Cardiomyopathy - restrictive
Description
- Stiff, non-compliant ventricle -> impaired diastolic filling with normal or near-normal EF and normal/small cavity size
- Haemodynamic signature: steep rise in ventricular pressure for a small volume increase
- -> inc filling pressures -> biatrial enlargement with normal-sized ventricles
- -> fixed, low stroke volume; cardiac output falls with tachycardia and with loss of atrial kick
- The rarest of the classical cardiomyopathies, but the one most often missed - "HFpEF that does not behave like HFpEF"
The picture
- Marked right-sided congestion out of proportion to left-sided symptoms
- Small ventricles + huge atria on echo = the pattern to recognise
- Wall thickening with LOW voltage on ECG = infiltration (a thickened wall should give big voltages; if it does not, the wall is full of something that is not myocyte)
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