A case of quadricuspid aortic valve is reported. The anomaly was unexpectedly found during surgery for aortic valve replacement in a 33-year-old man with aortic insufficiency. The supernumerary cusp was positioned between the right and the left coronary cusps. These three cusps were fairly uniform, although the non-coronary cusp was smaller. The cusps were excised and replaced with a Björk-Shiley valve. Congenital quadricuspid aortic valve is a rare abnormality, and only 39 cases have been reported. Most patients developed aortic insufficiency after reaching adulthood and nine patients had aortography or echocardiography. We propose to group congenital quadricuspid aortic valve into two types. Type 1: the supernumerary cusp is located anteriorly, that is, between the right and the left coronary cusps. The present case belongs to this type. Type 2: the supernumerary cusp is posterior and is situated between the right and the non-coronary cusps. In this type, the supernumerary cusp is always smaller than the other cusps.

Those cases in which a dissected lumen closes early in the onset of acute aortic dissection and produce a“dissected lumen with no blood flow”are regarded as a clinico-pathological entity and are called a“closing aortic dissection”, and the clinical picture and clinical course of 14 cases in which the clinical course could be observed from early onset were reported. Although 13 cases resulted in complete closure of the dissected lumen, one case initially showed incomplete closure, but subsequently closed completely. Two cases resulted in reopening of the blood flow, but the disease recurred, and by four and six weeks each had incompletely or completely reclosed. Consequently, there were three cases of entry observed and scars of entry were found in three other cases. And in eight cases, there was nothing observed at all. Although one patient died because of complications of secondary type I acute dissection, all the others survived. All told, the developmental mechanism of this disease was alluded to.

Two hundred fourteen cases treated surgically for aortic aneurysms between Jan. 1986 and Dec. 1991 at our hospital. Among them, 15 cases (7.0%) had multiple aortic aneurysms. In 10 cases, aneurysms were resected completely: 9 simultaneously (one-stage operation) and 1 separately (two-stage operation), although in 5 cases there remained another aneurysm left even after operation. In all cases who had one-stage operation, the combination of sites of aneurysms were descending thoracic aorta and infra-renal abdominal aorta. Temporary bypass (n=4), centrifugal pump (n=4) or cardiopulmonary bypass (n=1) were employed as supportive methods during aortic cross-clamp. There were neither operative nor late death in one-stage operation group, although one patient died due to rupture residual aneurysm 1.2 years after the first operation. In these patients, vascular disease are expected to be present systemically, so that operative method should be determined carefully under consideration of poor general condition and another risk factors. We recommend, however, that simultaneous one-stage operation for multiple aortic aneurysm might be safe and fully acceptable procedure, especially in case of those whose aneurysms exist in descending thoracic and infra-renal abdominal aorta.

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.

We describe two cases of aortic regurgitation with dove-coo murmur that required aortic valve replacements. In the first case, there were three small perforations of the cusps, two in the noncoronary cusp and one in the right coronary cusp. The cause of the cusp perforations was unclear. In the second case, there were two perforations of the cusps, one in the left coronary cusp and another small one in the right coronary cusp, along with a subannular pseudoaneurysm. The cause of the cusp perforations was an infective endocarditis. Before 1955, aortic regurgitations with dove-coo murmur were mostly reported to be originated by retroversion of the valve cusps due to syphilis. After 1960, syphilis was replaced by various diseases that bring about perforations, tears and retroversions of the cusps. The condition which is concerned in the development of the dove-coo murmur is that the aortic valve cusps have lesions without calcification and preserve the flexibility.

The authors examined the frequency of thromboembolism and bleeding complications in cases of mechanical valve replacement during the past 5 years in the Tokyo area. There were 21 cases of thromboembolism and 15 cases of bleeding complications. Analyzing these cases with regard to anticoagulant therapy, 71% of the thromboembolism cases and 47% of the bleeding complication cases had 10∼25% result on the thrombotest at the time of the event. Consequently, in cases of mechanical valve replacement it is necessary to reevaluate the therapeutic range of the thrombotest results. This was a retrospective study of a TAS (The Tokyo area anticoagulation study for cardiac valve replacement by using PT-INR) trial and we intend to carry out a prospective study on the therapeutic range of the thrombotest and PT-INR.

Recently, the demand for better cosmetic outcomes in pediatric cardiovascular operations has been growing. Between May 1998 and April 1999, six children aged 2 to 6 years with an ostium secundum type of atrial septal defect underwent reparative operations that used an approach consisting of a lower mid-line skin incision with full sternotomy. A 4.2-5.8cm vertical skin incision (mean, 4.9±0.3cm) was made between the level of the nipple and the xyphoid process. Comparison between this series and a group of weight-matched patients who underwent conventional operations revealed no significant differences in operation time (166.0±12.0vs. 147±8.4min), cardiopulmonary bypass time (33.2±4.0vs. 32.2±2.4min), aortic cross-clamp time (13.8±2.3vs. 12.3±1.3min), or the reduction in the hemoglobin concentration in blood on the first postoperative day (1.7±0.3vs. 2.9±0.6g/dl). The surgical wound was not associated with any complications in our series, including wound infection or subcutaneous hematoma. Our technique appears to be safe and provide satisfactory cosmetic outcome.

Intercostal artery aneurysm is a rare disease, and is usually associated with aortic coarctation, trauma and infection. Until recently, diagnosis of the aneurysm had not been possible before rupture of aneurysm. However, recent advances in computed tomography (CT) and magnetic resonance imaging (MRI) have made it possible to diagnose this lesion. A 68-year-old man was admitted with an abnormal shadow on chest X-ray film. A chest CT scan showed an aneurysm beside the descending aorta, suggestive of intercostal artery aneurysm. Intraoperative inspection confirmed the diagnosis. The aneurysm was shown to be atherosclerotic in origin by postoperative histological examination.

Among 232 patients undergoing repair for abdominal aortic aneurysms (AAA) during a 10-year period, 5 (2.2%) patients had evidence of inflammatory abdominal aortic aneurysms (IAAA). We examined their clinical course, laboratory, operative and histopathological findings, and considered possible correlations between their diagnosis and surgical treatment. Among these cases of IAAA, 2 patients complained of severe abdominal or back pain. The differential diagnosis from rupture of AAA was difficult in these cases. They also showed inflammatory signs in laboratory, operative and histopathological findings. Ultrasonography, computed tomography and magnetic resonance imaging appear to offer reliable means for diagnosing IAAA; In particular we could recognize the“Inflammatory mantle”in 3 cases, which indicated IAAA. In the surgical treatment, the presence of IAAA necessitates certain modifications in the surgical approach, in order to avoid injuring the retroperitoneal and abdominal structures. We could observe post-operative change in the thickness of the aortic wall, which decreases with time without anti-inflammatory agents.

Here we present a long-term follow-up of 50 operative survivors, who underwent surgery between December 1975 and March 1994 for the placement of an extracardiac conduit. Twenty-six patients received conduits with various valves (VC group). The valves used were the Hancock valve in 9 patients, the St. Jude Medical valve in 5, and a valved roll made of equine pericardium in 10. Twenty-four patients received valveless Dacron conduits (NVC group). Another group of patients, also with discontinuity between the right ventricle and the pulmonary artery, who were operated on without the use of a conduit, is presented here for comparison (NCR group: 16 patients). The follow-up period for the NCR group was shorter than for the other groups. There were a total of 4 late deaths in the conduit groups, and none in the NCR group. Freedom from reoperation due to conduit stenosis was analyzed by the Kaplan-Meier method. In the VC group, freedom from reoperation at 5, 10, and 15 years, was 87.8%, 50.8%, and 31.2% respectively. In the NVC group, freedom from reoperation at 5, 10, and 15 years was 100%, 95.7%, and 60.4%. There were statistically significant differences between the values in these 2 groups. In the NCR group, only one patient (6.25%) underwent reoperation due to stenosis in the right ventricular outflow tract. Although the rate of freedom from reoperation was lower in the valveless conduit group than in the valved conduit group, the majority of patients who receive a conduit between the right ventricle and the pulmonary artery will eventually require reoperation. Avoiding the use of an extracardiac conduit, and creating continuity between the right ventricle and pulmonary artery with autologous tissue is a useful alternative and may reduce the need for reoperation.