Surgical results for functional univentricular heart with total anomalous pulmonary venous connection over a 25-year experience.
BACKGROUND
Surgical results for functional univentricular heart with total anomalous pulmonary venous connection (TAPVC) have been unsatisfactory to date.


METHODS
During a 25-year period until December 2009, 207 TAPVC patients underwent surgical repair at our institute, including 56 with a univentricular heart. The 10-year survival rate was 51.1% with univentricular heart and 84.7% with biventricular heart (p<0.0001; log-rank, 27.6). Surgical outcomes and risk factors for early and late death after TAPVC repair in univentricular hearts were retrospectively analyzed.


RESULTS
Patients were aged 3.8±4.3 years and weighed 12.3±10.7 kg at operation. Preoperative diagnoses included heterotaxy syndrome in 55, asplenia in 48, preoperative pulmonary venous obstruction in 35, and pulmonary atresia in 20. TAPVC was classified as I in 22, II in 26, III in 5, and IV in 3. Concomitant procedures included Fontan procedure in 29, bidirectional Glenn procedure in 5, systemic-pulmonary shunt in 11, and pulmonary artery banding in 5. There were 17 hospital deaths and 11 late deaths. Fontan completion was undertaken in 31 (55.3%). Postoperative pulmonary venous obstruction was found in 15. Multivariate analysis identified TAPVC III and IV and pulmonary atresia as risk factors for hospital death. Univariate analysis identified postoperative pulmonary venous obstruction and concomitant systemic-pulmonary shunt as risk factors for hospital and late death.


CONCLUSIONS
TAPVC III, IV, and pulmonary atresia are risk factors for early postoperative death. Intensive intervention, including perioperative management and operation, is required in these complex patients.
Department of Cardiovascular Surgery, Heart Institute of Japan, Tokyo Women's Medical University, Tokyo, Japan. pulechann@yahoo.co.jp
Yuki
Y
Nakayama
Takeshi
T
Hiramatsu
Yusuke
Y
Iwata
Toru
T
Okamura
Takeshi
T
Konuma
Goki
G
Matsumura
Kenji
K
Suzuki
Kyoko
K
Hobo
Toshio
T
Nakanishi
Hiromi
H
Kurosawa
Kenji
K
Yamazaki
D000015:Abnormalities, Multiple / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D003914:Dextrocardia / Q000601:surgery; D004310:Double Outlet Right Ventricle / Q000601:surgery; D005260:Female; D018729:Fontan Procedure / Q000706:statistics & numerical data*; D006352:Heart Ventricles / Q000002:abnormalities* / Q000601:surgery; D059446:Heterotaxy Syndrome / Q000601:surgery; D006801:Humans; D007223:Infant; D007564:Japan / Q000453:epidemiology; D053208:Kaplan-Meier Estimate; D008297:Male; D011183:Postoperative Complications / Q000209:etiology / Q000401:mortality / Q000601:surgery; D018633:Pulmonary Atresia / Q000601:surgery; D011668:Pulmonary Veno-Occlusive Disease / Q000209:etiology / Q000401:mortality / Q000601:surgery; D012189:Retrospective Studies; D012307:Risk Factors; D012587:Scimitar Syndrome / Q000601:surgery*; D015996:Survival Rate; D014188:Transposition of Great Vessels / Q000601:surgery; D016896:Treatment Outcome