CardiomyopathyCraig Ernst MHS, PA-C

Lock Haven University

Cardiomyopathy

General term indicating disease of cardiac muscle resulting in abnormal function

Divided into three types: Dilated cardiomyopathy-ventricular dilation Hypertrophic cardiomyopathy-myocardial

hypertrophy Restrictive cardiomyopathy-impaired

ventricular filling Can have characteristics of more than one

Dilated Cardiomyopathy (DCM) Characterized by dilation and impaired

systolic function of left &/or right ventricle Most common DCM is ischemic

cardiomyopathy Idiopathic (ICM) next most common

Familial autosomal dominant in 20% of cases.

Role of coxsackie/adenovirus and immune mediated etiology unknown.

DCM Many cases of systemic heart muscle disease

present with features of DCM including: Ischemic/rheumatic CVD Generalized disease- hemochromatosis, sarcoid Connective tissue disease-SLE, systemic sclerosis Neuromuscular disease-Friederich’s ataxia etc Glycogen storage disease Primary heart muscle disease- amyloidosis Alcohol excess Cytotoxic drugs-doxorubicin, cyclophosphamide Pregnancy

Starling Curve

Starling Curve

Volume

Con

tract

ility

DCM Clinical features:

R/L heart failure Arrhythmia Emboli Cardiomegaly Tachycardia JVD 3rd/4th heart sounds basiler crackles displaced PMI

DCM Evaluation

CXR: cardiomegaly EKG: diffuse non specific ST-T wave changes,

LBBB common, tachycardia, conduction abnormalities, arrhythmias

Echo: poor chamber contraction and dilated chambers

If CAD suspected, cardiac catheterization Endomyocardial biopsy for research only.

DCM Treatment

Rx failure & arrhythmias Ace Inhibitors a must in failure management Non-specific Beta blockade:

Carvedilol, ??, ??

Anticoagulation for A.fib/mural thrombus. CRT-D (Bi-V AICD) Transplant

Sudden death – Due to V. Tach. or V. Fib

Hypertrophic Cardiomyopathy (HCM)

Complex heart disease due to the asymmetric left ventricular hypertrophy, left ventricular stiffness, mitral valve changes and cellular changes (myocardial disarray)

60 % inherited 40 % sporadic HTN, Aging, Unknown

Most autosomal dominant w/ variable penetrance

Other Terms Hypertrophic Obstructive Cardiomyopathy

(HOCM) Idiopathic hypertrophic subaortic stenosis (IHSS) Asymmetrical septal hypertrophy (ASH)

Systolic anterior motion (SAM) of mitral apparatus

Pathophysiology

HOCM is a subvalvular obstruction! Distinct from valvular Aortic Stenosis

(pressure gradient across valve) Gradient/obstruction increases with lower

LV volume HOCM pts here do better when full and slow

Standing after squatting/Valvsalva lower venous return & increase outflow

obstruction and intensity of murmur

HCM without obstruction

HCM with obstruction HOCM)

MV leaflet

HOCM: Clinical Features: Chest pain Dyspnea Syncope/Pre-syncope (typically with exertion) Palpitations Sudden Death (arrhythmia)

Typically occurs in asymptomatic young adults or adolescents (10-35 y/o)

Family history of sudden death, sustained ventricular tachycardia, & B/P response to exercise are recognized risk factors

Diastolic dysfunction with impaired filling Outflow tract obstruction occurs in 1/3 of cases

Physical Exam Systolic murmur; with little to no radiation to

neck vessels (increased by maneuvers that decrease preload such as Valvsalva or squatting) Decreases ventricular filling

May hear systolic murmur of MR Weak late carotid pulse (late obstruction) Diagnosed by echocardiogram.

Evaluation EKG-LVH with ST-T wave changes CXR-normal ECHO indicated if PE suggests IHSS Pedigree analysis (ECHO to screen 1st

relatives) Genetic analysis XST/Holter

Treatment of HOCM Relief of symptoms, prevention of endocarditis, arrhythmias and sudden death

B-Blockers or verapamil better filling, slow, bigger heart-less obstruction

Amiodarone or procainamide for A Fib need atrial kick to adequately fill LV

Avoid afterload reducing agents and vasodilators (no ACEI/A2RB, NITRO)-refractory hypotension These agents increase outflow obstruction actually do better with increased SVR Slow controlled emptying from increased SVR

SBE prophylaxis

Treatment of HOCM Implantable defibrillators

may be indicated if at risk for SCD

Dual-chamber pacemakers: reverse of resynchronization therapy for LVEF

Surgical: myotomy & myomectomy

Non surgical ablation of the septum (alcohol ablation through cath)

Sudden Cardiac Deaths

2-3 % per year Sudden & unexpected Sudden death may be the initial (only) presentation

NPR link Risk for SCD:

Extreme LVH Family history of SCD History of Vtach or syncope Failure of BP to rise with exercise

Treatments that lower gradient do not prevent SCD

Restrictive Cardiomyopathy (RCM)

May cause systolic & diastolic dysfunction All increase LV stiffness Characteristic ventricle filling pressures

Over time filling dramatically ceases

Restrictive Cardiomyopathy Some cardiomyopathies do not present with dilation

or hypertrophy but rather restricted ventricular filling (as with pericarditis)

Amyloidosis Sarcoidosis Hemochromoatosis Endomyocardial fibrosis Atrial dilation, atrial fibrillation and clot formation

common in restrictive

Restrictive Cardiomyopathy Clinical Features

Dyspnea Fatigue Embolic phenomena Elevated venous pressures

JVD Hepatomegaly Edema Ascites

Restrictive Cardiomyopathy CXR=cardiac enlargement EKG-low voltage and ST-T wave

abnormalities (Exaggerated Septal Q’s – Think MI)

Echo-symmetrical myocardial thickening Endomyocardial biopsy may be useful. Is it restrictive pericarditis?

Pre-op effusion Post op effusion

Restrictive Cardiomyopathy Treatment

No specific treatment Treat underlying cause… results? Those with amyloidosis may recur after

transplant

Amyloidosis Systemic disorder, but if DHF occurs

usually from multiple myeloma Fibrillar protein deposited throughout the

myocardium leading to rubbery consistency and concentric hypertrophy

RV & LV hypertrophy Absence of high voltage QRS on EKG

despite LVH on ECHO Appearance on Echo

Amyloidosis Fat pad aspirate or tissue biopsies for

systemic amyloidosis Endomyocardial biopsy if questionable

etiology

Poor prognosis

Hemochromatosis Hereditary disorder characterized by excess dietary iron

absorption and deposition in tissues with resulting end-organ damage.

Affects liver first and most frequently Pancreatic involvement results in DM Cardiac deposits leads to dilated cardiomyopathy Skin deposits leads to bronze discoloration that results

from increased melanin production. Hyperpigmentation Remember: liver, pancreas, heart

Dx: AST, ALT, serum iron, TIBC, ferritin