Restrictive cardiomyopathy

Last revised by Daniel J Bell on 1 Mar 2021

Restrictive cardiomyopathy is the least common subtype of cardiomyopathy and is characterized by a marked decrease in ventricular compliance. 

Patients can present with symptoms and signs of left ventricular failure and/or right ventricular failure 9

It is predominantly a disease of diastolic dysfunction where the systolic (contractile) of the myocardium is usually unaffected. 

Recognized causes which can give a restrictive type pattern include:

The heart size can be normal. Sometimes there may be evidence of biatrial dilatation.

Diastolic dysfunction may be observed with spectral Doppler interrogation of the transmitral filling velocities and tissue Doppler of the mitral annular velocity. The presence of restrictive physiology can be supported with the following:

  • transmitral flow velocities
    • amplitude of mitral E wave >100 cm/s
    • deceleration time (DT) <160 ms
    • ratio between E/A >2
  • tissue Doppler imaging (TDI) of the mitral annulus
    • characteristic, and pathognomonic, of restrictive filling physiology is the marked decrease in TDI velocities coupled with brisk reversal of hepatic venous Doppler flow upon inspiration
      • an early diastolic (e') septal annular velocity of <8 cm/s
        • lateral annular e' reduced when <10 cm/s
      • average E/e' ratio above 13 (using mean of septal and lateral e')
        • correlates with elevated left atrial pressure

Pulmonary venous Doppler may be used as an adjunctive measure in equivocal cases, which should demonstrate:

  • reversal of normally systolic dominant filling pattern, with an S/D ratio <1
  • elevation in peak atrial reversal (AR) velocities
    • above 35 cm/s considered elevated

Cardiac MRI is useful for differentiating between constrictive pericarditis. Biatrial enlargement with minimal or no ventricular enlargement may be present. Cine MRI will show altered diastolic filling. 

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