Restrictive cardiomyopathy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords: Infiltrative cardiomyopathy; RCM; stiff heart; stiffening of the heart; heart stiffening; stiffened heart
Overview
Historical Perspective
Classification
Pathophysiology
Causes
The main Causes of restrictive cardiomyopathy are enlisted below:[1][2]
- Amyloidosis (AL, ATTR, SSA)
- Sarcoidosis
- Hemochromatosis
- Eosinophilic myocardial disease
- Idiopathic RCM
- Progressive systemic sclerosis (scleroderma)
- Postradiation therapy (Hodgkin's lymphoma, breast cancer etc)
- Anderson Fabry disease
- Danon's disease
- Friedreich's ataxia
- Diabetic cardiomyopathy (restrictive phenotype)
- Drug induced (anthracycline toxicity, methysergide, ergotamine, mercurial agents, etc.)
- Mucopolysaccharidoses (Hurler's cardiomyopathy)
- Myocardial oxalosis
- Wegener's granulomatosis
- Metastatic malignancies
Differentiating restrictive cardiomyopathy from Other Diseases
Restrictive cardiomyopathy should be differentiated from dilated cardiomyopathy, hypertrophic cardiomyopathy, congestive heart failure ect [2],[1]
| Type of disease | History | Physical examination | Chest X-ray | ECG | 2D echo | Doppler echo | CT | MRI | Catheterization hemodynamics | Biopsy |
| Restrictive cardiomyopathy | Systemic disease (e.g., sarcoidosis, hemochromatosis). | ± Kussmaul sign, S3 and S4 gallop, murmurs of mitral and tricuspid regurgitation | Atrial dilatation | Low QRS voltages (mainly amyloidosis), conduction disturbances, nonspecific ST abnormalities | ± Wall and valvular thickening, sparkling myocardium | Decreased variation in mitral and/or tricuspid inflow E velocity, increased hepatic vein inspiratory diastolic flow reversal, presence of mitral and tricuspid regurgitation | Normal pericardium | Measurement of iron overload, various types of LGE (late gadolinium enhancement) | LVEDP – RVEDP ≥ 5 mmHg
RVSP ≥ 55 mmHg RVEDP/RVSP ≤ 0.33 |
May reveal underlying cause. |
| Constrictive pericarditis | Prior history of pericarditis or conditions affecting the pericardium. | Pericardial knock | Pericardial calcification | Nonspecific ST and T abnormalities, low QRS voltage (<50%) | ± Pericardial thickening, respiratory ventricular septal shift. | Increased variation in mitral and/or tricuspid inflow E velocity, hepatic vein expiratory diastolic reversal ratio ≥ 0.79 medial e′/lateral e′ ≥ 0.91 (Annulus Reversus) | Thickened/calcified pericardium | Thickened pericardium | LVEDP – RVEDP < 5 mmHg
RVSP < 55 mmHg RVEDP/RVSP > 0.33 Inspiratory decrease in RAP < 5 mmHg Systolic area index > 1.1 (Ref CP in the modern era) Left ventricular height of rapid filling wave > 7 mmHg |
Normal myocardium |
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications, and Prognosis
Diagnosis
Diagnostic Study of Choice
History and Symptoms
Physical Examination
Laboratory Findings
Electrocardiogram
X-ray
Echocardiography and Ultrasound
CT scan
MRI
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Interventions
Surgery
Primary Prevention
Secondary Prevention
References
- ↑ 1.0 1.1 Hong JA, Kim MS, Cho MS, Choi HI, Kang DH, Lee SE, Lee GY, Jeon ES, Cho JY, Kim KH, Yoo BS, Lee JY, Kim WJ, Kim KH, Chung WJ, Lee JH, Cho MC, Kim JJ (September 2017). "Clinical features of idiopathic restrictive cardiomyopathy: A retrospective multicenter cohort study over 2 decades". Medicine (Baltimore). 96 (36): e7886. doi:10.1097/MD.0000000000007886. PMC 6393124. PMID 28885342.
- ↑ 2.0 2.1 Rammos A, Meladinis V, Vovas G, Patsouras D (2017). "Restrictive Cardiomyopathies: The Importance of Noninvasive Cardiac Imaging Modalities in Diagnosis and Treatment-A Systematic Review". Radiol Res Pract. 2017: 2874902. doi:10.1155/2017/2874902. PMC 5705874. PMID 29270320.
Overview
Restrictive cardiomyopathy is the least common cardiomyopathy. It is called this because it restricts the heart from stretching and filling with blood properly. Rhythmicity and contractility of the heart may be normal, but the stiff walls of the heart chambers (atria and ventricles) keep them from adequately filling. So blood flow is reduced, and blood that would normally enter the heart is backed up in the circulatory system. In time, restrictive cardiomyopathy patients develop heart failure.
Causes
Life Threatening Causes
Life-threatening causes include conditions which may result in death or permanent disability within 24 hours if left untreated. There are no known life threatening causes of restrictive cardiomyopathy that may result in death within 24 hours if not treated.
Common Causes
Causes by Organ System
Causes in Alphabetical Order
Diagnosis
Electrocardiogram
Shown below is an example of restrictive cardiomyopathy with low voltage and flipped anterior T waves.
