Familial Aortic Dissection: A Report of Four Cases in Two Families / 日本心臓血管外科学会雑誌

There are rare reports of families with multiple members with aortic dissection in the absence of Marfan syndrome. We encountered four cases of aortic dissection in two families. The aortic dissection occurred in the mother and child of the first family and in sisters of the second family. All cases had systemic hypertension preoperatively and presented Stanford type A aortic dissection. All of them were operated successfully. None of them showed the characteristics of connective tissue disease affecting the skeletal, ocular, and cardiovascular system. However, many members of the two families had systemic hypertension and histopathological examination of the aorta showed cystic medial necrosis in all of the four cases. The present study suggests that the familial aortic dissection may be caused by weakness of the aortic wall related to heredity and systemic hypertension.

A Case of Unruptured Aneurysm of the Sinus of Valsalva / 日本心臓血管外科学会雑誌

A 36-year-old woman was admitted because of dyspnea on exertion and palpitations, during follow-up for ventricular septal defect since age 5. Physical examination revealed a grade IV/VI ejection systolic murmur at the second left intercostal space. Echocardiogram failed to recognize an unruptured aneurysm of the sinus of Valsalva protruding into the right ventricle which was seen on right ventriculogram (type I of Konno). Surgical treatment was successfully performed. Right ventriculography was much more effective rather than echocardiography in this case.

A Case of Ischemic Limb Perfusion during Cardiopulmonary Bypass / 日本心臓血管外科学会雑誌

A 64-year-old woman with chest pain and intermittent claudication was admitted to our hospital. Unstable angina pectoris and arteriosclerosis obliterans (ASO) of both leg were diagnosed. Angiography indicated total occlusion of the right external iliac artery and severe stenosis of the left external iliac artery, in addition to significant stenoses of the three major coronary arteries. The ankle pressure index was 0.49 in her right leg, and 0.74 in the left. Because coronary stenting was unsuccessful, emergency coronary artery bypass grafting was performed prior to arterial reconstruction of the lower extremities. To prevent exacerbation of limb ischemia during cardiopulmonary bypass (CPB), selective limb perfusion was performed with a 14-gauge intravenous catheter inserted into the right superficial femoral artery. There were no complications related to limb ischemia during or after the operation. Selective limb perfusion was considered to be useful to prevent limb ischemia during CPB in patients with ASO of the legs.