Disease relevance of Cardiomyopathy, Hypertrophic

• Asymmetric septal hypertrophy, an anatomic marker for this disease, was evident on thallium scans in the patients with idiopathic hypertrophic subaortic stenosis, with a ratio of septum to left ventricular free wall of 1.7 cm compared to 1.0 cm in eight normal volunteers, and 1.0 cm in eight patients with concentric left ventricular hypertrophy [1].
• In contrast, phospholamban ablation prevented systolic dysfunction and exercise intolerance, but not hypertrophy, in hypertrophic cardiomyopathy mice [2].
• Mild septal perforator compression (SPC) was observed in six other patients with IHSS, one patient with hypertrophic cardiomyopathy, 19 patients with AS, eight patients with severe stenosis of the LAD and one patient with myocardial bridge [3].
• Associated cardiac abnormalities were evident in six patients: two mitral prolapse (one Type II WPS and one Tpe III); one idiopathic hypertrophic subaortic stenosis (Type III); one congestive cardiomyopathy (Type III); one hypertrophic nonobstructive cardiomyopathy (Type I); and one atrial septal defect (Type II) [4].
• Human cardiac myosin isolated from operatively obtained samples of ventricular septum and left ventricular free wall of subjects with asymmetric septal hypertrophy (ASH) was compared, with respect to structural and enzymatic properties, to myosin isolated from hearts of subjects without heart disease [5].

Psychiatry related information on Cardiomyopathy, Hypertrophic

• BACKGROUND: The effect of calcium antagonists on regional left ventricular (LV) filling dynamics in patients with hypertrophic cardiomyopathy (HCM) is not well known, so the present study evaluated the results of echocardiography with color kinesis (CK) analysis during diltiazem infusion [6].
• Clinical use awaits technologic refinements, but potential applications include noninvasive detection of amyloid protein, of myofibrillar disorientation characteristic of idiopathic hypertrophic subaortic stenosis, and of various kinds of vascular pathology [7].

High impact information on Cardiomyopathy, Hypertrophic

• To determine whether thallium 201 myocardial imaging could be used to detect hypertrophic cardiomyopathy, 10 patients with idiopathic hypertrophic subaortic stenosis were studied [1].
• More significantly, genetic disruption of the calcineurin Abeta gene rescued hypertrophic cardiomyopathy and depressed functional capacity observed in p38-inhibited mice [8].
• Targeted inhibition of p38 MAPK promotes hypertrophic cardiomyopathy through upregulation of calcineurin-NFAT signaling [8].
• These oligomeric amyloid intermediates are present also in cardiomyocytes derived from many human dilated and hypertrophic cardiomyopathies [9].
• BACKGROUND: The class Ia antiarrhythmic drug disopyramide relieves the outflow tract obstruction of hypertrophic obstructive cardiomyopathy (HOCM) [10].

Chemical compound and disease context of Cardiomyopathy, Hypertrophic

• Verapamil improves exercise capacity in patients with hypertrophic cardiomyopathy (HCM), but its mechanism of action are unknown [11].
• BACKGROUND: We previously reported that 6 to 12 weeks of dual-chamber (DDD) pacing results in clinical and hemodynamic improvement in obstructive hypertrophic cardiomyopathy (HCM) [12].
• We examined the uptake and release of norepinephrine in the cardiac circulation and other regional vascular beds in 11 patients with hypertrophic cardiomyopathy (HCM) and in 10 control subjects during simultaneous infusion of tracer-labeled norepinephrine and isoproterenol [13].
• BACKGROUND: NSRT with intracoronary ethanol is a new promising treatment for patients with hypertrophic obstructive cardiomyopathy (HOCM) [14].
• OBJECTIVES: The aim of this study was to investigate the clinical expression of adenosine monophosphate-activated protein kinase (AMPK) gene mutations (PRKAG2) in adenosine monophosphate (AMP) kinase disease based on 12 years follow-up of known mutation carriers and to define the prevalence of PRKAG2 mutations in hypertrophic cardiomyopathy (HCM) [15].

Biological context of Cardiomyopathy, Hypertrophic

• Using a transgenic mouse model of hypertrophic cardiomyopathy as a tool, we assessed the relationship between the amount of ventricular ANF gene expression and the degree of hypertrophy as well as the relationship between the cells expressing ANF and tissue pathology [16].
• OBJECTIVES: We sought to determine the prevalence and phenotype of beta-myosin heavy chain gene MYH7 mutations in a large cohort of unrelated patients with hypertrophic cardiomyopathy (HCM) [17].
• Immunohistoselective sequencing (IHSS) of p53 tumor suppressor gene in human oesophageal precancerous lesions [18].
• In contrast, a missense mutation R94L in the vicinity of the strong tropomyosin-binding region associated with hypertrophic cardiomyopathy (HCM) resulted in an increase in the Ca(2+) sensitivity of force generation, as in the case of the other HCM-causing mutations in cTnT reported previously [19].
• A mouse model of hypertrophic cardiomyopathy (HCM) was created by expression of a cardiac alpha-myosin transgene including the R(403)Q mutation and a deletion of a segment of the actin-binding domain [20].

Anatomical context of Cardiomyopathy, Hypertrophic

• Two of the five patients had evidence of genetically transmitted hypertrophic cardiomyopathy, as evidenced by disorganized muscle cells in the ventricular septum or asymmetric septal hypertrophy in first degree relatives [21].
• Fractional myocardial uptake of thallium-201 was estimated by routine scintigraphic evaluation at rest and during exercise in 10 control subjects, 15 patients with coronary artery disease (CAD) and 12 patients with hypertrophic cardiomyopathy (HCM) [22].
• The finding at autopsy of typical pathologic features of hypertrophic heart disease (idiopathic hypertrophic subaortic stenosis, IHSS) and mitral valve prolapse (MVP) in a single patient prompted study of a number of close relatives of this patient [23].
• The configuration of the hypertrophied myocardium was evaluated by thallium-201 emission-computed tomography and 2-dimensional (2-D) sector scan in 10 patients with obstructive hypertrophic cardiomyopathy (HC), 10 with nonobstructive HC with giant negative T waves and 10 with concentric left ventricular (LV) hypertrophy [24].
• In this study, we investigate which pattern of immune response (Th1 or Th2) lies behind these diseases by analysing the basic cytokines secreted from PHA-cultured T lymphocytes and determining what differences, if any, exist between dilated cardiomyopathy (DMC) and hypertrophic cardiomyopathy (HCM) [25].

Gene context of Cardiomyopathy, Hypertrophic

• A role for NOS in cardiac hypertrophy has recently been demonstrated by studies which have shown hypertrophic cardiomyopathy (HCM) with altered contractility in constitutive NOS (cNOS) knockout mice [26].
• OBJECTIVES: This study examined plasma levels of certain matrix metalloproteinase (MMP) and tissue inhibitor of matrix metalloproteinase (TIMP) species before and after alcohol-induced myocardial infarction (MI) in patients with hypertrophic obstructive cardiomyopathy (HOCM) [27].
• Recently, we and others have shown that mutations in SCO2 are associated with a lethal infantile hypertrophic cardiomyopathy (HCMP) with COX-deficiency [28].
• BACKGROUND: Genotype-phenotype correlative studies have implicated 8 particular mutations that cause hypertrophic cardiomyopathy (HCM) as "benign defects," associated with near-normal survival: N232S, G256E, F513C, V606M, R719Q, and L908V of beta-myosin heavy chain (MYH7); S179F of troponin T (TNNT2); and D175N of alpha-tropomyosin (TPM1) [29].
• OBJECTIVES: The aim of this study was to evaluate the potential utility of genetic diagnosis in clinical management of families with hypertrophic cardiomyopathy (HCM) caused by mutations in the gene for cardiac troponin I (TNNI3) [30].

Analytical, diagnostic and therapeutic context of Cardiomyopathy, Hypertrophic

• The finding of asymmetric septal hypertrophy by echocardiography in the propositus suggested that the cause of the sudden death in the relatives was an undetected cardiomyopathy accompanying a mild and often subclinical myopathy [31].
• In the autopsy study, myocytes showed positivity for in situ nick end-labelling (TUNEL) and of Taq and Pfu polymerase-based in situ ligation assays not only in dilated cardiomyopathy (DCM, n = 9) with failure, but also in hypertrophic cardiomyopathy (HCM, n = 8) and hypertensive heart disease (HHD, n = 4) without failure [32].
• Differential diagnosis of hypertrophic cardiomyopathies: typical (subaortic) hypertrophic obstructive cardiomyopathy, atypical (mid-ventricular) hypertrophic obstructive cardiomyopathy and hypertrophic non-obstructive cardiomyopathy [33].
• PURPOSE: To assess first-pass magnetic resonance (MR) imaging in the evaluation of perfusion impairment in a genetically homogeneous population of patients with hypertrophic cardiomyopathy (HCM) and the Asp175Asn mutation of the alpha-tropomyosin gene and to evaluate the association between hypertrophy and perfusion [34].
• Three different subtypes of hypertrophic cardiomyopathy - resting obstructive (n = 5), latent obstructive (n = 3), and nonobstructive (n = 5) - were classified according to findings at catheterization in the 13 patients with asymmetric septal hypertrophy [35].

References