Chin L Poh1, Mary Xu2, John C Galati3, Ajay J Iyengar2, Michael Cheung4, Christian P Brizard5, Igor E Konstantinov5, Yves d'Udekem6. 1. University of Melbourne, Victoria, Australia; Department of Cardiac Surgery, Royal Children's Hospital, Victoria, Australia. 2. University of Melbourne, Victoria, Australia; Murdoch Children's Research Institute, Victoria, Australia. 3. The Clinical Epidemiology and Biostatistics Unit, Murdoch Children's Research Institute, Victoria, Australia; Department of Mathematics and Statistics, La Trobe University, Victoria, Australia. 4. Department of Cardiology, Royal Children's Hospital, Victoria, Australia. 5. Murdoch Children's Research Institute, Victoria, Australia; Department of Cardiac Surgery, Royal Children's Hospital, Victoria, Australia; Department of Pediatrics, University of Melbourne, Melbourne, Victoria, Australia. 6. Murdoch Children's Research Institute, Victoria, Australia; Department of Cardiac Surgery, Royal Children's Hospital, Victoria, Australia; Department of Pediatrics, University of Melbourne, Melbourne, Victoria, Australia. Electronic address: yves.dudekem@rch.org.au.
Abstract
BACKGROUND: Dextrocardia is found in a significant proportion of patients undergoing a single-ventricle repair. Surgical outcomes in this cohort are unclear. METHODS: The records of 41 consecutive patients with single-ventricle physiology and dextrocardia presenting in a single institution from 1990 to 2008 were reviewed. Of this cohort, 19 patients had heterotaxy syndrome. Twenty-five of the 41 patients had atrioventricular valve regurgitation (AVVR) on presentation (mild, 13; moderate, 9; severe, 3). RESULTS: One patient died before intervention. Initial surgical palliation was performed in 31 patients. Four patients died postoperatively and 4 interim deaths occurred between initial palliation and bidirectional cavopulmonary shunt (BCPS). Thirty of the surviving 32 patients underwent BCPS, with 2 perioperative deaths. There were 4 additional deaths before Fontan surgery. Twenty-two of the surviving 25 patients underwent a Fontan procedure. There was 1 postoperative mortality. Survival to the age of 15 years was 56% (95% confidence interval [CI], 39%-70%). Patients with moderate or severe regurgitation had higher mortality if they were managed conservatively rather than by surgery (5 of 6 vs 2 of 6; P=.24). Patients with bilateral BCPS had better operative outcomes and survival compared with peers with unilateral anastomosis (odds ratio, 27; P=.005; 95% CI, 2.7-269). The side of the systemic venous pathway did not seem to influence outcomes. CONCLUSIONS: Surgical outcomes of single-ventricle palliation seem poor in patients with dextrocardia. Aggressive management of congenital AVVR might improve the long-term prognosis.
BACKGROUND:Dextrocardia is found in a significant proportion of patients undergoing a single-ventricle repair. Surgical outcomes in this cohort are unclear. METHODS: The records of 41 consecutive patients with single-ventricle physiology and dextrocardia presenting in a single institution from 1990 to 2008 were reviewed. Of this cohort, 19 patients had heterotaxy syndrome. Twenty-five of the 41 patients had atrioventricular valve regurgitation (AVVR) on presentation (mild, 13; moderate, 9; severe, 3). RESULTS: One patient died before intervention. Initial surgical palliation was performed in 31 patients. Four patients died postoperatively and 4 interim deaths occurred between initial palliation and bidirectional cavopulmonary shunt (BCPS). Thirty of the surviving 32 patients underwent BCPS, with 2 perioperative deaths. There were 4 additional deaths before Fontan surgery. Twenty-two of the surviving 25 patients underwent a Fontan procedure. There was 1 postoperative mortality. Survival to the age of 15 years was 56% (95% confidence interval [CI], 39%-70%). Patients with moderate or severe regurgitation had higher mortality if they were managed conservatively rather than by surgery (5 of 6 vs 2 of 6; P=.24). Patients with bilateral BCPS had better operative outcomes and survival compared with peers with unilateral anastomosis (odds ratio, 27; P=.005; 95% CI, 2.7-269). The side of the systemic venous pathway did not seem to influence outcomes. CONCLUSIONS: Surgical outcomes of single-ventricle palliation seem poor in patients with dextrocardia. Aggressive management of congenital AVVR might improve the long-term prognosis.