Document Detail

Severe pulmonary hypertension without right ventricular failure: the unique hearts of patients with Eisenmenger syndrome.
MedLine Citation:
PMID:  11779519     Owner:  NLM     Status:  MEDLINE    
Why adults with the Eisenmenger syndrome fare so much better than other patients with severe pulmonary hypertension is not known, but may be related to unique hemodynamics found only in these patients and in normal fetuses. We used echocardiography to evaluate ventricular morphology and function in 80 subjects: 45 cyanotic adults and 5 cyanotic adolescents with Eisenmenger syndrome, 10 infants with nonrestrictive ventricular septal defect and left-to-right shunt flow (pre-Eisenmenger phase), and 20 fetuses with structurally normal hearts. Cross-sectional morphology of the hearts was the same in all 4 groups with a flat ventricular septum throughout the cardiac cycle and equal thickness of the right and left ventricular free walls (regression slope 0.98, r = 0.97, p <0.0001). This morphology was the same in patients independent of age, defect type, and ventricular function. Right ventricular fractional area change was slightly inferior to that of the left ventricle but normal in most patients with Eisenmenger syndrome (0.47 +/- 0.14 vs 0.51 +/- 0.13, p <0.01). Overall, there was a highly significant linear relation between right and left ventricular function (r = 0.81, p <0.0001). The hearts of patients with Eisenmenger syndrome are more like normal fetal hearts than normal adult hearts. Because of the unique cardiovascular hemodynamics, regression of right ventricular wall thickness does not occur and is likely the reason that patients with Eisenmenger syndrome fare so much better than other adults with severe pulmonary hypertension.
William E Hopkins; Alan D Waggoner
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The American journal of cardiology     Volume:  89     ISSN:  0002-9149     ISO Abbreviation:  Am. J. Cardiol.     Publication Date:  2002 Jan 
Date Detail:
Created Date:  2002-01-07     Completed Date:  2002-01-25     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  0207277     Medline TA:  Am J Cardiol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  34-8     Citation Subset:  AIM; IM    
Cardiology Unit, University of Vermont College of Medicine, Burlington, Vermont 05401, USA.
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MeSH Terms
Echocardiography, Doppler
Eisenmenger Complex / complications,  physiopathology*,  ultrasonography
Fetal Diseases / physiopathology
Hypertension, Pulmonary / complications,  physiopathology*
Infant, Newborn
Middle Aged
Ventricular Dysfunction, Right / physiopathology

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