Clinical analysis of 121 patients with hypertrophic cardiomyopathy (original) (raw)
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.
Ventricular Arrhythmias and Left Ventricular Hypertrophy in Hypertrophic Cardiomyopathy
Arquivos Brasileiros de Cardiologia, 2013
Background: In hypertrophic cardiomyopathy (HCM), the degree of left ventricular hypertrophy (LVH) could influence the development of ventricular arrhythmias. Objective: In HCM, analyze the association between the occurrence of ventricular arrhythmias on Holter electrocardiogram (Holter ECG) and the degree of LVH determined by maximum wall thickness (MWT) and mass index (MI) on echocardiography. Methods: Fifty-four consecutive patients with HCM underwent 24-hour Holter ECG and echocardiography for assessment of degree of LVH through MWT and MI. Two levels were established for the occurrence of ventricular arrhythmias: I-isolated or paired extrasystoles and II-non-sustained ventricular tachycardia (NSVT). Results: In 13 patients (24%) with NSVT (level II), there was a higher frequency of left ventricular (LV) MWT ≥ 21 mm (n = 10, 77%, 25 ± 4 mm) and LVMI≥144g/m² (n = 10, 77%, 200 ± 30 g/m²), in comparison with those presenting with only extrasystoles (level I) (n = 41, 76%), in which these measures were identified in, respectively, 37 % (n = 15, 23 ± 1 mm), p = 0.023, and 39% (n = 16, 192 ± 53 g / m²) of the cases p = 0.026. The cutoff values mentioned were determined by the ROC curve with a 95% confidence interval. NSVT was more common in patients with LVMWT ≥ 21 mm and LVMI ≥ 144 g/m² (8 of 13, 62%) than in those with one (4 of 13, 31%) or none (1 of 13; 8%) echocardiographic variables above cutoff values (p = 0.04). Conclusion: In HCM, the occurrence of ventricular arrhythmias on Holter-ECG was associated with the degree of LVH assessed by echocardiography through MWT and MI (Arq Bras Cardiol. 2013; 100(5):452-459).
Heart, 1983
The manifestations and workload in a district general hospital cardiac unit of 39 unselected cases of hypertrophic cardiomyopathy over a five year period are reported. The "typical" form with asymmetrical septal hypertrophy and a gradient was found in only one third of patients, serious ventricular arrhythmias were probably no more common than in the general population, and no deaths occurred during a relatively short follow up (mean 3 1 years). It is concluded that although hypertrophic cardiomyopathy occupies a not insignificant proportion of cardiac workload, unselected cases presenting to a district general hospital represent a relatively mild disease without a grave prognosis.
Background: Hypertrophic cardiomyopathy (HCM) is a genetic disorder with the prevalence of 1 in 500 globally. HCM is clinically characterized by thickening of the wall of the heart, predominantly left ventricle (LV), and interventricular septum (IVS). Our study aims to report the demographical, clinical and genetic profile of Indian HCM patients. Methods: HCM patients were recruited on the basis of WHO criteria. The clinical phenotypes were analyzed using electrocardiography, two-dimensional electrocardiography, and hotspot region of ...
The American Journal of Cardiology, 1981
Retrospective analysis of the clinical course of 254 patients with hypertrophic cardiomyopathy, followed up for 1 to 23 years (mean 6), disclosed that 58 had died, 32 of them suddenly. The 196 survivors were compared with the 32 patients who died suddenly and with the 38 who died suddenly or with heart failure. The combination of young age (14 years or less), syncope at diagnosis, severe dyspnea at last follow-up and a family history of hypertrophic cardiomyopathy and sudden death best predicted sudden death (false negative rate 30 percent, false positive rate 27 percent). A "malignant" family history was associated with poor prognosis, particularly in the younger patients; a family history of hypertrophic cardiomyopathy without sudden death was more frequent in the survivors (12 percent) than in the dead (5 percent). Patients who had a diagnosis in childhood were usually asymptomatic, had an unfavorable family history and a 5.9 percent annual mortality rate. In those aged 15 to 45 years at diagnosis, there was a 2.5 percent annual mortality rate and syncope was the only prognostic feature. Among those diagnosed between age 45 and 60 years, dyspnea and exertional chest pain were more common in the patients who died, and the annual mortality rate was 2.6 percent. Poor prognosis was better predicted by the history at the time of diagnosis and by changes in symptoms during follow-up than by any electrocardiographic or hemodynamic measurement. Hypertrophic cardiomyopathy, first recognized clinically by Brock,' was characterized by Goodwin et al." and Braunwald et a1.3 At the Royal Postgraduate Medical School sudden death was the most common mode of death.4 The purpose of this study is to relate specific clinical, electrocardiographic and hemodynamic features to prognosis and to examine the evidence that a family history of the disease," blackouts5 and diagnosis .in childhood6 were of ill omen. Methods Study patients: Retrospective analysis to assess the prognostic value of clinical, electrocardiographic and hemodynamic features at diagnosis and clinical features at last follow-up was performed on 254 patients with hypertrophic cardiomyopathy who were diagnosed and followed up at the Royal Postgraduate Medical School. Although noncardiac and cardiac surgical deaths are discussed, the comparative analysis included only those patients who died with heart failure or suddenly. The clinical information was always taken from observations by one of us. The diagnosis of hypertrophic cardiomyopathy was based on typical clinical features,2,3 M mode echocardiography and left ventricular angiography.* In 228 patients the diagnosis was established by left ventricular angiography. Twenty-six patients were diagnosed noninvasively: Ten patients were first degree relatives of propositi and had clinical, electrocardiographic or echocardiographic features consistent with hypertrophic cardiomyopathy, whereas 16 patients had the typical clinical and echocardiographic features of hypertrophic cardiomyopathy and a left ventricular outflow tract gradient.3*7*9 We do not believe that hypertrophic cardiomyopathy without a gradient can always be clinically or