Document Detail

Surgical outcome for patients with the mitral stenosis-aortic atresia variant of hypoplastic left heart syndrome.
MedLine Citation:
PMID:  18242265     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: We sought to identify and characterize a subgroup of patients with hypoplastic left heart syndrome who might be at higher risk for stage I failure. METHODS: From January 2001 through December 2006, all patients with hypoplastic left heart syndrome who underwent stage I palliation at Children's Hospital Boston were retrospectively reviewed. The subgroup with the mitral stenosis-aortic atresia variant was studied separately. We evaluated preoperative echocardiographic data, operative characteristics, and postoperative factors associated with death or the need for transplantation. The Kaplan-Meier method was used to assess survival. RESULTS: Thirty-eight (23%) of 165 patients had mitral stenosis-aortic atresia. Hospital mortality or need for transplantation for patients with mitral stenosis-aortic atresia was significantly higher than for other anatomic subgroups (29% vs 7.9%, P = .002). Left ventricle-subepicardial coronary artery communications were present in 20 (53%) patients with mitral stenosis-aortic atresia and were associated with a significantly higher hospital mortality (50% vs 6%, P = .004). No difference in outcome was demonstrated between different sources of pulmonary blood flow. A longer cardiopulmonary bypass time (P = .02) and the need for postoperative extracorporeal membrane oxygenation support (P < .001) were associated with a higher mortality rate. CONCLUSIONS: With improved outcomes in the management of neonates with hypoplastic left heart syndrome, those with the mitral stenosis-aortic atresia variant and left ventricle-subepicardial coronary artery fistulae have emerged as a higher-risk subgroup for failure of stage I palliation. Further investigation is required, and a change in clinical management strategy for this particular subgroup might be warranted.
Vladimiro L Vida; Emile A Bacha; Alesandro Larrazabal; Kimberly Gauvreau; Adam L Dorfman; Gerald Marx; Tal Geva; Audrey C Marshall; Frank A Pigula; John E Mayer; Pedro J del Nido; Francis Fynn-Thompson
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Publication Detail:
Type:  Journal Article     Date:  2007-12-20
Journal Detail:
Title:  The Journal of thoracic and cardiovascular surgery     Volume:  135     ISSN:  1097-685X     ISO Abbreviation:  J. Thorac. Cardiovasc. Surg.     Publication Date:  2008 Feb 
Date Detail:
Created Date:  2008-02-04     Completed Date:  2008-03-10     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0376343     Medline TA:  J Thorac Cardiovasc Surg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  339-46     Citation Subset:  AIM; IM    
Department of Cardiac Surgery, Children's Hospital Boston, Harvard Medical School, 300 Longwood Ave, Boston, MA 02115, USA.
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MeSH Terms
Abnormalities, Multiple / surgery*
Aorta / abnormalities*
Cardiac Surgical Procedures / methods*,  mortality*
Cause of Death
Follow-Up Studies
Heart Defects, Congenital / mortality,  surgery
Hospital Mortality / trends
Hypoplastic Left Heart Syndrome / mortality*,  surgery*
Infant, Newborn
Mitral Valve Stenosis / surgery
Palliative Care / methods
Retrospective Studies
Risk Assessment
Survival Analysis
Treatment Outcome

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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