Preceding Re-entry Closure for Chronic Thoracic Aortic Dissection in a Patient with Marfan Syndrome / 日本心臓血管外科学会雑誌

<p>A 42 year-old woman with Marfan syndrome, who had replacement of the ascending aorta for acute aortic dissection several years ago, was found to have distal aortic arch aneurysm. The aneurysm had small entries at cervical arterial branches and large re-entry at the left external iliac artery. It was necessary to perform two-staged operation Bentall procedure with total arch replacement and abdominal aortic replacement with re-entry closure. It was usually performed with a primary entry closure for chronic aortic dissection, but massive invasion was expected. We performed catheter angiography for entry and re-entry, and decided to perform preceding re-entry closure. First, we underwent replacement of the abdominal aorta, and then successfully performed the Bentall procedure with total aortic arch replacement. The catheter angiography was useful for decision-making for medical treatment.</p>

Waffle Procedure Improved Hemodynamic Circulation of Constrictive Epicarditis / 日本心臓血管外科学会雑誌

A 62-year-old woman who had suffered from malignant articular rheumatism since 2000 felt fatigue, exertional dyspnea and edema was admitted in May 2009. Cardiac catheterization demonstrated a dip-and-plateau pattern of the pressure curve of RV. Therefore we diagnosed constrictive pericarditis. She decided to have surgery as soon as possible. As she had also been troubled with constrictive pulmonary damage, chronic renal failure, and was taking an inotropic agent, we concluded that the use of extracorporeal circulation was risky. After median sternotomy, we recognized diastolic insufficiency of the heart due to hypertrophy of the epicardium. Using an ultrasonic scalpel and electrotome, we incised the thickened epicardium with a waffle procedure. The dip-and-plateau disappeared and as a result the cardiac index was improved immediately (1.9 <i>l</i>/min/m²→2.6 <i>l</i>/min/m²). She was discharged on postoperative day 25. In this way waffle procedure for constrictive pericarditis can an effective surgical procedure for a high risk patient.

A Surgical Case of Severe Aortic Valve Calcification Complicated by X-linked Hypophosphatemic Osteomalacia / 日本心臓血管外科学会雑誌

A 34-year-old woman had received a diagnosis of X-linked hypophosphatemic osteomalacia when she was born. As an adult she complained of general fatigue, palpitations dyspnea exertional and leg edema. Transthoracic echocardiography showed aortic stenosis and regurgitation with severe aortic valve calcification. Chest computed tomography revealed her ascending aorta to be circumferentially calcified. Surgery was performed through a median sternotomy via cardiopulmonary bypass, with perfusion from the right subclavian artery and the right femoral artery and drainage from the superior and the inferior venae cavae. A left ventricular venting cannula was inserted from the right superior pulmonary vein. When her bladder temperature had dropped to 28°C by central cooling, we stopped the perfusion from the right femoral artery and performed aortotomy. We examined the lumen and clamped the aorta at the usual site, while flushing with blood by femoral perfusion. Aortic valve calcification was observed to extend from the sinus of valsalva to the outflow tract of the left ventricle on the non-coronary cusp side. He was necessary to repair the aortic annulus due to a defect resulting from careful removal of the calcified valve. We then performed aortic valve replacement using a 19-mm Carpentier-Edwards perimount Magna. Her postoperative course was uneventful without any sign of neurological complications, and she was discharged 13 days after surgery. On pathological examination, localized ossification with calcification was observed in her aortic valve which seemed to be characteristic of X-linked hypophosphatemic osteomalacia.