dilated cardiomyopathy
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Introduction
Dilated cardiomyopathy is a disorder characterized by dilatation of the cardiac chambers & a reduction in ventricular contractility.
Etiology
- idiopathic (50%)
- stress cardiomyopathy
- exclusion of CAD, myocarditis, cardiac muscle disease
- toxic/drug-induced
- infectious myocarditis
- infiltrative & inflammatory disorders
- connective tissue disease - scleroderma
- hypersensitivity myocarditis
- hemochromatosis
- cardiac sarcoidosis
- peripartum state (peripartum cardiomyopathy)
- occurs during the last trimester or up to 6 months postpartum[2]
- commonly recurs in subsequent pregnancies
- giant cell myocarditis[2]
- metabolic abnormalities
- endocrine disorders
- tachycardia-induced myocarditis
- hypertension[5]
Pathology
- dilatation of cardiac chambers
- variable hypertrophy
- symptomatic heart failure often present
- tricuspid & mitral regurgitation are common
- atrial & ventricular arrhythmias in 50%
- high incidence of sudden death
- left ventricular non-compaction
Genetics
- alcoholic cardiomyopathy associated with homozygosity for angiotensin converting enzyme (ACE) deletion allele
- defects in LDB3 are a cause of dilated cardiomyopathy
- defects in titin are a cause of dilated cardiomyopathy (CMD1G, autosomal dominant)
- defects in ABCC9 are a cause of dilated cardiomyopathy (CMD1O), dilated cardiomyopathy with ventricular tachycardia
- expression of CAMK2D is significantly increased in patients suffering from dilated cardiomyopathy
- defects in LMNA are associated with type 1A
- defects in ACTC1 are associated with type 1R
- defects in ACTN2 are associated with type 1AA
- defects in TNNT2 are associated with type 1D
- defects in SCN5A are associated with type 1E
- defects in desmin are associated with type 1I
- defects in EYA4 are associated with type 1J
- defects in CSRP3 are associated with type 1M
- defects in TCAP are associated with type 1N
- defects in PLN are associated with type 1P
- defects in VCL are associated with type 1W
- defects in TPM1 are associated with type 1Y
- defects in MYH7 are associated with type 1S
- defects in thymopoietin-alpha are associated with type 1T
- defects in FTKN are associated with type 1X
- defects in TNNC1 are associated with type 1Z
- defects in TNNI3 are associated with type 2A
- defects in SGCD are associated with type 2F
- defects in dystrophin are associated with type 3B
Diagnostic procedures
- echocardiography
- 4 chamber dilation
- ventricular dysfunction
- valvular disease
- electrocardiogram generally abnormal, but changes are non-specific
- endomyocardial biopsy of limited value
Radiology
- radionuclide ventriculography
- evidence of coronary artery disease
- cardiac magnetic resonance imaging (CMRi) if cardiac sarcoidosis suspected
- late gadolinium enhancement associated with adverse clinical outcomes[7]
- 18F-fluorodeoxyglucose (FDG) PET scan if CMRi is negative
Differential diagnosis
Management
- general considerations
- similar to management of heart failure from any cause
- control of total body sodium & water
- vasodilators
- preload reduction
- afterload reduction
- ACE inhibitors are agents of choice
- low-dose beta blockers[2]
- statin may be of benefit, even in non-ischemic heart failure[6]
- counsel all women with previous peripartum cardiomyopathy to avoid pregnancy[2]
- treat peripartum cardiomyopathy with ACE inhibitors, beta-blockers & diuretics[2]
- specific considerations
- ventricular ectopy (excluding occasional PVCs)
- non-sustained ventricular tachycardia is common
- high incidence of sudden death
- antiarrhythmic agents do not improve survival in non-sustained ventricular ectopy
- anticoagulation
- high incidence of mural thrombi
- immunosuppressive therapy for biopsy proven myocarditis
- implantable defibrillators appropriate in about 1/3 of patients[3]
- ventricular ectopy (excluding occasional PVCs)
- evaluation for surgical options
- valvular disease
- coronary artery disease
- intra-aortic balloon counterpulsation to stabilize a patient before definitive surgical therapy
- prognosis:
- varies with etiology, chronicity & severity
- spontaneous improvement of ventricular function in 20-40% within 6-12 months of presentation
More general terms
More specific terms
- cardiac sarcoidosis
- dilated cardiomyopathy with quadriceps myopathy
- dilated cardiomyopathy with woolly hair & keratoderma; Carvajal syndrome; palmoplantar keratoderma with left ventricular cardiomyopathy & woolly hair (DCWHK)
- ischemic cardiomyopathy
References
- ↑ Manual of Medical Therapeutics, 28th ed, Ewald & McKenzie (eds), Little, Brown & Co, Boston, 1995, pg 131-32
- ↑ 2.0 2.1 2.2 2.3 2.4 2.5 Medical Knowledge Self Assessment Program (MKSAP) 11, 14, American College of Physicians, Philadelphia 1998, 2006
Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022 - ↑ 3.0 3.1 Journal Watch 22(10):77-78, 2002 Grimm W et al, Implantable defibrillator event rates in patients with idiopathic dilated cardiomyopathy, nonsustained ventricular tachycardia on Holter and a left ventricular ejection fraction below 30%. J Am Coll Cardiol 39:780, 2002 PMID: https://www.ncbi.nlm.nih.gov/pubmed/11869841
Kadish A Primary prevention of sudden death using ICD therapy: incremental steps. J Am Coll Cardiol 39:788, 2002 PMID: https://www.ncbi.nlm.nih.gov/pubmed/11869842 - ↑ Journal Watch 22(19):155, 2002 Fernandez-Sola J et al Angiotensin-converting enzyme gene polymorphism is associated with vulnerability to alcoholic cardiomyopathy. Ann Intern Med 137:321, 2002 PMID: https://www.ncbi.nlm.nih.gov/pubmed/12204015
- ↑ 5.0 5.1 UpToDate 2004 http://www.uptodate.com
- ↑ 6.0 6.1 Sola S et al, Atorvastatin improves left ventricular systolic function and serum markers of inflammation in nonischemic heart failure. J Am Coll Cardiol 2006, 47:332 PMID: https://www.ncbi.nlm.nih.gov/pubmed/16412856
Ramasubbu K and Mann DL The emerging role of statins in the treatment of heart failure. J Am Coll Cardiol 2006, 47:342 PMID: https://www.ncbi.nlm.nih.gov/pubmed/16412858 - ↑ 7.0 7.1 Eichhorn C, Koeckerling D, Reddy RK et al Risk Stratification in Nonischemic Dilated Cardiomyopathy Using CMR Imaging: A Systematic Review and Meta-Analysis. JAMA. 2024 Sep 19:e2413946. PMID: https://www.ncbi.nlm.nih.gov/pubmed/39298146 https://jamanetwork.com/journals/jama/fullarticle/2823869
Patient information
dilated cardiomyopathy patient information
Database
- OMIM: https://mirror.omim.org/entry/115200
- OMIM: https://mirror.omim.org/entry/302045
- OMIM: https://mirror.omim.org/entry/604145
- OMIM: https://mirror.omim.org/entry/608569
- OMIM: https://mirror.omim.org/entry/611407
- OMIM: https://mirror.omim.org/entry/607487
- OMIM: https://mirror.omim.org/entry/611878
- OMIM: https://mirror.omim.org/entry/611879
- OMIM: https://mirror.omim.org/entry/609909
- OMIM: https://mirror.omim.org/entry/611880
- OMIM: https://mirror.omim.org/entry/613424
- OMIM: https://mirror.omim.org/entry/601494
- OMIM: https://mirror.omim.org/entry/604765
- OMIM: https://mirror.omim.org/entry/612158
- OMIM: https://mirror.omim.org/entry/160760
- OMIM: https://mirror.omim.org/entry/601154
- OMIM: https://mirror.omim.org/entry/606685
- OMIM: https://mirror.omim.org/entry/607482
- OMIM: https://mirror.omim.org/entry/605362
- OMIM: https://mirror.omim.org/entry/613740
- OMIM: https://mirror.omim.org/entry/611615