Cardiomyopathie restrictive
Révision par le Dr Toni Hazell, MRCGPDernière mise à jour par le Dr Colin Tidy, MRCGPDernière mise à jour le 22 septembre 2023
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Dans cet article :
Cardiomyopathy is defined as a 'myocardial disorder in which heart muscle is structurally and functionally abnormal without coronary artery disease, hypertension, valvular or congenital heart diseases'.1
There are five types, namely hypertrophic, dilated, arrhythmogenic, restrictive and unclassified. Cardiomyopathy is a significant cause of sudden cardiac death in young people.
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What is restrictive cardiomyopathy?2
Restrictive cardiomyopathy is a heterogeneous group of diseases characterised by restrictive ventricular function with a rapid rise in ventricular pressure with only small increases in filling volume due to increased myocardial stiffness.
The defining feature is the coexistence of persistent restrictive pathophysiology, diastolic dysfunction, non-dilated ventricles, and atrial dilatation, regardless of ventricular wall thickness and systolic function.
Beyond this shared haemodynamic hallmark, the phenotypic spectrum of restrictive cardiomyopathy is wide. There are 4 main disease mechanisms:
Interstitial fibrosis and intrinsic myocardial dysfunction.
Infiltration of extracellular spaces.
Accumulation of storage material within cardiomyocytes.
Endomyocardial fibrosis.
There are separate articles which discuss Cardiomyopathies, Dilated Cardiomyopathies and Arrhythmogenic Right Ventricular Cardiomyopathy.
How common is restrictive cardiomyopathy? (Epidemiology)
Restrictive cardiomyopathy is the least common of the major cardiomyopathies, representing 2% to 5% of cases.3
However, restrictive cardiomyopathy is a leading cause of heart transplantation.
Familial inheritance is not characteristic of restrictive cardiomyopathy.
Restrictive cardiomyopathy is more prevalent in tropical Africa than in the Western world.
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Causes of restrictive cardiomyopathy
Often no underlying cause is found. The causes of restrictive cardiomyopathies include:2
Familial:
Familial, unknown gene.
Sarcomeric protein mutations.
Troponin I (restrictive cardiomyopathy ± hypertrophic cardiomyopathy).
Essential light chain of myosin.
Familial amyloidosis.
Transthyretin (restrictive cardiomyopathy and neuropathy).
Apolipoprotein (restrictive cardiomyopathy and nephropathy).
Desminopathy.
Pseuxanthoma elasticum.
Anderson–Fabry disease.
Non-familial:
Amyloid (AL/prealbumin).
Endomyocardial fibrosis.
Hypereosinophilic syndrome.
Metastatic cancers.
Rayonnement.
Drugs (anthracyclines).
Idiopathique.
Infiltrative myocardial disease.
Cardiac amyloidosis - the most common cause of restrictive cardiomyopathy in the Western World.
The myocardium may be infiltrated by iron in haemochromatosis, glycogen in Pompe’s disease and Cori’s disease, or glycolipids in Fabry’s disease.
Restrictive cardiomyopathy symptoms (presentation)
Usually presents with heart failure but normal systolic function: dyspnoea, fatigue, loud third heart sound, pulmonary oedema, murmurs due to valve incompetence.
Heart size is usually normal or slightly enlarged.
Features of right ventricular failure predominate: raised JVP, with prominent x and y descents, hepatomegaly, oedema, ascites.
The clinical presentation of restrictive cardiomyopathy and constrictive pericarditis patients may be strikingly similar.4
Up to 75% of patients with idiopathic restrictive cardiomyopathy develop atrial fibrillation.5
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Enquêtes2
Restrictive pathophysiology can be demonstrated by cardiac catheterisation or Doppler echocardiography.
The specific conditions may usually be diagnosed based on clinical data, 12-lead electrocardiogram, echocardiography, nuclear medicine, or cardiac MRI, but further investigations may be needed, such as endomyocardial biopsy and genetic evaluation.
Diagnostic différentiel
Apart from constrictive pericarditis, which is the main differential diagnosis to consider, other constrictive diseases may mimic restrictive cardiomyopathy, so the following are often considered:
Metastases.
Rayonnement.
Cardiac tamponade - constrictive only.
Restrictive cardiomyopathy treatment and management2
Disease-modifying treatments are available only for cardiac amyloidosis and, partially, for iron overload cardiomyopathy.
In children, restrictive cardiomyopathy is primarily idiopathic, and transplantation is the treatment of choice.
Management of heart failure, including diuretics and angiotensin-converting enzyme (ACE) inhibitors. Loop diuretics to reduce pulmonary and peripheral oedema and ascites.
Amiodarone can reduce ventricular arrhythmias in high-risk patients.
All patients with restrictive cardiomyopathy and atrial fibrillation should be anticoagulated unless contra-indicated.5
Beta-blockers and non-dihydropyridine calcium-channel blockers may be used for rate control in those with atrial fibrillation.5 Beta-blockades may not be tolerated due to their negative chronotropic and, to a lesser extent, inotropic impact.
A pacemaker may be required (patients are often not able to tolerate the cardiac dysfunction associated with arrhythmias).
Implantable cardioverter defibrillator: to prevent sudden death in high-risk patients.6
Heart transplantation may be indicated for some patients.
Pronostic
Variable, depending on the underlying cause.
Restrictive cardiomyopathy due to amyloid has a worse prognosis.7
Cardiac amyloidosis is difficult to treat due to poor tolerance of most cardiovascular medication and poor outcome for transplantation.8
Recent advances in diagnosis and treatment of amyloid are, however, associated with improved prognosis.9
Autres lectures et références
- Ciarambino T, Menna G, Sansone G, et alLes cardiomyopathies : An Overview. Int J Mol Sci. 2021 Jul 19;22(14). pii : ijms22147722. doi : 10.3390/ijms22147722.
- Brown KN, Pendela VS, Ahmed I, et al; Restrictive Cardiomyopathy. StatPearls, Jan 2023.
- Vio R, Angelini A, Basso C, et al; Hypertrophic Cardiomyopathy and Primary Restrictive Cardiomyopathy: Similarities, Differences and Phenocopies. J Clin Med. 2021 May 1;10(9):1954. doi: 10.3390/jcm10091954.
- Elliott P, Andersson B, Arbustini E, et alClassification of the cardiomyopathies : a position statement from the European Society Of Cardiology Working Group on Myocardial and Pericardial Diseases (Classification des cardiomyopathies : une prise de position du groupe de travail de la Société européenne de cardiologie sur les maladies du myocarde et du péricarde). Eur Heart J. 2008 Jan;29(2):270-6. Epub 2007 Oct 4.
- Rapezzi C, Aimo A, Barison A, et al; Restrictive cardiomyopathy: definition and diagnosis. Eur Heart J. 2022 Dec 1;43(45):4679-4693. doi: 10.1093/eurheartj/ehac543.
- Brieler J, Breeden MA, Tucker J; la cardiomyopathie : Une vue d'ensemble. Am Fam Physician. 2017 Nov 15;96(10):640-646.
- Zwas DR, Gotsman I, Admon D, et al; Advances in the differentiation of constrictive pericarditis and restrictive cardiomyopathy. Herz. 2012 Sep;37(6):664-73.
- Mohmand-Borkowski A, Tang WH; Atrial fibrillation as manifestation and consequence of underlying cardiomyopathies: from common conditions to genetic diseases. Heart Fail Rev. 2014 May;19(3):295-304. doi: 10.1007/s10741-014-9424-0.
- Maron BJ; Can sudden cardiac death be prevented? Cardiovasc Pathol. 2010 Apr 7.
- Esplin BL, Gertz MA; Current trends in diagnosis and management of cardiac amyloidosis. Curr Probl Cardiol. 2013 Feb;38(2):53-96. doi: 10.1016/j.cpcardiol.2012.11.002.
- Sharma N, Howlett J; Current state of cardiac amyloidosis. Curr Opin Cardiol. 2013 Mar;28(2):242-8. doi: 10.1097/HCO.0b013e32835dd165.
- Chaulagain CP, Comenzo RL; New insights and modern treatment of AL amyloidosis. Curr Hematol Malig Rep. 2013 Dec;8(4):291-8. doi: 10.1007/s11899-013-0175-0.
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Historique de l'article
Les informations contenues dans cette page sont rédigées et évaluées par des cliniciens qualifiés.
Date de la prochaine révision : 20 septembre 2028
22 Sept 2023 | Dernière version

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