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Reversible Cardiomyopathies
Dr Sajeer K T
Senior Resident,
Dept. of Cardiology, MCH Calicut
1
Dilated cardiomyopathy (DCM) :
- characterized by dilation and impaired contraction of one
or both ventricles
DCM - caused by a variety of specific diseases
≥ 50% of patients with DCM:
- an etiologic basis will not be identified
- Idiopathic DCM
One series- 1278 patients with congestive heart failure
 Idiopathic — 51 percent
 Idiopathic myocarditis — 9 percent
 Occult coronary disease — 8 percent
 Other identifiable causes — 32 percent
Felker, et al. The spectrum of dilated cardiomyopathy. The Johns Hopkins experience with 1,278 patients. Medicine
(Baltimore) 1999; 78:270
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Clinical Presentation
- Most patients present between age : 20-60 years
- Can occur in children and elderly
- Symptoms of CHF
- Incidental detection of asymptomatic cardiomegaly
- Symptoms related to coexisting arrhythmia, conduction
disturbance
- Thromboembolic complications
- Sudden death
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Reversible causes of dilated cardiomyopathy
-
Peripartum cardiomyopathy
Tachycardia-mediated cardiomyopathy
Takotsubo cardiomyopathy
Alcoholic cardiomyopathy
Cocaine
Medications
Ischemia
Endocrine dysfunction
SLE, Sarcoidosis
Nutritional deficiencies
Electrolyte abnormalities
Obstructive sleep apnea
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Peripartum cardiomyopathy (PPCM)
- Cardiomyopathy manifesting between the last month of pregnancy
and 6 months postpartum
- Toxic postpartal heart failure, Meadows synd., Zaria synd.
- Incidence in published series of 1:1300 to 1:4000 live births
Criteria for PPCM :
(1) Development of CHF secondary to deceased LV systolic
function in the last month of pregnancy or within 5 months
after delivery
(2) Absence of pre-exciting cardiac dysfunction
(3) Absence of determinable cause of cardiomyopathy
(4) LV systolic dysfunction:
- EF < 45%
- Fractional shortening of less than 30%
- LV end diastolic dimension of greater than 2.7 cm/m2
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- Risk factors for PPCM:
 Advanced age (>30 years)
 Multiparity
Twin gestation
 Use of tocolytic therapy
 Preeclampsia
 Obesity
 Chronic hypertension
 Black race
- Etiology - currently unknown
- Current hypotheses are:
(1) infectious
(2) immunologic
(3) nutritional
(4) drug-induced
(5) familial
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Infectious:
Felker et al : demonstrated a 62% prevalence of myocarditis
in patients PPCM who underwent endomyocardial biopsy
Immunologic:
- significant elevations of TNF-Alpha, IL-6 and Fas/APO-1
in patients with PPCM
- antibodies formed against actin can cross react with myocardium
- antibodies to cardiac muscle proteins have been identified in
patients with PPCM
Nutritional:
- Low selenium levels - a possible risk factor for PPCM
Drug-induced:
- prolonged terbutaline therapy
Familial:
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Echocardiography remains the tool for evaluation and follow-up
for women with postpartum cardiomyopathy
- Myocardial LV dysfunction - decrease in LV EF or FS
- LV thrombus is common with a LVEF less than 35%
Normal systolic function excludes postpartum cardiomyopathy
- should lead to an evaluation for high output failure
- anaemia
- thyrotoxicosis
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Treatment
Initial treatment of PPCM is similar to treatment for other forms of
heart failure
- diuretics, vasodilators (hydralazine and nitrates), digoxin
- (ACEI contraindicated in pregnancy)
Immunosuppression:
- Pentoxifylline: inhibit proinflammatory cytokines
(TNF-alpha, CRP, and Fas/Apo-133
- Intravenous immune globulin
Immunosuppressive therapy :
- considered if an endomyocardial biopsy indicates the presence
of myocarditis
- if there is no improvement after 2 weeks of standard HF therapy
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Outcome and prognosis
- Reported mortality rate for PPCM ranges from 15% to 50%
- Cardiac transplantation is an option if the patient fails to improve
- no consensus on whether subsequent pregnancies should be avoided
by women who survive PPCM
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Takotsubo Cardiomyopathy
A New Form of Acute, Reversible Heart Failure
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- a condition featuring symptoms and signs of AMI without
demonstrable coronary artery stenosis or spasm in which the
heart takes on the appearance of a Japanese octopus fishing pot
called a “takotsubo”
- Left ventricular dysfunction can be remarkably
depressed, recovers within a few weeks
- occurs predominantly in postmenopausal
women soon after exposure to sudden unexpected
emotional or physical stress.
Sato et al (1990) first described - reversible cardiomyopathy
- tako-tsubo-like left ventricular dysfunction
- Apical ballooning or stress cardiomyopathy
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- noncardiac surgery is one of the most frequent trigger for
cardiovascular events
( highest risk in those undergoing vascular surgery due to
coexisting severe coronary artery disease)
- incidence of ABS : 1% to 2% of patients with an acute MI
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Diagnostic Criteria
Mayo Clinic proposed diagnostic criteria in 2004
(1) - transient hypokinesis, akinesis, or dyskinesis in the LV mid
segments with or without apical involvement
- regional wall motion abnormalities that extend beyond a single
epicardial vascular distribution
- a stressful trigger
(2) - the absence of obstructive coronary disease or angiographic
evidence of acute plaque rupture
(3) - new ECG abnormalities (ST-segment elevation and/or T-wave
inversion) or modest elevation in cardiac troponin
(4) - absence of pheochromocytoma and myocarditis
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- syndrome much more common in women
- typically preceded by exposure to emotional or physical stressors
- most frequent clinical symptoms on admission are chest pain and
dyspnea resembling AMI
- most common ECG findings on admission : ST elevation in
precordial leads
- high levels of serum catecholamines and of plasma BNP
- Cardiac enzyme levels (eg, CKMB , Tn T) - slightly increased.
- Cardiac magnetic resonance imaging:
-lack of delayed contrast enhancement
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Coronary angiogram and cardiac imaging
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Apical sparing variant Takotsubo
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RV may develop similar regional wall motion abnormality in 30%
of patients
- develop congestive heart failure
Inverted Tako-tsubo :
- a rare variant presents with hypokinesis of the base of
the heart with preserved apical function
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- Cine sequences of CMR imaging
during systole (A) and diastole (B)
in the acute phase.
- Normal function could be documented
after 3 weeks (C, systole; D, diastole)
- Contrast-enhanced CMR image did not
show myocardial hyper enhancement
even in the delayed phase (E).
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Pathophysiology:
Myocardial biopsy:
- interstitial infiltrates( mononuclear lymphocytes, leukocytes,
and macrophages)
- myocardial fibrosis
- contraction bands with or without overt myocyte necrosis
Coagulation necrosis : seen in myocardial infarction
- Catecholamine Cardio toxicity:
- high local concentrations of nor-epinephrine might evoke
basal hyperkinesis
- increasing mechanical wall stress at the apex
- increasing end-diastolic pressure and BNP levels
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A Pathogenetic Concept About Takotsubo Cardiomyopathy
Possible underlying mechanism of classic takotsubo cardiomyopathy
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Management Considerations in Patients Presenting with ABS
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Prognosis
- Prognosis is generally favourable
- Fatal complication
- LV free wall rupture
- heart failure, with or without pulmonary oedema
- Recurrence rate of takotsubo cardiomyopathy : 10%
- Complete recovery - by 4 to 8 weeks
- Inhospital mortality from ABS is very low (1- 2%)
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Tachycardia-mediated cardiomyopathy
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Tachycardia-mediated cardiomyopathy
- impairment in LV function secondary to chronic tachycardia, which
is partially or completely reversible after normalization of heart rate
and/or rhythm irregularity
--- Gallagher JJ
Fenelon et al : Tachycardiomyopathy:
a) Pure type
b) impure Type
Pure type:
chronic tachycardia causes LV dysfunction in a normal heart and
completely recovers after termination of the tachycardia
Impure Type:
occurs in patients with structural heart diseases, and cardiac
dysfunction may only recover incompletely after termination of
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the tachycardia
Possible mechanisms for myocardial dysfunction
- Myocardial energy depletion
- Impaired energy utilization
- Myocardial ischemia
Abnormal calcium handling:
- the severity of calcium cycling abnormalities correlates with the
degree of ventricular dysfunction
(extensive abnormalities in calcium channel activity and
sarcoplasmic reticulum calcium transport )
- calcium availability to myocytes decreased
> reduction in contractility
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- Tachycardiomyopathy can occur at any age
- reported in infants, children ,adolescents and adults
- incidence of tachycardia-induced cardiomyopathy - unknown
- risk factors:
- type, rate and duration of tachyarrhythmia
- patient’s age
- underlying heart disease
- drugs
- coexisting medical conditions
- diagnosis of tachycardiomyopathy considered
- patient with LV systolic dysfunction and chronic or frequently
recurring cardiac arrhythmia
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Tachycardiomyopathy is induced by various supraventricular
and ventricular arrhythmias
- Ectopic atrial tachycardia (EAT)
- in children
- Permanent form of junctional reciprocating tachycardia (PJRT)
- often incessant and refractory to antiarrhythmic drugs
- commonly results in tachycardiomyopathy
Fenelon et al proposed the following criteria:
1) Dilatation of the heart or heart failure
2) Chronic or very frequent cardiac arrhythmias, including
incessant SVTs, AF or AFL and incessant VT
If chronic tachycardia continued more than 10-15% of the day, with
an atrial rate of more than 150% of that predicted for age,
- tachycardiomyopathy occurs.
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Treatment
- Basic concept for the treatment of tachycardiomyopathy
- controling the heart rate
- SVT : digitalis or verapamil : common drugs of choice
- Class I anti-arrhythmic drugs: negative inotropic effect
- clinical use should be careful
- In pediatric cases, EAT or PJRT : incessant
- Amiodarone
- class III
- dofetilide, sotalol,
- ibutilide or azimilide
- Catheter ablation : performed as the first-line therapy
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Alcoholic cardiomyopathy
- ethanol may induce asymptomatic LV systolic dysfunction
- 30% of asymptomatic chronic alcoholics have evidence of LV
systolic dysfunction
- ethanol abuse is the leading cause of nonischemic DCM in
industrialized countries
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Mechanisms of Ethanol-Induced
Myocardial Injury
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- likelihood of developing an ethanol-induced DCM correlates with
the amount of ethanol that is consumed in a lifetime
- Men – consumption more than 80 g of ethanol/day for at least 5 yrs
(1 L of wine, 8 std-sized beers, or 0.5 pint of hard liquor)
-Women - more susceptible to ethanol's cardiotoxic effects than men
- complete abstinence or reduction in ethanol consumption (less
than 60 g of ethanol/day): substantial improvement in LV
systolic function and symptoms of HF
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Cocaine and heart
Cocaine - commonest illicit drug used and the most frequent cause
of drug related deaths
Cocaine related complications :
1. Cardiac: myocardial ischaemia, coronary artery spasm,
acute MI, myocarditis, cardiomyopathy, arrhythmia,
hypertension
2. Vascular: aortic dissection and rupture, vasculitis
3. Gastrointestinal: mesenteric ischaemia or perforation
4. Pulmonary: pulmonary oedema, pulmonary infarction
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Dilated cardiomyopathy in cocaine users:
- attributable to the direct toxic effects
- heart failure results from:
- myofibrils destruction
- interstitial fibrosis
- myocardial dilatation
- cocaine induced hyper adrenergic state may contribute to the
cardiomyopathy (similar to pheochromocytoma)
- myocardial dysfunction is reversible with abstinence of cocaine
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Medications
- anthracycline-induced cardiomyopathy - most extensively studied
- cardiomyopathy and clinical heart failure are the main dose limiting
side effects of anthracyclines
- cardiomyopathy can occur within the 1st year and up to a decade
after completion of therapy
risk factors:
1 . cumulative dose of anthracyclines administered
(not to exceed 450 mg/m2 in adults)
2. extremes of age
3. concomitant chemotherapy and radiation
4. history of cardiovascular disease
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Endocrine dysfunction
- thyroid dysfunction
- excess sympathetic activity in pheochromocytoma
- rarely Cushing's syndrome
- growth hormone excess or deficiency
- cardiac dysfunction which can usually be reversed by
correction of the endocrine disorder
- hyperthyroidism or hypothyroidism:
- exact mechanisms of dilated cardiomyopathy not known
- thyroid hormone is known to alter preload, after load,
heart rate, and contractility
- excess T3 causes myocyte hypertrophy
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Pheochromocytoma :
- excess sympathomimetic amines cause focal direct myocyte
injury
- inflammation, down regulation of beta receptors
- reduction of viable myofibrils
Catecholamines –
- increase both intracellular calcium and free radicals
- contribute to myocyte injury
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Ischemia - cardiomyopathy
- coronary atherosclerosis is MC cause of DCM in US
- 50 to 75 percent of patients with HF
- usually irreversible due to myocardial infarction and subsequent
ventricular remodeling
- patients with ischemic DCM with hibernating myocardium
- benefit of revascularisation
- Revascularization of hibernating myocardium beneficial in
patients with ischemic DCM who have less severe heart disease
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Selenium deficiency and cardiomyopathy
Selenium deficiency:
- identified as a factor causing HF syndromes in areas of very
low selenium intakes( China )
- endemic selenium-responsive cardiomyopathy Keshan disease
Selenium deficiency :
- decreases the activity of glutathione peroxide
- resulting in increased free radicals > toxic to cardiac myocytes
Similar cases :
- HIV-infected patients
- subjects on parenteral nutrition
- Crohn’s disease
Possible causes of Keshan disease are:
- viral infection
- nutritional factors
(insufficient Zn or molybdenum, excessive barium or lead)
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Thiamine:
- plays an important role in normal oxidative phosphorylation and
myocardial energy production
- deficiency initially presents as a high output state secondary to
vasodilation
- followed by eventual depression of myocardial function –
- development of a low output state
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Obstructive sleep apnea - cardiomyopathy
- obstructive sleep apnea: contribute to the impairment of left
ventricular dysfunction
- history of snoring, daytime somnolence, and obesity should alert
the clinician to the diagnosis
- therapy : nasal continuous positive airway pressure during sleep
lead to a significant improvement in LV dysfunction
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Electrolyte abnormalities:
Chronic hypophosphatemia and hypocalcaemia:
- reversible cardiomyopathy
Infections :
- Bacterial, spirochetal, rickettsial, fungal, protozoal, helminthic
infections
- implicated as causes of necrotic or inflammatory lesions
- may lead to dilated cardiomyopathy
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THANK YOU
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MCQs
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1. All are causes of reversible cardiomyopathy
except?
A) Sarcoidosis
B) carnititine deficiency
C) hypercalcemia
D) hypothyroidism
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2. All are true about PPCM except?
a) More common in primi
b) Twin gestation
c) Preeclampsia
d) Age>30 years
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3.
a)
b)
c)
d)
True about treatment of PPCM?
ACEI can be used after delivery
Petoxyphiline used in all cases
Cardiac transplantation not indicated
biopsy done in all cases
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4. Apical ballooning syndrome true except?
a) More common in young females
b) Female gender predilection
c) Recovery over few weeks
d) No specific treatment
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5. Criteria of LV systolic dysfunction by echo in
PPCM:
a) EF < 45%
b) Fractional shortening of less than 30%
c) LV end diastolic dimension of greater than
2.7 cm/m2
d) ALL
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6. False about Takotsubo syndrome
a) Apical sparing can occur
b) preceded by exposure to emotional or physical stressors
c) most frequent clinical presentation on admission is acute
pulmonary edema
d) most common ECG findings on admission : ST elevation in
•
precordial leads
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7. True about Takotsubo syndrome pathogenesis
a) Catecholamine toxicityb) epicardial corornary artery spasm
c) Plaque rupture
d) all
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8. All are true regarding tachycardiomyopathy
except?
a) Only seen in infants and children
b) EAT and PJRT – are incessant
c) Abnormal calcium handling- mechanism
d) RF ablation is the preferred treatment
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9. False about keshans disease
a) Endemic in china
b) Only seen in selenium deficiency
c) LV function improves with selenium
supplement
d) Also in HIV infection
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10. Reversibility is maximum seen in which drug
related cardiomyopathy?
a) Dunorubicin
b) Epirubicin
c) Tratzumab
d) Imatinib
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• 1. All are causes of reversible cardiomyopathy
except?
• A) sarcoidosis
• B) carnititine deficiency
• C) hypercalcemia
• D) hypothyroidism
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2. All are true about PPCM except?
a) More common in primi
b) Twin gestation
c) Preeclampsia
d) Age>30 years
64
3.
a)
b)
c)
d)
True about treatment of PPCM?
ACEI can be used after delivery
Petoxyphiline used in all cases
Cardiac transplantation not indicated
biopsy done in all cases
65
4. Apical ballooning syndrome true except?
a) More common in young females
b) Female gender predilection
c) Recovery over few weeks
d) No specific treatment
66
5. Criteria of LV systolic dysfunction by echo in
PPCM:
a) EF < 45%
b) Fractional shortening of less than 30%
c) LV end diastolic dimension of greater than
2.7 cm/m2
d) ALL
67
6. False about Takotsubo syndrome
a) Apical sparing can occur
b) preceded by exposure to emotional or physical stressors
c) most frequent clinical presentation on admission is acute
pulmonary edema
d) most common ECG findings on admission : ST elevation in
•
precordial leads
68
7. True about Takotsubo syndrome pathogenesis
a) Catecholamine toxicityb) epicardial corornary artery spasm
c) Plaque rupture
d) all
69
8. All are true regarding tachycardiomyopathy
except?
a) Only seen in infants and children
b) EAT and PJRT – are incessant
c) Abnormal calcium handling- mechanism
d) RF ablation is the preferred treatment
70
9. False about keshans disease
a) Endemic in china
b) Only seen in selenium deficiency
c) LV function improves with selenium
supplement
d) Also in HIV infection
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10. Reversibility is maximum seen in which drug
related cardiomyopathy?
a) Dunorubicin
b) Epirubicin
c) Tratzumab
d) Imatinib
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