Restrictive cardiomyopathy

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List of terms related to Restrictive cardiomyopathy

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

Differentiating restrictive cardiomyopathy from Other Diseases

Differentiating restrictive cardiomyopathy from Other Diseases

Type of disease	History	Physical examination	Chest X-ray	ECG	2D echo	Doppler echo	CT	MRI	Catheterization hemodynamics	Biopsy
Restrictive cardiomyopathy
Constrictive pericarditis

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

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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

• Amyloidosis
• Chemotherapy
• Heart transplant
• Hemochromatosis
• Postirradiation fibrosis
• Sarcoidosis

Causes by Organ System

Cardiovascular	Amyloidosis, carcinoid heart disease, cardiac amyloidosis, endocardial fibroelastosis, endomyocardial fibrosis, heart transplant, hemochromatosis, Hurler syndrome, Noonan's syndrome
Chemical/Poisoning	Mercurial agents
Dental	No underlying causes
Dermatologic	Scleroderma, systemic sclerosis
Drug Side Effect	Anthracyclines, busulfan, chemotherapy, drugs, ergotamine, hydroxychloroquine, methysergide, serotonin
Ear Nose Throat	No underlying causes
Endocrine	Amyloidosis, carcinoid syndrome, carcinoid tumors, diabetes mellitus, carcinoid heart disease, hemochromatosis
Environmental	No underlying causes
Gastroenterologic	Alpha-L-iduronidase deficiency, glycogen storage disease, hemochromatosis, Hurler syndrome, sarcoidosis, carcinoid heart disease, carcinoid syndrome, scleroderma, systemic sclerosis
Genetic	Alpha-L-iduronidase deficiency, Anderson-Fabry disease, cystinosis, desminopathy, Fabry's disease, familial, Gaucher's disease, hemochromatosis, Hurler syndrome, mucopolysaccharidosis, Noonan's syndrome, pseudoxanthoma elasticum, sarcomeric protein mutations, Werner's syndrome
Hematologic	Carcinoid heart disease, carcinoid syndrome, carcinoid tumors, Gaucher's disease, hemochromatosis, hypereosinophilic syndrome
Iatrogenic	Postirradiation fibrosis, radiation
Infectious Disease	Loeffler's syndrome
Musculoskeletal/Orthopedic	Hemochromatosis, scleroderma, systemic sclerosis
Neurologic	No underlying causes
Nutritional/Metabolic	Alpha-L-iduronidase deficiency, amyloidosis, Anderson-Fabry disease, cystinosis, Fabry's disease, fatty infiltration, Gaucher's disease, generalized gangliosidosis GM1, glycogen storage disease, Hurler syndrome, I-cell disease, mucolipidosis II alpha/beta, mucopolysaccharidosis
Obstetric/Gynecologic	No underlying causes
Oncologic	Carcinoid heart disease, carcinoid syndrome, carcinoid tumors, hypereosinophilic syndrome, lymphoma, malignancy, metastatic cancers, paraneoplastic syndrome
Ophthalmologic	No underlying causes
Overdose/Toxicity	No underlying causes
Psychiatric	Hurler syndrome
Pulmonary	Churg-Strauss syndrome, Loeffler's syndrome, sarcoidosis, scleroderma, systemic sclerosis
Renal/Electrolyte	Scleroderma, systemic sclerosis
Rheumatology/Immunology/Allergy	Amyloidosis, cardiac amyloidosis, Churg-Strauss syndrome, Loeffler's syndrome, paraneoplastic syndrome, reactive arthritis, sarcoidosis, scleroderma, systemic sclerosis
Sexual	No underlying causes
Trauma	No underlying causes
Urologic	No underlying causes
Miscellaneous	Idiopathic

Causes in Alphabetical Order

• Alpha-L-iduronidase deficiency
• Amyloidosis
• Anderson-Fabry disease
• Anthracyclines
• Busulfan
• Carcinoid heart disease
• Carcinoid syndrome
• Carcinoid tumors
• Cardiac amyloidosis
• Chemotherapy
• Churg-Strauss syndrome
• Cystinosis
• Desminopathy
• Diabetes mellitus
• Drugs
• Endocardial fibroelastosis
• Endomyocardial fibrosis
• Ergotamine
• Fabry's disease
• Familial
• Fatty infiltration
• Gaucher's disease
• Generalized gangliosidosis GM1
• Glycogen storage disease
• Heart transplant
• Hemochromatosis

• Hurler syndrome
• Hydroxychloroquine
• Hypereosinophilic syndrome
• I-cell disease
• Idiopathic
• Loeffler's syndrome
• Lymphoma
• Malignancy
• Mercurial agents
• Metastatic cancers
• Methysergide
• Mucolipidosis II alpha/beta
• Mucopolysaccharidosis
• Noonan's syndrome
• Paraneoplastic syndrome
• Postirradiation fibrosis
• Pseudoxanthoma elasticum
• Radiation
• Reactive arthritis
• Sarcoidosis
• Sarcomeric protein mutations
• Scleroderma
• Serotonin
• Systemic sclerosis
• Werner's syndrome

Diagnosis

Electrocardiogram

Shown below is an example of restrictive cardiomyopathy with low voltage and flipped anterior T waves.

References

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