Muhammad S. Khan, Roosevelt Bryant III, Sung H. Kim, Kevin D. Hill, Jeffrey P. Jacobs, Marshall L. Jacobs, Sara K. Pasquali, David L. S. Morales
Cincinnati Children’s Hospital. Duke University School of Medicine. All Children’s Hospital. Johns Hopkins University School of Medicine. University of Michigan.
United States
Annals of Thoracic Surgery
Ann Thorac Surg 2015; 99: 2134-2139
DOI: 10.1016/j.athoracsur.2015.02.035
Abstract
Background: Total anomalous pulmonary venous connection (TAPVC) is prevalent in patients with atriovisceral heterotaxy. Although functionally univentricular heart defects are common in heterotaxy syndromes, the extent to which this association influences overall risk for TAPVC repair is undefined. This study examines multiinstitutional experience with TAPVC repair in infants with heterotaxy using a national clinical registry.
Methods: The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) (2002-2012) was queried for patients with heterotaxy syndrome who underwent TAPVC repair, with or without concomitant procedures at age of 90 days or younger. The cohort was divided into single ventricle (SV) and non-single ventricle (non-SV) groups based on STS-CHSD codes. Patient characteristics and in-hospital outcomes were described.
Results: Sixty-five centers reported 261 TAPVC repair operations (females, 115 [44%]; median [interquartile range] age and weight, 7 days [3-19 days] and 3.1 kg [2.7-3.5 kg]). Overall, 180 (69%) patients were identified with asplenia or right atrial isomerism, and 167 (64%) had SV diagnoses. Discharge mortality was 38%. Postoperatively, the median length of stay was 18 days (7-32 days), 20 (8%) patients required extracorporeal membrane oxygenation support, and 11 (4%) had reoperation for pulmonary vein stenosis. Mortality was higher for patients with SV defects (SV, 43% versus non-SV, 30%; p = 0.03). Length of stay, postoperative extracorporeal membrane oxygenation, and reoperation for pulmonary vein stenosis was similar between SV and non-SV groups. Overall, there was no difference in mortality for patients undergoing concomitant systemic-to-pulmonary artery shunt (p = 0.134) or surgery within 48 hours of birth (p = 0.876).
Conclusions: Total anomalous pulmonary venous connection repair in heterotaxy patients carries a high mortality risk, particularly with functionally univentricular physiology. These multiinstitutional data serve as an important benchmark and may be useful for risk stratification and counseling.
Category
Stenosis or Obstruction of Pulmonary Veins Following Surgical Repair of Anomalous Pulmonary Venous Connections
Patient Factors Influencing the Onset, Severity or Outcome of Disease
Length of Life Associated with Pulmonary Venous Obstruction
Year of Publication: 2015
Age Focus: Pediatric
Article Type: Retrospective Observational Cohort Studies (>10 patients)
Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: Yes