---
title: Restrictive cardiomyopathy
description: What restrictive cardiomyopathy is, why the ventricular walls become stiff, how it is detected, which treatment approaches clinical guidelines describe and when to call an ambulance.
type: disease
aliases:
  - RCM
sources:
  - tier: guideline
    org: esc
    title: 2023 ESC Guidelines for the management of cardiomyopathies
    year: 2023
    doi: 10.1093/eurheartj/ehad194
    url: https://doi.org/10.1093/eurheartj/ehad194
  - tier: guideline
    org: minzdrav-rf
    title: Хроническая сердечная недостаточность
    year: 2024
    doi: 10.15829/1560-4071-2024-6162
    url: https://doi.org/10.15829/1560-4071-2024-6162
  - tier: reference
    org: msd-manuals
    title: Restrictive Cardiomyopathy
    year: 2026
    url: https://www.msdmanuals.com/professional/cardiovascular-disorders/cardiomyopathies/restrictive-cardiomyopathy
  - tier: reference
    org: msd-manuals
    title: Restrictive Cardiomyopathy (Consumer Version)
    year: 2026
    url: https://www.msdmanuals.com/home/heart-and-blood-vessel-disorders/cardiomyopathy/restrictive-cardiomyopathy
sources_checked: 2026-10-12
review: null
---

Restrictive cardiomyopathy (RCM) is a group of heart disorders in which the walls of the ventricles become stiff, but not necessarily thickened, and do not fill properly with blood between heartbeats.<Cite n={4} /> Even a small increase in the volume of blood in a stiff ventricle raises the pressure inside it disproportionately.<Cite n={3} /> The <Structure id="left-ventricle">left ventricle</Structure> is affected most often, but both may be.<Cite n={3} /> It is the least common form of cardiomyopathy; the best known of the others are [dilated](../dilated-cardiomyopathy/) and [hypertrophic](../hypertrophic-cardiomyopathy/) cardiomyopathy.<Cite n={[3, 4]} />

<Diagram name="restrictive-cardiomyopathy">
  <p slot="normal">
    Between heartbeats the ventricles relax and fill with blood from the atria.<Cite n={4} /> The
    normal diagram shows none of the changes seen in RCM: the ventricular walls are elastic and
    stretch as the ventricles fill, and the atria are of normal size.<Cite n={[3, 4]} />
  </p>
  <p slot="disease">
    In RCM the stiff ventricular walls stretch poorly, the ventricles fill less well and the filling
    pressure inside them rises.<Cite n={3} /> The left ventricle is usually not enlarged; its wall
    thickness may be normal or increased, and in the diagram it is normal.<Cite n={3} /> Because
    the filling pressure stays high, both atria enlarge.<Cite n={3} />
  </p>
</Diagram>

## Why it happens

<Patient>

A ventricular wall becomes stiff when the heart muscle is gradually replaced by scar tissue or when substances that do not belong there build up in it.<Cite n={4} /> In more than half of cases no specific cause is found.<Cite n={4} />

Scarring of the heart muscle can follow radiation treatment of a tumour in the chest or be caused by some medicines.<Cite n={4} /> Substances that can build up in the heart muscle include iron when the body has too much of it (haemochromatosis), white blood cells called eosinophils in hypereosinophilic syndrome, and an unusual protein, amyloid, in amyloidosis.<Cite n={4} /> Infants and children can have a congenital form, and in tropical countries there is endomyocardial fibrosis, which affects both ventricles.<Cite n={4} /> There are also inherited forms linked to changes in genes.<Cite n={[1, 3]} />

</Patient>

<Student>

MSD classifies the causes of RCM into four groups: infiltrative (amyloidosis, sarcoidosis), non-infiltrative (idiopathic RCM, systemic sclerosis), storage diseases (Fabry disease, haemochromatosis) and endomyocardial disorders (endomyocardial fibrosis, hypereosinophilic syndrome, carcinoid syndrome, metastatic cancer, some medications); radiation injury is placed in the same group.<Cite n={3} />

The ESC figure "Spectrum of restrictive heart diseases" divides the causes into intrinsic myocyte dysfunction, endomyocardial disorders and myocardial extracellular matrix disorders.<Cite n={1} /> In the figure, RCM proper comprises primary RCM, variants in sarcomeric, cytoskeletal and several other genes (including filamin and titin), desminopathy, endomyocardial fibrosis, hypereosinophilia, hyperoxaluria and radiation.<Cite n={1} /> Amyloidosis, sarcoidosis, Anderson–Fabry disease, Danon disease, glycogenoses, iron overload and several other conditions are shown as myocardial diseases with occasional restrictive physiology, often in the context of LV hypertrophy.<Cite n={1} />

The principal haemodynamic abnormality is diastolic dysfunction: the stiff, non-compliant LV fills poorly, filling pressures rise, pulmonary venous hypertension develops, and the RV may become involved over time; systolic function is typically preserved in the early stages.<Cite n={3} /> Stroke volume is often relatively fixed, so cardiac output depends on heart rate.<Cite n={3} /> Chronically raised filling pressures lead to biatrial enlargement, atrial arrhythmias and functional atrioventricular valve regurgitation.<Cite n={3} /> The Russian Ministry of Health guideline lists RCM among the rarer causes of heart failure with preserved ejection fraction (HFpEF).<Cite n={2} />

</Student>

<HeartModel highlight={['left-ventricle', 'right-ventricle', 'left-atrium', 'right-atrium']} />

## Symptoms

<Patient>

RCM causes [heart failure](../heart-failure/): shortness of breath on exertion and when lying flat, fluid build-up in the tissues and swelling.<Cite n={4} /> Tiredness, an awareness of the heartbeat (palpitations) and a swollen abdomen can occur.<Cite n={[3, 4]} />

At rest there are usually no symptoms: even a stiff heart supplies the body with enough blood at rest.<Cite n={4} /> Symptoms appear during exertion, when the pressure in the stiff heart rises quickly and it cannot pump as much blood as the body needs.<Cite n={4} /> Heart rhythm problems are common, especially [atrial fibrillation](../atrial-fibrillation/); chest pain and fainting are less likely than in hypertrophic cardiomyopathy.<Cite n={4} />

</Patient>

<Student>

Typical features are fatigue, exercise intolerance, exertional dyspnoea, orthopnoea, palpitations, abdominal distension and peripheral oedema; atrial and ventricular arrhythmias and conduction abnormalities are common.<Cite n={3} /> Involvement of the sinoatrial and atrioventricular nodes may cause sinus node dysfunction and varying degrees of AV block.<Cite n={3} /> According to the ESC, the prevalence of atrial fibrillation in RCM is 45–51%.<Cite n={1} />

Examination shows pulmonary crackles and marked jugular venous distension with a rapid y descent without respiratory variation (no Kussmaul sign); pulsus paradoxus is typically absent.<Cite n={3} /> The clinical picture closely resembles constrictive pericarditis, a disease of the [pericardium](../pericarditis/).<Cite n={3} />

</Student>

## How it is diagnosed

<Patient>

RCM is one of the causes a doctor looks for in a person with heart failure.<Cite n={4} /> The diagnosis is based on the combined results of a physical examination, an [ECG](../../diagnostic/ecg/), a chest X-ray and a [heart ultrasound (echocardiography)](../../diagnostic/echocardiography/).<Cite n={4} /> An ECG usually finds changes, but they are not specific enough for a diagnosis.<Cite n={4} />

A heart ultrasound shows enlarged atria: the heart may still squeeze normally, but the muscle is stiff, does not relax properly and does not fill with enough blood.<Cite n={4} /> Cardiac MRI can show changes in the heart tissue caused by a build-up of, for example, iron or amyloid.<Cite n={4} />

Sometimes a thin tube (catheter) is passed through a blood vessel to the heart to measure the pressures in the heart chambers and to take a small sample of heart muscle for examination (biopsy).<Cite n={4} /> Genetic testing is done for some forms or if the cause is unknown.<Cite n={4} />

</Patient>

<Student>

RCM should be suspected in HFpEF, particularly in a patient with a systemic disorder causing myocardial infiltration or fibrosis.<Cite n={3} /> The initial work-up is an ECG, chest radiography and transthoracic echocardiography, followed by cardiac MRI; right and left heart catheterisation and endomyocardial biopsy are reserved for cases where non-invasive tests are inconclusive or a specific diagnosis is needed to guide therapy.<Cite n={3} /> The ESC recommends multimodality imaging to differentiate RCM from hypertrophic (HCM) or dilated (DCM) cardiomyopathy with restrictive physiology (class I).<Cite n={1} />

The ECG is non-specific and may show biatrial enlargement, ST-T abnormalities and conduction disturbances; the latter raise suspicion of an infiltrative process.<Cite n={3} /> According to the ESC, AV block in RCM should raise suspicion of desminopathy or amyloidosis.<Cite n={1} />

Echocardiography shows a non-dilated LV with preserved ejection fraction; biatrial dilatation is common, and wall thickness may be normal or increased, especially with infiltrative processes.<Cite n={3} /> Cardiac MRI provides superior tissue characterisation and is particularly useful in differentiating RCM from constrictive pericarditis; patterns of late gadolinium enhancement and T1, T2 and T2* mapping may point to a specific cause, such as Fabry disease, amyloidosis or iron overload.<Cite n={3} />

Catheterisation shows raised diastolic filling pressures, rapid early diastolic filling and equalisation of ventricular diastolic pressures, the "square root" sign; unlike constrictive pericarditis, ventricular interdependence and respiratory variation in pressures are minimal.<Cite n={3} /> The Russian Ministry of Health heart failure guideline recommends right heart catheterisation when heart failure is thought to be caused by RCM (ESC class IIa).<Cite n={2} /> The ESC recommends catheterisation in all children with RCM to measure pulmonary artery pressures and pulmonary vascular resistance (class I).<Cite n={1} />

According to the ESC, endomyocardial biopsy should be considered in RCM to exclude specific diagnoses (iron overload, storage disorders, mitochondrial cytopathies, amyloidosis, granulomatous myocardial diseases) and to diagnose restrictive myofibrillar disease caused by desmin variants (class IIa).<Cite n={1} /> Laboratory tests look for systemic causes: iron studies, serum and urine protein electrophoresis with immunofixation, serum free light chains, and a full blood count to detect eosinophilia.<Cite n={3} />

</Student>

## How it is treated

<Patient>

Treatment is aimed at the cause, at relieving fluid congestion and at preventing blood clots and heart rhythm problems.<Cite n={3} /> Sometimes treating the cause can stop the heart damage from getting worse or partly reverse it: for example, excess iron is removed from the body in iron overload, and inflammation in the heart is suppressed in sarcoidosis.<Cite n={4} /> But many people with RCM have no specific treatment.<Cite n={4} />

Water tablets (diuretics) reduce swelling and breathlessness, but they also reduce the amount of blood entering the heart, so the doctor adjusts the dose especially carefully.<Cite n={4} /> Some medicines usually given for heart failure are poorly tolerated in RCM because they lower blood pressure too much.<Cite n={[3, 4]} /> In atrial fibrillation, medicines that reduce blood clotting are given to prevent clots.<Cite n={4} />

A pacemaker may be needed for a slow heart rhythm or severe conduction problems.<Cite n={3} /> An implantable cardioverter-defibrillator is recommended for people who have survived a cardiac arrest or a ventricular arrhythmia that disrupted the circulation.<Cite n={1} /> In severe disease, carefully selected patients may be offered a heart transplant.<Cite n={[3, 4]} />

The outlook depends on the cause but is often poor, because the diagnosis is frequently made very late.<Cite n={4} /> The ESC recommends that people with cardiomyopathy and their relatives have access to a team of cardiomyopathy specialists.<Cite n={1} /> The doctor chooses the treatment together with the patient.

</Patient>

<Student>

Specific therapy when the cause is known: immunosuppressive therapy for cardiac sarcoidosis, iron chelation for iron overload, enzyme replacement for Fabry disease, and disease-modifying therapy for amyloidosis.<Cite n={3} />

Diuretics are the cornerstone of symptomatic treatment of congestion but are used cautiously: patients with RCM are highly preload dependent, and excessive diuresis can reduce cardiac output and cause hypotension.<Cite n={3} /> Afterload-reducing agents (nitrates, inhibitors of the renin–angiotensin–aldosterone system and neprilysin) may be poorly tolerated because of hypotension.<Cite n={3} />

Atrial fibrillation is poorly tolerated because the contribution of atrial contraction to ventricular filling is lost; a rhythm control strategy with antiarrhythmic drugs or ablation is therefore generally preferred, although maintaining sinus rhythm can be difficult.<Cite n={3} /> The ESC also prefers rhythm control in RCM but notes that there are no data on ablation in this disease.<Cite n={1} /> The ESC states that oral anticoagulation should be considered in patients with RCM and atrial fibrillation or flutter unless contraindicated (class IIa).<Cite n={1} /> Anticoagulation is also indicated for an intracardiac thrombus.<Cite n={3} />

Bradyarrhythmias and advanced conduction system disease may require a permanent pacemaker.<Cite n={3} /> An ICD is recommended for patients with RCM who have survived a cardiac arrest or a ventricular arrhythmia causing haemodynamic instability (class I).<Cite n={1} /> Heart transplantation is considered in carefully selected patients without severe irreversible pulmonary hypertension or other end-organ dysfunction.<Cite n={3} /> Prognosis is determined by the cause; in idiopathic RCM it is generally poor.<Cite n={3} />

</Student>

## When to call an ambulance

RCM often progresses gradually to advanced heart failure.<Cite n={3} /> Life-threatening complications of decompensation include pulmonary oedema and cardiogenic shock, and hospital admission is also needed for dangerous heart rhythm disturbances.<Cite n={2} /> Call your local emergency number (112 in the EU, 911 in the US and Canada, 999 in the UK) if breathlessness suddenly gets much worse, especially at rest or lying down, if attacks of breathlessness start, if you faint or lose consciousness, or if chest pain or discomfort lasts more than 15 minutes.<Cite n={2} />

If you wake up at night short of breath or gain more than 2 kg in 3 days, tell your doctor straight away.<Cite n={2} /> Atrial fibrillation increases the risk of stroke and other thromboembolic events.<Cite n={1} /> The signs of a stroke are described in the article on [atrial fibrillation](../atrial-fibrillation/).

<Quiz />