Infiltrative CM

Question 1

What do you see with your CVP waveform with infiltrative cardiomyopathies?

Answer 1

Prominent a and v waves with rapid X and Y descents

Question 2

What kind of heart failure do you normally have with infiltrative cardiomyopathies?

Answer 2

Heart failure with preserved EF (diastolic dysfunction)

Question 3

What can you see on EKG with infiltrative cardiomyopathies?

Answer 3

Infiltration within the conduction system can cause high-degree AV blocks, ventricular arrhythmias, and bifascicular blocks

Question 4

What are some common echo findings with infiltrative cardiomyopathies?

Answer 4

Biatrial enlargement + normal LVEF + thickened walls as disease progresses

Question 5

What are some common right heart cath findings in infiltrative CM?

Answer 5

Square root sign (rapid ventricular filling resulting in a drop in LVEDP followed by an early and high plateau - also seen with constrictive pericarditis)

Question 6

What is a common left heart cath finding in infiltrative CM that differentiates it from CP?

Answer 6

LVEDP > RVEDP by 5 mmHg or more (different from constrictive pericarditis where chamber pressures are equal and have interdependence with filling)

Question 7

How can MRI tell restrictive CM from constrictive pericarditis?

Answer 7

Late gadolinium enhancement on MRI suggests infiltrative pathology

Question 8

What is the most common infiltrative CM?

Answer 8

Amyloidosis

Question 9

What do you see in the myocardium with amyloidosis?

Answer 9

Deposits of abnormally organized protein multimers (amyloid fibrils) + misfolding pattern (antiparallel beta-sheets)

Question 10

How does amyloidosis first present as (cardiac)?

Answer 10

Diastolic heart failure with progressive obliteration of the ventricular cavity –> eventually leading to systolic heart failure + arrhythmias and autonomic instability

Question 11

What is primary/AL amyloidosis?

Answer 11

Plasma cell proliferation resulting in overproduction of immunoglobulin light chains and multiorgan deposition (autonomic and peripheral nervous systems, liver, kidney)

Question 12

What medications are primary/AL amyloidosis patients sensitive to?

Answer 12

Increased sensitivity to beta blockers and ACE inhibitors

Question 13

What is the treatment for primary/AL amyloidosis?

Answer 13

Chemo and immunotherapeutic agents + stem cell transplant

Question 14

What do you need to know about familial/ATTR amyloid?

Answer 14

Affects multiple organ systems + senile variant is specific to myocardial deposition + liver is the site of transthyretin (precursor protein) production

Question 15

What do you need to know about secondary/AA amyloid?

Answer 15

Reactive process triggered by inflammation/infection + typically mild disease

Question 16

What is isolated atrial amyloidosis?

Answer 16

Deposition of ANP in the atria –> leads to increased incidence of atrial fibrillation (otherwise little clinical significance)

Question 17

What are some EKG findings found in amyloidosis?

Answer 17

Low voltage EKG despite increased LV wall thickness + conduction abnormalities + inferoseptal ST abnormalities (pseudoinfarction pattern)

Question 18

What are some TEE findings in amyloidosis?

Answer 18

Starry sky appearance with impaired global longitudinal strain with apical sparing

Question 19

What tests can you do to diagnose amyloidosis?

Answer 19

Monoclonal light chain assays (kappa or lambda suggests AL amyloidosis) + scintigraphy with technetium-99m pyrophosphate (100% sensitive for TTR amyloidosis) + endomyocardial biopsy (congo red staining for amyloid)

Question 20

What is the pathophysiology of cardiac sarcoidosis?

Answer 20

Non-caseating granulomatous disease + activated macrophages within the granulomas produce excessive 1,25-hydroxy vitamin D which increases Ca levels –> macrophages transform into epitheloid cells that proceduce ACE (correlates with granuloma burdon and disease severity)

Question 21

What organ system is most affected by sarcoidosis?

Answer 21

The lungs (>90% of patients), can cause end-stage pulmonary fibrosis leading to pulmonary hypertension

Question 22

What is the treatment for sarcoidosis?

Answer 22

Corticosteroids; other immunomodulator treatments include methotrexate, azathioprine, and infliximab

Question 23

What are common cardiac findings with sarcoidosis?

Answer 23

MRI late gad enhancement seen in the basal and lateral LV walls + arrhythmias (a.fib in 18% and atrial tachycardiacs in 7%) + conduction abnormalities (prolonged QT, bifascicular block, high grade AV blocks + ventricular tachycardias) + restrictive CM

Question 24

How do you diagnose cardiac sarcoidosis?

Answer 24

1. Histologic diagnosis from myocardial tissue (non-caseating granulomas seen in heart tissue) 2. Clinical diagnosis (histologic diagnosis of other organs + one or more of the following: steroid-responsive CM or heart block, unexplained reduced LVEF, unexplained sustained VT, high-grade heart block, and imaging consistent with cardiac sarcoid

Question 25

What disease states do you see fatty infiltration of the myocardium?

Answer 25

Arrhythmogenic RV cardiomyopathy + myotonic dystrophy + myocardial infarction + a. fib + LV hypertrophy + Fabry disease

Question 26

What is lipomatous hypertrophy a risk factor for?

Answer 26

Atrial fibrillation

Question 27

What is the hallmark of cardiac fatty infiltration?

Answer 27

Arrhythmias and conduction abnormalities

Question 28

What is Gaucher disease?

Answer 28

Lysosomal storage disorder; cardiac involvement is rare but can present as restrictive CM and pulmonary HTN

Question 29

What do you see on cardiac MRI with Gaucher disease

Answer 29

You do NOT see late myocardial gad enhancement

Question 30

What is Hunter and Hurler Syndrome?

Answer 30

Mucopolysaccharidoses (deposition of glycoasminoglycans throughout the body) + myocardial involvement is frequently silent but can present as conduction abnormalities

Question 31

What cardiac findings are most common in Hunter and Hurler syndrome?

Answer 31

Valvular disease (stenosis or regurgitation) and coronary artery involvement

Question 32

How do you differentiate infiltrative cardiomyopathy from hypertrophic cardiomyopathy via EKG?

Answer 32

HCM presents with high voltage EKG while infiltrative CMs present with low-voltage EKGs

Question 33

What common finding of infiltrative cardiomyopathies is unique to cardiac amyloidosis?

Answer 33

Autonomic instability