Article Text

CARDIOMYOPATHY
Diagnosis and management of dilated cardiomyopathy
Free
  1. Perry Elliott
  1. Department of Cardiological Sciences, St George's Hospital Medical School, London, UK
  1. Dr PM Elliott, Department of Cardiological Sciences, St George's Hospital Medical School, Cranmer Terrace, London SW17 0RE, UK email:pelliott{at}sghms.ac.uk

Statistics from Altmetric.com

Dilated cardiomyopathy is a heart muscle disorder defined by the presence of a dilated and poorly functioning left ventricle in the absence of abnormal loading conditions (hypertension, valve disease) or ischaemic heart disease sufficient to cause global systolic impairment. A large number of cardiac and systemic diseases can cause systolic impairment and left ventricular dilatation, but in the majority of patients no identifiable cause is found—hence the term “idiopathic” dilated cardiomyopathy (IDC). There are experimental and clinical data in animals and humans suggesting that genetic, viral, and immune factors contribute to the pathophysiology of IDC.

Causes of dilated cardiomyopathy

Young

  • Myocarditis (infective/toxic/immune)

  • Carnitine deficiency

  • Selenium deficiency

  • Anomalous coronary arteries

  • Arteriovenous malformations

  • Kawasaki disease

  • Endocardial fibroelastosis

  • Non-compacted myocardium

  • Calcium deficiency

  • Familial IDC

  • Barth syndrome

Adolescent/adults

  • Familial IDC

  • X linked

  • Alcohol

  • Myocarditis (infective/toxic/immune)

  • Tachycardiomyopathy

  • Mitochondrial

  • Arrhythmogenic right ventricular cardiomyopathy

  • Eosinophilic (Churg Strauss syndrome)

  • Drugs—anthracyclines

  • Peripartum

  • Endocrine

  • Nutritional—thiamine, carnitine deficiency, hypophosphataemia, hypocalcaemia

Diagnosis

Clinical presentation

The first presentation of IDC may be with systemic embolism or sudden death, but patients more typically present with signs and symptoms of pulmonary congestion and/or low cardiac output, often on a background of exertional symptoms and fatigue for many months or years before their diagnosis. Intercurrent illness or the development of arrhythmia, in particular atrial fibrillation, may precipitate acute decompensation in such individuals. Increasingly, IDC is diagnosed incidentally in asymptomatic individuals during routine medical screening or family evaluation of patients with established diagnosis.

A careful family history facilitates diagnosis of inherited causes of IDC by characterising the family phenotype, and also defines the scope of family screening.1 At least 25% of patients have evidence for familial disease with predominantly autosomal dominant inheritance. Clinically, familial disease is defined by the presence of two or more affected individuals in a single family and should also be suspected in all patients with …

View Full Text

Supplementary materials

  • Additional references for: "Diagnosis and management of dilated cardiomyopathy" by Perry Elliott. Heart 2000;84:106-12.
     
    1. Mancini DM, Beniaminovitz A, Levin H, et al. Low incidence of myocardial recovery after left ventricular assist device implantation in patients with chronic heart failure. Circulation 1998;98:2383-9.
    2. Waagstein F, Bristow MR, Swedberg K, et al. Beneficial effects of metoprolol in idiopathic dilated cardiomyopathy. Lancet 1993;342:1441-6.
    3. Sütsch G, Kiowski W, Yan X, et al. Short-term oral endothelin-receptor antagonist therapy in conventionally treated patients with symptomatic severe chronic heart failure. Circulation 1998;98:2262-8.
    4. Bozkurt B, Villaneuva F, Holubkov R, et al. Intravenous immune globulin in the therapy of peripartum cardiomyopathy. J Am Coll Cardiol 1999;34:177-80.
    5. Oz MC, Argenziano M, Catanese K, et al. Bridge experience with long-term implantable left ventricular assist devices. Are they an alternative to transplantation? Circulation 1997;95:1844-5.
    6. Anon. Consensus recommendations for the management of chronic heart failure. Am J Cardiol 1999;83:1A-38A.
    7. Keeling PJ, Gang Y, Smith G, et al. Familial dilated cardiomyopathy in the United Kingdom. Br Heart J 1995;73:417-21.
    8. Dorffel WV, Felix SB, Wallukat G, et al. Short-term hemodynamic effects of immunoadsorption in dilated cardiomyopathy. Circulation 1997;95:1994-7.
    9. Muller J, Wallukat G, Dandel M, et al. Immunoglobulin adsorption in patients with idiopathic dilated cardiomyopathy. Circulation 2000;101:385-91.
    10. Baig MK, Goldman JH, Caforio AL, et al. Familial dilated cardiomyopathy: cardiac abnormalities are common in asymptomatic relatives and may represent early disease J Am Coll Cardiol 1998;31:195-20.
    11. Caforio AL, Grazzini M, Mann JM, et al. Identification of alpha- and beta-cardiac myosin heavy chain isoforms as major autoantigens in dilated cardiomyopathy. Circulation 1992;85:1734-42.
    12. Gold MR Optimization of ventricular pacing: where should we implant the leads? J Am Coll Cardiol 1999;33:324-6.
    13. Brecker SJ, Gibson DG. What is the role of pacing in dilated cardiomyopathy? Eur Heart J 1996;17:819-24.
    14. Torre-Amione G, Kapadia S, Benedict C, et al. Proinflammatory cytokine levels in patients with depressed left ventricular ejection fraction: a report from the studies of left ventricular dysfunction (SOLVD). J Am Coll Cardiol 1996;27:1201-6.
    15. Henry WL, Gardin JM, Ware JH. Echocardiographic measurements in normal subjects from infancy to old age. Circulation 1980;62:1054-61.

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.