Restrictive (least common)

  • Walls don't necessarily get bigger just stiffer. Stiff walls don't fill well ➔ diastolic dysfunction (they still kinda contract well)

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

SSX

  • 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

  • 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

  • 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