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ARRYTHMOGENIC RIGHT VENTRICULAR

CARDIOMYOPATHY

Inherited condition(chances to get it vary)


The cells of theheart muscleare held together by proteins.
In people with ARVC, these proteins do not develop
properly and so cannot keep the heart muscle cells
together. The muscle cells become detached and fatty
deposits build up in an attempt to repair the damage.
Usually more affected on right sided but can affect both
sided
Lead to ventricular arrythmia(impulse distrupted as it pass
the affected area)

Important cause ofsudden cardiac death (SCD) in young adults,


accounting for 11% of all cases and 22% of cases among
athletes.
Male : Female = 1:3
Typically present between the teenage years and the forties.

CLINICAL PRESENTATION
Mostly asymptomatics
Common symptoms :
Palpitation (27%-67%)
Syncope (26%-32%)
Dizziness(low CO)
SOB(if left ventricle affected)
Ascites
Pitting edema

ECG-usually normal but sometimes can see RBBB


Echocardiography-may demonstrate right ventricular
dilatation/decrease of ejection fraction
Genetic testing shows genetic abnormalities in the
genes that encode for these desmosomal proteins
Right Heart Catheterization with Heart Tissue Biopsy

2 major criteriaor1 major and 2 minor criteriaor4


minor criteria.

MANAGEMENT
Suppression of symptomatic cardiac arrhythmias
Control heart rate-b blocker,ca channel blocker
Control heart rhythm-amiodarone
Heart transplant-long term treatment

HYPERTENSIVE
CARDIOMYOPATHY

ETIOLOGY
Hypertensive heart disease is caused by chronically
elevated blood pressure.
It can be due to:
-primary(essential hypetension)-most common
-secondary hypertension

HYPERTENSIVE HEART DISEASE

Symptomatic

Abnormalities in hypertensive heart disease that includes

Left ventricular hypertrophy


Systolic & Diastolic dysfunction
Clinical presentation:
- Arrhythmias (palpitation, syncope)
- Symptomatic heart failure (shortness of breath, reduced
effort tolerance, fatigue, orthopnea, paroxysmal nocturnal
dyspnea)

LEFT VENTRICULAR
HYPERTROPHY

ARRHYTHMIAS
Occurs because the electrophysiological changes
associated with left ventricular hypertrophy are not
uniform throughout the hypertrophied tissue
Myocardial fibrosis could also cause local variations in the
conduction velocities precipitating arrhythmias

INVESTIGATION
CHEST X-RAY

(A B C D E)

- Dilated prominent upper lobe


vessels
- Cardiomegaly
- Alveolar oedema (Bats wing)
- Interstitial oedema (Kerley B
lines)
- Pleural effusion (Blunting of
costophrenic angle)

ECHOCARDIOGRAM
Left ventricular
hypertrophy symmetrical
dilated LV
low LV ejection fraction
Usually associated with
diastolic dysfunction
Increased left atrium
diameter

Two-dimensional echocardiogram
(parasternal long axis view) from a 70-yearold woman showing concentric left
ventricular hypertrophy and left atrial
enlargement.

ELECTROCARDIOGRAM

LVH strain pattern

Tall R waves in V4 and V5 with down sloping ST segment depression and T wave
inversion are suggestive of left ventricular hypertrophy (LVH) with strain pattern. LVH
with strain pattern usually occurs in pressure overload of the left ventricle as in systemic
hypertension or aortic stenosis.

MANAGEMENT
Lifestyle modification (diet, exercise, weight loss)
Pharmacological intervention
ACEi
B-blocker
CCBs
ARBs
Diuretics
Anti- arrhythmias
Anti- coagulant