完全性肺静脉异位引流术后肺静脉狭窄的病理类型及手术治疗

Morphological patterns and surgical treatment of pulmonary vein stenosis after total anomalous pulmonary venous connection repair

目的探讨完全性肺静脉异位引流（TAPVC）术后肺静脉狭窄（PPVS）不同病理类型与临床表现、发病机制、手术方法和预后的关系。方法2004年3月至2011年9月，11例PPVS患儿进行再手术，心上型5例，心内型3例，混合型1例，单心室伴TAPVC2例，其中心上和心下型各1例。初次手术年龄23天～28个月；体质量5～19k，平均（10．7±5．1）kg；再手术年龄4个月～6年，平均（27．1±22．6）个月；两次手术间隔82天-5年。分析PPVS形态学病理特点，根据PPVS术前影像学检查和术中发现，将PPVS形态学病理类型分为4种：Ⅰ型——吻合口狭窄，Ⅱ型——肺静脉口狭窄，Ⅲ型——肺静脉节段性狭窄，Ⅳ型——肺静脉闭锁。每个患儿存在一种或多种病理类型；病变可涉及一支或多支肺静脉。手术采用中度低温体外循环8例，深低温停循环3例。内膜剥脱术7例；无缝线技术5例；吻合口补片扩大术3例；纤维瘢痕切除1例。结果PPVS病理类型Ⅰ型2例，Ⅱ型2例，Ⅰ型＋Ⅱ型1例，Ⅱ型＋Ⅲ型2例，Ⅰ型＋Ⅱ型＋Ⅲ型1例，Ⅱ型＋Ⅳ型1例，Ⅳ型2例。无术后早期死亡，吻合口梗阻1例，分支肺静脉梗阻2例。术后中期（17个月）死亡1例，因肺静脉再次狭窄手术1例，长期服用枸橼酸西地那非和波生坦各1例。余发育良好。结论PPVS是一种进展性疾病，其形态学与临床表现和发病机制有关，形态学分类有助于选择手术方法和判断预后。

Objective Postoperative pulmonary vein stenosis（PPVS） is a severe complication after total anomalous pul- monary venous connection （TAPVC） repair, and represents a significant surgical challenge with a high recurrence rate and poor prognosis. This study was performed to analysis the morphological patterns of pulmonary vein stenosis after TAPVC, and to dem- onstrate the relationships between the pathological patterns with their clinical manifestations, pathogenesis, surgical options and their prognosis. Methods Between March, 2004 to September, 2011, 11 consecutive patients with PPVS underwent reopera- tions. Supracardiac type in 5 cases, intracardiac type in 3 cases, mixed type in 1 case, single ventricle associated with supracar- diac type in 1 case and infraeardiac type in 1 cases. The age was 23 days to 28 months, body weight at the first operation was 5 - 19 kg, the median weight （ 10.7 ＋ 5.1 ） kg. The age at reoperation was 4 months to 6 years, the interval between the first and second operation was 82 days to 5 years. Morphological patterns of PPVS was classified into 4 types based on the preopera- tive imaging examinations and surgical findings ： （ t ） anastomic stenosis （ type Ⅰ ）, （ 2 ） ostial stenosis of individual pulmonary vein （type Ⅱ ） , （3）segemental stenosis of individual pulmonary vein （type Ⅲ ） , （4）complete occulsion of individual pulmo- nary vein（ type ⅣⅠ）. One or more pathological patterns may exit in one patient, and may involved one or more individual pulmo- nary veins. Fibrous resection in 1 case, patch augmentation in 3 cases,individual pulmonary endarterectomy in 7 cases and su- tureless technique in 5 cases. Cardiopulmonary bypass with moderate hypothermia were used in 8 cases,with deep hypothermia and circulatory arrest in 3 cases. Results Type Ⅰ in 2 cases, type 11 in 2 cases, type Ⅰ and type Ⅱ in 1 cases, type Ⅰ , type Ⅱ and type IH in 1 case, type Ⅱ and Ⅲ in 2 cases, type Ⅱ and 1V in 1 case and type Ⅳ in 2 cases were found in our series. There was no early mortality, 1 case with residual anastomic obstruction and 2 cases with individual pulmonary vein obstruction. One patient died 17 monthes after reoperation, and the intermediate mortality was 10%.