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Cardiomyopathy, Dilated
Updated: Sep 30, 2008
Introduction
Background
Cardiomyopathies are an important and heterogeneous group of diseases. The concept of heart muscle disease has a notable and evolving history. In the mid 1850s, chronic myocarditis was the only recognized cause of heart muscle disease. In 1900, the designation of primary myocardial disease was introduced, and it was not until 1957 that the term "cardiomyopathy" was used for the first time. Over the subsequent years, a number of definitions for cardiomyopathies have been advanced in concert with an increasing understanding of these diseases.
Cardiomyopathies have traditionally been divided into 3 types (ie, dilated, hypertrophic, restrictive); however, the classification of cardiomyopathies continues to evolve based on the rapid evolution of molecular genetics as well as the introduction of recently described diseases. For the purposes of this article, dilated cardiomyopathy refers to the subset of cardiomyopathies characterized by dilation and contractile dysfunction of the left and right ventricles that is not due to hypertension or ischemic heart disease.
Pathophysiology
A diverse range of conditions that promote cardiomyocyte injury or loss may cause dilated cardiomyopathy. Dilated cardiomyopathy is characterized by ventricular chamber enlargement and systolic dysfunction with normal left ventricle (LV) wall thickness. Ventricular enlargement and dysfunction generally leads to progressive heart failure with further decline in LV contractile function. Sequelae include ventricular and supraventricular arrhythmias, conduction system abnormalities, thromboembolism, and sudden or heart failure–related death.
Compensatory mechanisms associated with low cardiac output induce reflex upregulation of sympathetic tone and the renin-angiotensin axis, causing increased release of vasopressin, aldosterone, and atrial natriuretic peptide. Stimulation of these hormonal tracts results in volume expansion, which induces vasoconstriction. Vasoconstriction increases afterload that, in turn, decreases stroke volume. As cardiac output depends on stroke volume and heart rate, vasoconstriction ultimately can contribute to decreased cardiac output. Treatment of dilated cardiomyopathy is directed at interrupting this negative cycle.
Frequency
United States
Dilated cardiomyopathy is a common and largely irreversible form of heart muscle disease with an estimated prevalence of 1:2500. It is the third most common cause of heart failure and the most frequent cause of heart transplantation.
In total, an estimated 5 million Americans experience heart failure (including those with left ventricular failure), with more than 500,000 new cases diagnosed annually.
Mortality/Morbidity
From 1993-2003 deaths from heart failure increased by 20.5%. The 2003 death rate was 19.7 per 100,000 overall.1 Demographic distribution of the death rate was as follows:
- White males - 20.5 per 100,000
- White females - 18.4 per 100,000
- Black males - 23.4 per 100,000
- Black females - 20.4 per 100,000
The Framingham study2 Long-term survival was found to be inversely proportional to the severity of disease on initial diagnosis.
Hyponatremia at the time of presentation was found to be a marker of increased stimulation of the renin-angiotensin axis and of worsening of the disease course and prognosis.
Age
Dilated cardiomyopathy may manifest clinically at a wide range of ages (most commonly in the third or fourth decade but also in young children).
Clinical
History
- Determine the severity of disease, possible causes, and symptomatology when taking the history of a patient with suspected cardiomyopathy. Symptoms are a good indicator of the severity of the disease and may include the following:
- Fatigue
- Dyspnea on exertion, shortness of breath
- Orthopnea, paroxysmal nocturnal dyspnea
- Increasing edema, weight, or abdominal girth
- Note other important patient information, including the following:
- Age
- Sex
- Race
- Medical history, especially the following:
- Hypertension
- Angina
- Coronary artery disease
- Anemia
- Thyroid dysfunction
- Breast cancer
- Prior history of heart failure or myocardial injury
- Medications (especially new medications or lack of compliance with current medications)
- Social history (eg, tobacco, alcohol, illicit drug use)
- Family history of cardiomyopathy or sudden cardiac death
Physical
- On physical examination, look for signs of heart failure and volume overload. Assess vital signs with specific attention to the following:
- Tachypnea
- Tachycardia
- Hypertension
- Other pertinent findings include the following:
- Signs of hypoxia
- Jugular venous distension (JVD)
- Pulmonary edema (crackles and/or wheezes)
- S3 gallop
- Enlarged liver or presence of hepatojugular reflex
- Peripheral edema
Causes
About 20-30% of dilated cardiomyopathy cases have been reported as familial, although with incomplete and age-dependent penetrance, and linked to a diverse group of more than 20 loci and genes. Although genetically heterogeneous, the predominant mode of inheritance for dilated cardiomyopathy is autosomal dominant, with X-linked, autosomal recessive, and mitochondrial inheritance less frequent. Several of the mutant genes linked to autosomal dominant dilated cardiomyopathy encode the same contractile sarcomeric proteins that are responsible for hypertrophic cardiomyopathy, including α-cardiac actin; α-tropomyosin; cardiac troponin T, I, and C; and β- and α-myosin heavy chain. Research to determine further disease loci is ongoing.
The dilated cardiomyopathy phenotype with sporadic occurrence may derive from a particularly broad range of primary (and secondary) causes, including the following:
- Chronic excessive alcohol consumption
- Other drugs
- Heavy metals
- Emetine
- Anthracyclines (daunorubicin and doxorubicin)
- Cocaine
- Methamphetamine
- Cobalt
- Infections
- Viral endocarditis/myocarditis (coxsackievirus, adenovirus, parvovirus, human immunodeficiency virus [HIV])
- Parasites
- Protozoa
- Chagas disease (most common cause in parts of South America)
- High-output states
- Anemia
- Thyrotoxicosis
- Pregnancy
- Collagen vascular disease
- Glycogen storage disease, type IV also known as Andersen disease is associated with DCM.
- Thiamine deficiency and zinc deficiency
- Hypophosphatemia
- Amyloidosis
- Neuromuscular disorders (Duchenne/Becker and Emery-Dreifuss muscular dystrophies)
- Pheochromocytoma
More on Cardiomyopathy, Dilated |
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| Follow-up: Cardiomyopathy, Dilated |
| References |
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Further Reading
Keywords
dilated cardiomyopathy, cardiomyopathy, disease of the myocardium, heart disease, low cardiac output, cardiac failure, heart failure, congestive heart disease, CHD, congestive heart failure, CHF, contractile dysfunction, ventricular chamber enlargement, systolic dysfunction, ventricular arrhythmias, supraventricular arrhythmias, conduction system abnormalities, thromboembolism, sudden death
Overview: Cardiomyopathy, Dilated