Aortic dissection with multiple familial members is rare. It is commonly associated with Marfan syndrome. Several authors have reported familial aortic dissection without Marfan syndrome. We encountered 4 cases of aortic dissection in a family. The aortic dissection occurred in the mother and all of her children. No case had clinical manifestations of Marfan syndrome or other connective tissue disease. Histopathological examination of the aorta did not show cystic medial necrosis in 2 operated cases. Many members in the family had systemic arterial hypertension. The presence of multiple incidence of aortic dissection in one family suggests underlying connective tissue disease, irrespective of the absence of typical features of Marfan syndrome. Therefore we propose that other close relatives as well as the members with aortic dissection should be followed-up in the same way used for families with typical connective tissue disease like Marfan syndrome.

An 80-year-old man felt a loss of strength and sharp pain in both lower limbs while playing gate-ball, consulted a nearby doctor, and was followed up. Because the sharp pains in both lower limbs became aggravated the next day, he was given a previously prescribed medication. Both femoral pulses were absent and acute arterial obstruction of the lower limbs was suspected. A contrast-enhanced CT scan showed a thrombosed infrarenal abdominal aortic aneurysm with a maximum transverse diameter of 37 mm, and both external iliac arteries were contrast imaged by collateral circulation pathways. We diagnosed acute thrombosis of an abdominal aortic aneurysm, and was urgently transported to our hospital. We classified his lower limbs as Balas grade III and TASC classification grade IIb and Rutherford classification grade IIb. He exhibited no abdominal symptoms and since we confirmed the blood flow of his lower limbs, we decided to perform revascularization. An extra-anatomical bypass (axillo-bifemoral bypass) was conducted because he had dementia, and was old. After the operation, myonephropathic metabolic syndrome (MNMS) did not develop, and the patient was discharged on foot on the 16th postoperative day. Acute thrombosis of an abdominal aortic aneurysm is a rare disease. Because the ischemic area widens, often causing serious MNMS after the revascularization, it has a poor prognosis. Here, we report a case in which one such patient was rescued.

A 71-year-old man had sudden onset of left lower limb swelling and consulted an orthopedic surgeon 14 days later. Venous echography demonstrated compression of the left iliac vein and the thrombus of the common iliac vein. After emergency admission, conservative therapy was given for 7 days, but the symptoms did not sufficiently diminish and a thrombus was also present. We therefore performed femoro-iliac cross-over vein bypass using a 10mm ringed ePTFE graft. Symptoms were completely improved and the graft was shown to be patent by echography after 3 months.

Causative factors for thrombi formation in left atria of 38 patients with mitral stenosis who underwent mitral valve surgery (open mitral commissurotomy or mitral valve replacement) alone or in combination with other procedures were studied. There were 9 cases of left atrial thrombosis (LAT). Left atrial diameter was increased in LAT(+) group (6.1±1.6cm) compared with LAT(-) group (4.6±0.7cm). There was significant difference in the left atrial diameter between the two groups of patients (p<0.01). Cardiac output was decreased in LAT(+) group (3.04±0.74l/min) compared with LAT(-) group (3.99±1.07l/min). Cardiac output of LAT (-) group was significantly larger than that of LAT(+) group (p<0.05). Mean transition time of blood through left atrium (MTT_LA) was calculated using left atrial volume and cardiac output. In LAT (+) group, MTT_LA was significantly increased (6.2±3.9sec) compared with LAT(-) group (2.9±1.6sec). It is considered that, in mitral stenosis, prolongation of MTT_LA is one of the risk factors for thrombi formation in the left atrium.

Cardiac surgery associated with idiopathic thrombocytopenic purpura (ITP) is rare, and only 10 cases have been reported in the literature. In this report, we described the successful surgical management of a patient with ITP, diabetes mellitus and malfunction of mitral bioprosthetic valve. A 62-year-old male, who underwent mitral valve replacement (MVR) by means of a Carpentier-Edwards valve prosthesis and CABG ten years ago, developed malfunction of mitral prosthetic valve. The preoperative platelet count was 52, 000/mm³ and PA-IgG elevated markedly. The diagnosis of ITP was based on findings of bone marrow examinations. Thrombocytopenia was treated by steroids for 4 weeks and large dose γ-globulin (20g/day) for 5 days preoperatively, but platelet count did not increase. Platelet rich plasma (PRP) was transfused prior to cardiopulmonary bypass (CPB) and fresh blood was added to the priming material of CPB. Re-MVR was performed by means of mechanical valve prosthesis. After operation, large doses of γ-globulin and transfusion of PRP were performed for 3 days, and the postoperative course was uneventful. Other reports in addition to this study reveal that cases of cardiac surgery associated with ITP should be initially controlled preoperatively with steroids or high-dose γ-globulin, and if these treatments are harmful or ineffective, splenectomy should be considered.

Fifty-five adult patients with atrial septal defect (ASD) were surgically treated. In the preoperative study, 6 patients showed high pulmonary artery systolic pressure (>50mmHg). However, there was no linear relation between PAP and age, nor between Q_p/Q_s and PAP. As for the additional surgical procedures, MVR (1), MAP (1), TAP (3), OPC (2) were carried out with ASD closure in 7 patients. Post-operative evaluation with echocardiography revealed increase in the left ventricular chamber size, decrease in the severity of tricuspid regurgitation and same grade mitral regurgitation compaired with pre-operative level. From these data, the prediction of the atrioventricular valve regurgitation after ASD closure seemed to be difficult just from the preoperative evaluation, Transesophageal echocardiography was useful for the evaluation of residual atrioventricular valve regurgitation during operation in the cases of ASD with over II grade regurgitation preoperatively.

A thirteen-day-old neonate was admitted because of systolic heart murmur, tachycardia, tachypnea and sucking weakness. The chest X-ray film demonstrated remarkable cardiomegaly and pulmonary congestion. Echocardiography detected marked thickening and stenosis of the aortic valve, and left ventricular dysfunction (EF=10%). The pressure gradient between left ventricle and ascending aorta was presumed 130mmHg with pulsed Doppler echocardiography, Since he did not respond to conservative treatment, an emergency open aortic valvular commissurotomy under cardiopulmonary bypass was performed the day after admission. We made incisions of 1mm in the left side and 0.5mm in the right side commissure of the adherent bicuspid aortic valve. After the procedure, left ventricular function improved (EF=57%), and the pressure gradient was reduced to 62mmHg. He showed good recover from the congestive heart failure. There are few reports about operative treatment of congenital aortic valve stenosis in neonates. This is considered to be the third youngest successful operative case of open aortic valvular commissurotomy in Japan.

Prostaglandin E₁ (PGE₁) was used continuously in adults from immediately after induction of anesthesia, during extracorporeal circulation, to the acute phase after open heart surgery. Using blood flow in the toe determined by laser Doppler flowmeter and the temperature difference between periphery and core as indices, the effects of afterload reduction and improvement of peripheral circulation were investigated. Subjects were 17 adults who underwent open heart surgery. PGE₁ was used in 7 patients and not used in 10. In the group using PGE₁, continuous injection of 0.015μg/kg/min of PGE₁ was started immediately after induction of anesthesia and was maintained during extracorporeal circulation until the acute phase after surgery. During extracorporeal circulation, perfusion pressure was kept at 50∼60mmHg and PGE₁ injection was controlled within the range of 0.015∼0.030μg/kg/min. At completion of extracorporeal circulation, the dose was fixed at 0.015μg/kg/min again. The degree of improvement of peripheral circulation was evaluated on the basis of hemodynamics, blood flow in the toe determined by laser Doppler flowmeter and the temperature difference between periphery and core, at induction of anesthesia (before using PGE₁) on completion of extracorporeal circulation, and in the acute phase after surgery. The value of blood flow in the toe determined by laser Doppler flowmeter was significantly higher in the PGE₁ group than in the non-PGE₁ group, from completion of extracorporeal circulation to the acute phase after surgery. Moreover, peripheral temperature was significantly higher in the PGE₁ group than in the non-PGE₁ group at completion of the extracorporeal circulation as well as immediately after surgery, and the temperature difference between periphery and core was significantly smaller. Continuous injection of PGE₁ enabled smooth control of perfusion pressure during extracorporeal circulation. Although there was no significant difference in peripheral vascular and total pulmonary resistance, the coefficients tended to be lower in the PGE₁ group. The use of PGE₁ during open heart surgery seems to be an effective method to improve peripheral circulation.

Ischemic heart disease (IHD) poses a major complicating factor for abdominal aortic aneurysm (AAA) repair. To identify patients with IHD, we evaluated patients scheduled to undergo AAA repair with dipyridamole-thallium scintigraphy (DTS) and coronary angiography (CAG). If indicated, coronary revascularization was performed. Finally, an assessment of the effectiveness of these preventive measures was made. One hundred and ten patients scheduled to undergo AAA repair were identified and treated accordingly over a 20-year period. As the pre-operative evaluation and prophylactic surgical revascularization strategies were instituted in 1983, the patients were divided into 2 groups: 25 patients between 1973-1982 (group A) and 85 patients between 1983-1992 (group B). The mean age of patients in group A was 65.3 years. The male/female ratio within this group was 21:4. One patient in the group had a history of IHD and 9 had hypertention. The mean age of patients in group B was 67.7 years. The male/female ratio within this group was 77:8. Fourteen patients in this group had a history of IHD and 27 had hypertension. Screening and treatment of IHD in group B was as follows. All patients with a history of IHD underwent CAG. Of the 32 patients with cardiac risk factors, including hypertension and hyperlipidemia, or ECG abnormalities who underwent DTS, 8 were referred for CAG. Thirty-nine patients with no risk factors and a normal ECG proceeded to AAA repair without further workup. Perioperative myocardial infarction occurred in 2 patients in grouzp A, leading to death in 1 patient. Coronary revascularization was performed in 5 patients in group B. No perioperative myocardial infarction occurred in this group. Pre-operative identification of high-risk cases with DTS, CAG, and coronary revascularization in patients with IHD may prevent cardiovascular complications in patients undergoing AAA repair.

A 62-year-old man was referred to our hospital because of dyspnea. Electrocardiogram showed chronic atrial fibrillation and echocardiogram revealed severe tricuspid regurgitation. His history included a motorbike accident at age 17, and a heart murmur was pointed out in the following year. He developed paroxysmal atrial fibrillation when he was 45 years old. Heart failure was not controlled by medication and tricuspid valve repair was indicated. At surgery, the anterior leaflet of tricuspid valve was widely prolapsed due to chordal rupture. We performed chordal reconstruction with 4 expanded polytetrafluoroethylene (CV-5®) sutures, and ring annuloplasty. Furthermore, a small fenestration at the tricuspid annulus was noticed and was closed with a direct suture. The biatrial modified Maze procedure was performed subsequently. The patient is doing well without TR recurrence, and restored sinus rhythm is maintained. We report successful repair of traumatic tricuspid regurgitation.