Document Detail

Left Ventricular Diastolic Dysfunction in Type I Gaucher Disease: An Echo Doppler Study.
MedLine Citation:
PMID:  25250513     Owner:  NLM     Status:  Publisher    
Type I Gaucher disease (GD1) is an autosomal recessive lysosomal storage disease characterized by multiorgan damage. Left ventricular (LV) involvement has been rarely reported. Accordingly, the aim of the study was to evaluate LV geometry and function in a series of patients with GD1. Eighteen patients with GD1, 18 age- and sex-matched normal controls, and 18 age- and sex-matched hypertensive patients (HTN) were compared by standard echo Doppler examination. LV mass index, relative wall thickness and ejection fraction, transmitral E/A ratio, E velocity deceleration time (DT), atrial filling fraction (AFF = time-velocity integral of A velocity/time-velocity integral of total diastole × 100), E/e' ratio, and left atrial volume index were determined. Nine GD1 patients also exhibited arterial hypertension. The intergroup difference of LV mass index and relative wall thickness was not significant. Transmitral E/A ratio was lower in HTN than in normal controls and GD1 (P < 0.05). GD1 exhibited longer DT than NC and HTN (P = 0.009). AFF was higher in GD1 and HTN compared to NC (P = 0.034). After adjustment for heart rate, GD1 was associated with longer DT (P < 0.001) and greater AFF (P = 0.036), while HTN was associated only with AFF (P = 0.013). No interaction was found between GD1 and HTN. In conclusion, GD1 is associated with subclinical LV diastolic dysfunction, which is independent of the coexistence of arterial hypertension. Subclinical LV impaired relaxation in the context of myocardial infiltrative damage could be the mechanism underlying these alterations.
Francesco Lo Iudice; Antonio Barbato; Riccardo Muscariello; Carlo Di Nardo; Francesco de Stefano; Michelina Sibilio; Pasquale Strazzullo; Giovanni de Simone; Maurizio Galderisi
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2014-9-24
Journal Detail:
Title:  Echocardiography (Mount Kisco, N.Y.)     Volume:  -     ISSN:  1540-8175     ISO Abbreviation:  Echocardiography     Publication Date:  2014 Sep 
Date Detail:
Created Date:  2014-9-24     Completed Date:  -     Revised Date:  2014-9-25    
Medline Journal Info:
Nlm Unique ID:  8511187     Medline TA:  Echocardiography     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
© 2014, Wiley Periodicals, Inc.
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