Type IV Total Anomalous Pulmonary Venous Connection

Abstract

Background: 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. Methods: 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). Results: 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. Conclusion: 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.