Restrictive (least common)
Restrictive (least common)
Walls don't necessarily get bigger just stiffer. Stiff walls don't fill well ➔ diastolic dysfunction (they still kinda contract well)
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PATHO
PATHO
Amyloidosis (MC), sarcoidosis, hemochromatosis, systemic sclerosis, chemo/radiation
Deposits in myocardium ➔ proliferation of connective tissue ➔ ↓ elasticity/compliance (ability to fill)
Blood backs up ➔ atrial congestion ➔ atrial enlargement (more blood can stay in atrium) ➔ systemic venous congestion ➔ RIGHT HF
Familial/primary amyloidosis ➔ light-chain amyloid proteins toxic to cardiomyocytes
Sarcoidosis ➔ granulomatous deposits
Hemochromatosis deposits excess Fe in heart, usually ➔ DCM
Scleroderma ➔ connective tissue replaces myocytes
RHF: JVD, Peripheral edema, Hepatomegaly, Ascites
If the blood fills up the right ventricle, the excess stays in right atrium, more blood comes into filled atria ➔ dilation of atria
Adding more water to a full cup just spills out. Think of the water all over the counter as the blood going back into vena cava
This backup causes right sided HF sx. Right MC than left sided sx because contractility is somewhat preserved (initially)
Amyloidosis: macroglossia
Scleroderma (rapid progression): cutaneous manifestations spread trunk ➔ elbow; dysphagia
Hemochromatosis: bronze skin diabetes
DX
DX
ECHO shows diastolic dysfunction WITH atrial enlargement/dilation without ventricle enlargement/dilation
Cardiac MRI
Amyloidosis DX: endo/myocardium biopsy. Stained with congo-red ➔ apple green bifringence (with polarized light)
EKG shows low voltage QRS and conduction problems like LBB
Cardiac cath shows ↑ atrial pressure
Do the ECHO first
Will also show reduced diastolic filling but EF is preserved (might even be ↑) because diastolic volume is so low that what squirts out (EF) is also low
Biopsy if they already told you the echo or they ask for definitive and they have infiltrative process
Deposits don't really have electrical activity so QRS amplitude is no bueno
TX
TX
Treat underlying
Amyloidosis/sarcoidosis needs steroids
Hemochromatosis needs chelation/phlebotomy
Can use β-blockers/CCB for rate control to ↑ ventricle filling time
Diuretics for fluid overload
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