Hadjo A, Jimenez M, Baudet E, Roques X, Laborde N, Srour S, Surrel C, Choussat A
Hôpital Cardiologique du Haut-Lévêque, Université de Bordeaux II, Pessac, France.
Eur Heart J. 1995 Nov;16(11):1668-74. doi: 10.1093/oxfordjournals.eurheartj.a060793.
This study reviews the long-term course of 52 patients with pulmonary atresia and ventricular septal defect seen in a single institution and followed for a mean period of 8.6 years (range 2 days to 20 years). Before the first operation, pulmonary blood supply was provided by ductus arteriosus supplying confluent pulmonary arteries in 26 patients (50%, group I), and was partially or entirely dependent on systemic collateral arteries in the other 26 patients (group II). The angiographic mean ratio of diameters of the right and left pulmonary artery/descending aorta (McGoon ratio) was significantly lower in group II than in group I, 0.76 +/- 0.42 vs 1.04 +/- 0.17 (P = 0.006). Severe arborization defects (with fewer than 10 pulmonary vascular segments connected to central pulmonary arteries) were present only in group II patients (eight patients: 15%), six of whom had congenital absence of the central pulmonary arteries. Corrective surgery was performed in 23 patients (44%, 14 in group I, nine in group II). All but one, who died later, had a McGoon ratio > or = 1 (mean 1.19 +/- 0.18) at time of repair. There was one hospital death (4%) and two late deaths (9%). All but one of the surviving corrected patients were in functional class I or II. Conduit replacement reoperation was performed in three patients (14%), 6, 10 and 13 years, respectively, after repair. At the end of the study, among the 37 patients (71%) who were alive (17 in group I, 20 in group II), 20 (39%) were corrected (12 in group I, eight in group II), four await corrective surgery, and six (11.5%) are estimated inoperable (all in group II) because of very hypoplastic or absent pulmonary arteries. This study confirms the estimated rate in the published literature of long-term survival in patients with pulmonary atresia and ventricular septal defect, and the good results of corrective surgery whenever the size and distribution of pulmonary arteries are satisfactory. The problem of very hypoplastic pulmonary arteries and severe arborization defects remains contentious.
本研究回顾了在单一机构中诊治的52例肺动脉闭锁合并室间隔缺损患者的长期病程，平均随访时间为8.6年（范围2天至20年）。首次手术前，26例患者（50%，I组）的肺血供由供应融合肺动脉的动脉导管提供，另外26例患者（II组）的肺血供部分或完全依赖体循环侧支动脉。II组右肺动脉与左肺动脉/降主动脉直径的血管造影平均比值（麦戈恩比值）显著低于I组，分别为0.76±0.42和1.04±0.17（P = 0.006）。严重的分支缺陷（与中央肺动脉相连的肺血管段少于10个）仅出现在II组患者中（8例：15%），其中6例先天性中央肺动脉缺如。23例患者（44%）接受了矫正手术（I组14例，II组9例）。除1例术后死亡外，所有患者修复时的麦戈恩比值均≥1（平均1.19±0.18）。有1例医院死亡（4%）和2例晚期死亡（9%）。除1例外，所有存活的矫正患者心功能均为I或II级。3例患者（14%）分别在修复后6年、10年和13年进行了管道置换再次手术。研究结束时，在存活的37例患者（71%）中（I组17例，II组20例），20例（39%）已矫正（I组12例，II组8例），4例等待矫正手术，6例（11.5%）因肺动脉发育极差或缺如估计无法手术（均在II组）。本研究证实了已发表文献中肺动脉闭锁合并室间隔缺损患者的长期生存率估计，以及只要肺动脉大小和分布满意，矫正手术效果良好。肺动脉发育极差和严重分支缺陷的问题仍然存在争议。
