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At least 739 records · Page 41Linked to original sources
PubMed2005

The genetic basis for cardiac remodeling.

Cardiomyopathies are primary disorders of cardiac muscle associated with abnormalities of cardiac wall thickness, chamber size, contraction, relaxation, conduction, and rhythm. They are a major cause of morbidity and mortality at all ages and, like acquired forms of cardiovascular disease, often result in heart failure. Over the past two decades, molecular genetic studies of humans and analyses of model organisms have made remarkable progress in defining the pathogenesis of cardiomyopathies. Hypertrophic cardiomyopathy can result from mutations in 11 genes that encode sarcomere proteins, and dilated cardiomyopathy is caused by mutations at 25 chromosome loci where genes encoding contractile, cytoskeletal, and calcium regulatory proteins have been identified. Causes of cardiomyopathies associated with clinically important cardiac arrhythmias have also been discovered: Mutations in cardiac metabolic genes cause hypertrophy in association with ventricular pre-excitation and mutations causing arrhythmogenic right ventricular dysplasia were recently discovered in protein constituents of desmosomes. This considerable genetic heterogeneity suggests that there are multiple pathways that lead to changes in heart structure and function. Defects in myocyte force generation, force transmission, and calcium homeostasis have emerged as particularly critical signals driving these pathologies. Delineation of the cell and molecular events triggered by cardiomyopathy gene mutations provide new fundamental knowledge about myocyte biology and organ physiology that accounts for cardiac remodeling and defines mechanistic pathways that lead to heart failure.

Actomyosin↗
PubMed2005

Array lessons from the heart: focus on the genome and transcriptome of cardiomyopathies.

Our understanding of the cardiovascular system has evolved through the years by extensive studies emphasizing the identification of the molecular and physiological mechanisms involved in its normal function and disease pathogenesis. Major discoveries have been made along the way. However, the majority of this work has focused on specific genes or pathways rather than integrative approaches. In cardiomyopathies alone, over 30 different loci have shown mutations with varying inheritance patterns, yet mostly coding for structural proteins. The emergence of microarrays in the early 1990s paved the way to a new era of cardiovascular research. Microarrays dramatically accelerated the rhythm of discoveries by giving us the ability to simultaneously study thousands of genes in a single experiment. In the field of cardiovascular research, microarrays are having a significant contribution, with the majority of work focusing on end-stage cardiomyopathies that lead to heart failure. Novel molecular mechanisms have been identified, known pathways are seen under new light, disease subgroups begin to emerge, and the effects of various drugs are molecularly dissected. This cross-study data comparison concludes that consistent energy metabolism gene expression changes occur across dilated, hypertrophic, and ischemic cardiomyopathies, while Ca2+ homeostasis changes are prominent in the first two cardiomyopathies, and structural gene expression changes accompany mostly the dilated form. Gene expression changes are further correlated to disease genetics. The future of microarrays in the cardiomyopathy field is discussed with an emphasis on optimum experimental design and on applications in diagnosis, prognosis, and drug discovery.

Calcium↗
PubMed1989

Epidemiology of idiopathic dilated and hypertrophic cardiomyopathy. A population-based study in Olmsted County, Minnesota, 1975-1984.

Using the records linkage system of the Mayo Clinic and of the Rochester Epidemiology Project, which accesses diagnostic data on the entire population of Olmsted County, Minnesota, we identified 45 new cases of idiopathic dilated cardiomyopathy (DCM) and 19 new cases of hypertrophic cardiomyopathy (HCM) among county residents for the years 1975-1984. Overall age- and sex-adjusted incidence rates were 6.0/100,000 and 2.5/100,000 person-years, respectively. The incidence of DCM doubled from 3.9/100,000 in the first 5 years to 7.9/100,000 person-years in the last 5 years of study. The corresponding change for HCM was from 1.4 to 3.6/100,000 person-years. Age- and sex-adjusted prevalence rates as of January 1, 1985, for DCM and HCM were 36.5/100,000 and 19.7/100,000 population, respectively. The prevalence of DCM in persons less than 55 years old was 17.9/100,000, over a third of whom were New York Heart Association functional Class III or IV at diagnosis. These estimates may be of value in determining the potential use of health care resources, particularly cardiac transplantation.

Age Factors↗
PubMed1998

Echocardiography-guided ethanol septal reduction for hypertrophic obstructive cardiomyopathy.

BACKGROUND: Left ventricular outflow tract (LVOT) obstruction is frequently responsible for symptoms in hypertrophic obstructive cardiomyopathy (HOCM). Medical therapy is often not sufficient to control these symptoms, and surgical myotomy-myomectomy is required. METHODS AND RESULTS: We enrolled 33 symptomatic patients with HOCM and obstruction (>/=40 mm Hg gradient at rest or >/=60 mm Hg dobutamine-provoked). By contrast echocardiography, the bulging septum was localized and infarcted by injection of 2 to 5 mL of absolute ethanol into the septal artery(ies) supplying the hypertrophied area. Baseline echocardiograms with Doppler, myocardial perfusion tomograms, and treadmill exercise or pharmacological testing were compared with those at 6 weeks and 6 months. The mean rise in creatine kinase was 1964+/-796 U. All patients experienced symptomatic relief; NYHA class decreased from 3. 0+/-0.5 to 0.9+/-0.6 (P<0.001). Exercise time increased from 286+/-193 to 421+/-181 seconds (P=0.03). The resting and dobutamine-provoked gradient decreased from 49+/-33 and 96+/-34 mm Hg to 9+/-19 (P<0.001) and 24+/-31 mm Hg (P<0.001), respectively. Echocardiograms repeated at 6 weeks after the procedure showed a 28% reduction in septal thickness and 17% reduction in left ventricular mass. Myocardial perfusion imaging showed a "septal amputation pattern," with scarring in the upper and middle septal areas. Complete heart block developed in 11 patients, who then required permanent pacemaker implantation. CONCLUSIONS: Echocardiography-guided ethanol septal reduction in patients with HOCM is a safe, minimally invasive procedure that provides symptomatic relief with improved hemodynamic and left ventricular parameters.

Adult↗
PubMed2004

Pergolide use in Parkinson disease is associated with cardiac valve regurgitation.

OBJECTIVE: To determine if pergolide injures heart valves, by comparing echocardiographic findings in pergolide-treated patients with those of a historical control group. METHODS: Letters were sent to all patients in the authors' practice believed to be taking pergolide, and those responders who wished to continue it were urged to undergo echocardiography. Echocardiograms were obtained on 46 patients, and scores for valvular regurgitation were compared with those from an age-matched control group derived from the Framingham Study. The composite valve regurgitation score was modeled as a linear function of total milligrams lifetime use of pergolide, controlling for age. RESULTS: Eighty-nine percent of pergolide-treated patients had some degree of valvular insufficiency. For each of the three valves for which there are control data, we found an approximately 2- to 3-fold increased risk of abnormal valves in the pergolide patients (odds ratio [OR] approximately 3) and an estimated 14-fold increased risk of concerning tricuspid regurgitation (OR = 18.4). The composite valve score (the sum of valve scores for each of the four valves) was a function of lifetime pergolide use. CONCLUSION: Pergolide may injure cardiac valves, resulting most commonly in tricuspid regurgitation.

Aged↗
PubMed2001

Heart transplantation and the Batista operation for children with refractory heart failure.

Medically refractory heart failure may be present in children with cardiomyopathy (CMP) or complex congenital heart disease (CHD). In adults, the surgical management of this condition is either heart transplantation or the Batista operation. From March 1995 to January 2000, a total of 6 children, aged from 1 to 16 years, with medically refractory heart failure associated with CMP or complex CHD underwent cardiac transplantation and one of them also had the Batista operation as a bridge to transplantation. One of the 6 patients died of intractable sepsis 17 days after the operation, but the other 5 were discharged with satisfactory hemodynamics. Immunosuppressive agents, including azathioprine, cyclosporin or FK-506, were given. One patient experienced moderate acute rejection, but it was controlled by FK-506, OKT-3 and solumedrol. However, another suffered from lymphoproliferative disease 8 months after transplant, but it was controlled by intravenous immunoglubulin, alpha-interferon and acyclovir. Cardiac function during serial follow-up (range, 1 month to 5 years) revealed normal systolic and diastolic function and none received any anticongestive medications. Almost all patients received an oversized donor heart. The left ventricle (LV) mass was remodeled, initially as an decrease and later as an increase. The patient who underwent the Batista operation was discharged 1 month after the operation with an increased LV ejection fraction (from 10% to 22%). She was successfully bridged to heart transplantation 7 months after the Batista operation. The results of cardiac transplantation in growing children are satisfactory and remain the mainstay of surgical treatment for medically refractory heart failure in these patients. However, with a shortage of donor hearts, the Batista operation may be adopted as a bridge to heart transplant with a fair response.

Adolescent↗
PubMed2004

Noncompaction of the left ventricular myocardium diagnosed in pregnant woman and neonate.

Noncompaction of the left ventricular myocardium (NCLV) is an uncommon congenital cardiomyopathy with poor prognosis. We describe a case of NCLV that developed in a pregnant woman and her neonate. A nulliparous woman was referred at 24 weeks' gestation due to dyspnea and fetal hydrops. Maternal echocardiography demonstrated NCLV with characteristic findings of prominent and excessive ventricular trabeculations and deep intertrabecular recesses in the left ventricle. An M-mode echocardiography suggested depressed left ventricular systolic function. A fetal echocardiography at 24 weeks' gestation demonstrated cardiomegaly, but morphologic findings were not definitive for NCLV. An emergency cesarean section was performed due to maternal heart failure. A neonatal echocardiography diagnosed NCLV with depressed left ventricular systolic function. The neonate died of cardiac failure on the second day of life. Autopsy confirmed the echocardiographic findings. Since patients with NCLV may develop heart failure, multidisciplinary management is mandatory. In addition, awareness of familial occurrence of NCLV should be kept in mind for early diagnosis in the fetus and neonate.

Adult↗
PubMed1990

Cardiomyopathies in children. Clinical, epidemiological and prognostic evaluation.

In this article, the clinical and epidemiological characteristics of 137 children with cardiomyopathy admitted to the Hacettepe Pediatric Cardiology Unit were studied and the prognosis was evaluated after a follow-up period of 24 +/- 5 months. It was found that the highest proportion of patients were residents of Ankara, followed by the Northern and the Central Anatolian regions, and consanguinity between their parents was more common than the proportion for Turkey as a whole. Most of the patients had dilated cardiomyopathy (78.9%) and the age at which symptoms appeared varied according to the type of cardiomyopathy. A high proportion of patients came to the hospital with complaints of dyspnea and a decrease in effort capacity. The most common findings on the physical examination were hepatomegaly and tachycardia. Electrocardiographic and echocardiographic evaluations were made for all patients and cardiac catheterization and endomyocardial biopsy were performed when necessary. The outcome was as follows: 34.5% improved, 45.7% remained stable, 9.5% deteriorated and 10.3% died.

Adolescent↗
PubMed1994

AA-amyloidosis presenting with chronic diarrhea and cardiac manifestations.

In secondary amyloidosis (AA type), clinically significant cardiac and gastrointestinal involvement are uncommon, in contrast to the primary type. We report a case presenting with chronic diarrhea and cardiac manifestations who was diagnosed as having AA-amyloidosis with unknown predisposing illness based on endomyocardial, rectal and subcutaneous fat tissue biopsies.

Aged↗
PubMed1998

[Heart involvement in systemic amyloidosis. In vivo diagnosis].

This is the case of a 42 year-old male with systemic amyloidosis, probably primary, and heart involvement. Physiopathological and clinical aspects are described as well as the rarity of the diagnosis in vivo. The limited aspects of therapy are also described.

Adult↗
PubMed2000

Impaired Ca2+-ATPase oligomerization and increased phospholamban expression in dilated cardiomyopathy.

Although primary genetic defects have been identified for some forms of inherited cardiomyopathy, it is not well understood how secondary abnormalities actually lead to muscle cell destruction. Since cardiomyopathies significantly influence morbidity and mortality rates world-wide, it is important to improve the differential diagnosis of these disorders and develop potential treatments for inherited diseases of the heart. Elucidation of the secondary molecular mechanisms underlying cardiac cell necrosis might help linking a specific mutation in a cardiac gene to acute heart failure. As disturbed Ca2+-homeostasis may contribute to heart failure, we have investigated the relative abundance and oligomeric status of the sarcoplasmic reticulum Ca2+-ATPase and phospholamban in various cardiomyopathies. These two proteins represent important factors in cardiac relaxation. The SERCA2 isoform of the Ca2+-ATPase represents a major Ca2+-removal system in cardiac muscle fibres and phospholamban is a regulator of Ca2+-pump activity. Although Ca2+-ATPase expression did not seem to be markedly altered, the comparative immunoblot analysis presented here clearly shows that phospholamban expression is increased in dilated cardiomyopathy, possibly explaining the decreased Ca2+-uptake in the disease. In contrast to the normal enzyme, the Ca2+-pump was demonstrated to exhibit an impairment of crosslinker-stabilized oligomerization in dilated cardiomyopathy. Since Ca2+-ATPase oligomerization is important for co-operative kinetics and protection against proteolytic degradation, the monomeric Ca2+-ATPase may trigger an abnormal contraction-relaxation cycle in dilated cardiomyopathy leading to heart failure.

Blotting, Western↗