Furlanetto Gláucio, Furlanetto Beatriz H S, Henriques Sandra R C, Lopes Lilian M, Miranda Eduardo T, Porto Carolina M G, Pereira Maria Emilia N T, Davini Claudia, Espinosa Elssi C
Furlanetto Institute, São Paulo, Brazil
Instituto Furlanetto - Real e Benemérita Associação Portuguesa de Beneficência, São Paulo, Brazil.
World J Pediatr Congenit Heart Surg. 2015 Jan;6(1):26-32. doi: 10.1177/2150135114554660.
The mixed total anomalous pulmonary connection is a rare type of congenital cardiopathy. The occurrence of multiple connections of the pulmonary veins has important implication in the diagnosis and surgical planning. These types of total anomalous pulmonary venous connection (TAPVC) and the infracardiac have higher surgical mortality.
Between December 1994 and July 2013, a total of 58 children underwent surgical treatment of TAPVC. Twenty-five (43.1%) patients had supracardiac connection, 15 (25.8%) patients had intracardiac type, 12 (20.6%) patients had a mixed type, and 6 (10.3%) patients had an infracardiac type. In children with mixed TAPVC, four (33.3%) patients had type I, five (41.6%) patients had type II, and three (25.0%) patients had type III. The diagnosis of TAPVC was performed in all patients using Doppler echocardiography, and they underwent cardiopulmonary bypass with aorta-bicaval cannulation and lately we use mild hypothermia with normal flow. The drainage channels were ligated in all patients.
The immediate surgical results were as follows: one (16.6%) death in the infracardiac group, four (16.0%) deaths in the supracardiac group, one (8.3%) death in the mixed group, and no death in the cardiac group (no statistical difference, P = .488).
In this series of patients, the mixed TAPVC could be corrected only with preoperative two-dimensional Doppler echocardiography with good immediate results, and we believe that the use of computed angiotomography combined with three-dimensional reconstruction may improve the surgical outcome mainly in the mixed and infracardiac group of TAPVC.
混合型完全性肺静脉异位连接是一种罕见的先天性心脏病类型。肺静脉存在多处连接对诊断和手术规划具有重要意义。这类完全性肺静脉异位连接（TAPVC）及心内型的手术死亡率较高。
1994年12月至2013年7月，共有58例儿童接受了TAPVC手术治疗。25例（43.1%）患者为心上型连接，15例（25.8%）患者为心内型，12例（20.6%）患者为混合型，6例（10.3%）患者为心下型。在混合型TAPVC患儿中，4例（33.3%）为I型，5例（41.6%）为II型，3例（25.0%）为III型。所有患者均通过多普勒超声心动图进行TAPVC诊断，并在体外循环下采用主动脉-双腔静脉插管，近期我们采用浅低温及正常流量。所有患者均结扎引流通道。
手术即刻结果如下：心下型组1例（16.6%）死亡，心上型组4例（16.0%）死亡，混合型组1例（8.3%）死亡，心内型组无死亡（无统计学差异，P = 0.488）。
在这组患者中，混合型TAPVC仅通过术前二维多普勒超声心动图即可得到较好的即刻纠正效果，我们认为计算机血管造影结合三维重建的应用可能主要改善TAPVC混合型和心下型组的手术效果。
