Non ischemic Cardiomyopathies

and Cardiac MRI

Mahi L. Ashwath MD, MBA, FACC,

FASE, FSCMR

President, Iowa ACC

Associate Professor of Medicine and Radiology

Division of Cardiology

University of Iowa Hospitals and Clinics1

Normal Cardiac MRI

Normal Cardiac MRI

LAD infarct – ischemic cardiomyopathy

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Cardiomyopathies

Genetic Mixed Acquired

Hypertrophic

Cardiomyopathy

Dilated Cardiomyopathy Myocarditis

Arrhythmogenic right

ventricular

cardiomyopathy

Restrictive

Cardiomyopathy

Stress / Takotsubo

Cardiomyopathy

Non compaction

cardiomyopathy

Peripartum

Cardiomyopathy

Storage

Cardiomyopathy

Tachycardia Mediated

Cardiomyopathy

Dilated Cardiomyopathy

Dilated Cardiomyopathy

• Dilation and impaired contraction of one or both ventricles

• Usually associated with increase in total cardiac mass

• Incidence 5-8 cases per 100,000 population

• Upto 14% of middle aged and elderly population have asymptomatic

LV systolic dysfunction

• Causes:

▪ Viruses

▪ Gene mutations

• Distinguish from

▪ Ischemic Cardiomyopathy

▪ Valvular Cardiomyopathy

Hypertrophic Cardiomyopathy

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• Variety of mutations associated with hypertrophy of the LV and occasionally the RV

• Prevalence 0.2 – 0.5%

• Histology

• Hypertrophied myocytes with

• Myocardial disarray

• Microvasculature with decreased luminal cross sectional area

• Impaired vasodilatory capacity

• Abnormalities

• LV outflow obstruction

• Diastolic dysfunction

• Myocardial ischemia

• Mitral regurgitation

Hypertrophic Cardiomyopathy

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Hypertrophic Cardiomyopathy – Presentation -

Symptoms

• Heart failure

• Fatigue

• Dyspnea

• Myocardial ischemia

• Chest pain

• Arrhythmia

• Palpitations

• Pre-syncope or syncope

• Systolic murmurs:

• Obstruction murmur –

▪ Harsh crescendo – decrescendo murmur

▪ apex and LLSB –

▪ increases with increase in obstruction – upright

posture, Valsalva, post PVC or with nitroglycerin

▪ Decrease with sitting, handgrip and following passive

elevation of the legs

• Mitral regurgitation with a posterior jet

▪ mid – late systolic murmur at apex

Hypertrophic Cardiomyopathy – Presentation -

Signs

Diagnostic testing

• Goals:

• To establish the diagnosis of HCM

• To identify the presence or severity of obstruction

• Assess mitral regurgitation

• Risk of arrhythmia

• Overall LV function

Tests performed:

• EKG

• Prominent abnormal Q waves in the inferior and lateral leads – due to

septal depolarization of the hypertrophied myopathic tissue

• Deeply inverted and prominent T waves with apical variant of HCM

• Echo

• Left ventricular hypertrophy

• unexplained increased LV wall thickness ≥15 mm systolic anterior motion

(SAM) of the mitral valve

• LVOT obstruction

• Ambulatory EKG monitoring

• assessment for ventricular arrhythmias and risk for sudden cardiac death

• Exercise testing

• abnormal blood pressure [BP] response to exercise) and for the assessment

of LVOT gradient

• Cardiovascular magnetic resonance

• Genetic Testing

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Management - To Decrease Obstruction

• Decrease contraction

• Beta-blockers

• Verapamil

• Diltiazem

• Disopyramide

• Maintain afterload

• Avoid vasodilators

• Avoid diuretics

• Increase volume

• Hydration

• Ongoing angina

• Ranolazine

• Persistent Obstruction

• Myectomy

• Alcohol Septal Ablation

• Indication for Myectomy

• LVOT obstruction

• Angina, dyspnea, and/ or syncope resulting in significant impairment in quality

of life

• Symptoms persist despite appropriate medical therapy

• Procedure involves

• Direct removal of septal muscle

• Mitral valve repair

• Apical approach if significant hypertrophy of distal portion of LV

• Complications:

• Excessive removal of tissue – VSD

• LBBB or CHB

• Aortic regurgitation rarely

• Long term outcomes

• Improvement of symptoms

• Lower rate of appropriate ICD discharges

• Improvement of Pulm HTN

• Improved survival compared to obstructed patients

Surgical Myectomy

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Ablation

• Creates a localized myocardial infarction

• Abnormalities of MV and pap muscle cannot be addressed

• Significant reduction in LVOT gradient, minimal requirement for repeat procedures

• Complications

• Coronary artery dissection

• Pericardial effusion

• Larger MI

• Complete heart block

• Ventricular tachycardia

• ? Arrhythmic death

2011 ACCF/ AHA guidelines for diagnosis and treatment of Hypertrophic Cardiomyopathy

2011 ACCF/ AHA guidelines for diagnosis and treatment of Hypertrophic Cardiomyopathy

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Risk factors for SCD

2011 ACCF/ AHA guidelines for diagnosis and treatment of Hypertrophic Cardiomyopathy

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Screening

• Genetics:

• Autosomal Dominant

• Genetic Testing:

• Screen first degree relatives

• H&P, ECG, Echo, genetic screening of first degree relatives if a definite HCM

mutation identified

• No screening up to age 12, unless high risk

• Annually ages 12-18 years

• Adults – every 5 years, usually up to age 40-50 years

Apical Hypertrophic Cardiomyopathy

EKG

Strain pattern – Blueberry on top

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Apical Hypertrophic Cardiomyopathy

• No LVOT obstruction, can have mid cavitary obstruction

• Common in East Asian populations

• No or mild symptoms

• Audible and palpable S4

• Giant negative T waves on EKG

• Spade like configuration of the LV on echo and MRI

• Associated apical wall motion abnormalities, including

hypokinesis and aneurysm

Fabry Cardiomyopathy

Fabry Cardiomyopathy

Fabry Cardiomyopathy

• HCM mimicker

• Glycogen storage disorder

• Abnormal scar pattern with inferolateral patchy

enhancement

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Other mimickers

• Aortic Stenosis

• Hypertension

• Athlete’s Heart

• Volume Depletion

• Sub aortic membrane

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HCM Take Home…

• Diagnosis and differential diagnosis

• Treatment of symptoms

• Avoid vasodilation, dehydration

• Risk of SCD

• Family Screening

Restrictive Cardiomyopathies

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Amyloidosis

• Amylum - starch (Latin)

• Extracellular deposition of amyloid

• Deposits stained with Congo Red dye display an apple green

birefringence

• Types of Amyloid

• Light chains (AL) - “Primary”

• Systemic AL amyloidosis involves the liver, kidneys,

autonomic and peripheral nervous systems, lung and heart

• Cardiac infiltration is present in most patients with AL

amyloidosis

• Transthyretin (ATTR) - “Senile” or “Familial”

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Cardiac Amyloidosis

• Heart failure

• Diastolic dysfunction > systolic dysfunction

• Lower extremity edema

• Hepatic congestions, ascites, dyspnea

• Low cardiac output

• Electrophysiologic

• Syncope due to bradyarrhythmias or Advanced AV block or ventricular

arrhythmia

• Heart block

• Tachyarrhythmias

• Low voltages on EKG

• Imaging / Laboratory

• Left ventricular “hypertrophy”

• Elevated troponin

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Amyloidosis - EKG

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Amyloidosis - Echo

Strain pattern – apical sparing – cherry on top

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Amyloidosis – Treatment Strategy

• Treatment of Heart failure

• Diuretics

• Treatment for ATTR Cardiomyopathy

• Tafamidis for – ATTR-ACT trial

• Liver transplant

• Need biopsy for exact type of Amyloidosis

• Affects prognosis

• Treatment of clonal plasma cell disorder

• Chemotherapy

• Stem cell transplant

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Treatment - cardiac specific

• Diuretics/ salt restriction

• Usually have peripheral edema/ ascites

• Avoid

• Digoxin, beta - blockers, vasodilators

• Orthostatic hypotension

• Midodrine

• Treatment of atrial and ventricular arrhythmias

• Pacemakers

• Bradyarrhythmias - esp ATTR

Endomyocardial Fibrosis

Endomyocardial Fibrosis

Takotsubo Cardiomyopathy

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Takotsubo Cardiomyopathy

• Synonyms:

• Apical ballooning syndrome

• Broken heart syndrome

• Stress induced cardiomyopathy

• Transient regional dysfunction of the left ventricle

• By definition,

• Absence of angiographic evidence of obstructive CAD or acute plaque rupture, pheochromocytoma, myocarditis

• New EKG abnormalities

• Modest troponin elevation

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Takotsubo Cardiomyopathy

• Mechanisms:

• Catecholamine excess

• Microvascular dysfunction

• Multivessel coronary artery spasm

• Common WMA - apical,

• can be mid-ventricular, basal, focal and global types

• May have myocardial edema on MRI, but no scar

• Can be due to physiological (males) or psychological (females) stressor

• Supportive and standard CHF care

• Recovery of function

Myocarditis

Myocarditis

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• Increased risk of sudden cardiac death

• No competitive sports for 3-6 months

• Echo, Holter monitor and stress test prior to clearance

Pericarditis with pericardial inflammation

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Left ventricular non compaction

• Altered myocardial wall

• Continuity between the LV cavity and deep intra trabecular recesses

• Genetics:

• Sporadic or familial

• Autosomal dominant inheritance is more common than X-linked inheritance or

autosomal recessive inheritance

• Yield of genetic testing in patients with LVNC is around 40 to 50 percent

• Overlap in the genetic loci implicated in the major cardiomyopathies

• LVNC and hypertrophic cardiomyopathy

• LVNC and apical hypertrophic cardiomyopathy

• Co-occurs with congenital heart disease or Wolff-Parkinson-White syndrome

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Left ventricular Non compaction

• Exercise:

• LVEF <50 percent,

• ECG abnormalities,

• arrhythmia,

• symptomatic presentation,

• and/or a positive family history of cardiomyopathy

• counseled to refrain from competitive endurance sports or weight lifting (similar to patients with hypertrophic cardiomyopathy)

• LV non compaction may be associated with

• Heart failure,

• Thromboembolism

• Ventricular arrhythmias in adults

• Family counseling and screening

• All patients with LVNC should receive family and genetic counseling

Sarcoidosis

Circulation. 2009;120:1969-1977

Pathological correlation of sarcoidosis and sudden death

Tavora et al. Am J Cardiol 2009;104:571–577)

Pathological correlation of sarcoidosis and sudden death

Tavora et al. Am J Cardiol 2009;104:571–577)

ARVC

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Arrhythmogenic Right Ventricular Cardiomyopathy

• Prevalence of ARVC - 1 in 2000 to 1 in 5000

• Inherited

• Autosomal dominant – most common

• Autosomal recessive

• Part of a syndrome – Naxos disease, Carajal syndrome -

have hyperkeratosis of palms and soles and woolly hair

• Significant number of first degree relatives

• Especially siblings

• Localized or generalized dilatation of the RV and myocardial

thinning in the regions of dilatation - typical findings

• Macroscopically - scarred appearance with fibrous or fibro fatty

replacement of the myocardium

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Clinical Manifestations

• Many clinically silent and asymptomatic for decades

• Can include

• Palpitations

• syncope

• Chest pain

• Dyspnea

• Sudden cardiac death

• Frequent presentations:

• Palpitations

• Ventricular ectopy

• PVCs, NSVT

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EKG - Epsilon wave

5-30%

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ARVC - Management

• Arrhythmias:

• Implantable Cardioverter Defibrillator

• Genetic testing - family screening

• Exercise restriction

• Heart transplant

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ARVC - Management

• High risk features

• Younger age

• Syncope

• Cardiac arrest or VT

• 2 or more mutations

• LV involvement

• Naxos disease

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Cardiomyopathies

Genetic Mixed Acquired

Hypertrophic

Cardiomyopathy

Dilated

Cardiomyopathy

Myocarditis

Arrhythmogenic right

ventricular

cardiomyopathy

Restrictive

Cardiomyopathy

Stress / Takotsubo

Cardiomyopathy

Non compaction

cardiomyopathy

Peripartum

Cardiomyopathy

Storage

Cardiomyopathy

Tachycardia

Mediated

Cardiomyopathy

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Thank you!!!