Restrictive cardiomyopathy resident survival guide
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Restrictive cardiomyopathy is defined as heart-muscle disease with impaired ventricular filling usually due to increased stiffness. The diastolic volume of either or both ventricles is normal or decreased,the systolic function usually remains normal and wall thickness may be normal or increased. The symptoms and signs may consist of right (jugular venous pressure, peripheral edema, and ascites) or left ventricular failure (breathlessness and evidence pulmonary edema).
- Idiopathic cardiomyopathy
- Endomyocardial fibrosis
- Toxic effects of anthracycline
Complete Diagnostic Approach
A complete diagnostic approach should be carried out after a focused initial rapid evaluation is conducted and following initiation of any urgent intervention.
History and symptoms:
❑ Hints for etiology (history of pericarditis/ tuberculosis/ trauma DD:contrictive pericarditis or amyloidosis/ sarcoidosis)
❑ Vital signs:
❑ General appearance:
❑ Complete blood count
Imaging and additional tests:
❑ Noninvasive imaging and tests:
Invasive imaging and tests:
- Loop diuretics (low to medium dose): to relieve venous congestion in the pulmonary and systemic circulation, may cause signs of hypotension and hypoperfusion. Monitor systemic perfusion with physical examination, blood urea nitrogen and serum creatinine concentration
- Calcium channel blockers: may increase diastolic function
- Beta blockers: may have benefit by improving ventricular relaxation
- Angiotensin-converting enzyme inhibitors and/or angiotensin II receptor blockers: may improve diastolic filling
- Digoxin: increases intracellular calcium and therefore should be used with caution
- Treat atrial fibrillation: atrial fibrillation with the removal of the atrial contribution to ventricular filling may worsen existing diastolic dysfunction, patients with atrial fibrillation should be anticoagulated
- Advanced conduction-system disease (i.e. advanced AV-block): Treat by the implantation of a pacemaker
- Malignant ventricular arrhythmias:May require treatment with an automatic implantable defibrillator or an antitachycardia device
- Cardiac transplantation should be performed in eligible patients with intractable heart failure
- Kushwaha SS, Fallon JT, Fuster V (1997). "Restrictive cardiomyopathy". N Engl J Med. 336 (4): 267–76. doi:10.1056/NEJM199701233360407. PMID 8995091.
- DePasquale EC, Nasir K, Jacoby DL (2012). "Outcomes of adults with restrictive cardiomyopathy after heart transplantation". J Heart Lung Transplant. 31 (12): 1269–75. doi:10.1016/j.healun.2012.09.018. PMID 23079066.