Hypertrophic cardiomyopathy with aortic dilation: a novel observation.

Published on Dec 1, 2017in European Journal of Echocardiography4.841
· DOI :10.1093/EHJCI/JEW292
Rayan Yousefzai3
Estimated H-index: 3
(UW: University of Wisconsin-Madison),
Anushree Agarwal5
Estimated H-index: 5
(UW: University of Wisconsin-Madison)
+ 12 AuthorsA. Jamil Tajik15
Estimated H-index: 15
(UW: University of Wisconsin-Madison)
Sources
Abstract
Aims Our goal was to identify the prevalence of aortic dilation in patients with hypertrophic cardiomyopathy (HCM), the most prevalent (0.2%) heritable, genetic cardiovascular disease. Aortic dilation also represents a spectrum of familial inheritance. However, data regarding the prevalence of aortic dilation in HCM patients is lacking. Methods and results This is an observational retrospective study of all patients referred to our HCM centre. Aortic dilation was defined based on recent American Society of Echocardiography and European Association of Cardiovascular Imaging published guidelines. Of the 201 HCM patients seen between Jan. 1, 2011 and March 31, 2014, 18 (9.0%) met the definition of aortic dilation. Mean age was 56.3 ± 9.3 years, 77.8% were male, mean ascending aorta diameter was 4.0 ± 0.4 cm in males and 3.8 ± 0.2 cm in females, mean sinuses of Valsalva diameter was 4.2 ± 0.2 cm in males and 3.8 ± 0.4 cm in females, and 13 (72.2%) had left ventricular outflow tract obstruction. HCM patients with dilated aorta were more likely males, less likely hypertensive and had larger left ventricle diameter and more aortic valve regurgitation; remaining characteristics were similar. Conclusion We report a novel observation with 9.0% prevalance of dilated aorta in HCM patients. Further studies are needed to help define the genetic and pathophysiologic basis as well as the clinical implications of this association in a larger group of HCM patients.
References20
Newest
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#2A. Evangelista (Hebron University)H-Index: 1
Last. Marc A.A.M. SchepensH-Index: 41
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OBJECTIVE : Previous studies re - vealed that hypertrophic cardiomyopathy (HCM) patients have impaired aortic elastic properties with contrasting data about aortic dimensions. We aimed to extend our knowledge about this topic, considering tissue Doppler imaging (TDI) and tissue strain. PATIENTS AND METHODS: 25 HCM patients and 25 healthy volunteers matched for age and sex were enrolled.They underwent transthoracic echocardiography to measure aortic dimensions at four levels (Valsalva sinuses, si...
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