Document Detail

Success and limitations of right ventricular sinus myectomy for pulmonary atresia with intact ventricular septum.
MedLine Citation:
PMID:  18805279     Owner:  NLM     Status:  MEDLINE    
OBJECTIVES: Right ventricular sinus myectomy has been proposed for pulmonary atresia with intact ventricular septum for morphology falling within the uncertain area for eventual biventricular repair. Our objective was to evaluate right ventricular sinus myectomy by characterizing the morphologic spectrum of these patients, determining whether biventricular repair was achieved, ascertaining growth of right-sided structures, and assessing survival. METHODS: We evaluated medical records, all imaging studies, and follow-up data (complete in all but 1 patient) from 43 patients with pulmonary atresia with intact ventricular septum treated from October 1993 to July 2005, 16 of whom underwent right ventricular sinus myectomy. Serial echocardiographic measurements of right-sided cardiac structures were converted to Z values to estimate their growth relative to somatic growth. RESULTS: Patients undergoing right ventricular sinus myectomy had mild-to-moderate right ventricular size diminution (grade -1.2 +/- 3.2) and a tricuspid valve Z value of -4.9 +/- 1.9. Thirteen (87%) of the 16 patients achieved biventricular repair. After right ventricular sinus myectomy, mean right ventricular cavity size grade increased to 1.4 +/- 0.66, but the tricuspid valve Z value did not change appreciably over time. Five-year survival after sinus myectomy was 85%; late deaths were in patients with the smallest tricuspid valves at presentation (Z value < -7). CONCLUSIONS: Right ventricular sinus myectomy in the uncertain area for biventricular repair of pulmonary atresia with intact ventricular septum leads to immediate increase in right ventricular volume. It, in combination with establishing right ventricle-pulmonary trunk continuity, allowed early biventricular repair in 87% of patients. However, tricuspid valve growth in relation to somatic growth was minimal. Thus, small tricuspid valve size might limit the long-term success of biventricular repair achieved by means of right ventricular sinus myectomy.
Roosevelt Bryant; Edward R Nowicki; Roger B B Mee; Jeevanantham Rajeswaran; Brian W Duncan; Geoffrey L Rosenthal; Uthara Mohan; Muhammad Mumtaz; Eugene H Blackstone
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Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, Non-U.S. Gov't     Date:  2008-06-09
Journal Detail:
Title:  The Journal of thoracic and cardiovascular surgery     Volume:  136     ISSN:  1097-685X     ISO Abbreviation:  J. Thorac. Cardiovasc. Surg.     Publication Date:  2008 Sep 
Date Detail:
Created Date:  2008-09-22     Completed Date:  2008-10-16     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0376343     Medline TA:  J Thorac Cardiovasc Surg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  735-42, 742.e1-2     Citation Subset:  AIM; IM    
Department of Thoracic and Cardiovascular Surgery, Cleveland Clinic, Cleveland, Ohio 44195, USA.
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MeSH Terms
Cardiac Surgical Procedures / methods
Child, Preschool
Heart Septum
Heart Ventricles / surgery*
Pulmonary Atresia / mortality,  surgery*
Tricuspid Valve / pathology

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