Repair of “Simple” Total Anomalous Pulmonary Venous Connection: A Review From the Pediatric Cardiac Care Consortium

Abstract

Background Outcomes for repair of Total Anomalous Pulmonary Venous Connection (TAPVC) from individual institutions suggest a significant improvement in mortality over the last several decades. The purpose of this study is to review the outcomes following repair of TAPVC from a large multi-institutional registry. Methods A retrospective review of the multi-institutional database, the Pediatric Cardiac Care Consortium (PCCC) was used to identify patients with the diagnosis of TAPVC who underwent complete correction between the years of 1982 to 2007. Data reviewed included age, decade of primary operation, anatomic type, presentation, and in-hospital mortality. Results Of the 118,084 surgical procedures submitted to the PCCC, 2191 (1.9%) were primary surgical correction of TAPVC. Sixty one percent of the cohort was male with 6.8% reported as premature. Overall in-hospital surgical mortality for simple TAPVC was 13%. Mortality was 20% from 1982 to 1989, 16% from 1990 to 1999, and 8% from 2000 to 2007. Obstruction to the anomalous pulmonary venous connection occurred in 29% with a mortality of 26%. Conclusions Surgical outcomes from repair of congenital cardiac anomalies have significantly improved over the last several decades. Multi-institutional large databases are needed to confirm results published from single institutional experiences. Although improvements in surgical repair of TAPVC have occurred over the last three decades, specific subtypes still experience significant mortality.

Surgical Outcomes of Total Anomalous Pulmonary Venous Connection Repair: A 22-Year Experience

“…In addition, there was a slight tendency for a greater incidence of postoperative PVO (30% vs. 7.4%, p = 0.11, Table ), which may be related to the higher incidence of preoperative obstruction (Table ). Several authors have confirmed our findings, that when TAPVC is associated with major CHD, early and long‐term outcomes remain poor. Primary sutureless technique repair in this group has been suggested in order to avoid PVO, and it has been advocated to repair PVO as early as possible to avoid pulmonary hypertension.…”

“…[1][2][3][4] Surgical repair is usually performed in the neonatal age and there has been a continuous improvement in mortality and morbidity of surgery for isolated TAPVC. 1,2,7,8 On the contrary, when compared to complex CHD (Group 2 in our series), especially UVH anomalies and preoperative PVO, TAPVC repair is still associated with significant operative mortality and late morbidity. [7][8][9][10][11][12][13] We have reviewed our experience of more than two decades, in order to analyze early and late results, and predictors of outcomes.…”

“…1,2,7,8 On the contrary, when compared to complex CHD (Group 2 in our series), especially UVH anomalies and preoperative PVO, TAPVC repair is still associated with significant operative mortality and late morbidity. [7][8][9][10][11][12][13] We have reviewed our experience of more than two decades, in order to analyze early and late results, and predictors of outcomes. As described previously, low weight at operation (<3 kg), 14 use of DHCA, and prolonged ACC clamp time (> 60 minutes) 9,10 have been associated with increased operative mortality ( Table 4).…”

“…TAPVC is a rare form of CHD that can occur isolated or in association with various cardiac malformations, including UVH cardiac anomalies, and heterotaxy syndrome, and it is almost always lethal within the first months of life if no surgical treatment is performed . Surgical repair is usually performed in the neonatal age and there has been a continuous improvement in mortality and morbidity of surgery for isolated TAPVC . On the contrary, when compared to complex CHD (Group 2 in our series), especially UVH anomalies and preoperative PVO, TAPVC repair is still associated with significant operative mortality and late morbidity …”

“…Surgical repair is usually performed in the neonatal age and there has been a continuous improvement in mortality and morbidity of surgery for isolated TAPVC . On the contrary, when compared to complex CHD (Group 2 in our series), especially UVH anomalies and preoperative PVO, TAPVC repair is still associated with significant operative mortality and late morbidity …”