hypertrophic cardiomiopathy

INTRODUCTION AND OBJECTIVES: Molecular characterization of congenital heart diseases now includes the not infrequent dysmorphic Noonan syndrome. A study of 6 genes of the RAS-MAPK pathway in Spanish patients is presented: the impact of heart disease, clinical expressivity, and diagnostic yield are investigated. METHODS: The study included 643 patients (and 182 family members) diagnosed by dysmorphologists, cardiologists, and pediatric endocrinologists from 74 tertiary hospitals...

UNLABELLED: Retrospectively the Perinatal Post-Hypoxic Cardiac disorder, was researched in 32 newborn (ages 0-14 days), having a normal weight, with hypoxia at birth, Apgar score 3-7, requiring resuscitation at birth. The patients did not show signs of major cardiac suffering, except a systolic murmur, the neurologic manifestations prevailing. RESULTS: The investigations showed: cardiomegaly (46.9%); ECG: repolarization disorders of the left ventricle (LV) (43.5%); echocardiographic modifications in 67...

The response of the body to vigorous physical activity is a multiorgan system phenomenon. As a result, the body undergoes profound morphologic and functional alterations, but as there are different kinds of physical activities, the degree of these changes is highly variable as well. Considering many sudden cardiac deaths in sport, it is needless to say how important it is to know where the border of normal changes of the body due to physical activity is and when these changes become unhealthy. Also it is very important to distinguish physiological changes of the body due to physical activity and pathological changes due to some cardiac diseases...

Variability of C-structural heterochromatin segments in chromosome 1,9 and 16 has been studied in lymphocyte cultures of peripheral blood taken from the patients with the hypertrophy (HC) and dilatate (DC) forms of cardiomiopathy and their 1st degree relatives (32 individuals, in total). 10 healthy individuals composed the control group. C-segments were sorted according to Patil and Lubs: a<0.5 x 16p; b>0.5-1 x 16p; c>1.5 x 16p; d>1.5-2 x 16p; e>2 x 16p. The total amount of C-heterochromatin in all the studied chromosomes was tended to increase for DC patients and the relatives of the patients with the two forms of cardiomiopathy...

AIM: The aim of this study was to assess the relationship between echocardiographic indexes of left ventricular (LV) hypertrophy with LV mass (LVM) obtained at cardiac magnetic resonance (CMR) in a population of patients with hypertrophic cardiomiopathy (HCM). METHODS: Thirty-nine patients with HCM underwent echocardiography and CMR. By echocardiography maximal wall thickness (MWT), Spirito' and Maron's hypertrophy index and the Wigle's score were obtained. Absolute LVM was measured through CMR and indexed to body surface area (LVMi)...

This is an update that aimed to describe some aspects of physiopathology of Hypertrophic Cardiomyopathy (HCM) and its therapeutics correlating them to nursing assistance. Data source was based on LILACS bibliographical database using the search expression "hypertrophic cardiomiopathy" and "nursing care". The HCM is a disease that appears from cromossomic alterations that causes hypertrophy of the left ventricule and can cause heart failure, functional class III and IV. Treatment consists in using palliative medications or other procedures like Pace-Maker implantation, ventriculoplasty and heart transplantation...

Complex clinical-laboratory investigation of children with congestive heart failure (CHF) developed on the basis of dilated cardiomyopathy and hypertrophic cardiomyopathy has been carried out. The development of CHF in children with cardiomyopathy was accompanied by changes of blood serum lactate, MB isoform of creatine phosphokinase, TNF-6, interleukin-6 (IL-6) (but not IL-2), and soluble receptors for IL-2 and IL-6.

INTRODUCTION: Despite remarkable advances in medicine and sports, sudden cardiac death remains a significant problem. INCIDENCE OF SUDDEN CARDIAC DEATH: The incidence of sudden cardiac death varies in different studies and there are no systematic data about it. It varies in different types of sports, with age and sex. SUDDEN CARDIAC DEATH AND PHYSICAL ACTIVITY: Many changes in cardiac morphology and function represent an adaptive response to physical activity...

BACKGROUND: Many enteroviruses as well as hepatitis C have been increasingly found in association with cardiomyopathies, even though, the evidence has been inconclusive. METHODS: From 1998 to 2001, at the liver clinic of our community-based hospital, in the cohort of hepatitis C patients, infection evidence by PCR confirmation and echocardiographic measures of left ventricular (LV) function were obtained. RESULTS: One hundred-eight patients were identified...

The impact of molecular genetics in the diagnosis and management of various forms of heritable cardiac or vascular disorders is continuously increasing thanks to the newly available laboratory tools. Familial hypertrophic cardiomyopathy (FHC), an autosomal dominant inherited disease characterized by unexplained left ventricular hypertrophy and a wide range of clinical symptoms, is the first cardiac disorder whose genetic bases have been elucidated. Linkage analysis studies have shown a statistically significant association between the disease status and at least seven genetic loci, all coding for sarcomeric proteins, in unrelated kindreds...

Hypertrophic cardiomiopathy is a peculiar process with different anatomical and functional abnormalities which are present in different degrees in each case. Echocardiography and Doppler techniques have contributed definitively to the knowledge of this process and these procedures are choices for establishing the diagnosis of hypertrophic cardiomyopathy and to evaluate the clinical and morphological diversity of this process consisting of a spectrum of abnormalities with a variable presence in each case. Disproportionate septal hypertrophy is the most frecuent finding but the hypertrophy can involve other segments and different patterns can be present; concentric hypertrophy, apical, involving right ventricle, inverted asymetric, etc...

Hypertrophic cardiomyopathy (HCM) is phenotypically and genotypically a heterogeneous disease. Since 1989, four chromosomal loci have been identified for HCM and the genes residing on three of these have been identified as beta-myosin heavy chain (beta-MHC), cardiac troponin-T and alpha-tropomyosin. These genes code for sarcomeric proteins and exhibit the same phenotype, suggesting that HCM is a disease of the sarcomere. Over 40 missense mutations and one deletion of the beta-MHC gene have been identified. Similarly, missense mutations in the alpha-tropomyosin gene and the cardiac troponin-T gene have been identified...

A case of hypertrophic cardiomiopathy (HCM) mimicking athlete heart, is reported. Performing competitive activity was followed by progression of HCM to cardiac dilation and hypokinesis so that transplant was needed at young age. The Authors suggest a more aggressive approach possibly inclusive of cardiac biopsy when doubtful cases of athlete heart require permission for competitive sports.

In this paper eight patients with myogenic or neurogenic muscle disorders are presented, in whom cardiomiopathy was also found. Six patients developed a dilated cardiomiopathy associated with neurogenic atrophies or progressive muscular dystrophy. In patients with Mb. Friedreich and HSNM type II together with the total dilatation of the septum hypertrophy was observed and in patients with spinal muscular amyotrophy of scapuloperoneal type atriomyopathy dominated. In two patients with mitochondrial disorders a hypertrophic cardiomyopathy was found...

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