Pathophyiology of restrictive cardiomyopathy

Restrictive cardiomyopathy (RCM) is a rare heart condition characterized by stiffness and impaired relaxation of the heart muscle, leading to impaired ventricular filling. The pathophysiology involves:

1. Myocardial fibrosis: Scarring and stiffening of the heart muscle, often due to:
- Infiltrative diseases (e.g., amyloidosis, sarcoidosis)
- Genetic mutations (e.g., desmin, troponin)
- Radiation therapy
2. Impaired relaxation: Reduced ability of the heart muscle to relax and fill with blood during diastole
3. Diastolic dysfunction: Elevated filling pressures and reduced ventricular volumes
4. Systolic function: Often preserved, but can be impaired in advanced cases
5. Increased stiffness: Reduced compliance of the heart muscle, leading to:
- Elevated end-diastolic pressures
- Reduced cardiac output
6. Neurohormonal activation: Activation of stress hormones (e.g., norepinephrine, renin-angiotensin-aldosterone system)
7. Cellular changes: Alterations in cardiomyocyte structure and function, including:
- Hypertrophy
- Apoptosis
- Fibrosis

Consequences:

1. Heart failure: Reduced cardiac output and increased filling pressures
2. Arrhythmias: Atrial fibrillation, ventricular tachycardia
3. Thromboembolism: Increased risk due to stagnant blood flow
4. Progressive disease: Worsening symptoms and reduced survival

Note: RCM can be primary (idiopathic) or secondary to various conditions. Accurate diagnosis and management are crucial to improve outcomes.


#Cardiomyopathy