Contemporary Outcomes of Surgical Repair of Total Anomalous Pulmonary Venous Connection in Patients With Heterotaxy Syndrome.
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.
Cardiothoracic Surgery, Cincinnati Children's Hospital, Cincinnati, Ohio.
Muhammad S
MS
Khan
Cardiothoracic Surgery, Cincinnati Children's Hospital, Cincinnati, Ohio.
Roosevelt
R
Bryant
Duke Clinical Research Institute, Duke University School of Medicine, Durham, North Carolina.
Sung H
SH
Kim
Duke Clinical Research Institute, Duke University School of Medicine, Durham, North Carolina.
Kevin D
KD
Hill
Johns Hopkins Children's Heart Surgery, All Children's Hospital, St. Petersburg, Florida.
Jeffrey P
JP
Jacobs
Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland.
Marshall L
ML
Jacobs
Department of Pediatrics and Communicable Diseases, University of Michigan, Ann Arbor, Michigan.
Sara K
SK
Pasquali
Cardiothoracic Surgery, Cincinnati Children's Hospital, Cincinnati, Ohio. Electronic address: david.morales@cchmc.org.
David L S
DL
Morales
D005260:Female; D005500:Follow-Up Studies; D059446:Heterotaxy Syndrome / Q000150:complications* / Q000401:mortality / Q000601:surgery; D017052:Hospital Mortality / Q000639:trends; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D009656:North America / Q000453:epidemiology; D011652:Pulmonary Circulation; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000601:surgery; D011668:Pulmonary Veno-Occlusive Disease / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D016896:Treatment Outcome; D014656:Vascular Surgical Procedures / Q000379:methods*