Clinical analysis of 121 patients with hypertrophic cardiomyopathy (original) (raw)
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Journal of the American College of …, 1983
The distribution of left ventricular hypertrophy was assessed by M-mode and two-dimensional echocardiography in 89 patients with hypertrophic cardiomyopathy. Myocardial thickness was measured in the septum and the free and posterior wall in both the proximal and distal left ventricle. All patients had at least one myocardial region that was hypertrophied. The predominant pattern of hypertrophy was defined as symmetr ic (31%), asymmetric septal (55% ) and distal ventricular (14%). The spectrum of wall thickness measurements between patients with symmetric hypertrophy was wide (1.5 to 4.5 em) and was not related to age. In patients with asymmetric septal hypertrophy , the distribution of hypertrophy conformed to previously described patterns; hypertrophy was localized to the anterior septum (14%) Hypertrophic cardiomyopathy is defined as a heart muscle disorder of unknown origin that is characterized by unexplained hypertrophy of a nondilated left ventricle ( I). During the past decade, the most widely applied diagnostic criteria were derived from the M-mode echocardiogram. These criteria emphasized the demonstration of asymmetric hypertrophy between the upper anterior septum and the left ventricular posterior wall as well as features associated with Icft ventricular pressure gradients, such as systolic anterior motion of the mitral valve and mid-systolic closure of the aortic valve. Recent studies, however, have shown that myocardial regions that are not visualized by the M-mode beam may be hypertrophied in the absence of asymmetric septal hypertrophy (2,3). Thus, using the M-mode technique, the diagnosis and pattern of myocardial hypertrophy may be undetected. Two-dimensional echocardiography permits evaluation of the entire left ventricle (4-6). The or the anterior and posterior septum (35%) or involved both the septum and the left ventricular free wall (51%). The patients with distal ventricular hypertrophy had marked papillary muscle thickening, and only 1 of 12 patients could be correctly diagnosed using M·mode echocardiography ,
The American Journal of Cardiology, 2008
In clinical practice, differential diagnosis among different forms of left ventricular (LV) hypertrophy is not always easy, and hypertrophic cardiomyopathy (HC) can be misdiagnosed. In this study, it was hypothesized that a 3-dimensional echocardiographically derived index of LV regional mass distribution could be useful in differentiating HC from other forms of LV hypertrophy. Sixty-eight subjects underwent 2-and 3-dimensional echocardiography; of these, 20 were healthy volunteers, 18 were top-level athletes, 15 had essential hypertension, and 15 had HC. In off-line analysis, a 12-segment model was generated for segmental mass measurement. A mass dispersion index (MDI) was calculated as the average of the SDs of segmental mass values at the basal, middle, and apical layers. The ratio of ventricular septal thickness to posterior wall thickness was also calculated using 2-dimensional echocardiography. Patients with HC had significantly higher MDI values (1.75 ؎ 0.43) than healthy volunteers (0.39 ؎ 0.13) (p <0.0001), athletes (0.49 ؎ 0.12) (p <0.0001), and patients with hypertension (0.38 ؎ 0.10) (p <0.0001). The ratio of ventricular septal thickness to posterior wall thickness was significantly higher in patients with HC (1.31 ؎ 0.23) than normal subjects (1.04 ؎ 0.05) (p <0.0001), highly trained athletes (1.03 ؎ 0.06) (p ؍ 0.001), and patients with hypertension (1.06 ؎ 0.06) (p ؍ 0.002). However, receiver-operating characteristic analysis showed a higher sensitivity for MDI (93.3% for the cut-off value of 1.13) than the ratio of ventricular septal thickness to posterior wall thickness (66.7% for the cut-off value of 1.20), with excellent specificity for both (100%) in identifying patients with HC. In conclusion, the 3-dimensional echocardiographically derived MDI could be considered a useful and reliable additional tool in differentiating HC from other forms of LV hypertrophy.
American Journal of Cardiology, 2011
is a risk factor for sudden death and a determinant of high mortality. We determined its prevalence, clinical parameters, long-term outcome, and pathologic findings of explanted hearts. We retrospectively analyzed 382 patients with HC; ILVSF was characterized by LV ejection fraction <50% at rest and was identified in 24 patients (6.3%). Patients with ILVSF were younger than patients with normal SF (43.5 ؎ 14.1 vs 55.3 ؎ 20.4 years, p ؍ 0.001) and had larger LV end-diastolic cavity diameter (53.2 ؎ 12.2 vs 43.8 ؎ 6.2 mm, p ؍ 0.001), larger left atrium (51.2 ؎ 6.5 vs 44.3 ؎ 8 mm, p <0.001), and lower fractional shortening (30.7 ؎ 11.1% vs 45.5% ؎ 10.3%, p <0.001). A combined end point (heart failure death or heart transplantation) was considered. Median follow-up was 3 years (1.2 to 6.3). Fourteen patients with ILVSF (58.3%) had the end point compared to 3 (0.8%) with normal SF (p <0.001). In explanted hearts, fibrosis represented 30.5 ؎ 12.5% of the left ventricle; we observed a direct correlation between fibrosis and ventricular dilation (r ؍ 0.794, p ؍ 0.001) and an inverse correlation between fibrosis and ejection fraction (r ؍ ؊0.623, p ؍ 0.023). Number and length density of small arterioles (<50 m in diameter) were significantly decreased. In conclusion, ILVSF in HC has a poor prognosis and is associated with fibrosis and selective decreased development of small arterioles.
Thirty Controversies and Considerations in Hypertrophic Cardiomyopathy
Structural Heart, 2021
More than 60 years after the first description of "asymmetric septal hypertrophy of the heart", hypertrophic cardiomyopathy (HCM) as a disease, has continued to provide clinicians and researchers fertile grounds for debate and investigation. HCM, as a complex disease requiring a multidisciplinary approach, runs the gamut of all we learned in medical school and beyond-physiology, clinical medicine, genetics, imaging, diagnostics, pharmacotherapies, electrophysiology, interventional cardiology, and cardiothoracic surgical interventions. In this review, we discuss thirty contemporary controversies and considerations in HCM.
Journal of the American College of Cardiology, 1986
Ten patients with nonobstructive hypertrophic cardiomyopathy and only mild localized left ventricular hypertrophy who had severe symptoms of cardiac failure are described. During a mean follow-up period of7 years, 6 of these 10 patients showed a substantial increase in left ventricular internal dimension (6 to 15 mm, mean 10) as assessed with M-mode echocardiography, although absolute left ventricular cavitysizeremained within normal limits in 5 of the 6. Four patients demonstrated substantial septal thinning (5 to 14 mm, mean 8). Left ventricular diastolic function, assessed by radionuclide angiography in nine patients, was impaired in eight who showed decreased peak filling rate « 2.5 end-diastolic volumes/s) and prolonged time to peak rate of filling (~180 ms). Furthermore, left ventricular systolic function, usually supernormal in patients with hypertrophic
Clinical and morphologic expression of hypertrophic cardiomyopathy in patients ≥ 65 years of age
The American Journal of Cardiology, 1994
(HC) is most often identified in patients in the second through f'iih decades of life, but has been increasingly recognized in older patients. The present report char acterizes morphologic and clinical features of HC in 134 consecutively studied patients aged 265 years referred to a tertiary center. Echocardiographic or clinical evaluation, or both, was pep formed in 134 patients aged 65 to 65 years (mean 72) at most recent evaluation. Selected findings were compared with those in 64 youthful patients with HC aged 15 to 35 years (mean 25). Most elderly patients (120 of 134,90%) developed marked symptoms that usually became evident after age 55 years; 64 of 120 experienced sustained improvement with medical treatment or operation. Elderly patients had relatively mild left ventricular (LV) wall thickening (20 + 3 mm), generally confined to the septum. In most (i.e., 66%), septal hypertrophy was uniformly distributed with parallel right and left bolt ders and associated with elliptical LV cavity shape; however, in 32%, an inhomogeneously hypertrophied septum bulged into the left ventricle, disrupting normal cavity shape. Dynamic subaortic obstruction was present under basal or provocable conditions in a particularly small LV oufflow tract in 103 of 134 patients (77%), and was usually produced by relatively restricted excursion of the anteriorly displaced mitral leaflets and posterior septal motion. HC is characterized by agmlated differences in both clinical and morphologic expression. Elderly patients with HC characteristically demonstrate onset of cardiac symptoms late in life, as well as distinctive LV morphology and dynamics of oufflow obstruction.
Risk factors for systolic dysfunction and ventricular dilatation in hypertrophic cardiomyopathy
International Journal of Cardiology, 1994
The history of an 18-year-old male with hypertrophic cardiomyopathy (HCM) and ventricular dilatation is presented and the literature on systolic dysfunction and ventricular dilatation in patients with HCM is StatisticaIly analyzed in search of risk factors. The patient was followed for '7 years when he developed recurrent ventricular fibrillation, left ventricular dilatation and low cardiac output. An automatic cardioverter-defibrillator was implanted but the patient died of electro-mechanical dissociation.