Ricci M, Elliott M, Cohen G A, Catalan G, Stark J, de Leval M R, Tsang V T
Cardiothoracic Unit, Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London WC1N 3JH, UK.
Eur J Cardiothorac Surg. 2003 Jul;24(1):28-36; discussion 36. doi: 10.1016/s1010-7940(03)00180-5.
Recurrent pulmonary venous obstruction (PVO) occurs in 0-18% of infants undergoing correction of total anomalous pulmonary venous connection (TAPVC). Limited published data suggest that PVO usually develops within 6 months of primary repair, and that outcomes of reoperations are poor. This study aimed to review our experience of reoperations for PVO post-TAPVC repair and to identify risk factors for adverse outcome.
Twenty patients underwent reoperation for PVO between 1982 and 2002. Clinical data were reviewed. TAPVC was mostly infracardiac (11 patients). TAPVC was obstructed in nine patients. PVO developed early (<6 months) in seven patients, and late in 13 (>6 months). Time of presentation was unrelated to type of PVO (anastomotic vs. ostial). Repair was accomplished using various techniques (anastomotic enlargement with native atrial tissue, enlargement with pericardium, free or in situ, or other prosthetic material). Follow-up ranged from 1 month to 15 years (average 44 months).
Thirteen patients received one reoperation, while seven had multiple reoperations. In 13 patients, PVO was defined as new onset (no obstruction post-TAPVC repair), and in seven patients as residual (minimal obstructive changes post-TAPVC repair that progressed to PVO). Ten patients presented with anastomotic PVO, six with anastomotic and ostial PVO (involving the PVs), three with ostial PVO, and one with coronary sinus-left atrial junction stenosis. Mortality was 25% (5/20). Six of the ten patients with anastomotic PVO underwent one reoperation (2/6 died); the other four developed ostial PVO after reoperation, requiring multiple procedures (2/4 died). Mode of presentation (new onset vs. residual), site of obstruction (anastomotic vs. ostial), preoperative RV pressure (<0.8 vs. >0.8 systemic), number of reoperations (single vs. multiple), residual obstruction (presence or absence), and operative approach (Gore-tex or not) did not seem to affect outcomes. Risk factors for death were early presentation (<6 months) and persistence of pulmonary hypertension after reoperation; early presentation was also a risk factor for multiple reoperations.
Our findings support the conclusion that early presentation and postoperative pulmonary hypertension have the greatest adverse impact on outcome. Of these, failure to achieve a low-pressure pulmonary vascular system seems to be the variable that most strongly prevents survival. In our series, neither ostial PVO nor multiple re-interventions significantly increased surgical risk. The negative impact of postoperative residual obstruction on outcome was not striking. However, an aggressive surgical approach to this disease is still warranted. Although the role of each technique in obtaining long-lasting relief of PVO remains to be established, the use of artificial material seems unwise.
在接受完全性肺静脉异位连接（TAPVC）矫治术的婴儿中，复发性肺静脉梗阻（PVO）的发生率为0%至18%。有限的已发表数据表明，PVO通常在初次修复后的6个月内发生，且再次手术的预后较差。本研究旨在回顾我们对TAPVC修复术后PVO再次手术的经验，并确定不良结局的危险因素。
1982年至2002年间，20例患者因PVO接受了再次手术。回顾了临床资料。TAPVC大多为心内型（11例患者）。9例患者的TAPVC存在梗阻。7例患者的PVO发生较早（<6个月），13例患者发生较晚（>6个月）。出现症状的时间与PVO的类型（吻合口处与开口处）无关。采用多种技术完成修复（用自体心房组织扩大吻合口、用心包扩大吻合口，游离或原位使用，或使用其他人工材料）。随访时间为1个月至15年（平均44个月）。
13例患者接受了一次再次手术，7例患者接受了多次再次手术。13例患者的PVO被定义为新发（TAPVC修复术后无梗阻），7例患者为残留（TAPVC修复术后有轻微梗阻改变，进展为PVO）。10例患者表现为吻合口处PVO，6例患者表现为吻合口处和开口处PVO（累及肺静脉），3例患者表现为开口处PVO，1例患者表现为冠状窦-左心房连接部狭窄。死亡率为25%（5/20）。10例吻合口处PVO患者中有6例接受了一次再次手术（2/6死亡）；另外4例患者在再次手术后出现开口处PVO，需要多次手术（2/4死亡）。出现症状的方式（新发与残留）、梗阻部位（吻合口处与开口处）、术前右心室压力（<0.8与>0.8体循环压力）、再次手术次数（单次与多次）、残留梗阻（存在或不存在）以及手术方式（是否使用戈尔特斯材料）似乎均不影响预后。死亡的危险因素为出现症状较早（<6个月）和再次手术后持续存在肺动脉高压；出现症状较早也是多次再次手术的危险因素。
我们的研究结果支持以下结论：出现症状较早和术后肺动脉高压对预后的不良影响最大。其中，未能实现低压肺血管系统似乎是最强烈阻碍生存的变量。在我们的系列研究中，开口处PVO和多次再次干预均未显著增加手术风险。术后残留梗阻对预后的负面影响并不显著。然而，对这种疾病仍需采取积极的手术方法。尽管每种技术在获得PVO的持久缓解方面的作用仍有待确定，但使用人工材料似乎并不明智。
