ABSTRACT
Objective Postoperative pulmonary vein stenosis(PPVS) is a severe complication after total anomalous pulmonary 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 demonstrate 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 reoperations.Supracardiac type in 5 cases,intracardiac type in 3 cases,mixed type in 1 case,single ventricle associated with supracardiac type in 1 case and infracardiac 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 preoperative imaging examinations and surgical findings:(1) 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 pulmonary vein (type Ⅳ).One or more pathological patterns may exit in one patient,and may involved one or more individual pulmonary veins.Fibrous resection in 1 case,patch augmentation in 3 cases,individual pulmonary endarterectomy in 7 cases and sutureless 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 Ⅱ in 2 cases,type Ⅰ and type Ⅱ in 1 cases,type Ⅰ,type Ⅱ and type Ⅲ in 1 case,type Ⅱ and Ⅲ in 2 cases,type Ⅱ and Ⅳ in 1 case and type Ⅳ in 2 cases were found in our series.There was no early mortality,I 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％.Reoperation was done in 1 case for recurrent pulmonary vein obstruction.Two patients need ViagTa or Bosentan for long time.The others did well during follow-up.Conclusion PPVS is a kind of progressive disease.The morphological patterns of PPVS are related to their clinical presentations and its pathogenesis,and are very important to make surgical decisions and pridict the patient's prognosis.