Infiltrative CM Flashcards
What do you see with your CVP waveform with infiltrative cardiomyopathies?
Prominent a and v waves with rapid X and Y descents
What kind of heart failure do you normally have with infiltrative cardiomyopathies?
Heart failure with preserved EF (diastolic dysfunction)
What can you see on EKG with infiltrative cardiomyopathies?
Infiltration within the conduction system can cause high-degree AV blocks, ventricular arrhythmias, and bifascicular blocks
What are some common echo findings with infiltrative cardiomyopathies?
Biatrial enlargement + normal LVEF + thickened walls as disease progresses
What are some common right heart cath findings in infiltrative CM?
Square root sign (rapid ventricular filling resulting in a drop in LVEDP followed by an early and high plateau - also seen with constrictive pericarditis)
What is a common left heart cath finding in infiltrative CM that differentiates it from CP?
LVEDP > RVEDP by 5 mmHg or more (different from constrictive pericarditis where chamber pressures are equal and have interdependence with filling)
How can MRI tell restrictive CM from constrictive pericarditis?
Late gadolinium enhancement on MRI suggests infiltrative pathology
What is the most common infiltrative CM?
Amyloidosis
What do you see in the myocardium with amyloidosis?
Deposits of abnormally organized protein multimers (amyloid fibrils) + misfolding pattern (antiparallel beta-sheets)
How does amyloidosis first present as (cardiac)?
Diastolic heart failure with progressive obliteration of the ventricular cavity –> eventually leading to systolic heart failure + arrhythmias and autonomic instability
What is primary/AL amyloidosis?
Plasma cell proliferation resulting in overproduction of immunoglobulin light chains and multiorgan deposition (autonomic and peripheral nervous systems, liver, kidney)
What medications are primary/AL amyloidosis patients sensitive to?
Increased sensitivity to beta blockers and ACE inhibitors
What is the treatment for primary/AL amyloidosis?
Chemo and immunotherapeutic agents + stem cell transplant
What do you need to know about familial/ATTR amyloid?
Affects multiple organ systems + senile variant is specific to myocardial deposition + liver is the site of transthyretin (precursor protein) production
What do you need to know about secondary/AA amyloid?
Reactive process triggered by inflammation/infection + typically mild disease
What is isolated atrial amyloidosis?
Deposition of ANP in the atria –> leads to increased incidence of atrial fibrillation (otherwise little clinical significance)
What are some EKG findings found in amyloidosis?
Low voltage EKG despite increased LV wall thickness + conduction abnormalities + inferoseptal ST abnormalities (pseudoinfarction pattern)
What are some TEE findings in amyloidosis?
Starry sky appearance with impaired global longitudinal strain with apical sparing
What tests can you do to diagnose amyloidosis?
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)
What is the pathophysiology of cardiac sarcoidosis?
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)
What organ system is most affected by sarcoidosis?
The lungs (>90% of patients), can cause end-stage pulmonary fibrosis leading to pulmonary hypertension
What is the treatment for sarcoidosis?
Corticosteroids; other immunomodulator treatments include methotrexate, azathioprine, and infliximab
What are common cardiac findings with sarcoidosis?
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
How do you diagnose cardiac sarcoidosis?
- 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
What disease states do you see fatty infiltration of the myocardium?
Arrhythmogenic RV cardiomyopathy + myotonic dystrophy + myocardial infarction + a. fib + LV hypertrophy + Fabry disease
What is lipomatous hypertrophy a risk factor for?
Atrial fibrillation
What is the hallmark of cardiac fatty infiltration?
Arrhythmias and conduction abnormalities
What is Gaucher disease?
Lysosomal storage disorder; cardiac involvement is rare but can present as restrictive CM and pulmonary HTN
What do you see on cardiac MRI with Gaucher disease
You do NOT see late myocardial gad enhancement
What is Hunter and Hurler Syndrome?
Mucopolysaccharidoses (deposition of glycoasminoglycans throughout the body) + myocardial involvement is frequently silent but can present as conduction abnormalities
What cardiac findings are most common in Hunter and Hurler syndrome?
Valvular disease (stenosis or regurgitation) and coronary artery involvement
How do you differentiate infiltrative cardiomyopathy from hypertrophic cardiomyopathy via EKG?
HCM presents with high voltage EKG while infiltrative CMs present with low-voltage EKGs
What common finding of infiltrative cardiomyopathies is unique to cardiac amyloidosis?
Autonomic instability
