Van Praagh Stella, Mayer John E, Berman Norman B, Flanagan Michael F, Geva Tal, Van Praagh Richard
Department of Surgery, Children's Hospital and Harvard Medical School, Boston, Massachusetts 02115, USA.
Ann Thorac Surg. 2002 Jan;73(1):48-56; discussion 56-7. doi: 10.1016/s0003-4975(01)03249-0.
Apical ventricular septal defects (VSDs) are difficult to visualize and close transatrially. We described their distinctive anatomic features, which have seldom been documented angiocardiographically and pathologically, in order to develop an effective approach for their surgical management.
Fourteen postmortem cases, two explanted hearts, 9 successfully operated patients, and 1 unoperated living patient were included in this report. Angiocardiographic documentation of the apical VSD was available in 14 of 16 (87.5%) of the postmortem and transplanted cases, and in 6 of 10 (60%) of the living patients. Echocardiograms were available in 23 of all 26 cases (88%).
Severe associated malformations were present in 14 of 16 (87%) of the pathologically documented cases. Large VSDs allowed extensive communication between the left ventricular and the right ventricular sinuses in 4 patients. In 12 of the pathologically documented cases and in the 10 living patients, the left ventricular apex communicated with the right ventricular apical infundibular recess.
Extremely large apical VSDs with severe biventricular dysplasia and dysfunction may require cardiac transplantation. Large apical VSDs can be successfully closed through a small apical infundibulotomy. This approach, applicable even in small infants, can avoid pulmonary artery banding or left ventriculotomy.
心尖部室间隔缺损(VSD)经心房难以观察及闭合。我们描述了其独特的解剖特征,这些特征在心血管造影和病理学方面鲜有记录,目的是为其手术治疗开发一种有效的方法。
本报告纳入了14例尸检病例、2例心脏移植切除的心脏、9例手术成功的患者以及1例未手术的存活患者。16例尸检和移植病例中的14例(87.5%)以及10例存活患者中的6例(60%)有心血管造影记录的心尖部VSD。所有26例病例中有23例(88%)有超声心动图检查结果。
16例经病理记录的病例中有14例(87%)存在严重的相关畸形。4例患者中,大的VSD导致左心室和右心室窦之间广泛交通。在12例经病理记录的病例以及10例存活患者中,左心室心尖与右心室心尖漏斗部隐窝相通。
伴有严重双心室发育不良和功能障碍的极大的心尖部VSD可能需要心脏移植。大的心尖部VSD可通过小的心尖漏斗部切开术成功闭合。这种方法甚至适用于小婴儿,可避免肺动脉环扎术或左心室切开术。