BACKGROUND: Aortic atresia has been implicated as a risk factor for decreased survival after stage 1 palliation. Prior studies evaluating the association of anatomic subtypes and mortality report conflicting results. Our objective was to determine if mitral valve patency with aortic atresia is associated with increased mortality in hypoplastic left heart syndrome (HLHS). METHODS: All patients (n = 72) with classic HLHS born between August 1996 and May 2002, who underwent stage I Norwood palliation, had presenting echocardiograms reviewed for patency of the mitral and aortic valves. The cohort was divided into three groups: aortic atresia-mitral atresia (AA-MA), aortic stenosis-mitral stenosis (AS-MS), and aortic atresia-mitral stenosis (AA-MS). Analysis included analysis of variance techniques for continuous variables and the 2-tailed Fisher exact test for categoric variables. Survival analysis was performed using the Kaplan-Meier method with log-rank testing. RESULTS: Of the 72 patients, 36 had AA-MA, 17 had AS-MS, and 19 had AA-MS. The stage 1 hospital survival was 92% for the entire cohort, 97% for AA-MA, 94% for AS-MS, and 79% for AA-MS (p = 0.05). Interstage mortality was 8% (6 of 72) overall, but was 21% (4 of 19) for AA-MS versus 6% (2 of 36) for AA-MA and 0% for AS/MS. Overall survival to date was 79% for the entire cohort but was 58% for AA-MS, 86% for AA/MA, and 88% for AA-MS (p = 0.015). Aortic atresia alone was not associated with increased mortality (p = 0.2). CONCLUSIONS: In patients with HLHS, aortic atresia was associated with increased mortality only in the presence of a patent mitral valve. The highest incidence of death was observed primarily during the interstage period.
BACKGROUND:Aortic atresia has been implicated as a risk factor for decreased survival after stage 1 palliation. Prior studies evaluating the association of anatomic subtypes and mortality report conflicting results. Our objective was to determine if mitral valve patency with aortic atresia is associated with increased mortality in hypoplastic left heart syndrome (HLHS). METHODS: All patients (n = 72) with classic HLHS born between August 1996 and May 2002, who underwent stage I Norwood palliation, had presenting echocardiograms reviewed for patency of the mitral and aortic valves. The cohort was divided into three groups: aortic atresia-mitral atresia (AA-MA), aortic stenosis-mitral stenosis (AS-MS), and aortic atresia-mitral stenosis (AA-MS). Analysis included analysis of variance techniques for continuous variables and the 2-tailed Fisher exact test for categoric variables. Survival analysis was performed using the Kaplan-Meier method with log-rank testing. RESULTS: Of the 72 patients, 36 had AA-MA, 17 had AS-MS, and 19 had AA-MS. The stage 1 hospital survival was 92% for the entire cohort, 97% for AA-MA, 94% for AS-MS, and 79% for AA-MS (p = 0.05). Interstage mortality was 8% (6 of 72) overall, but was 21% (4 of 19) for AA-MS versus 6% (2 of 36) for AA-MA and 0% for AS/MS. Overall survival to date was 79% for the entire cohort but was 58% for AA-MS, 86% for AA/MA, and 88% for AA-MS (p = 0.015). Aortic atresia alone was not associated with increased mortality (p = 0.2). CONCLUSIONS: In patients with HLHS, aortic atresia was associated with increased mortality only in the presence of a patent mitral valve. The highest incidence of death was observed primarily during the interstage period.
Authors: James S Tweddell; Lynn A Sleeper; Richard G Ohye; Ismee A Williams; Lynn Mahony; Christian Pizarro; Victoria L Pemberton; Peter C Frommelt; Scott M Bradley; James F Cnota; Jennifer Hirsch; Paul M Kirshbom; Jennifer S Li; Nancy Pike; Michael Puchalski; Chitra Ravishankar; Jeffrey P Jacobs; Peter C Laussen; Brian W McCrindle Journal: J Thorac Cardiovasc Surg Date: 2012-02-15 Impact factor: 5.209
Authors: Anastasios C Polimenakos; Shyam K Sathanandam; Tarek S Husayni; Chawki F El Zein; David A Roberson; Michel N Ilbawi Journal: Pediatr Cardiol Date: 2011-06-05 Impact factor: 1.655
Authors: Meryl S Cohen; Nicholas Dagincourt; Victor Zak; Jeanne Marie Baffa; Peter Bartz; Andreea Dragulescu; Gul Dudlani; Heather Henderson; Catherine D Krawczeski; Wyman W Lai; Jami C Levine; Alan B Lewis; Rachel T McCandless; Richard G Ohye; Sonal T Owens; Steven M Schwartz; Timothy C Slesnick; Carolyn L Taylor; Peter C Frommelt Journal: J Am Soc Echocardiogr Date: 2018-07-03 Impact factor: 5.251
Authors: Bhavya Trivedi; P Brian Smith; Piers C A Barker; James Jaggers; Andrew J Lodge; Ronald J Kanter Journal: Am Heart J Date: 2011-01 Impact factor: 4.749
Authors: Shyam Sathanandam; Wei Cui; Nguyen Vu Nguyen; Tarek S Husayni; Andrew H Van Bergen; Imran Sajan; Chawki El-Zein; Anastasios Polimenakos; Michel N Ilbawi; David A Roberson Journal: Pediatr Cardiol Date: 2010-09-05 Impact factor: 1.655
Authors: Matthew V Park; Raymond T Fedderly; Peter C Frommelt; Michele A Frommelt; Andrew N Pelech; Robert D B Jaquiss; Kathleen Mussatto; James S Tweddell Journal: Pediatr Cardiol Date: 2012-11-21 Impact factor: 1.655