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Ischemic cardiomyopathy: endomyocardial biopsy and ventriculographic evaluation of patients with congestive heart failure, dilated cardiomyopathy and coronary artery disease.

OBJECTIVES: This study was designed to define clinical and pathophysiologic similarities and differences between patients with ischemic and idiopathic dilated cardiomyopathy. BACKGROUND: Significant coronary artery disease in patients with new onset congestive heart failure due to dilated cardiomyopathy has important prognostic and therapeutic implications. METHODS: Clinical, histologic, ventriculographic and hemodynamic features of patients with dilated cardiomyopathy who underwent coronary angiography were reviewed. RESULTS: Patients with ischemic cardiomyopathy (n = 21) compared with those with idiopathic cardiomyopathy (n = 40) had similar presenting symptoms, durations of illness, and coronary risk factor profiles, with the exception of a greater prevalence of cigarette smoking (71% vs. 39%, p = 0.028) and male gender (100% vs. 70%, p = 0.014). Endomyocardial biopsy specimens from patients with ischemic cardiomyopathy demonstrated a greater prevalence of replacement fibrosis (48% vs. 8%, p = 0.001) and a lesser degree of histologically assessed myocyte hypertrophy (mean grade 0.5 +/- 0.7 vs. 1.3 +/- 1.3, p = 0.015). Although ventriculographically determined regional dyskinesia was present in both groups, there was a higher prevalence of two or more adjacent segments in the ischemic cardiomyopathy group (50% vs. 10%, p = 0.03). This ischemic group had hemodynamic variables associated with a worse prognosis: higher pulmonary artery wedge pressure (23 +/- 10 vs. 15 +/- 9 mm Hg, p = 0.006) and lower cardiac index (2.0 +/- 0.5 vs. 2.3 +/- 0.5 liters/min per m2, p = 0.044). Also, in this group, patients had a mean of 2.6 +/- 0.7 diseased vessels; 15 (71%) of 21 patients had triple-vessel disease and 18 (86%) of 21 had at least one occluded or suboccluded artery. CONCLUSIONS: 1) Patients with ischemic and idiopathic cardiomyopathy may be clinically indistinguishable unless coronary angiography is performed. 2) A greater prevalence of replacement fibrosis and a lesser degree of myocardial hypertrophy in patients with ischemic cardiomyopathy may account for the greater extent of hemodynamic decompensation observed at presentation.

Biopsy↗

[Latent cardiomyopathy - an early form of congestive cardiomyopathy].

Ten patients with a latent or subclinical form of congestive cardiomyopathy were selected from a group of 70 patients with congestive cardiomyopathy. Latent cardiomyopathy was diagnosed in view of the slightly decreased left ventricular myocardial function with no signs of cardiomegaly or left ventricular heart failure. Physical working capacity amounted to 89% (normal range greater than or equal to 80%) in the 10 patients. Hemodynamic evaluation showed slightly decreased systolic myocardial function with normal left ventricular volume. In 3 patients left ventricular end-diastolic pressure was significantly increased. The clinical follow-up showed a stable course in 7 patients and a slight deterioration in 2 patients, 1 patient died after 16 months. 5-year survival rate was 89% in patients with latent cardiomyopathy and 26% in the 60 patients with congestive cardiomyopathy. It is concluded that latent cardiomyopathy is a rather rare early form of congestive cardiomyopathy It is usually diagnosed by invasive evaluation because of the slightly reduced myocardial function and normal size of the left ventricle. Clinical follow-up in patients with latent cardiomyopathy is reasonably good compared to patients with congestive cardiomyopathy.

Adult↗

Primary prophylaxis of sudden death in hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, and dilated cardiomyopathy.

We present an evidence-based overview of primary prevention of sudden cardiac death. Several recent studies have provided important data regarding pharmacologic and device-based therapy for patients with conditions that confer high risk for sudden death. A rational approach to these therapies, with emphasis on implanted cardiovertor defibrillators, is discussed.

Anti-Arrhythmia Agents↗

[Stetho-acoustic cardiomyopathy--characteristics in cardiomyopathies studied by echocardiography].

In 4 different forms of cardiomyopathy which had been diagnosed echocardiographically we performed stethoacoustic investigations. Hereby each of these 4 different forms of cardiomyopathy developed a specific noise. In the patients with hypertrophic non-obstructive cardiomyopathy we found a mesosystolic to protomesosystolic interval murmur in 2 L 1 and called it hypertrophic murmur. In the apical hypertrophic cardiomyopathy we found a mesosystolic murmur above the apex and called it apical hypertrophic murmur. In the obstructive cardiomyopathy (hypertrophic obstructive cardiomyopathy) we found a mesotelesystolic murmur in 4 L 3 and called it obstruction murmur. In the dilatative cardiomyopathy (COCM) existed an immediate systolic murmur above the apex and we called it dilatation murmur.

Cardiomyopathies↗

Distinguishing ischemic cardiomyopathy from nonischemic dilated cardiomyopathy with coronary echocardiography.

Transthoracic echocardiographic examination of the proximal left coronary system was performed in 59 patients who had dilated cardiomyopathy to determine if this technique could distinguish between ischemic and nonischemic dilated cardiomyopathy. With use of annular array transducers (3.5 or 5 MHz) and digital image processing, echocardiographic visualization of the coronary arteries was successful in 55 (93%) of 59 patients. As assessed by coronary angiography, 32 subjects had ischemic cardiomyopathy and 27 had nonischemic cardiomyopathy. Twenty-seven (84%) of the 32 patients who had coronary artery disease and 24 (89%) of the 27 patients with nonischemic cardiomyopathy were correctly identified. The accuracy of coronary echocardiography was 86% in the entire study group and 93% when patients with inadequate studies were excluded. All subjects who had ischemic cardiomyopathy had evidence of disease by coronary echocardiography or segmental wall motion abnormalities. Multivariate analysis permitted correct classification of 93% of all subjects based on the results of the coronary echocardiogram, evaluation of segmental wall motion and a history of prior myocardial infarction. The correct diagnosis was made in 86% when the results of coronary echocardiography were excluded from analysis using all other echocardiographic and clinical variables. Transthoracic coronary echocardiography can be performed with a high degree of success in patients with dilated ventricles and the technique can reliably distinguish between ischemic and nonischemic dilated cardiomyopathy.

Cardiomyopathy, Dilated↗

The frequency of familial dilated cardiomyopathy in a series of patients with idiopathic dilated cardiomyopathy.

BACKGROUND: Dilated cardiomyopathy is characterized by an increase in ventricular size and impairment of ventricular function. Most cases are believed to be sporadic, and familial dilated cardiomyopathy is usually considered to be a rare and distinct disorder. We studied the proportion of cases of idiopathic dilated cardiomyopathy that were familial in a large sequential series of patients whose first-degree relatives were investigated regardless of whether these relatives had cardiac symptoms. METHODS: We studied relatives of 59 index patients with idiopathic dilated cardiomyopathy of obtaining a family history and performing a physical examination, electrocardiography, and two-dimensional, M-mode, and Doppler echocardiography. A total of 315 relatives were examined. RESULTS: Eighteen relatives from 12 families were shown to have dilated cardiomyopathy. Thus, 12 of the 59 index patients (20.3 percent) had familial disease. There was no difference in age, sex, severity of disease, exposure to selected environmental factors, or electrocardiographic or echocardiographic features between the index patients with familial disease and those with nonfamilial disease. A noteworthy finding was that 22 of 240 healthy relatives (9.2 percent) with normal ejection fractions had increased left ventricular diameters during systole or diastole (or both), as compared with 2 of 112 healthy control subjects (1.8 percent) who were studied separately. CONCLUSIONS: Dilated cardiomyopathy was found to be familial in at least one in five of the patients in this study, a considerably higher percentage than in previous reports. This finding has important implications for family screening and provides direction for further investigation into the causes and natural history of dilated cardiomyopathy.

Aged↗

Ischemic cardiomyopathy: lack of clinical applicability of the WHO/ISFC classification of cardiomyopathies.

BACKGROUND: The classification of cardiomyopathies proposed by the WHO/ISFC Task Force defines ischemic cardiomyopathy as "a dilated cardiomyopathy with impaired contractile performance not explained by the extent of coronary disease or ischemic damage". The aim of this study was to verify the clinical applicability of the WHO/ISFC definition of ischemic cardiomyopathy. METHODS: Retrospective analysis of the clinical characteristics of patients with a left ventricular ejection fraction < 40%, in whom coronary angiography showed a) stenosis < or = 50% of a main coronary artery and/or b) stenosis > 50% of a distal portion of a main coronary artery or of a secondary branch. The patients with a clinical diagnosis of previous myocardial infarction were excluded. RESULTS: Fourteen patients with the angiographic characteristics listed above were identified. Twelve patients were males, mean age 59 years. They represented 3.8% of all the patients with left systolic ventricular dysfunction who underwent coronary angiography in the same period. The left ventricular end-diastolic volume was 170 +/- 45 ml/m2 and the ejection fraction was 27 +/- 6%. The cause of systolic left ventricular dysfunction was systemic arterial hypertension in 3 patients, diabetes mellitus in 2, a combination of these diseases in 4, chronic alcohol abuse in 1, a previous clinically silent myocardial infarction in 1, and idiopathic dilated cardiomyopathy in 3. CONCLUSIONS: In conclusion, in all our patients with severe left ventricular dysfunction which was not explained by the extent of coronary artery disease, at least one possible cause of impaired contractile performance could be identified. Thus the definition of ischemic cardiomyopathy according to the new WHO/ISFC classification of cardiomyopathies appears to be of scarce utility on clinical grounds and should be redefined and if necessary reclassified.

Adult↗

Chronic respiratory illness as a predictor of survival in idiopathic dilated cardiomyopathy: the Washington, DC, Dilated Cardiomyopathy Study.

Although bronchial asthma and emphysema have been associated with idiopathic dilated cardiomyopathy in case-control studies, little is known about the prognostic importance of chronic respiratory disease in idiopathic dilated cardiomyopathy. To study this, we examined history of bronchial asthma, emphysema and chronic bronchitis, and respiratory medication use as possible predictors of survival in idiopathic dilated cardiomyopathy using data from a Washington, DC, population-based study (n = 129). The cumulative survival rates among patients with a history of emphysema or chronic bronchitis were 60% and 48% at 12 and 36 months, respectively, compared with 81.8% and 67.2% among patients without emphysema or chronic bronchitis. The survival rates of idiopathic dilated cardiomyopathy patients with and without a history of bronchial asthma at the time of idiopathic dilated cardiomyopathy diagnosis were similar. In multivariate analysis using the proportional hazards model, only ventricular arrhythmias and ejection fraction were found to be statistically significant predictors of survival in idiopathic dilated cardiomyopathy. The adjusted relative risk estimate for emphysema and chronic bronchitis was close to one. Thus, the results of this population-based study do not suggest that history of chronic respiratory illness is an independent predictor of survival in idiopathic dilated cardiomyopathy.

Adult↗

Response to isoproterenol as a prognostic indicator of evolution from hypertrophic cardiomyopathy to a phase resembling dilated cardiomyopathy.

OBJECTIVES: We sought to assess whether isoproterenol stress echocardiography could detect in advance in which patients hypertrophic cardiomyopathy would progress to a phase resembling dilated cardiomyopathy. BACKGROUND: In a few patients, hypertrophic cardiomyopathy has been reported to progress to a phase characterized by systolic dysfunction and left ventricular dilation, resembling dilated cardiomyopathy. METHODS: Echocardiograms were recorded before and immediately after intravenous infusion of isoproterenol (0.02 microgram/kg body weight per min) for 5 min in 18 patients with typical hypertrophic cardiomyopathy (i.e., hypertrophied, hyperdynamic and nondilated) to determine the difference in fractional shortening. The patients were categorized into those with a good response (difference in fractional shortening > 7%, 14 patients) and those with a poor response (difference < or = 7%, 4 patients). Changes in left ventricular end-diastolic diameter and fractional shortening were evaluated by using serial echocardiography over an average follow-up period of 5.4 years. RESULTS: In the good response group, neither end-diastolic diameter nor fractional shortening changed significantly during the follow-up period. In the poor response group, end-diastolic diameter significantly increased from a mean +/- SD of 41 +/- 5 to 53 +/- 5 mm (p < 0.05), and fractional shortening significantly decreased from 40 +/- 12% to 29 +/- 10% (p < 0.05). All patients in the poor response group showed a substantial decrease (> or = 5%) in fractional shortening and an increase (> or = 5 mm) in end-diastolic diameter. One patient developed congestive heart failure due to systolic dysfunction during the observation period. CONCLUSIONS: The present study confirmed that impaired responses to isoproterenol infusion are related to future deterioration of left ventricular performance in patients with typical hypertrophic cardiomyopathy.

Cardiomyopathy, Dilated↗

[Hereditary cardiomyopathies: a review. Mutation of structural proteins a common cause of hereditary cardiomyopathy].

Cardiomyopathy is a disorder of the cardiac muscle and can be either primary or secondary. The primary disorders have been classified by WHO into 4 groups based on structure and function; hypertrophic, dilated and restricted cardiomyopathies and arrythmogenic right ventricle dysplasia. During the last decade the familial nature of many of these cardiomyopathies has been elucidated and different genes have been found to be mutated and causative of disease. Certain patterns can be distinguished in the mutated genes, e.g. in general the genes causing hypertrophic cardiomyopathies code for proteins involved in the contractile apparatus, the sarcomere, and the genes causing dilated cardiomyopathy code for proteins that anchor the sarcomere to the cell membrane and extracellular matrix. This article reviews these recent genetic findings and discusses their potential clinical applicability.

Cardiomyopathies↗

The spectrum of left ventricular size in dilated cardiomyopathy: clinical correlates and prognostic implications. SPIC (Italian Multicenter Cardiomyopathy Study) Group.

To address the issues of variability and prognostic role of left ventricular dimensions in dilated cardiomyopathy (DCM), 144 patients with DCM were studied. They were arbitrarily assigned to two groups according to an echocardiographic left ventricular end-diastolic diameter index < or = 15% (45 patients with mildly dilated cardiomyopathy) and above 15% (99 patients with typically dilated cardiomyopathy) of the upper normality range. Among the patients with mildly dilated cardiomyopathy, there were more men (89% vs 66%; p < 0.01). This group of patients also had a greater prevalence of atrial fibrillation (22% vs 3%; p < 0.001) higher left ventricular fractional shortening (15 +/- 6% vs 13 +/- 5%; p < 0.05), higher ejection fraction (28 +/- 8% vs 24 +/- 8%; p < 0.01), and a lower exercise tolerance (5 +/- 2 MET vs 6 +/- 2 MET; p < 0.05). At the time of follow-up examination (30 +/- 15 months), event-free survival was not significantly different between patients with mildly dilated cardiomyopathy and those with typically dilated cardiomyopathy. Pulmonary capillary wedge pressure (p < 0.001) and left atrial dimension index (p < 0.01) were significant predictors of prognosis as determined by Cox multivariate analysis. Minimal or mild ventricular dilatation is not uncommon in DCM, and it identifies a heterogenous group of patients--some who are in the early stages of disease and others with severe pump dysfunction and persistently small hearts. Ventricular dilatation is not an independent predictor of prognosis.

Adult↗

Five candidate genes for hamster cardiomyopathy did not map to the cardiomyopathy locus by FISH analysis.

The Syrian cardiomyopathic hamster (BIO14.6), that develops both muscular dystrophy and progressive cardiomyopathy, is widely used as an animal model of autosomal recessive cardiomyopathy mimicking human hypertrophic cardiomyopathy, and five genes have been proposed as strong candidates for the cause of cardiomyopathy. We recently mapped the cardiomyopathy locus of the hamster to the centromeric region of chromosome 9qa2.1-b1 by construction of a genetic linkage map of the Syrian hamster. Thus, we analyzed the loci of the five candidate genes, alpha tropomyosin, cardiac troponin T, adhalin, calpain 3 and cardiac myosin binding protein-C, by the FISH method, and found that these genes were mapped on the distal portion of chromosome 12qa5 and 4pa2 and the proximal portion of chromosomes 9qb7, 1qc1.1 and 1qb3, respectively. These results provide strong evidence that the five candidate genes previously proposed are not related to the hamster cardiomyopathy.

Animals↗

Mechanocardiographic evaluation of left ventricular diastolic function in hypertrophic cardiomyopathy and hypertensive cardiomyopathy.

Usefulness of computerized mechanocardiography and echocardiography in the diagnosis of impaired left ventricular diastolic function is shown in a study comparing 17 hypertrophic obstructive cardiomyopathy and 17 hypertensive cardiomyopathy patients to 20 normal subjects. Mechanocardiography allows the evaluation of three different parameters of diastolic function: isovolumic relaxation evaluated by S2a-O or better by t-dr/dt and dr/dt/A2 ratio, left ventricular compliance by the A/H ratio and time of rapid filling. All the parameters are impaired in both pathological populations. Isovolumic relaxation being more depressed in hypertensive cardiomyopathy and duration of rapid filling being prolonged especially in the hypertrophic obstructive cardiomyopathy patients. Although the differences are small indicating only trends computerized mechanocardiography gives some evidence for difference in the alteration of diastolic function in hypertrophic obstructive cardiomyopathy and hypertensive cardiomyopathy.

Cardiomyopathy, Hypertrophic↗

Measuring serum aminoterminal type III procollagen peptide, 7S domain of type IV collagen, and cardiac troponin T in patients with idiopathic dilated cardiomyopathy and secondary cardiomyopathy.

OBJECTIVE: To identify new prognostic indicators in idiopathic dilated cardiomyopathy (DCM) and secondary cardiomyopathy. DESIGN AND PATIENTS: Serum concentrations of aminoterminal propeptides of type III procollagen and the 7S domain of type IV collagen (7S collagen)--which have recently been used as indicators of collagen matrix turnover in other diseases--and of cardiac troponin T were measured in 17 consecutive patients with DCM and in four patients with secondary cardiomyopathy (one associated with hyperthyroidism, two with chronic renal failure, one with amyloidosis), confirmed by endomyocardial biopsy. The correlation of these variables with short term prognosis was then assessed prospectively. RESULTS: 11 of the patients were positive for type III procollagen, 7S collagen, or troponin T even though their creatine kinase concentrations were within the normal range. These patients had a poor short term prognosis (p < 0.001). CONCLUSIONS: Within the DCM and secondary cardiomyopathy groups, there was a subgroup of patients with raised concentrations of serum collagen and troponin T, for whom short term prognosis was poor. Although it is unclear whether these serum peptide levels reflect ongoing myocyte degeneration and interstitial fibrosis, they may serve as useful new prognostic indicators for cardiomyopathy.

Adult↗

Review of dilated cardiomyopathies. Dilated cardiomyopathies and altered prothrombotic state: a point of view of the literature.

Heart failure is an enormously important clinical problem that, if not faced, may overwhelm health care resources. Primary and secondary cardiomyopathies cause the majority of cases of clinical heart failure, which is thus better approached from the utility point of view of myocardial failure. Furthermore, the risk of thromboembolic complications presenting in such disease may be higher than in ischemic cardiomyopathy. Intracardiac thrombi and mural endocardial plaques (from the organization of thrombi) are present at necropsy in more than 50% of patients with dilated cardiomyopathy (DCM). Several studies have shown that systemic and pulmonary emboli are more frequent in patients with ventricular thrombi or plaques. Dilated cardiomyopathy has been associated with left ventricular thrombosis which leads to substantial morbidity and mortality as a site for peripheral emboli. There are some studies on patients with dilated cardiomyopathy showing altered hemostasis and platelet behavior despite sinus rhythm. Platelet activation, thrombin activation and fibrinolytic activity are increased in patients with DCM compared to normal subjects. However, these markers reflecting coagulation activation in patients with left ventricle thrombus are comparable to those in patients without thrombus in the left ventricle. The pathophysiology and clinical issues concerning the susceptibility to develop left ventricular (LV) thrombosis and its complications like cerebrovascular disease in patients with DCM are summarized and the most recent articles present in the medical literature are reviewed.

Animals↗

[Comparison of histologic changes in Chagas' cardiomyopathy and dilated cardiomyopathy].

In this study the histopathologic features of Chagas' disease and idiopathic dilated cardiomyopathy were compared in 38 patients with congestive heart failure, classes II or III. Biopsy specimens were obtained with the Stanford model needle for right ventricle. Twenty-two patients presented non-chagasic dilated cardiomyopathy, and 16 were carriers of Chagas' disease. Twenty-three were male and 15 were female; their ages varied 18 and 49 years (mean 35.5). Fibrosis was slightly more frequently and intense in Chagas' disease than in dilated cardiomyopathy. Myocarditis aspect also was more frequent in Chagas' disease than in dilated cardiomyopathy (62.4% vs. 50%), with all cases showing a chronic form; acute myocarditis was observed only in dilated myocardiopathy. These differences, however, did not reach statistical significance. In conclusion, the presence of fibrosis, hypertrophy and moderate to severe degrees of chronic inflammatory infiltrate are suggestive of but not pathognomonic of Chagas' disease. On the other hand, dilated myocardiopathies are more frequently associated with less fibrosis or hypertrophy and the presence of mild degree of inflammatory infiltrate. The arrangement of this inflammatory infiltrate tends to be diffuse in dilated cardiomyopathy and in chagasic myocarditis it is multifocal, very similar to the histopathological aspect found in myocardial rejections episodes of transplanted hearts.

Adolescent↗

Progression from hypertrophic cardiomyopathy to dilated cardiomyopathy.

The incidence of hypertrophic cardiomyopathy progressing to dilated cardiomyopathy is rare. A patient diagnosed at age 22 and followed for 24 years who progressed to a dilated cardiomyopathy with severe congestive heart failure is reported. Left ventricular dilatation in hypertrophic cardiomyopathy may be due to a complication of surgery, beta-blocker therapy, or myocardial infarction. It may also represent the natural history in a subset of patients with hypertrophic cardiomyopathy.

Adult↗

Differentiation of cardiac amyloidosis and hypertrophic cardiomyopathy. A comparison of familial amyloidosis with polyneuropathy and hypertrophic cardiomyopathy by electrocardiography and echocardiography.

The clinical and echocardiographic features of cardiac amyloidosis may closely resemble those of hypertrophic cardiomyopathy, and the disorders may thus be mixed up. The present study was undertaken in an attempt to identify features separating the two conditions by analysis of electro- and echocardiographic findings in patients with familial amyloid polyneuropathy and hypertrophic cardiomyopathy. Twenty-nine patients with familial amyloidosis and 22 with hypertrophic cardiomyopathy were studied. Particular attention was given to the sum of the S wave in V1 and R wave in V5 or V6, the echocardiographic left ventricular mass and cross-sectional area, the presence or absence of asymmetrical septal thickening, granular and sparkling myocardial appearance, thickened heart valves, systolic anterior motion of the mitral valve, and pericardial effusion. A granular and sparkling appearance of the myocardium and thickened heart valves were found to be the best predictors of cardiac amyloidosis, while low QRS amplitudes in relation to echocardiographic left ventricular mass and a pericardial effusion seemed less important. The presence of systolic anterior movement of the mitral valve, a large left ventricular mass and a sum of S in V1 and R in V5 or V6 greater than 35 mm indicated hypertrophic cardiomyopathy. When the four strongest predictors (left ventricular mass, thickened heart valves, a granular sparkling myocardial appearance, and systolic anterior movement of the mitral valve) were used to reclassify the present patients, 28 of 29 amyloidosis patients and 21 of 22 patients with hypertrophic cardiomyopathy were correctly categorized. Noninvasive methods may thus be useful for detecting the myocardial infiltrative process, and cardiac amyloidosis may be confidently diagnosed by typical noninvasive findings together with histopathological documentation of amyloid in an organ other than the heart.

Adult↗