Caldarone C A, Najm H K, Kadletz M, Smallhorn J F, Freedom R M, Williams W G, Coles J G
Division of Cardiovascular Surgery, The Hospital for Sick Children, University of Toronto and University of Toronto Faculty of Medicine, Ontario, Canada.
Ann Thorac Surg. 1998 Nov;66(5):1521-6. doi: 10.1016/s0003-4975(98)00951-5.
Recent reports have cited improving results for surgical management of isolated total anomalous pulmonary venous drainage. Complex cases (with other cardiac anomalies) are less frequently reported and are associated with higher mortality.
Retrospective review identified 170 consecutive patients treated for total anomalous pulmonary venous drainage from 1982 to 1996: 44 cases were "complex" (with significant associated cardiac lesions) and 126 cases were "simple."
Operative mortality for simple cases decreased from 26% to 8%, and mortality for complex cases remained constant at 52%. Age, size, and the presence of atrial isomerism were univariate predictors of mortality. Multivariable analysis identified only univentricular hearts and associated cardiac lesions as predictors of operative mortality. Pulmonary artery (n = 16) and arteriopulmonary (n = 7) shunting strategies for complex cases resulted in less than 30% long-term survival.
Despite improvement in survival for simple cases, management of total anomalous pulmonary venous drainage with single-ventricle hearts or other associated cardiac lesions remains problematic.
近期报告指出，孤立性完全性肺静脉异位引流的手术治疗效果有所改善。复杂病例（合并其他心脏畸形）的报告较少，且死亡率较高。
回顾性研究纳入了1982年至1996年间连续接受完全性肺静脉异位引流治疗的170例患者：44例为“复杂”病例（合并严重相关心脏病变），126例为“简单”病例。
简单病例的手术死亡率从26%降至8%，复杂病例的死亡率保持在52%不变。年龄、体型和心房异构的存在是死亡率的单因素预测指标。多变量分析仅确定单心室心脏和相关心脏病变是手术死亡率的预测指标。复杂病例采用肺动脉（n = 16）和动脉肺（n = 7）分流策略，长期生存率低于30%。
尽管简单病例的生存率有所提高，但单心室心脏或其他相关心脏病变的完全性肺静脉异位引流的治疗仍然存在问题。
