St Louis James D, Turk Elizabeth M, Jacobs Jeffrey P, O'Brien James E
1 Division of Cardiac Surgery, Department of Surgery, Children's Mercy Hospitals and Clinics, Kansas City, MO, USA.
2 Division of Cardiac Surgery, Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
World J Pediatr Congenit Heart Surg. 2017 Mar;8(2):142-147. doi: 10.1177/2150135116682456.
Mortality associated with correction of type IV total anomalous pulmonary venous connection (TAPVC) is generally reported in combination with other anatomic types. The objective of this study is to review surgical outcomes associated with the repair of type IV TAPVC by analyzing a multi-institutional cohort specific for this group. We also analyze patient-specific variables that may contribute to poor operative outcomes.
A retrospective review of the Pediatric Cardiac Care Consortium (PCCC) registry identified patients who underwent repair of type IV TAPVC between 1982 and 2007. Variables reviewed included gender, prematurity, age at repair, anatomic pattern, presence of obstruction, associated anomalies, and operative mortality. Subclassifications were defined as type IV A (2+2 pattern), type IV B (3+1 pattern), and type IV C (bizarre).
Of the 2,248 patients with the diagnosis of TAPVC, 215 belonged to type IV. For type IV, the overall unadjusted mortality was 26%. There was no difference in mortality based on the particular anatomic drainage pattern. Twenty-eight percent had partial obstruction of the pulmonary venous return, with no patient having complete obstruction. Patients with obstruction had a significantly greater mortality than those without obstruction (39% vs 20%, P = .005). Approximately 16% of patients who present with obstruction of some pulmonary vein(s) underwent an emergency repair.
Type IV TAPVC is a rare disease with a diverse anatomic presentation. Even though a small number of the patients with obstruction underwent emergent repair, mortality remained significant. This likely represents the intrinsic lung pathology that must be considered in the postoperative period.
与IV型完全性肺静脉异位连接（TAPVC）矫治相关的死亡率通常与其他解剖类型合并报道。本研究的目的是通过分析针对该组的多机构队列，回顾IV型TAPVC修复的手术结果。我们还分析了可能导致手术效果不佳的患者特异性变量。
对儿童心脏护理联盟（PCCC）登记处进行回顾性研究，确定1982年至2007年间接受IV型TAPVC修复的患者。回顾的变量包括性别、早产、修复时年龄、解剖模式、梗阻情况、相关异常和手术死亡率。亚分类定义为IV A型（2+2模式）、IV B型（3+1模式）和IV C型（奇异型）。
在2248例诊断为TAPVC的患者中，215例属于IV型。对于IV型，总体未经调整的死亡率为26%。基于特定解剖引流模式的死亡率无差异。28%的患者存在部分肺静脉回流梗阻，无患者完全梗阻。有梗阻的患者死亡率显著高于无梗阻的患者（39%对20%，P = .005）。约16%出现部分肺静脉梗阻的患者接受了急诊修复。
IV型TAPVC是一种罕见疾病，解剖表现多样。尽管少数有梗阻的患者接受了急诊修复，但死亡率仍然很高。这可能代表了术后必须考虑的内在肺部病理情况。
