Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0205700 (ash)
 15,125 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Three cases of hypertrophic cardiomyopathy (HCM) which presented with different modes of appearance of left ventricular hypertrophy are reported. Case 1: A 24-year-old man had three relatives with HCM. At 13 years of age, he showed no electrocardiographic or echocardiographic abnormalities characteristic of HCM. During the ensuing 11 years, he developed asymmetric septal hypertrophy (ASH) and systolic anterior motion of the mitral valve (SAM), with right bundle branch block and T-wave inversion. Cardiac catheterization confirmed the diagnosis of hypertrophic obstructive cardiomyopathy by demonstrating an intraventricular pressure gradient of 25 mmHg. These observations indicate that this case developed abnormal hypertrophy during adolescence on the basis of genetic predisposition of an autosomal dominant trait. Case 2: A 51-year-old woman had three proven and three possibly affected relatives. At 35 years of age, she had a normal electrocardiogram, although the echocardiogram was not available. Now, 16 years later, she had developed ASH with abnormal Q-waves and was diagnosed as having non-obstructive HCM. These suggest that ASH can be manifested as late as during middle-age, even in those with genetic predisposition. Case 3: A 47-year-old woman was diagnosed as having hypertension and her blood pressure was 190/100 mmHg at 40 years of age, though she had no abnormal electrocardiographic findings and heart murmurs. Now, at 47 years of age, she had developed T-wave inversion, ASH, SAM, and an intraventricular pressure gradient of 50 mmHg. Thus, her ASH appeared during middle-age, and was probably provoked by hypertension, though a complete family survey could not be conducted. These three patients' findings indicate that there may be various modes of appearance of left ventricular hypertrophy in HCM. In the majority of patients with genetic predisposition, abnormal hypertrophy may develop during adolescence as in Case 1. In others, it may develop in middle-age, as it did in Case 2. The disease spectrum of HCM may additionally include those who develop abnormal hypertrophy during middle-age, following provocation by hypertension, as in Case 3.
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PMID:[Hypertrophic cardiomyopathy manifesting different modes of illness: report of three cases]. 342 21

Thirteen asymptomatic adults less than 40 years old who showed tall right precordial R waves on the ECG were examined by VCG, M-mode and two-dimensional echocardiography (2D Echo). Common causes of QRS anterior displacement, such as right ventricular enlargement or right bundle branch block, were excluded in each subject. Although each subject was normal at physical examination, 2D Echo revealed areas of left ventricular hypertrophy in eight of these 13 subjects. Four had a prevailing hypertrophy of the basal portion of the interventricular septum, three had an isolated apical hypertrophy, and one had a diffuse concentric left ventricular hypertrophy. Results were normal in five cases. 2D Echo classification was confirmed by heart catheterization findings, when available. The subjects with asymmetric septal hypertrophy showed low-voltage QRS leftward forces on the ECG and VCG. ECGs and VCGs were not useful in differentiating the subjects with atypically distributed left ventricular hypertrophy from the normals: high-voltage QRS leftward forces and T wave abnormalities were evident in some subjects of both groups. Tall right precordial R waves may constitute a marker of hypertrophic cardiomyopathy in asymptomatic young adults. 2D Echo is useful to exactly classify these subjects.
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PMID:Significance of high voltage QRS anterior forces in young asymptomatic adults. Evaluation by wide-angle two-dimensional echocardiography. 622 16

Familial hypertrophic cardiomyopathy is an autosomal dominant genetic disease considered the most common cause of sudden cardiac death in individuals under 35 years old, especially the athletes. This study aimed to investigate the association between the presence of late potentials and a family history of sudden death, syncope, and complex ventricular arrhythmias on patients with hypertrophic cardiomyopathy. A case series study was carried out from March 2001 to December 2002, including 22 patients with hypertrophic cardiomyopathy according to transthoracic echocardiogram criteria. Patients on a cardiac pacemaker, right bundle branch block, cardiac transplant, and under no possibilities to realize the exams were excluded. The results showed that asymmetric septal hypertrophy was the most common type (73%), 63% had a positive familial history of hypertrophic cardiomyopathy, 55% sudden cardiac death, and 23% syncope. Also, complex ventricular arrhythmias were detected in 14% and late potentials in 23% of patients. According to this study, the presence of late potentials was not associated with familial sudden death, syncope, and complex ventricular arrhythmias.
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PMID:Familial Hypertrophic Cardiomyopathy: Late Potentials and Other Prognostic Markers. 3204 15