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Twenty-one Years and 626 Patients Later: Contemporary Outcomes After Bidirectional Cavopulmonary Anastomosis
*Lauren C Kane, Luis E De León, Ruth Ackah, Rohini R Sigireddi, Carlos M. Mery, Iki Adachi, Jeffrey S. Heinle, E.Dean McKenzie, Charles D. Fraser, Jr.
Texas Childrens Hospital/Baylor College of Medicine, Houston, TX

OBJECTIVES:
The bidirectional cavopulmonary connection (BCPC) is a standard procedure in the multistage palliation of complex congenital heart disease (CHD), with varying reported rates of interstage mortality among centers. The purpose of this study was to determine the outcomes following BCPC and examine risk factors affecting transplant-free survival to Fontan palliation in a large single-institutional cohort.
METHODS:
Patients undergoing BCPC from 1995 to 2016 were included. Competing risk analysis was performed to model events after BCPC (failure and Fontan completion). Transplant-free survival to Fontan was analyzed using the Kaplan-Meier method, and risk factors for transplant or death were examined using log-rank tests and Cox-regression models.
RESULTS:
The cohort included 626 patients. Median age at surgery was 9 months, and median weight was 7 kg. Median follow-up was 3.4 years (5 days - 14.6 years). Dominant ventricular morphology was left in 269 (43%), right in 313 (50%) and biventricular (BV) in 44 (7%) patients. Seventy-nine (13%) patients had primary BCPC and 547 (87%) had prior palliation. Operative mortality was 2%. Five years following BCPC, 370 (59%) patients have undergone Fontan or BV repair, 55 (9%) have failed (takedown, transplant or death), and 202 (32%) were alive waiting or were not deemed candidates for Fontan (Figure). Seventy-six patients (12%) underwent BCPC during the same hospitalization as stage I palliation. When comparing these patients to those who had surgery electively (n=550), outcomes were worse in the perioperative period [operative mortality: 8/76 (10%) vs 6/550 (1%), p<0.001]. Long-term results however, were comparable as evidenced by similar reintervention rates [24 (32%) vs 169 (31%), p=0.88] and equivalent survival after Fontan [35/37 (95%) vs 374/383 (98%), p=0.51]. Overall interstage attrition in our cohort was 9%. Actuarial 2-, 5-, and 8-year transplant-free survival following BCPC was 93%, 90% and 83%. On multivariate analysis, risk factors for death or transplant prior to Fontan were moderate or severe atrioventricular valve regurgitation (p<0.001), moderate or severe ventricular dysfunction (p=0.04), and a dominant right ventricle (RV) (p=0.003).
CONCLUSIONS:
Survival after BCPC for complex CHD in infants has increased with improvement in surgical techniques and perioperative management of these patients. Patients who undergo BCPC during their index hospitalization have a more complicated perioperative course, however there appears to be no difference in long-term outcomes. Causes of interstage attrition are varied and the majority of deaths and transplantations appear to be influenced by ventricular function and morphological dominance. Patients with moderate or severe ventricular dysfunction, moderate or severe atrioventricular valve regurgitation and a dominant RV, appear to be at higher risk for failure.


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