Starnes V A, Luciani G B, Wells W J, Allen R B, Lewis A B
Division of Cardiothoracic Surgery, Children's Hospital Los Angeles, USC School of Medicine, Los Angeles 90027, USA.
Ann Thorac Surg. 1996 Aug;62(2):442-8; discussion 448-9.
The optimal surgical treatment of complex (multiple level or recurrent) left ventricular outflow tract obstruction (LVOTO) in infancy is controversial. Staged procedures expose the children to the need for reoperation, and currently available techniques of aortoventriculoplasty are associated with the morbidities of biological and mechanical prostheses.
Between July 1992 and January 1996, we have performed 24 aortic root replacements with the pulmonary autograft in pediatric patients (< 18 years). Of this group, 8 were infants and children with complex LVOTO aged 9 days to 22 months (mean, 8.6 +/- 8 months) and weighing 3.3 to 10.2 kg (mean, 6.3 +/- 2.6 kg). The diagnoses were interrupted aortic arch/ventricular septal defect/subaortic stenosis in 3, recurrent aortic stenosis in 2, aortic stenosis and subaortic stenosis in 1, and aortic stenosis/subaortic stenosis/mitral stenosis/regurgitation in 2. All patients had undergone one to three previous operative procedures (mean, 1.5 +/- 0.8 procedures/patient). Preoperative echocardiographic peak LVOT gradient was 71.7 +/- 25 mm Hg (range, 40 to 110 mm Hg) and aortic annulus size was 7.2 +/- 2.3 mm (range, 4 to 10.6 mm). The surgical technique included replacement of the aortic root with the pulmonary autograft combined with incision of the conal septum to relieve subaortic stenosis or accommodate for size discrepancy between the aortic and pulmonary autograft root and a pulmonary homograft placed in the right ventricular outflow tract.
There were no perioperative or late deaths at follow-up (range, 2 to 25 months; mean, 13.5 +/- 8 months). Mean hospital stay was 15 +/- 17 days (range, 4 to 53 days). Three children had the following complications: diaphragmatic paresis (1), delayed pericardial effusion (1), and atrioventricular block requiring a pacemaker (1). In follow-up, echocardiographic findings showed absent aortic regurgitation in 3 and trivial aortic regurgitation in 5, and no significant LVOTO (mean peak gradient, 6.2 +/- 7.6 mm Hg; range, 0 to 16 mm Hg). Pulmonary homograft regurgitation was absent in 5, trivial in 2, and moderate in 1. Peak right ventricular outflow tract gradient by echocardiogram was trivial in 7, and a significant gradient of 55 mm Hg has developed in 1 infant. There were no infective or embolic complications during follow-up.
Our experience shows that aortic root replacement with the pulmonary autograft can be performed in children with excellent clinical results. The technique of root replacement combined with ventriculoplasty allows definitive and simultaneous relief of complex and multiple-level obstructive lesions. Considering the growth potential of the pulmonary autograft, this should be regarded as the optimal treatment modality in infants with complex LVOTO:
婴儿期复杂（多平面或复发性）左心室流出道梗阻（LVOTO）的最佳手术治疗存在争议。分期手术使患儿需要再次手术，而目前可用的主动脉心室成形术技术与生物和机械假体的并发症相关。
1992年7月至1996年1月，我们对小儿患者（<18岁）进行了24例肺动脉自体移植主动脉根部置换术。该组中，8例为9天至22个月（平均8.6±8个月）、体重3.3至10.2 kg（平均6.3±2.6 kg）的复杂LVOTO婴儿和儿童。诊断为主动脉弓中断/室间隔缺损/主动脉瓣下狭窄3例，复发性主动脉瓣狭窄2例，主动脉瓣狭窄和主动脉瓣下狭窄1例，主动脉瓣狭窄/主动脉瓣下狭窄/二尖瓣狭窄/反流2例。所有患者此前均接受过一至三次手术（平均每位患者1.5±0.8次手术）。术前超声心动图显示左心室流出道峰值梯度为71.7±25 mmHg（范围40至110 mmHg），主动脉瓣环大小为7.2±2.3 mm（范围4至10.6 mm）。手术技术包括用肺动脉自体移植置换主动脉根部，同时切开圆锥间隔以缓解主动脉瓣下狭窄，或适应主动脉和肺动脉自体移植根部之间的大小差异，并在右心室流出道置入肺动脉同种异体移植物。
随访期间（范围2至25个月；平均13.5±8个月）无围手术期或晚期死亡。平均住院时间为15±17天（范围4至53天）。3例患儿出现以下并发症：膈肌麻痹（1例）、延迟性心包积液（1例）、需要起搏器的房室传导阻滞（1例）。随访中，超声心动图显示3例无主动脉瓣反流，5例有微量主动脉瓣反流，且无明显左心室流出道梗阻（平均峰值梯度6.2±7.6 mmHg；范围0至16 mmHg）。5例无肺动脉同种异体移植物反流，2例有微量反流，1例有中度反流。超声心动图显示7例右心室流出道峰值梯度微量，1例婴儿出现55 mmHg的明显梯度。随访期间无感染或栓塞并发症。
我们的经验表明，肺动脉自体移植主动脉根部置换术可在儿童中进行，临床效果良好。根部置换联合心室成形术技术可明确并同时缓解复杂和多平面梗阻性病变。考虑到肺动脉自体移植的生长潜力，这应被视为复杂左心室流出道梗阻婴儿的最佳治疗方式。
