Restrictive Cardiomyopathy Flashcards
Restrictive cardiomyopathy can be classified into how many categories? Do they overlap?
4 categories
Yes
What are the four categories of RCM? (Restrictive cardiomyopathy)
Infiltrative cardiomyopathy
Storage disorders
Endomyocardial disorders
Noninfiltrative restrictive cardiomyopathy
The pneumonic “PLEASe Help” are the causes of restrictive cardiomyopathy. What does it stand for?
Postradiation/Post surgery fibrosis
Löffler endocarditis
Endocardial fibroelastosis
Amyloidosis
Sarcoidosis
Hemochromatosis
What is the etiology of infiltrative cardiomyopathy?
- what are they caused by?
Amyloidosis: caused by an accumulation of abnormal proteins in the myocardium
Sarcoidosis: secondary to deposition of granulomas in the myocardium
Primary hyperoxaluria
Etiology of storage disorders in RCM?
-what causes this etiology?
Hereditary hemochromatosis: increased absorption of iron from GI tract (more commonly causes dilated cardiomyopathy)
Some storage disorders, e.g. _________ (most common lysosomal lipid storage disease), can also cause infiltrative cardiomyopathy
Gaucher disease
What Endomyocardial disorders can cause RCM?
Hypereosinophilia (Loeffler endocarditis)
Endocardial fibroelastosis
Radiotherapy to the chest
What is löffler endocarditis (loeffler endocarditis)?
Hypereosinophilia
Eosinophilic infiltration of the myocardium
What is Endocardial fibroelastosis?
A diffuse thickening of the left ventricle endocardium from the proliferation of fibrous and elastic tissue
Etiology of noninfiltrative cardiomyopathy?
Idiopathic RCM
Systemic sclerosis
Pathophysiology of RCM?
Infiltration or proliferation of connective or fibrotic tissue —> decreased elasticity of myocardium —> decreased ventricular compliance (sever diastolic dysfunction) leading to hypotension
Clinical features of RCM?
-symptoms
-physical examination
-Auscultation findings
-features of any underlying diseases
Symptoms: Dyspnea on exertion, leg swelling, palpitations, syncope
Physical examination: features of right sides HF (JVD, peripheral edema, hepatomegaly and ascites, kussmaul sign)
Auscultation: prominent S4 gallop, signs of HF
Underlying:
•Amyloidosis: carpal tunnel syndrome, large tongue
•hemochromatosis: bronze skin
•Sarcoidosis: Erythema nodosum
Diagnostic tests to confirm RCM?
EKG, CXR, lab studies, TTE
What will the EKG show with suspected RCM?
Low voltage QRS complexes
Conduction disorders: bundle branch block and afib
What with the CXR show?
Evidence of underlying disease (sarcoidosis)
