Myocardial Disease - Arrhythmogenic Right Ventricular Cardiomyopathy

Question 1

What is ARVC?

Answer 1

A form of inherited cardiovascular disease that is the 2nd commonest cause of sudden cardiac death in the young (after hypertrophic cardiomyopathy.

Question 2

Pathophysiology of ARVC (2).

Answer 2

1. Autosomal Dominant with Variable Expression.

2. Replacement of Right Ventricular Myocardium with Fatty and Fibrofatty Tissue.

Question 3

Clinical Presentation of ARVC (3).

Answer 3

1. Palpitations.
2. Syncope.
3. Sudden Cardiac Death.

Question 4

ECHO Findings in ARVC (2).

Answer 4

1. Subtle in Early Stages.

2. Enlarged Hypokinetic Right Ventricle with a Thin Free Wall.

Question 5

ECG Findings in ARVC.

Answer 5

1. T Wave Inversion - V1 -V3.

2. Epsilon Wave : Terminal Notch in QRS Complex.

Question 6

Management of ARVC.

Answer 6

1. Sotalol - Antiarrhythmic.
2. Catheter Ablation : Prevent VT.
3. ICD.

Question 7

What is Naxos Disease?

Answer 7

Triad of ARVC (Autosomal Recessive Variant), Palmoplantar Keratosis, Woolly Hair.