Non-modifiable Patient Factors at Bidirectional Cavopulmonary Anastomosis are Associated with Failure of Successful Palliation for Single Ventricle Physiology
W. Hampton Gray1, Michael E Bowdish1, John D Cleveland1, Wendy J Mack2, *Winfield J Wells1, Vaughn A Starnes1, *S. Ram Kumar1
1Division of Cardiac Surgery, Department of Surgery, Keck School of Medicine of the University of Southern California; Heart Institute, Children's Hospital of Los Angeles, Los Angeles, CA;2Department of Preventative Medicine, Keck School of Medicine of the University of Southern California, Los Angeles, CA
OBJECTIVE: Outcomes of surgical palliation for complex single ventricle (SV) physiology continue to improve. Despite these improvements, a subset of these patients fails to reach final stage palliation. Early identification of these patients will allow for appropriate modification of therapeutic approaches to improve outcomes. We sought to determine factors at second stage SV palliation, bidirectional cavopulmonary anastomosis (BDCA), that can predict failure of final stage palliation.
METHODS: We retrospectively reviewed patients who underwent BDCA at our institution between 2007 and 2016. Preoperative patient, clinical and hemodynamic variables at BDCA were analyzed. Successful palliation was defined as hospital survival following completion Fontan or conversion to 1.5- or two-ventricle physiology. Mortality prior to or at final stage palliation or need for transplantation was designated as failed palliation. Continuous data are presented as median and interquartile range and cox regression analysis was used to identify predictive variables.
RESULTS: 424 patients (193 girls) underwent BDCA at 7 (5.5 - 8.9) months of age and a weight of 6.5 (5.7 - 7.7) kg. Of these, 44 (10%) had primary BDCA while 380 (90%) underwent prior palliative procedures. 212 (50%) patients had a dominant right ventricle, 61 (14%) heterotaxy, 35 (8%) a genetic syndrome and 70 (16%) prematurity. Prior to BDCA, 119 (28%) patients required home oxygen and 36 (9%) sildenafil. Mean pulmonary artery pressure was 13 (10-15) mm Hg, pulmonary vascular resistance 1.8 (1.4-2.3) Wood Units/sqm, and 56 (13%) patients had more than mild ventricular dysfunction. 340 (80%) patients have been successfully palliated - 315 (74%) Fontan, 22 (5%) one-and-a-half and 3 (1%) two-ventricle repairs. 31 (7%) patients are candidates for and are awaiting final stage palliation and 16 (3.8%) were lost to follow-up. Failure to complete final stage palliation was 8.7% (n=37) - 13 (3%) died in-hospital after BDCA, 18 (4%) died following discharge prior to final palliation, 5 (1%) died in-hospital following final stage palliation and 1 underwent transplantation. Median time from BDCA to final stage palliation was 34.6 (28.1-42.5) months. Freedom from failed palliation at 12, 24, and 36 months following BDCA were 94%, 93% and 91%, respectively. On Cox regression analysis, risk factors for failed palliation were dominant right ventricle [hazard ratio (HR)=3.39, 95% confidence interval (CI) 1.3-8.7], lower weight (corrected for age) at BDCA (HR=0.67, 95% CI 0.45-0.98), and Down's Syndrome (HR=7.32, 95% CI 1.5-36.4).
CONCLUSION: A large majority of patients with complex SV physiology can be successfully surgically palliated. In our analysis, non-modifiable patient factors prior to BDCA predict failure of successful palliation. Our data allow us to appropriately risk stratify patients prior to BDCA and identify patients in whom alternative management strategies may be required to improve outcomes.
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