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Restrictive cardiomyopathy

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Restrictive cardiomyopathy
Other namesObliterative cardiomyopathy, infiltrative cardiomyopathy, constrictive cardiomyopathy[1]
Micrograph of cardiac amyloidosis, a cause of restrictive cardiomyopathy. Congo red stain.
SpecialtyCardiology Edit this on Wikidata

Restrictive cardiomyopathy (RCM) is a form of cardiomyopathy in which the walls of the heart are rigid (but not thickened).[2][3] Thus the heart is restricted from stretching and filling with blood properly. It is the least common of the three original subtypes of cardiomyopathy: hypertrophic, dilated, and restrictive.[1]

It should not be confused with constrictive pericarditis, a disease which presents similarly but is very different in treatment and prognosis.[1]

Signs and symptoms

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Untreated hearts with RCM often develop the following characteristics:[4]

  • M or W configuration in an invasive hemodynamic pressure tracing of the RA
  • Square root sign of part of the invasive hemodynamic pressure tracing Of The LV
  • Biatrial enlargement
  • Thickened LV walls (with normal chamber size)
  • Thickened RV free wall (with normal chamber size)
  • Elevated right atrial pressure (>12mmHg),
  • Moderate pulmonary hypertension,
  • Normal systolic function,
  • Poor diastolic function, typically Grade III - IV Diastolic heart failure.

Those affected by RCM will experience decreased exercise tolerance, fatigue, jugular venous distention, peripheral edema, and ascites.[3] Arrhythmias and conduction blocks are common.

Causes

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RCM can be caused by genetic or non-genetic factors.[5][6][7] Thus it is possible to divide the causes into primary and secondary.[8] The common modern organization is into Infiltrative, storage diseases, non-infiltrative, and endomyocardial etiologies:[9]

The most common cause of restrictive cardiomyopathy is amyloidosis.[3]

Mechanism

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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, reducing preload and end-diastolic volume. Thus, blood flow is reduced, and blood volume that would normally enter the heart is backed up in the circulatory system. In time, restrictive cardiomyopathy patients develop diastolic dysfunction and eventually heart failure.[13]

Diagnosis

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Diagnosis is typically made via echocardiography. Patients will demonstrate normal systolic function, diastolic dysfunction, and a restrictive filling pattern.[9] 2-dimensional and Doppler studies are necessary to distinguish RCM from constrictive pericarditis. If a patient has restrictive cardiomyopathy, the Doppler study should present poorly maintained ventricular filling on the E-wave and little to no late ventricular filling on the A-wave leading to the dip and plateau pattern of the early diastolic pressure marks seen on the ECG.[14] Cardiac MRI and transvenous endomyocardial biopsy may also be necessary in some cases.[3][9] Reduced QRS voltage on EKG may be an indicator of amyloidosis-induced restrictive cardiomyopathy.[9]

Treatment

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Treatment of restrictive cardiomyopathy should focus on management of causative conditions (for example, using corticosteroids if the cause is sarcoidosis), and slowing the progression of cardiomyopathy.[9] Salt-restriction, diuretics, angiotensin-converting enzyme inhibitors, and anticoagulation may be indicated for managing restrictive cardiomyopathy.[15] As a consequence of reduced blood flow through the heart, there is an elevation in filling pressures, aimed at sustaining optimal blood circulation throughout the body. However, the excessive use of diuretics may lead to inadequate blood perfusion in body tissues and, consequently, tissue hypoperfusion due to a reduction in overall blood volume.[16]

Calcium channel blockers are generally contraindicated due to their negative inotropic effect, particularly in cardiomyopathy caused by amyloidosis.[17][18] Digoxin, calcium channel blocking drugs and beta-adrenergic blocking agents provide little benefit, except in the subgroup of restrictive cardiomyopathy with atrial fibrillation.[19] Vasodilators are also typically ineffective because systolic function is usually preserved in cases of RCM.[3]

Heart failure resulting from restrictive cardiomyopathy will usually eventually have to be treated by cardiac transplantation or left ventricular assist device.[15]

Epidemiology

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Endomyocardial fibrosis is generally limited to the tropics and sub-saharan Africa.[9] The highest incidence of death caused by cardiac sarcoidosis is found in Japan.[20]

References

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  1. ^ a b c Hancock, EW (September 2001). "Differential diagnosis of restrictive cardiomyopathy and constrictive pericarditis". Heart. 86 (3): 343–9. doi:10.1136/heart.86.3.343. PMC 1729880. PMID 11514495.
  2. ^ "restrictive cardiomyopathy" at Dorland's Medical Dictionary
  3. ^ a b c d e Pathophysiology of heart disease : a collaborative project of medical students and faculty. Lilly, Leonard S., Harvard Medical School. (5th ed.). Baltimore, MD: Wolters Kluwer/Lippincott Williams & Wilkins. 2011. ISBN 978-1605477237. OCLC 649701807.{{cite book}}: CS1 maint: others (link)
  4. ^ Muchtar, E; Blauwet, L; Gertz, M (2017). "Restrictive Cardiomyopathy: Genetics, Pathogenesis, Clinical Manifestations, Diagnosis, and Therapy". Circulation Research. 121 (7): 819–837. doi:10.1161/CIRCRESAHA.117.310982. PMID 28912185.
  5. ^ Brodehl, Andreas; Ferrier, Raechel A.; Hamilton, Sara J.; Greenway, Steven C.; Brundler, Marie-Anne; Yu, Weiming; Gibson, William T.; McKinnon, Margaret L.; McGillivray, Barbara (March 2016). "Mutations in FLNC are Associated with Familial Restrictive Cardiomyopathy". Human Mutation. 37 (3): 269–279. doi:10.1002/humu.22942. ISSN 1098-1004. PMID 26666891. S2CID 35455240.
  6. ^ Brodehl, Andreas; Gaertner-Rommel, Anna; Klauke, Bärbel; Grewe, Simon Andre; Schirmer, Ilona; Peterschröder, Andreas; Faber, Lothar; Vorgerd, Matthias; Gummert, Jan (2017). "The novel αB-crystallin (CRYAB) mutation p.D109G causes restrictive cardiomyopathy". Human Mutation. 38 (8): 947–952. doi:10.1002/humu.23248. ISSN 1098-1004. PMID 28493373. S2CID 13942559.
  7. ^ Brodehl, Andreas; Pour Hakimi, Seyed Ahmad; Stanasiuk, Caroline; Ratnavadivel, Sandra; Hendig, Doris; Gaertner, Anna; Gerull, Brenda; Gummert, Jan; Paluszkiewicz, Lech; Milting, Hendrik (2019-11-11). "Restrictive Cardiomyopathy is Caused by a Novel Homozygous Desmin (DES) Mutation p.Y122H Leading to a Severe Filament Assembly Defect". Genes. 10 (11): 918. doi:10.3390/genes10110918. ISSN 2073-4425. PMC 6896098. PMID 31718026.
  8. ^ Crawford, Michael H. (2003). Current diagnosis & treatment in cardiology. New York: Lange Medical Books/McGraw-Hill. pp. 188. ISBN 978-0-8385-1473-3.
  9. ^ a b c d e f Muchtar, Eli; Blauwet, Lori A.; Gertz, Morie A. (2017-09-15). "Restrictive Cardiomyopathy: Genetics, Pathogenesis, Clinical Manifestations, Diagnosis, and Therapy". Circulation Research. 121 (7): 819–837. doi:10.1161/CIRCRESAHA.117.310982. ISSN 0009-7330. PMID 28912185.
  10. ^ Brodehl, Andreas; Pour Hakimi, Seyed Ahmad; Stanasiuk, Caroline; Ratnavadivel, Sandra; Hendig, Doris; Gaertner, Anna; Gerull, Brenda; Gummert, Jan; Paluszkiewicz, Lech; Milting, Hendrik (2019-11-11). "Restrictive Cardiomyopathy is Caused by a Novel Homozygous Desmin (DES) Mutation p.Y122H Leading to a Severe Filament Assembly Defect". Genes. 10 (11): 918. doi:10.3390/genes10110918. ISSN 2073-4425. PMC 6896098. PMID 31718026.
  11. ^ Brodehl, Andreas; Gaertner-Rommel, Anna; Klauke, Bärbel; Grewe, Simon Andre; Schirmer, Ilona; Peterschröder, Andreas; Faber, Lothar; Vorgerd, Matthias; Gummert, Jan; Anselmetti, Dario; Schulz, Uwe (August 2017). "The novel αB-crystallin ( CRYAB ) mutation p.D109G causes restrictive cardiomyopathy". Human Mutation. 38 (8): 947–952. doi:10.1002/humu.23248. ISSN 1059-7794. PMID 28493373. S2CID 13942559.
  12. ^ Brodehl, Andreas; Ferrier, Raechel A.; Hamilton, Sara J.; Greenway, Steven C.; Brundler, Marie-Anne; Yu, Weiming; Gibson, William T.; McKinnon, Margaret L.; McGillivray, Barbara; Alvarez, Nanette; Giuffre, Michael (March 2016). "Mutations in FLNC are Associated with Familial Restrictive Cardiomyopathy". Human Mutation. 37 (3): 269–279. doi:10.1002/humu.22942. PMID 26666891. S2CID 35455240.
  13. ^ "Restrictive Cardiomyopathy". The Lecturio Medical Concept Library. Retrieved 28 June 2021.
  14. ^ Nihoyannopoulos, P and D Dawson. 2009. Restrictive cardiomyopathies. European Journal of Echocardiography , Volume 10, Issue 8, Pages iii23–iii33, https://doi.org/10.1093/ejechocard/jep156
  15. ^ a b "Restrictive Cardiomyopathy Treatment & Management". 2014-12-18. Retrieved 2015-06-10.
  16. ^ Muchtar, E, LA Blauwet and MA Gertz. 2017. Restrictive cardiomyopathy: genetics, Ppathogenesis, clinical manifestations, diagnosis, and therapy. Circulation Research. 121:819–837. https://doi.org/10.1161/CIRCRESAHA.117.310982
  17. ^ Pollak, A; Falk, R H (1993-08-01). "Left ventricular systolic dysfunction precipitated by verapamil in cardiac amyloidosis". Chest. 104 (2): 618–620. doi:10.1378/chest.104.2.618. ISSN 0012-3692. PMID 8339658.
  18. ^ Gertz, Morie A.; Falk, Rodney H.; Skinner, Martha; Cohen, Alan S.; Kyle, Robert A. (1985-06-01). "Worsening of congestive heart failure in amyloid heart disease treated by calcium channel-blocking agents". American Journal of Cardiology. 55 (13): 1645. doi:10.1016/0002-9149(85)90995-6. ISSN 0002-9149. PMID 4003314.
  19. ^ Artz, Gregory; Wynne, Joshua (October 2000). "Restrictive Cardiomyopathy". Current Treatment Options in Cardiovascular Medicine. 2 (5): 431–438. doi:10.1007/s11936-000-0038-6. ISSN 1092-8464. PMID 11096547. S2CID 45162583.
  20. ^ Hulten, Edward; Aslam, Saira; Osborne, Michael; Abbasi, Siddique; Bittencourt, Marcio Sommer; Blankstein, Ron (February 2016). "Cardiac sarcoidosis—state of the art review". Cardiovascular Diagnosis and Therapy. 6 (1): 50–63. doi:10.3978/j.issn.2223-3652.2015.12.13. ISSN 2223-3652. PMC 4731586. PMID 26885492.
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