DILATED CARDIOMYOPATHY

DR ADEKUNLE VICTOR O.
CARDIOLOGY UNIT
MEDICINE DEPARTMENT
ABUTH SHIKA
ZARIA
 OUTLINE
• Introdution
• Epidemiology
• Aetiology
• Clinical Manifestation
• Investigation
• Complications
• Management
• Prognosis
 Introduction
• Dilated Cardiomyopathy has been recognised as a
cause of Heart Failure since the mid 19th century’.

• In the mid 1850s, myocarditis was the only known

cause of Heart Muscle disease

• In 1884 Takaki Kanehiro a British-trained Japanese

Naval Medical Officer observed that Beriberi was
endemic among low-ranking crew who ate nothing
but rice but not among crews of Western Navies or
Officers who consumed a western diet.
 Introduction
• In 1884 Bollinger described a syndrome of Heart
Enlargement seen in chronic alcoholics referred to
then as the ‘alcoholic-plethoric beer heart’.

• In 1893 Steell commented ‘ Not only do I recognise

alcoholism as one of the causes of muscle failure of
the heart but I find it to be a rather common one
 Introduction
Cardiomyopathies are primary heart muscle diseases (not
valves, endocardium or pericardium) resulting in cardiac
dysfunction that is not attributable to other causes e.g
Hypertensive heart disease or Myocardial Infarction.(WHO
1980)
2 classification systems exist –
WHO (1980) – Hypertrophic
Dilated
Restrictive

- (1990) Primary : Hypertrophic/Dilated/ Restrictive

Specific: Infections, Metabolic, Systemic
 Introduction
• Definition(s)

-Heart Muscle diseases of unknown etiology.

(1980 WHO)

-Heart Muscle Diseases associated with cardiac

dysfunction.
(1995 WHO/ISFC)
 Introduction Contd
• Dilated Cardiomyopathy is that form of myocardial
disease (primary or secondary) in which there is
gross LV dilatation , increased heart size and systolic
dysfunction manifesting as Congestive Heart
Failure.

• It is now regarded as the final common pathway for

a variety of myocardial insults leading eventually to
congestive cardiac failure
 Epidemiology
• Commonest form of cardiomyopathy
Idiopathic
Incidence – 3-10 cases per 100,000
Prevalence – 1:2500

Age – Peak incidence -5th decade of life

90% of cases seen between 2nd and 6th decades of life

Sex- 2x commoner in males

Race – 3x commoner in blacks

 Etiology

• Primary or Idiopathic

• Secondary:
- Hypertensive Heart Disease
- Infections : HIV, Poliovirus, Influenza virus, Coxsackie A and
B viruses, Echovirus, Hepatitis B virus, CMV, EBV
- Toxins: Alcohol, Anthracycline cytotoxics, Lead, Mercury,
Cobalt
- Endocrine: Acromegaly, Phaeochromocytoma,
Hypothyroidism, Hyperthyroidism
- Metabolic: H ypocalcemia, hypophosphatemia, thiamine
deficiency, pregnancy , selenium deficiency
 Etiology contd
• Reversible causes include alcohol, thiamine deficiency,
hypothyroidism, hyperthyroidism, selenium deficiency,
hypocalcemia, hypophosphatemia and pregnancy.

• Alcohol causes DCM directly through cardiotoxicity as well

as through associated thiamine deficiency

• Urbano –Marquez et al(1989) found significant structural

and functional myocardial and skeletal muscle changes in
individuals consuming >90g of alcohol per day for up to 20
years1.
 Etiology contd
• Lazerevic et al2 studied structural and functional
characteristics among asymptomatic individuals consuming
>3 drinks/day for >4 days/wk,dividing them into 3 groups
based on duration: A- 5-9 years, B- 10-14 years, C- >15 years.
They found significant changes including including
Increased LV EDD and isovolumic relaxation times - A

A + significant posterior wall+septal hypertrophy

+decreased deceleration time -B

B+ significant reduction in amplitude of E/A waves during

transmitral velocity flow studies -C
 Etiology contd
• Overall consensus is that diastolic dysfunction
occurs early in alcoholic cardiomyopathy following
which significant cardiac dilatation and systolic
dysfunction ensues and symptoms develop.
 Etiology contd
• Peripartum Cardiomyopathy is defined as an idiopathic
cardiomyopathy presenting with Heart Failure 2o to LV
systolic dysfunction late in pregnancy (last trimester)or in
the immediate months following delivery (6 months) where
no other cause of HF is found. It is a diagnosis of exclusion.
(European Society Of Cardiology working group on PPCM
2010)
Systolic dysfunction (EF <45%) must be present to make a
diagnosis.
Exact cause is unknown but proposed etiologies include
selenium deficiency, viral myocarditis.
In our environment, volume overload from ingestion of dried
lake salt (kunun kanwa), lying on heated mud beds and
taking hot baths twice a day for up to 40 days postpartum
have been implicated as causes
 Pathogenesis
Cardiac Insult

Myocardial Inflammation

Myocyte and connective tissue injury /loss from enzymes

elaborated by inflammatory cells e.g neutrophil elastase,
myeloperoxidase

Myocardial thinning and dilatation

 Functional Pathophysiology
Cardiac Dilatation

Increased LV End Diastolic Volume

and Reduced Ejection Fraction

Activation of compensatory mechanisms viz Sympathetic

stimulation and Activation of RAAS

Decompensation from inablility of

compensatory mechanisms to maintain cardiac output
 Manifestation
• DCM accounts for up to a third of all cases of Heart
Failure.
Symptoms and signs of Left and Right Sided Heart
Failure viz
Exertional Dyspnea
Orthopnea
Paroxysmal Nocturnal Dyspnea
Body Swelling
Easy satiety
 Manifestation contd
Dyspneic @ rest
Cyanosis
Ankle Edema
tachycardia might be irregularly irregular
Hyper/Normo/Hypotensive
Raised JVP with a normal wave pattern
Displaced non-heaving apex
Soft S1, S2, S3 or S4 with murmurs of MR and TR
Bibasal Crepitations
Tender smooth Hepatomegaly
Ascites
Clinicals to suggest specific cause
 Investigation
• FBC – Polycythemia, macrocytosis without hypochromia
• LFTs – significant GGT elevation
• CXR – Enlarged Heart Shadow with pulmonary edema
• ECG – LAE, repolarization abnormalities(ST-T wave changes)

• Echocardiogram - findings would depend on stage

Increased LV Diameter in diastole and systole –
Normal or Increased Wall thickness –
Increased LV End Diastolic Volume
Reduced Ejection Fraction - <50%
+/- Diastolic dysfunction
Mitral/ Tricuspid Regurgitation
 Investigation contd
Urinary BNP and NT-pro BNP > 400 and 2000pg/ml respectively

Endomyocardial Biopsy – Lymphocytic myocarditis.

 Treatment
• Natural History of Idiopathic DCM is chronic Refractory Heart Failure
Reversiblility of structural and functional changes seen is
achievable in some causes
• Anti-Failure regimen
- low salt diet
- graded exercise
- Loop diuretics – IV or oral frusemide,
- Spironolactone( Aldosterone antagonist)
- ACE inhibitors/ARBs: lisinopril, losartan
- Antiplatelets: aspirin, clopidogrel
- Anticoagulation: enoxaparin, warfarin
- Inotropics: dopamine, dobutamine
 Treatment contd
• Loop diuretics – Reduce preload by urinary Na excretion
Frusemide – Onset :
Duration:

ACE inhibitors/ AR Blockers –

- Cardiac remodelling
- reduce preload by inhibiting aldosterone production
- reduce afterload by inhibiting Angiotensin II (vasoconstrictor)
production or action
Spironolactone – Reduces preload by antagonising the effects of
aldosterone(Na retention)

Antithrombotic therapy – Low-Dose Aspirin, clopidogrel

 Treatment contd
• Inotropes
- Digoxin: especially in Atrial Fibrillation
- Dopamine
- Dobutamine
Indications: cardiogenic shock
low cardiac index in the presence of hypoperfusion or
congestion.

Anticoagulation is indicated in
- Atrial Fibrillation
- Previous thromboembolic event
- Newly formed LV thrombus
 Treatment contd
Ventricular Assist Devices and Total Artificial Hearts
- VADs are mechanical circulatory devices that partially or
completely replace the function of a failing heart. Used in 3
clinical scenarios:
-Ventricular Dysfunction following Cardiac Surgery
- Bridge to Transplant
- Destination Therapy

Bridge to transplant indications:

- approval for cardiac transplantation (UNOS Status 1)
- imminent risk of dying before donor heart is available
- on Intraaortic Baloon pump
 Ventricular Assist Devices
• - Hemodynamic criteria
- Left Atrial or Pulmonary capillary wedge pressure > 20 mmHg
with either systolic BP<80mmHg or cardiac index of <2L/min/m 2

Contraindications- Contraindications to transplant + uncorrected

aortic regurgitation.

Destination therapy indications:

- NYHA Class IV Ht failure for > 60 days
- NYHA Class III/IV for up to 28 days with
a. received IABP for >14 days
b. dependent on IV inotropic agents with > 2 failed weaning
attempts.
AND
 Ventricular Assist Devices contd
• - Has peak O2 consumption < 14 ml/kg

AND
- Must not be a candidate for transplant for one of the
following reasons
- Age>65 YRS OR
- Has IDDM with end-organ damage OR
- CRF serum creatinine (>2.5mg/dl)> 90 days OR
- Presence of other clinically significant condition.
Patients with Total Artificial Hearts have their ventricles
removed in addition to having biventricular assist devices
implanted.
Prognostic Factors in Idiopathic Dilated Cardiomopathy

Factor Good Bad

LVEF >32% <32%

LVEDP <22mmHg >22mmHg

LAV

Arrhythmias Absent Present

Others Peripartum , metabolic and

endocrine causes