The purpose of this echocardiographic study was to evaluate the frequency of restrictive left ventricular filling pattern in dilated cardiomyopathy, as well as its clinical and echocardiographic correlations and prognostic implications. The study included 49 pts (39 males, 10 females) aged 32 to 56 years (mean age: 46 years), with a diagnosis of dilated cardiomyopathy, according to the World Health Organization criteria. Pulsed Doppler echocardiographic analysis was performed at discharge and during a two year follow-up and compared with clinical and 2-dimensional echocardiographic findings. The patients were divided into two groups according to measurement of E decelaration time (DT) of transmitral flow patterns: a restrictive group (Group A-21 pts) with DT < 115 ms. and a non-restrictive group (Group B-28 pts) with DT > 115 ms. Of 49 pts, 15 died during a two-year follow-up, 12 in the restrictive group and only 3 in the non-restrictive group. Eleven of these pts (73%) died of worsening of congestive heart failure and four (27%) died suddenly. The restrictive filling pattern was associated at Doppler study with a higher E wave velocity, lower A wave velocity and higher E/A ratio. Pts in the restrictive group were in a higher New York Heart Association functional class, and had a lower ejection fraction and more severe mitral regurgitation. In addition, they had a significantly larger right ventricle and higher mean pulmonary artery pressure. Mortality rate in the restrictive group was markedly higher than that in the non-restrictive group at 1 year (24% vs. 0%, respectively, p < 0.001) and at 2 years (57% vs. 11%, respectively, p < 0.001). The results from this study indicate that a restrictive left ventricular filling pattern is frequent in dilated cardiomyopathy, and is associated with more severe disease and is the single best clinical predictor for cardiac death in pts with dilated cardiomyopathy.
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Gene
January 2025
Department of Cardiology, Children's Hospital of Nanjing Medical University, Nanjing 210008, China. Electronic address:
Backgroud: The ALMS1 gene is predominantly localized to cilia, particularly in the photoreceptor cells of the retina, auditory neurons, kidneys, and other ciliated structures. Pathogenic mutations in this gene cause Alstrom syndrome (AS), which is characterized by dilated cardiomyopathy, retinal degeneration, neurodeafness, and centripetal obesity. However, the genetic mechanism of the ALMS1 gene remains unclear.
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