Department of Pediatric Cardiovascular Surgery, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan.
Ann Thorac Surg. 2012 Sep;94(3):833-41; discussion 841. doi: 10.1016/j.athoracsur.2012.04.071. Epub 2012 Jul 20.
We aimed to reveal late clinical features of patients with pulmonary atresia with intact ventricular septum (PA/IVS) or critical pulmonary stenosis (cPS) after biventricular repair (BVR) based on preoperative right ventricular (RV) end-diastolic volume (RVEDV) findings.
Since 1985, 23 of 73 patients with PA/IVS (n=22) or cPS (n=1) with a tripartite RV and without major sinusoidal communication underwent BVR with a hybrid approach. The mean age and weight at BVR were 1.4±2.1 years and 6.9±5.9 kg, respectively. Mean follow-up was 10.1±6.4 years (range, 1.1 to 24.6 years).
Overall survival, reintervention-free, and arrhythmia-free rates at 20 years were 90.6%, 75.4%, and 50.4%, respectively. In 19 patients with preoperative RVEDV of 60% to 120% of normal, echocardiography at 10 years after BVR showed well-maintained RV systolic function. However, RV volume was quantitatively dilated in 16 (88.9%) due to moderate or greater tricuspid regurgitation in 8 (44.4%), pulmonary regurgitation in 12 (66.7%), or both, which caused arrhythmia in 3 patients more than 10 years after BVR. Two patients with preoperative RVEDV of greater than 120% of normal required tricuspid valve replacement after BVR, after which refractory atrial tachyarrhythmia developed in both patients. Furthermore, 2 patients with preoperative RVEDV of less than 60% of normal showed a cardiac index value within 2.5 L/min/m2 at 1 year after BVR, which did not improve.
Patients with PA/IVS or cPS and adequately sized RV showed good late clinical features after BVR. However, long-term follow-up examinations are necessary for RV dilatation and late-onset arrhythmia.
我们旨在基于术前右心室（RV）舒张末期容积（RVEDV）发现，揭示接受双心室修复（BVR）后伴有完整室间隔的肺动脉闭锁（PA/IVS）或严重肺动脉瓣狭窄（cPS）患者的晚期临床特征。
自 1985 年以来，73 例具有三尖瓣 RV 且无主要窦房沟通的 PA/IVS 患者（n=22）或 cPS 患者（n=1）中，有 23 例行 BVR 治疗，采用混合方法。BVR 时的平均年龄和体重分别为 1.4±2.1 岁和 6.9±5.9kg。平均随访时间为 10.1±6.4 年（范围为 1.1 至 24.6 年）。
20 年时的总生存率、无再干预生存率和无心律失常生存率分别为 90.6%、75.4%和 50.4%。在术前 RVEDV 为正常的 60%至 120%的 19 例患者中，BVR 后 10 年的超声心动图显示 RV 收缩功能保持良好。然而，由于 8 例（44.4%）中度或重度三尖瓣反流、12 例（66.7%）肺动脉瓣反流或两者均导致 RV 容积定量扩张，16 例（88.9%）患者出现心律失常。BVR 后 10 年以上，3 例患者出现心律失常。2 例术前 RVEDV 大于正常的 120%的患者需要行三尖瓣置换术，此后两名患者均出现难治性房性心动过速。此外，2 例术前 RVEDV 小于正常的 60%的患者在 BVR 后 1 年时心脏指数值在 2.5 L/min/m2 以下，且未改善。
PA/IVS 或 cPS 且 RV 大小适当的患者在接受 BVR 后表现出良好的晚期临床特征。然而，需要对 RV 扩张和迟发性心律失常进行长期随访检查。
