管外科学会雑誌

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.

A case report of a successful staged operation for a patinet with annuloaortic ectasia and debakey type II+IIIb aortic dissection associated with Marfan's syndrome / 日本心臓血管外科学会雑誌

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.