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Dilated cardiomyopathy

Dilated cardiomyopathy or DCM (also known as congestive cardiomyopathy), is a disease of the myocardium (the muscle of the heart) in which a portion of the myocardium is dilated, often without any obvious cause. About one in three cases of congestive heart failure (CHF) is due to dilated cardiomyopathy.1 more...

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A cardiomyopathy is any disease that primarily affects the muscle of the heart. In DCM, left and/or right ventricular systolic pump function of the heart is impaired, leading to progressive cardiac enlargement and hypertrophy, a process called remodeling.1

Dilated cardiomyopathy is the most common form of cardiomyopathy. It occurs more frequently in men than in women, and is most common between the ages of 20 and 60 years.2


Although no cause is apparent in many cases, dilated cardiomyopathy is probably the end result of myocardial damage produced by a variety of toxic, metabolic, or infectious agents. It may be the late sequel of acute viral myocarditis, possibly mediated through an immunologic mechanism. Alcohol abuse is also strongly associted with the development of dilated cardiomyopthy in some cases. Autoimmune mechanisms are also suggested as a cause for dilated cardiomyopathy.3

A reversible form of dilated cardiomyopahty may be found with alcohol abuse, pregnancy, thyroid disease, cocaine use, and chronic uncontrolled tachycardia.


About 20-40% of patients have familial forms of the disease, with mutations of genes encoding cytoskeletal, contractile, or other proteins present in myocardial cells.4 The disease is genetically heterogenous, but the most common form of its transmission is an autosomal dominant pattern. Autosomal recessive, X-linked, and mitochondrial inheritance of the disease is also found.5

Althought the disease is more common in African-Americans than in whites, it may occur in any patient population.

Associated symptoms

Symptoms of left- and right-sided congestive heart failure develop gradually in most patients. Left ventricualr dilatation may be present for months or even years before the patient becomes symptomatic.

Vague chest pain may be present, but typical angina pectoris is unusual and suggests the presence of concomitant ischemic heart disease. Syncope due to arrhythmias, and systemic embolism may occur.

Physical examination

The patients may present variable degrees of cardiac enlargement, and findings of congestive heart failure. In advance stages of the disease, the pulse pressure is narrowed and the jugular venous pressure is elevated. Third and fourth heart sounds are common. Mitral or tricuspid regurgitation may occur, presented by systolic murmurs upon auscultation (see mitral regurgitation and tricuspid insufficiency for more details about the findings).

Laboratory examinations

Generalized enlargement of the heart is seen upon normal chest X-ray. Pleural effusion may also be noticed, which is due to pulmonary venous hypertension.


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Assessment of prognosis in idiopathic dilated cardiomyopathy - Editorial
From CHEST, 4/1/02 by Robert C. Bahler

Determining the prognosis in each patient with idiopathic dilated cardiomyopathy (IDC) has been an important goal since the introduction of cardiac transplantation. Although heart transplants continue to be limited to critically ill patients, the triage of IDC patients will assume greater importance as the era of an implantable artificial heart nears. How successful are current approaches to identifying patients with high short-term mortality rates?

The New York Heart Association-rated class IV patient who experiences repeated hospitalizations and has a need for inotropic support is clearly at a high risk of death. Thus, the consideration for a heart replacement is straightforward. Most patients with IDC are not critically ill and do not require such an urgent intervention, yet perhaps 20% of such patients will die within 1 year. Early follow-up studies (1-6) have identified a number of variables that are associated with an adverse outcome. Some of the better predictors were New York Heart Association class rating, increasing age, a low left ventricular ejection fraction (LVEF), high left ventricular filling pressures, a very dilated left ventricle, exercise peak oxygen uptake (V[O.sub.2]) of < 11 to 16 mL/kg/min, marked intraventricular conduction delay (including a permanent pacemaker), and complex ventricular arrhythmias. Later studies that included echocardiography confirmed the importance of these factors and identified additional variables associated with adverse outcomes, including a relatively low left ventricular mass, (7) the presence of moderate or greater mitral regurgitation, (8) an increased left atrial size, right ventricular enlargement, (9) and a reduced right ventricular ejection fraction. (10) Echocardiographic parameters of diastolic function that reflect a high left atrial pressure were also predictive of adverse outcomes and included a high early diastolic mitral inflow velocity, a short early diastolic mitral inflow velocity deceleration time, (11,12) and a reduced pulmonary venous inflow velocity during ventricular systole. (13) A "Doppler index," which was defined using the formula (isovolumic contraction time + isovolumic relaxation time)/ejection time, was introduced to assess both right and left ventricular function and, as would be anticipated, the reduced function of either ventricle identified patients with unfavorable outcomes. (14,15)

As each discipline examined patients with IDC, the number of variables associated with adverse outcomes or a high mortality rate grew. An increase in the late potentials of the QRS complex, as seen in the signal-averaged ECG, was shown to be an independent predictor of all-cause cardiac death. (16) The presence of chronic and excessive cardiac sympathetic stimulation implies an adverse prognosis and can be identified by high serum levels of circulating catecholamines, a decrease in the heart rate variability during Holter monitoring, (17) or down-regulation of the myocardial [beta]-adrenergic receptors, as assessed by nuclear metaiodobenzylguanidine imaging. (18) Ventricular tachycardia and complex ventricular arrhythmias during a 24-h Holter recording, when associated with a low LVEF, place patients at a higher risk of death. (19) Markers of myocardial cell death, such as an elevation of serum troponin T levels, (20) an increased ratio of creatine kinase M[B.sub.2]/M[B.sub.1], (21) and scintigraphic evidence of an abnormal myocardial uptake of a monoclonal antibody to myosin, (22) all identify IDC patients who are at high risk.

With the exception of peak V[O.sub.2], the many variables reviewed above assessed only resting cardiac function. Perhaps ventricles that are able to favorably respond to a transient inotropic stimulus (ie, contractile reserve) would be capable of long-term improvement and would be associated with a better prognosis. Conversely, the irreversibly damaged ventricle would not exhibit enhanced function, and the prognosis would be poor. The utility of assessing the contractile reserve of severely hypokinetic myocardial regions in patients with chronic ischemic heart disease is now well-established. Revascularization of the myocardial segments exhibiting contractile reserve leads to improved regional function. Whether evidence of contractile reserve in IDC patients also would identify those most likely to respond favorably to medical therapies has only been explored in the last 10 years. Dubois-Rande et al (23) studied 36 patients and reported in 1992 that patients demonstrating an increase in left ventricular dP/dt with intracoronary dobutamine infusion had favorable responses to medical therapy. Subsequently, five reports (24-28) involving a total of 223 patients have shown the utility of contractile reserve as an indicator of prognosis. Exercise was the inotropic stimulus in one study, (24) and IV dobutamine infusion was used in the others. (25-28) Protocols for the infusion of dobutamine differed, and the measurements used to assess contractile reserve varied. Nevertheless, the results of these studies are consistent in that patients exhibiting enhanced left ventricular function had better clinical outcomes over relatively short periods of follow-up (ie, 6 to 36 months). Interestingly, the report by Kitaoka et al (28) found that the LVEF during dobutamine infusion and the LVEF at follow-up were closely related.

In this issue of CHEST, Drozdz and his colleagues (see page 1216) extend these observations in a larger group of 77 patients who were observed for a remarkably long (mean [+ or -] SD) period of 63 [+ or -] 7 months. The failure of ventricular function to improve during dobutamine infusion at 10 [micro]g/kg/min was defined as either a left ventricular end-systolic volume of > 150 mL or no decrease in the left ventricular end-diastolic volume. Importantly, the failure to show improved ventricular function was a significant predictor of a fatal outcome and replaced other known clinical and echocardiographic predictors of death in a multivariate analysis. The clinical variables of male gender and atrial fibrillation remained potent indicators of a poor outcome.

Recently, Scrutinio et al (27) reported their studies of contractile reserve in 60 IDC patients. They measured contractile reserve as the percentage change in the end-systolic volume index during dobutamine infusion, and this parameter was significantly related to the patient's exercise capacity, which was expressed as peak V[O.sub.2]. Either a small percentage decrease in the end-systolic volume index or a low peak V[O.sub.2] was predictive of the occurrence of clinical events. Unfortunately, this study was underpowered to assess the utility of these variables to predict death.

Despite all these studies, the relative value of each test remains unclear, and we continue to be uncertain as to which tests should be performed routinely. The majority of the reported observations have been in relatively small numbers of patients, the variables that were measured differ, selection bias cannot always be discerned, definitions of unfavorable outcomes have differed, and the follow-up intervals generally have been brief. Nevertheless, there are some common themes running through these and other studies (29,30) that are consistent with clinical experience. A favorable prognosis is likely under the following conditions: (1) when the diseased left ventricle exhibits improved systolic function either during inotropic stimulation or in association with treatment; (2) when exercise capacity, as measured by peak V[O.sub.2] or the results of a 6-min walk test, is reasonably preserved; (3) when signs of a high left atrial pressure are absent; (4) when there is no evidence of a continued hypersympathetic state; and (5) when there are no major arrhythmic events. Although the LVEF is useful in assessing the prognosis for groups of patients, it is less helpful as a single variable in assessing the prognosis of an individual patient.

Recognition of the IDC patient who is at very high risk for death in the near term is more problematic. Confidence in our ability to identify these patients correctly will come when the many univariate predictors of death are applied to a large cohort in a multicenter trial. This will allow a robust multivariate analysis of the relative utility of our many tests and should lead to a more focused and accurate evaluation of each patient with IDC. A measure of the contractile reserve of the left ventricle is very likely to be one of the important variables in predicting outcome. The best indicator of contractile reserve needs to be identified, but it is probable that changes in end-systolic dimensions or volume will provide useful prognostic information.

Dr. Bahler is affiliated with Case Western Reserve University School of Medicine, and the Heart and Vascular Center, MetroHealth Medical Center.

Correspondence to: Robert C. Bahler, MD, Heart and Vascular Center, MetroHealth Medical Center, 2500 MetroHealth Dr, Cleveland, OH 44109; e-mail:


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(12) Boni A, Cortigiani L, Nannini E. Echocardiographic evaluation of left ventricular diastolic function in patients with dilated cardiomyopathy: correlation among doppler, hemodynamic and clinical findings. G Ital Cardiol 1998; 28:1120-1127

(13) Dini FL, Dell'Anna R, Micheli A, et al. Impact of blunted pulmonary venous flow on the outcome of patients with left ventricular systolic dysfunction secondary to either ischemic or idiopathic dilated cardiomyopathy. Am J Cardiol 2000; 85:1455-1460

(14) Dujardin KS, Tei C, Yeo TC, et al. Prognostic value of a Doppler index combining systolic and diastolic performance in idiopathic-dilated cardiomyopathy. Am J Cardiol 1998; 82:1071-1076

(15) Ikeda R, Yuda S, Kobayashi N, et al. Usefulness of right ventricular Doppler index for predicting outcomes in patients with dilated cardiomyopathy. J Cardiol 2001; 37:157-164

(16) Fauchier L, Babuty D, Cosnay P, et al. Long-term prognostic value of time domain analysis of signal-averaged electrocardiography in idiopathic dilated cardiomyopathy. Am J Cardiol 2000; 85:618-623

(17) Yi G, Goldman JH, Keeling PJ, et al. Heart rate variability in idiopathic dilated cardiomyopathy: relation to disease severity and prognosis. Heart 1997; 77:108-114

(18) Merlet P, Benvenuti D, Moyse D, et al. Prognostic value of MIBG imaging in idiopathic dilated cardiomyopathy. J Nucl Med 1999; 40:917-923

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