Restrictive cardiomyopathy

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Restrictive cardiomyopathy
Other namesObliterative cardiomyopathy, infiltrative cardiomyopathy, constrictive 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

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

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

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

Diagnosis is typically made via echocardiography. Patients will demonstrate normal systolic function, diastolic dysfunction, and a restrictive filling pattern.

EKG may be an indicator of amyloidosis-induced restrictive cardiomyopathy.[9]

Treatment

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.

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

left ventricular assist device.[15]

Epidemiology

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

  1. ^
    PMID 11514495
    .
  2. ^ "restrictive cardiomyopathy" at Dorland's Medical Dictionary
  3. ^
    OCLC 649701807.{{cite book}}: CS1 maint: others (link
    )
  4. PMID 28912185
    .
  5. S2CID 35455240
    .
  6. S2CID 13942559
    .
  7. PMID 31718026
    .
  8. ISBN 978-0-8385-1473-3
    .
  9. ^
    PMID 28912185
    .
  10. PMID 31718026
    .
  11. S2CID 13942559
    .
  12. 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. {{cite journal}}: Cite journal requires |journal= (help)
  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. PMID 8339658
    .
  18. PMID 4003314
    .
  19. S2CID 45162583
    .
  20. PMID 26885492
    .

External links
Retrieved from "https://en.wikipedia.org/w/index.php?title=Restrictive_cardiomyopathy&oldid=1217654441"