more rare ekg tracings

Question 1

brugada syndrome

Answer 1

diagnostic:

The term brugada sign is used, if we have a coved, greater than 2 mm descending type of st elevetation, followed by a negative T wave in at least 2 leads out of V1, V2 and V3.

The right ventricle is most affected in Brugada syndrome, and particularly (but not specifically) the right ventricular outflow tract .
Aetiology

In a nutshell, Brugada syndrome is due to a mutation in the cardiac sodium channel gene. This is often referred to as a sodium channelopathy. Over 60 different mutations have been described so far and at least 50% are spontaneous mutations, but familial clustering and autosomal dominant inheritance has been demonstrated.

Question 2

arrhythmogenic right ventricular cardiomyopathy

Answer 2

EKG tracing:

• > Epsilon wave (most specific finding, seen in 30% of patients)
• > T wave inversions in V1-3 (85% of patients)
• > Prolonged S-wave upstroke of 55ms in V1-3 (95% of patients)
• > Localised QRS widening of 110ms in V1-3
• > Paroxysmal episodes of ventricular tachycardia with a LBBB morphology (i.e. right ventricular VT).

General explanation:
An inherited myocardial disease associated with paroxysmal ventricular arrhythmias and sudden cardiac death.
Characterized pathologically by fibro-fatty replacement of the right ventricular myocardium.
The second most common cause of sudden cardiac death in young people (after HOCM), causing up to 20% of sudden cardiac deaths in patients < 35 yrs of age.
Typically inherited as an autosomal dominant trait, with variable penetrance and expression (there is an autosomal recessive form called Naxos Disease, which is associated with woolly hair and skin changes).
More common in men than women (3:1) and in people of Italian or Greek descent.
Estimated to affect approximately 1 in 5,000 people overall.

***https://www.youtube.com/watch?v=V9Qao-ZPOJs

Clinical features:
ARVC causes symptoms due to ventricular ectopic beats or sustained ventricular tachycardia (with LBBB morphology) and typically presents with palpitations, syncope or cardiac arrest precipitated by exercise.
The first presenting symptom may be sudden cardiac death.
Over time, surviving patients also develop features of right ventricular failure, which may progress to severe biventricular failure and dilated cardiomyopathy.
There is usually a family history of sudden cardiac death.