Di Gioia Giuseppe, Mega Simona, Miglionico Marco, Di Sciascio Germano
Department of Cardiovascular Sciences, Campus Bio-Medico University of Rome, Rome, Italy.
J Cardiovasc Echogr. 2016 Jan-Mar;26(1):16-18. doi: 10.4103/2211-4122.178466.
Ostium primum defect is a congenital malformation involving atrial septum contiguous with atrioventricular valve annulus; it is accompanied by abnormalities in the development of the endocardial cushions, often resulting in associated atrioventricular valves malformations. Few cases have been reported in adulthood because these patients frequently come to medical attention at an earlier age when symptoms such as dyspnea, fatigue, cyanosis, and tendency to underweight occur. Various factors affect the timing of clinical presentation, but the most important is the degree of mitral/tricuspid insufficiency; when valve regurgitation remains moderate, the appearance of symptoms may be delayed for decades. In adult patients, deterioration of clinical status and death are mainly due to the development of arrhythmias or heart block. We present the case of a 67-year-old patient, without previous cardiovascular events, with a new onset of atrial fibrillation, who developed dyspnea and fatigue; echocardiography showed a large interatrial defect localized in the basal portion of the septum, associated with anterior mitral valve cleft and moderate regurgitation. The patient underwent surgical closure of the defect (intraoperatory measures 1,9 × 3 cm) with autologous pericardium patch; a permanent epicardial pacemaker was implanted for the development of complete atrioventricular block in the early postoperative period.
原发孔型缺损是一种先天性畸形,累及与房室瓣环相邻的房间隔;它伴有心内膜垫发育异常,常导致相关的房室瓣畸形。成年期报道的病例很少,因为这些患者在出现呼吸困难、疲劳、发绀和体重偏轻等症状时,往往在较早年龄就引起了医疗关注。多种因素影响临床表现出现的时间,但最重要的是二尖瓣/三尖瓣关闭不全的程度;当瓣膜反流仍为中度时,症状的出现可能会延迟数十年。在成年患者中,临床状况恶化和死亡主要是由于心律失常或心脏传导阻滞的发生。我们报告一例67岁患者,既往无心血管事件,新发房颤,出现呼吸困难和疲劳;超声心动图显示一个大的房间隔缺损位于间隔的基部,伴有二尖瓣前叶裂和中度反流。患者接受了自体心包补片修补缺损手术(术中测量为1.9×3cm);术后早期因发生完全性房室传导阻滞植入了永久性心外膜起搏器。