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DCMP

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Dilated

Cardiomyopathy
Dilated Cardiomyopathy – diagnosis and management (Nov ’11)

•Dilation and impaired contraction of ventricles:

•Reduced systolic function with or without heart failure

•Characterized by myocyte damage

•Multiple etiologies with similar resultant pathophysiology


CAUSES
🠶 Most common cause of DCM is Idiopathic (≈50%)
🠶 Myocarditis (46%)
🠶 Neuromuscular diseases : Duchenne muscular dystrophy
🠶 Familial cardiomyopathy
In inherited familial DCM autosomal dominant inheritance pattern is most frequent
X-linked, autosomal recessive, and mitochondrial inheritance patterns are less common

Other causes of DCM include


🠶 Inflammatory
Infectious :Viral infection(Cox-sackie, CMV ,Adeno ,HIV ) Bacterial(Diphtheria),
Fungal, Protozoan, Rickettsial)
Non infectious : Collagen vascular disease: SLE, RA
🠶 Endocrine–Hyper- and Hypothyroidism, Diabetes, Hypocalcaemia, Hypophosphatemia,
🠶 Metabolic : Glycogen storage disease, Mucopolysaccharidoses
🠶 Nutritional : Kwashiorkor, Beriberi /Thiamine, Selenenium , Carnitine deficiency).
🠶 Toxic agents :Doxorubicin (anthracyclines ) ,alcohol, cocaine
Pathology and Pathophysiology
🠶 In DCM, a weakening of systolic contraction is associated with dilatation of all four
cardiac chambers.

🠶 Dilatation of the atria is in proportion to ventricular dilatation.

🠶 The ventricular walls are not thickened, although heart weight is increased.

🠶 Intracavitary thrombus formation is common in the apical portion of the ventricular cavities
and in atrial appendages and may give rise to pulmonary and systemic emboli.

🠶 Histologic examinations from endomyocardial biopsies show varying degrees of myocyte


hypertrophy and fibrosis.
PATHOLOGY
🠶 Cardiac dilatation

– cane be adaptive due to increased loading conditions or maladaptive in Idiopathic DCM

Myocellular hypertrophy and cell death

– Cardiac hypertrophy (adaptive response): increase in collagen content preserves myocardial


performance

– Cumulative loss of myofibrils and cardiac myocytes :(apoptosis, cellular necrosis )decrease in the wall
thickness

🠶 Extracellular matrix remodelling

🠶 – Cardiac fibroblast proliferate

🠶 – Mechanically stable cross linked collagen is degraded by metalloproteinases

🠶 – Excess of poorly cross-linked collagen is deposited into interstitium

🠶 – Increase myocardial mass, intersitial fibrosis, ventricular dilatation


Pathology and Pathophysiology
Pathology and Pathophysiology
CLINICAL MANIFESTATIONS
HISTORY
🠶 History of fatigue, weakness, and symptoms of left-sided heart failure (dyspnea on
exertion, orthopnea)
🠶 History of prior viral illness occasionally

PHYSICAL EXAMINATION
🠶 Signs of CHF (tachycardia, pulmonary crackles, weak peripheral pulses, distended neck
veins, hepatomegaly) are present. The apical impulse usually is displaced to the left and
inferiorly.
🠶 S2 may be normal or narrowly split with accentuated P2 if pulmonary hypertension
develops.
🠶 A prominent S3 is present with or without gallop rhythm.
🠶 soft regurgitant systolic murmur (caused by MR or TR tricuspid regurgitation [TR])
may be present
CLINICAL MANIFESTATIONS
🠶 Heart failure

– Congestion : Edema, Orthopnea, PND paroxysmal nocturnal dyspnea

– Reduced cardiac output : Fatigue, Dyspnea on exertion

🠶 Arrhythmias and/or conduction system disease

🠶 Thromboembolic disease (from left ventricular mural thrombus)- stroke

Heart failure symptoms 75%-85%

Anginal chest pain 8%-20%

Emboli (systemic or pulmonary) 1%-4%

Syncope <1%

Sudden cardiac death <1%


DIAGNOSIS
 History

 Clinical examination

 Chest x-ray

 12-lead ECG

 Echocardiography

 Blood tests

 Family screening and genetic testing

 Endomyocardial biopsy

 Cardiac CT Scan

 MRI (magnetic resonance imaging)

 Cardiac catheterization
DIAGNOSIS
CXR Radiography

🠶 Generalized cardiomegaly is usually present

🠶 with or without signs of pulmonary venous hypertension or pulmonary edema.

ECG Electrocardiography

🠶 1. Sinus tachycardia, LVH, and ST-T changes are the most common findings.

🠶 Left or right atrial hypertrophy [LAH] or [RAH]) may be present.

🠶 Atrial or ventricular arrhythmias and atrioventricular (AV) conduction disturbances


may be seen
DIAGNOSIS
ECHOCARDIOGRAPHY
🠶 shows a marked LV enlargement and poor contractility
🠶 The LA may also be enlarged.
🠶 Occasionally, an intracavitary thrombus may be found,
especially in the left atrial appendage and cardiac apex.
🠶 Pericardial effusion may be seen.

On M-mode echocardiography,
🠶 End-diastolic and end-systolic dimensions of the LV are
increased
🠶 markedly reduced fractional shortening and ejection
fraction of the LV
🠶 for serial assessment of patients with DCM.

🠶 Mitral inflow Doppler tracing :reduced E velocity and a


decreased E/A ratio (ratio of E-wave to A-wave velocity)
Natural History and Prognosis
🠶 Progressive deterioration is the rule rather than the exception.
🠶 About two thirds of patients die from intractable heart failure within 4 years after the
onset of symptoms of CHF.
🠶 Atrial and ventricular arrhythmias develop with time (in ≈50% of patients studied by 24-hour
Holter)
🠶 Systemic and pulmonary embolism resulting from dislodgment of intracavitary thrombi
occurs in the late stages of the illness.
🠶 Causes of death are CHF, sudden death resulting from arrhythmias, and massive
embolization.

Prognosis
🠶 Approximately one third die, one third recover completely, and one third improve with
some residual cardiac dysfunction.
DIAGNOSIS
Cardiac Catheterization can be helpful to
🠶 (1) exclude anomalous coronary artery
🠶 (2) predict etiology and prognosis by obtaining endomyocardial biopsy
🠶 (3) evaluate for cardiac transplantation
🠶 (4) measurement of pulmonary vascular resistance.

Endomyocardial biopsy typically shows varying degrees of myocyte


hypertrophy and fibrosis without significant lymphocytic infiltration

Other Laboratory Tests


🠶 Urine for organic and amino acids; 3-methylglutaconic acid (i.e., Barth syndrome)
🠶 Blood studies for lactate, calcium, magnesium, carnitine, and acylcarnitine
MANAGEMENT
🠶 If no identifiable and treatable cause of DCM is found, therapy is supportive and consists of
(1)Anti-congestive regimen, (2) Control of significant arrhythmias, and(3)Minimizing the risk of
thromboembolic complications.
TREATMENT FOR UNDERLYING HEART FAILURE consists of
🠶 Diuretics :furosemide, spironolactone),
🠶 Digoxin
🠶 ACE inhibitors (captopril, enalapril
🠶 Bed rest or restriction of activity.
MINIMIZING THE RISK OF THROMBOEMBOLIC COMPLICATIONS
🠶 Antiplatelet agents (aspirin) should be initiated as propensity for thrombus formation in
patients with dilated cardiac chambers and blood stasis
🠶 If thrombi are detected, they should be treated aggressively with heparin initially and later
switched to long-term warfarin therapy
CONTROL OF SIGNIFICANT ARRHYTHMIAS
🠶 Patients with arrhythmias may be treated with amiodarone
🠶 Amiodarone is effective and relatively safe in children.
MANAGEMENT
🠶 Recently, the use of β-adrenergic blocker therapy in children with chronic heart
failure has been shown to improve LV ejection fraction. Carvedilol is a β-adrenergic
blocker with additional vasodilating action
🠶 Carnitine supplementation : If carnitine deficiency is considered
🠶 Studies have shown beneficial effects of growth hormone in adult patients with
DCM. Some of these studies reported that treatment with growth hormone for 3 to 6
months resulted in increased LV wall thickness, reduction of the chamber size, and
improved cardiac outputarted.
🠶 study involving children reported that administration of recombinant human growth
hormone (0.025–0.04 mg/kg/day for 6 months) showed a trend toward improved LV
ejection fraction
🠶 Children with severe decompensation may require mechanical circulatory support
therapy with a ventricular assist device, intraaortic balloon counterpulsation, or
ECMO extracorporeal membrane oxygenation.
🠶 Children with DCM may become candidates for cardiac transplantation
🠶 Utility of immunosuppressive agents, including steroids, cyclosporine, and
azathioprine, remains unproved

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