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PubMed1995

The natural history and spectrum of idiopathic dilated cardiomyopathy, including HIV and peripartum cardiomyopathy.

Idiopathic dilated cardiomyopathy (IDCM) is an often fatal heart disease characterized by ventricular dilation and reduced systolic function. Despite advances in diagnostic and therapeutic techniques that have enabled earlier identification of patients with IDCM, the etiology of the disease in most patients remains unknown. The current 5-year survival rate for patients with asymptomatic IDCM approaches 80%; this is a significant improvement compared with earlier reports of the natural history of IDCM. Men are more commonly afflicted with IDCM, but women with IDCM tend to present with more advanced disease. Recent analysis of 3-year transplant-free survival reveals no gender differences, however. Survival in children with IDCM is variable; 30% of infants die within 2 years of diagnosis, but 5-year survival for childhood IDCM is 60% to 84%. An association of IDCM with the peripartum period is well recognized. The etiology of peripartum cardiomyopathy remains unknown, and some cases are familial. This disease is reversible in approximately 50% of patients, and in patients with intractable congestive heart failure, cardiac transplantation is a viable treatment option. AIDS has been more recently associated with IDCM. Acute left ventricular dysfunction and consequent dilated cardiomyopathy occur with increased frequency in patients with advanced AIDS. The etiology of dilated cardiomyopathy in HIV-infected patients is presently poorly understood. Survival for patients with AIDS after development of left ventricular dysfunction is extremely poor.

Cardiomyopathy, Dilated↗
PubMed2006

Unusual cardiomyopathies: ventricular noncompaction and takotsubo cardiomyopathy.

With improved imaging techniques, such as cardiac magnetic resonance imaging and computed tomography, 2 unusual cardiomyopathies have been added to the differential diagnosis of nonischemic dilated cardiomyopathies. Ventricular noncompaction (VNC) classically affects the left ventricle, although right ventricular involvement can also be seen. Symptoms can be absent or can be consistent with varying degrees of heart failure and arrhythmias. VNC can initially present in all age groups, from neonates to the elderly. In takotsubo cardiomyopathy, the characteristic appearance of the left ventricle involves transient regional dysfunction of the apex and mid-ventricle, with hyperkinesis of the basal segments. Classically, it occurs after an emotionally stressful event, and it predominantly affects postmenopausal women. This article reviews characteristics of these unique cardiomyopathies.

Cardiomyopathy, Dilated↗
PubMed1985

[Apical hypertrophic cardiomyopathy: an element in the continuum of hypertrophic cardiomyopathies].

Fourteen cases of apical hypertrophic cardiomyopathy (i.e. hypertrophy localized to the distal half of the left ventricular wall) are reported. There were 12 men and 2 women aged from 21 to 84 years. Only one of the patients presented, at first consultation, with severe functional symptoms, namely stage IV dyspnoea. ECG tracings were always abnormal, but the classical giant T waves were found in only 7 patients. In the 9 patients who had cardiac catheterization the left ventricular end-diastolic pressure was raised, and angiography showed an "ace of spades" diastolic image of the left ventricle with systolic obliteration of its tip. The distribution of parietal hypertrophy was best studied by two-dimensional echocardiography: the left ventricular apex was affected alone in 7 patients and concomitantly with the adjacent segments of the left or right ventricle in the other 7 patients. A family study showed that 3 patients had a descendant with obstructive cardiomyopathy. At a 4.6 years' follow-up the course of the disease was usually favourable. Apical hypertrophic cardiomyopathy is not a particular entity but one of different possible forms of hypertrophic cardiomyopathy. It seems to be benign in most cases.

Adult↗
PubMed1996

[Cardiomyopathies (VII). Natural history of hypertrophic cardiomyopathy].

After a short historic review of conceptual developments in hypertrophic cardiomyopathy, the natural history of the disease is analyzed according to each of its morphologic and functional abnormalities. The lack of association between hypertrophic morphology and sudden death is considered. Diastolic dysfunction and LV obstruction, although a frequent cause of dyspnea and heart failure, is not a risk factor for sudden death. Something similar occurs with the infrequent appearance in this disease of contractile failure. Myocardial ischemia is frequent in hypertrophic cardiomyopathy and general prognostic information about it is still lacking. Nevertheless, in young patients with family history of sudden death, a positive Thallium effort test may be a marker of sudden death (without an arrhythmogenic substrate), and may respond to verapamil. Finally, the new knowledge about genetic mutations in hypertrophic cardiomyopathy are analized. We conclude with some futuristic comments about hypertrophic cardiomyopathy.

Adolescent↗
PubMed1997

[Clinical course of hypertrophic cardiomyopathy in a non selected population. The Experience of the Italian Multicenter Cardiomyopathy Study].

BACKGROUND: Most of the information available on the clinical course and prognosis of hypertrophic cardiomyopathy (HCM) is based on data generated from international referral centres and as a result, it constitutes a potentially biased perspective of the disease process in this complex and diverse condition. A multicentric study was therefore set up with the aim of providing information on unselected patient populations with HCM. METHODS: The study group comprised 330 patients from 5 non-referral hospitals (mean age 42 +/- 16 years, M/F 226/104, 74-22%-obstructive, 299-91%-in NYHA class I-II) who were followed up regularly for 9.5 +/- 5.6 years. RESULTS: The vast majority of patients (n = 272, 82%) remained asymptomatic or mildly symptomatic during the follow-up period, whereas the remaining patients (n = 58, 18%) experienced clinical deterioration or died. Of the 18 patients (5%) who died of cardiovascular causes related to hypertrophic cardiomyopathy, 14 had progressive congestive heart failure and only 4 died suddenly. The annual mortality rate for cardiovascular disease was 0.57%, while the mortality rate due to sudden cardiac death was only 0.1%. The cumulative survival rate was 98, 95 and 93%, at 5, 10 and 15 years of follow-up respectively. Atrial fibrillation proved to be a relatively common (n = 81, 24%) and particularly unfavourable clinical feature, with higher mortality rate for cardiovascular causes related to hypertrophic cardiomyopathy. Syncope occurred in 47 patients (14%) but did not appear to have prognostic significance. CONCLUSIONS: In an unselected population, hypertrophic cardiomyopathy had a relatively benign prognosis that was inconsistent with its prior characterization as a generally progressive disorder, based primarily on the experience of selected referral institutions. Sudden unexpected cardiac death was distinctly uncommon, although a sizable proportion of patients, particularly the subset prone to atrial fibrillation, did experience clinical deterioration.

Adolescent↗
PubMed2003

Transformation of myocarditis and inflammatory cardiomyopathy to idiopathic dilated cardiomyopathy: facts and fiction.

There is broad evidence that enteroviruses and adenoviruses can induce an acute inflammation of the myocardium without cardiac dysfunction (i.e. myocarditis) or with cardiac dysfunction (i.e. inflammatory cardiomyopathy) that can transform to a virus-negative dilated cardiomyopathy. In the adult patient neither other viruses (parvo-B 19 virus, hepatitis C virus, cytomegalovirus) nor post-infection autoimmunity are likely to induce idiopathic dilated cardiomyopathy.

Autoimmunity↗
PubMed2005

Hypertrophic cardiomyopathy in Noonan Syndrome closely mimics familial hypertrophic cardiomyopathy due to sarcomeric mutations.

A 27 year old female with Noonan syndrome and hypertrophic cardiomyopathy underwent cardiovascular magnetic resonance imaging. These images showed asymmetrical septal hypertrophy with maximal left ventricular end-diastolic wall thickness of 25 mm. Following administration of gadolinium, areas of hyperenhancement were seen in the anterior, anteroseptal and lateral walls. This is the first report of focal gadolinium hyperenhancement in hypertrophic cardiomyopathy due to Noonan syndrome and suggests that myocardial fibrosis can be imaged by MR hyperenhancement as seen previously in sarcomeric hypertrophic cardiomyopathy.

Adult↗
PubMed2004

Tcap gene mutations in hypertrophic cardiomyopathy and dilated cardiomyopathy.

OBJECTIVES: We sought to explore the relationship between a Tcap gene (TCAP) abnormality and cardiomyopathy. BACKGROUND: Hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM) cause severe heart failure and sudden death. Recent genetic investigations have revealed that mutations of genes encoding Z-disc components, including titin and muscle LIM protein (MLP), are the primary cause of both HCM and DCM. The Z-disc plays a role in establishing the mechanical coupling of sarcomeric contraction and stretching, with the titin/Tcap/MLP complex serving as a mechanical stretch sensor. Tcap interacts with the calsarcin, which tethers the calcineurin to the Z-disc. METHODS: The TCAP was analyzed in 346 patients with HCM (236 familial and 110 sporadic cases) and 136 patients with DCM (34 familial and 102 sporadic cases). Two different in vitro qualitative assays-yeast two-hybrid and glutathion S-transferase pull-down competition-were performed in order to investigate functional changes in Tcap's interaction with MLP, titin, and calsarcin-1 caused by the identified mutations and a reported DCM-associated mutation, R87Q. RESULTS: Two TCAP mutations, T137I and R153H, were found in patients with HCM, and another TCAP mutation, E132Q, was identified in a patient with DCM. It was demonstrated by the qualitative assays that the HCM-associated mutations augment the ability of Tcap to interact with titin and calsarcin-1, whereas the DCM-associated mutations impair the interaction of Tcap with MLP, titin, and calsarcin-1. CONCLUSIONS: These observations suggest that the difference in clinical phenotype (HCM or DCM) may be correlated with the property of altered binding among the Z-disc components.

Asian People↗
PubMed1997

Electrocardiographic features differentiating dilated cardiomyopathy from hypertrophic cardiomyopathy.

To determine the usefulness of electrocardiographic (ECG) features in differentiating between hypertrophic cardiomyopathy with features mimicking dilated cardiomyopathy (D-HCM) and true dilated cardiomyopathy (DCM), we compared ECGs of 52 consecutive patients (11 with D-HCM, 41 with DCM). Left atrial dimension, left ventricular internal dimension, and septal and posterior wall thickness were employed as echocardiographic indexes, while QRS duration, amplitude of RV5 or V6 + SV1, number of abnormal Q waves, P-terminal force in V1, and frontal plane QRS axis were used as ECG parameters. The patients with D-HCM demonstrated a larger number of abnormal Q waves (P < .0001), greater prolongation of QRS duration (P < .0001), and lower amplitude of RV5 or V6 + SV1 (P < .0001). In all cases of D-HCM, atrial overload was observed and abnormal QRS axis in 9 (82%) of the 11 patients. These features were noted in 21 (51%) and 17 (41%), respectively, of the 41 DCM patients (P < .005 and P < .05, respectively). Despite significant differences in the echocardiographic parameters between D-HCM and DCM, excluding left ventricular end-diastolic dimension, ECG abnormalities were more significant between the two groups. The results indicate that ECG features are extremely useful in differentiation between DCM and D-HCM.

Cardiomyopathy, Dilated↗
PubMed1994

Cardiomyopathy in childhood and adult life, with emphasis on hypertrophic cardiomyopathy.

Over 60 entries in the genetic catalog have cardiomyopathy features--32 autosomal dominant, 35 autosomal recessive and X-linked. Over 40 present in, or can have survival into, adult life. Major clinicopathologic categories of these cardiomyopathic disorders included: sudden death (13 entities); cardiac conduction disturbance important feature; associated myopathy or motor dysfunction; storage diseases with cardiac involvement; cardiac amyloidoses; and, other categories. Genes, abnormality of which can cause hypertrophic cardiomyopathy (HCM), have been identified on chromosomes 1, 14 and 15, the locus on chromosome 14 involving the B-myosin heavy chain gene, but at least one unidentified locus is known to exist and there is a suggestive locus on chromosome 16, so that HCM is not a single disease but a group of disorders with clinicopatholopic similarities. To investigate these aspects of HCM in some detail, sixty-six patients with "sharply demarcated" differential myocardial fiber bundle hypertrophy (DMBH), considered to be of significant degree, from a pediatric autopsy data base of approximately 8,000 cases, were reviewed. Twenty-three of the patients died suddenly, without antecedent significant cardiac dysfunction, seven had clinical congestive heart failure of varying duration, three were stillborn, six showed evidence of aspiration of amniotic sac content (three had history of fetal distress), five had ischemic bowel disease, three (two with clinical cerebral palsy and one with Ondine's curse syndrome) had cerebral atrophy and sclerosis and one had extensive more acute encephalomalacia, and a variety of other major "causes of death" were present. Whether all infants and children with DMBH meeting the criteria used, who do not have congenital heart disease, have dominant hypertrophic cardiomyopathy (HCM) cannot be established by studies of this type, but the "concentration" of a gene or genes for HCM in pediatric autopsy series because the strong effect of HCM on life expectancy is relevant to this possibility. The data raise the question that stillbirth, fetal distress with aspiration of amniotic sac content, ischemic bowel disease and cerebral atrophy and sclerosis may be hitherto underappreciated features of HCM in childhood, and that patients with HCM may be peculiarly liable to die with certain types of septic shock, such as acute meningococcemia. In the material of this study, sudden death was statistically more frequent in females than in males in childhood (p < .029).

Adolescent↗
PubMed1993

Pathological analysis of hypertrophic cardiomyopathy simulating dilated cardiomyopathy.

The pathomorphologic features of hypertrophic cardiomyopathy simulating dilated cardiomyopathy in the late stage (HCM-DCM) were compared with those of ordinary hypertrophic cardiomyopathy (HCM). Seven autopsied hearts with HCM-DCM and 11 with HCM were assessed quantitatively using an image analyzer. Unlike HCM, significant left ventricular enlargement and wall thinning were observed in HCM-DCM, and the percentage areas of massive fibrosis and disarray were significantly greater. In HCM-DCM, the disarray was distributed diffusely, whereas massive fibrosis was distributed more intensively in the ventricular septum and anterior wall than in the lateral and posterior wall. Narrowing of intramyocardial small arteries was observed more frequently in HCM-DCM, especially in the ventricular septum and anterior wall, than in HCM. These results suggest that the enlargement and wall thinning of the left ventricle in HCM-DCM are attributable to non-uniform progression of massive fibrosis, which is closely related to small-arterial lesions.

Adult↗
PubMed1992

[Two cases of hypothyroidism with echocardiographic features similar to cardiomyopathy: one simulates hypertrophic and the other dilated cardiomyopathy].

Two cases of hypothyroidism with echocardiographic features similar to cardiomyopathy were presented. In case 1 (a 68 year-old woman), moderate pericardial effusion and myocardial hypertrophy were observed on admission. In case 2 (a 69 year-old woman), dilation of the left ventricle, hypokinesis of the interventricular septum and the left ventricular free wall, and reduced left ventricular systolic function were observed on admission. These echocardiographic findings were similar to hypertrophic or dilated cardiomyopathy. In the two cases, after recovery to euthyroid state, these echocardiographic abnormalities returned to normal. We concluded that hypothyroidism led to a reversible hypertrophic or dilated cardiomyopathy, and that echocardiographic study was useful for diagnosis and for following up cardiac manifestations of hypothyroidism.

Aged↗
PubMed1987

[Evolution of dilated cardiomyopathy from hypertrophic obstructive cardiomyopathy: a case report].

A 20-year-old woman whose echocardiograms showed a rare evolution from hypertrophic to dilated cardiomyopathy during a nine year observation period is described. This patient was initially diagnosed as having hypertrophic obstructive cardiomyopathy (HOCM) at the age of 12 years. Her echocardiogram showed marked thickening of the interventricular septum (IVS) and left ventricular posterior wall (LVPW), asymmetric septal hypertrophy (ASH) and systolic anterior motion of the mitral valve (SAM). Chest radiography revealed a prominent left ventricular border and a cardiothoracic ratio of 0.52. At 18 years of age she experienced onset of palpitation during the 16th week of pregnancy. Her echocardiogram recorded in June 1980 revealed a thickened IVS and LVPW with resolution of the SAM and of the narrow cavity. At 20 years of age she became pregnant again and was admitted to our hospital for the third time at 24 weeks gestation. On admission her blood pressure was 122/60 and her pulse was 56, and moist rales were audible over both lung fields. Peripheral edema was noted. Chest radiography revealed moderate cardiomegaly, a cardiothoracic ratio of 0.66, and congestion of the pulmonary vasculature. An echocardiogram showed thinning of the IVS and LVPW with hypokinesis and dilatation of the cavity compatible with dilated cardiomyopathy (DCM). She delivered a boy on July 15th 1982 at 32 weeks gestation following which she developed marked congestive heart failure. She expired one month later. A chest radiograph made one day before death revealed marked cardiomegaly, a cardiothoracic ratio of 0.76, prominent pulmonary vasculature and a pleural effusion. Autopsy was refused; therefore the terminal DCM-like features were not clarified pathologically. However, the slow progression from HOCM to HCM during eight years and then rapid progression from HCM to DCM during four months was most impressive.

Adult↗
PubMed1983

[Histological aspects of congestive cardiomyopathy caused by alcohol. Comparison with so-called primary cardiomyopathies].

The endoventricular biopsies of nine patients with alcoholic congestive cardiomyopathy were studied under the light microscope and under the electron microscope. These were compared with the results of nine cases of primary congestive cardiomyopathy, at a similar clinical stage. In the absence of specific signs, the overall appearance on both light and electron microscopy is sufficiently suggestive to allow differentiation of the two forms of cardiomyopathy. Repeated biopsies revealed disappearance of the nuclear abnormalities after withdrawal of alcohol, which introduces a prognostic element.

Adult↗
PubMed1998

Evolution of dilated cardiomyopathy from hypertrophic obstructive cardiomyopathy in a child.

Hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM) are two different diseases and have distinct etiologies. HCM is rare in children and rarely progresses to DCM. We report a 5-year-old Taiwanese girl with a definite diagnosis of hypertrophic obstructive cardiomyopathy. The diagnosis was based on echocardiograms, cardiac catheterization, and endomyocardial biopsy. However, the clinical features progressed to a picture of nonobstructive HCM at age 9, and the patient developed dilated poorly contractile ventricles with intractable congestive heart failure at age 10. She died from ventricular tachycardia at age 13. This report emphasizes the importance of periodic reevaluation for children with HCM.

Cardiomyopathy, Dilated↗
PubMed1996

Quality of life on treatment with metoprolol in dilated cardiomyopathy: results from the MDC trial. Metoprolol in Dilated Cardiomyopathy trial.

Quality of life in heart failure patients is receiving increased attention as a reflection of a treatment's potential secondary benefit of general well-being and daily functioning. The Metoprolol in Dilated Cardiomyopathy (MDC) trial was conducted as a large, multicenter trial to establish the effects of metoprolol on mortality and need for heart transplantation in patients with symptomatic idiopathic cardiomyopathy. It was found that metoprolol was well tolerated, improved symptoms and cardiac function, and prevented clinical deterioration in patients with symptomatic idiopathic dilated cardiomyopathy. Quality of life was evaluated as a secondary endpoint in 345 out of 383 randomized patients using a disease-specific questionnaire, the Quality of Life in Heart Failure Questionnaire, depicting physical activity, somatic symptoms, emotions, and life satisfaction. In a comparison of patients treated with metoprolol or placebo, patients treated with metoprolol noted a significantly more favorable response than those treated with placebo in terms of the overall treatment evaluation (p < 0.05). Additionally, an analysis of the changes from baseline to 18 months, using 95% confidence intervals, revealed that patients treated with metoprolol showed a significant improvement from baseline to 18 months in life satisfaction, physical activity, and the total score, while patients treated with placebo did not change at all. The improvement in quality of life was supported by the correlations with improvement in traditional clinical parameters.

Adrenergic beta-Antagonists↗
PubMed2000

Prediction of major arrhythmic events and sudden cardiac death in dilated cardiomyopathy. The Marburg Cardiomyopathy Study design and description of baseline clinical characteristics.

The Marburg Cardiomyopathy Study (MACAS) is a prospective observational study designed to determine the value of the following potential non-invasive arrhythmia risk predictors in more than 200 patients with idiopathic dilated cardiomyopathy (IDC) over a 5-year follow-up period: New York Heart Association functional class, left ventricular end-diastolic diameter and ejection fraction, left bundle branch block and atrial fibrillation on ECG, QTc and JTc-dispersion on 12-lead ECG, abnormal time-domain analysis and spectral turbulence analysis of the signal-averaged ECG, ventricular arrhythmias and heart-rate variability on 24-hour Holter ECG, baroreflex sensitivity, and microvolt T wave alternans during exercise. This report describes the rationale of MACAS as well as the clinical characteristics of the first 236 patients enrolled between March 1996 and October 1999. The prognostic significance of the potential arrhythmia risk predictors in MACAS will be determined by multivariate Cox analysis at the end of 5-year follow-up. Primary endpoints are total mortality and major arrhythmic events defined as sustained ventricular tachycardia, ventricular fibrillation or sudden cardiac death. The results of MACAS will have important implications for the design of future studies evaluating the role of prophylactic defibrillator therapy in idiopathic dilated cardiomyopathy.

Adolescent↗
PubMed1993

Beneficial effects of metoprolol in idiopathic dilated cardiomyopathy. Metoprolol in Dilated Cardiomyopathy (MDC) Trial Study Group.

Several small studies have suggested beneficial effects of long-term beta-blocker treatment in idiopathic dilated cardiomyopathy. Our large multicentre study aimed to find out whether metoprolol improves overall survival and morbidity in this disorder. 383 subjects with heart failure from idiopathic dilated cardiomyopathy (ejection fraction < 0.40) were randomly assigned placebo or metoprolol. 94% were in New York Heart Association functional classes II and III, and 80% were receiving background treatment. A test dose of metoprolol (5 mg twice daily) was given for 2-7 days; those tolerating this dose (96%) entered randomisation. Study medication was increased slowly from 10 mg to 100-150 mg daily. There were 34% (95% CI -6 to 62%, p = 0.058) fewer primary endpoints in the metoprolol than the placebo group; 2 and 19 patients, respectively, deteriorated to the point of needing transplantation and 23 and 19 died. The change in ejection fraction from baseline to 12 months was significantly greater with metoprolol than with placebo (0.13 vs 0.06, p < 0.0001). Pulmonary capillary wedge pressure decreased more from baseline to 12 months with metoprolol than with placebo (5 vs 2 mm Hg, p = 0.06). Exercise time at 12 months was significantly greater (p = 0.046) in metoprolol-treated than in placebo-treated patients. In patients with idiopathic dilated cardiomyopathy, treatment with metoprolol prevented clinical deterioration, improved symptoms and cardiac function, and was well tolerated.

Adolescent↗