Backer C L, Idriss F S, Zales V R, Ilbawi M N, DeLeon S Y, Muster A J, Mavroudis C
Division of Cardiovascular-Thoracic Surgery, Children's Memorial Hospital, Chicago, Ill.
J Thorac Cardiovasc Surg. 1991 Aug;102(2):288-95; discussion 295-6.
Surgical management of the conal (supracristal) ventricular septal defect differs significantly from the management of the perimembranous (infracristal) ventricular septal defect. The absence of a portion of the conal septum can lead to prolapse of the right cusp of the aortic valve, which predisposes these patients to aortic insufficiency. Between January 1980 and December 1989, 36 children with conal ventricular septal defect underwent intracardiac repair. Diagnosis was by echocardiography, cardiac catheterization, and intraoperative exploration. Preoperative evaluation showed that 26 patients (72%) had aortic valve prolapse and 16 (44%) had aortic insufficiency. Pulmonary-to-systemic flow ratios ranged from 1:1 to 3.5:1 (mean 2.0:1.0). Ten patients (27%) were believed to have clinical congestive heart failure. Age at the time of operation ranged from 2 weeks to 18 years (mean 5.5 years). Operative exposure was through the pulmonary artery (26), aorta (4), right ventricle (3), or right atrium (3). Simultaneous aortic valve suspension for aortic insufficiency was performed in four patients. Operative survival was 100%. Follow-up is complete in all patients and ranges from 0.5 to 9 years (mean 4.3 years). All patients are in normal sinus rhythm. No residual ventricular septal defects have been identified. Twenty-three of 36 patients (64%) have no evidence of aortic insufficiency; 12 of 36 (33%) have trivial or mild aortic insufficiency. One patient with initial severe aortic insufficiency underwent repeat aortic valvuloplasty 3 years after ventricular septal defect closure and aortic valve suspension. No patients have required aortic valve replacement. Surgical management of the conal ventricular septal defect differs from that of the perimembranous ventricular septal defect in two critical aspects. The operative approach should be through the pulmonary artery. This allows the best exposure of the remaining conal septum and the pulmonary and aortic valve leaflets, facilitating closure of the defect without injury to the valves or conduction system. Conal ventricular septal defects should undergo early closure, regardless of shunt volume, to prevent progressive aortic insufficiency.
圆锥（嵴上型）室间隔缺损的外科治疗与膜周（嵴下型）室间隔缺损的治疗有显著差异。圆锥间隔部分缺如可导致主动脉瓣右冠瓣脱垂，使这些患者易患主动脉瓣关闭不全。1980年1月至1989年12月，36例圆锥室间隔缺损患儿接受了心内修复术。诊断通过超声心动图、心导管检查和术中探查。术前评估显示，26例患者（72%）有主动脉瓣脱垂，16例（44%）有主动脉瓣关闭不全。肺循环与体循环血流量之比为1:1至3.5:1（平均2.0:1.0）。10例患者（27%）被认为有临床充血性心力衰竭。手术时年龄为2周至18岁（平均5.5岁）。手术显露途径包括经肺动脉（26例）、主动脉（4例）、右心室（3例）或右心房（3例）。4例因主动脉瓣关闭不全同时行主动脉瓣悬吊术。手术生存率为100%。所有患者均完成随访，随访时间为0.5至9年（平均4.3年）。所有患者均为正常窦性心律。未发现残余室间隔缺损。36例患者中有23例（64%）无主动脉瓣关闭不全证据；36例中有12例（33%）有轻微或轻度主动脉瓣关闭不全。1例最初有严重主动脉瓣关闭不全的患者在室间隔缺损修补和主动脉瓣悬吊术后3年接受了再次主动脉瓣成形术。无患者需要行主动脉瓣置换术。圆锥室间隔缺损的外科治疗在两个关键方面与膜周室间隔缺损不同。手术入路应经肺动脉。这样能最佳显露残余圆锥间隔以及肺动脉瓣和主动脉瓣叶，便于在不损伤瓣膜或传导系统的情况下闭合缺损。圆锥室间隔缺损无论分流大小均应尽早闭合，以防止主动脉瓣关闭不全进展。
