Alboliras E T, Mavroudis C, Pahl E, Gidding S S, Backer C L, Rocchini A P
Children's Memorial Hospital, Department of Pediatrics, Northwestern University Medical School, Chicago, Illinois 60614, USA.
Ann Thorac Surg. 1999 Aug;68(2):549-55. doi: 10.1016/s0003-4975(99)00621-9.
Models that predict survival in neonates with left ventricular hypoplasia and critical aortic stenosis may not be applicable to neonates with left ventricular hypoplasia and coarctation.
We report 8 infants with severe aortic coarctation and left ventricular hypoplasia. Mean age was 18 days (range 1-48 days), and mean weight was 3.5 kg (range 2.7-4.3 kg). Associated diagnoses included mild aortic stenosis (4), ventricular septal defect (2), and venous anomalies (2). All had coarctation repair as a primary procedure (3 of these had concomitant intracardiac procedures); 7 had subsequent operations. All are alive and well 1.1-6.7 years (mean 3.1 years) after the first surgery. Progressive increases were observed in aortic and mitral diameters, and in left ventricular dimensions, areas, and volumes when the preoperative, earliest postoperative, and most recent echocardiograms were compared.
Despite severe left ventricular hypoplasia, a two-ventricle repair is possible in selected cases. The prognostic criteria for left ventricular hypoplasia in critical aortic stenosis may not be applicable to infant coarctation. Relief of coarctation may result in the growth of the very small left ventricle, especially when the aortic root and mitral diameters are satisfactory.
预测左心室发育不全和严重主动脉狭窄新生儿存活率的模型可能不适用于左心室发育不全合并主动脉缩窄的新生儿。
我们报告了8例患有严重主动脉缩窄和左心室发育不全的婴儿。平均年龄为18天(范围1 - 48天),平均体重为3.5千克(范围2.7 - 4.3千克)。相关诊断包括轻度主动脉狭窄(4例)、室间隔缺损(2例)和静脉异常(2例)。所有患儿均以主动脉缩窄修复作为主要手术(其中3例同时进行了心内手术);7例患儿接受了后续手术。首次手术后1.1 - 6.7年(平均3.1年),所有患儿均存活且状况良好。比较术前、术后最早及最近的超声心动图发现,主动脉和二尖瓣直径以及左心室大小、面积和容积均有逐渐增加。
尽管左心室严重发育不全,但在某些病例中仍可进行双心室修复。严重主动脉狭窄中左心室发育不全的预后标准可能不适用于婴儿主动脉缩窄。解除主动脉缩窄可能会使非常小的左心室生长,尤其是当主动脉根部和二尖瓣直径令人满意时。