Thursday 4/12/2014 Hassan Alahmadi Medical Resident ( R1)

 35 years old male patient who presented with frequent syncopal episode with palpitation and his ECG is shown Interpret the ECG  What it is your diagnosis ?

 The Brugada syndrome is an autosomal dominant genetic disorder associated with increased risk of ventricular tachyarrhythmias and sudden cardiac death  the ECG findings consist of a pseudo-right bundle branch block and persistent ST segment elevation in leads V1 to V3  QT interval prolongation may be seen in the right precordial leads

 Prevalence — The prevalence of the typical ECG changes of the Brugada pattern has been evaluated in a number of different populations. Studies in heterogeneous populations suggest that the majority of affected individuals are of Asian descent  Gender — The Brugada pattern is much more common in men than in women  Age at diagnosis — Brugada syndrome is usually diagnosed in adulthood. While rarely diagnosed in children

Brugada pattern versus Brugada syndrome

 Asymptomatic  AF  Nocturnal agonal respiration  VT  Syncope  Sudden cardiac death

 (A) This typical coved pattern present in V1-V2 shows the following:  In the classic Brugada type 1 ECG, the elevated ST segment (≥2 mm) descends with an upward convexity to an inverted T wave  The duration of QRS is longer than in RBBB, and there is a mismatch between V1 and V6.

 (B) This typical saddle-back pattern present in V1-V2 shows the following:  the ST segment is ≥2 mm elevated and has a "saddle back" ST-T wave configuration, in which the elevated ST segment descends toward the baseline but remains at least 0.5 mm above the isoelectric baseline and then rises again to an upright or biphasic T wave.

 Type 1  Appearance of type 1 ST segment elevation (coved type) plus at least one of the following :-  Documented ventricular fibrillation  Polymorphic ventricular tachycardia (VT)  Family history of sudden cardiac death at less than 45 years of age  Family history of type 1 Brugada pattern ECG changes  Inducible VT during electrophysiology study  Unexplained syncope suggestive of a tachyarrhythmia  Nocturnal agonal respiration

 Type 2 :  Appearance of type 2 ST segment elevation (saddle-back type) in more than one right precordial lead under baseline conditions, with conversion to type 1 following challenge with a sodium channel blocker, plus at least one of the following:  Documented ventricular fibrillation  Polymorphic ventricular tachycardia (VT)  Family history of sudden cardiac death at less than 45 years of age  Inducible VT during electrophysiology study  Unexplained syncope suggestive of a tachyarrhythmia  Nocturnal agonal respiration

 TREATMENT — Treatment for patients diagnosed with the Brugada syndrome is primarily focused around termination of any ventricular arrhythmias with :- 1. Therapy with an ICD 2. pharmacologic therapy for arrhythmia prevention has been tried but with relatively little success.

 Antiarrhythmic drug therapy in patients with the Brugada syndrome may be considered in two circumstances:  In patients who refuse ICD implantation or are not a candidate for ICD implantation due to reduced life expectancy or significant comorbidities, initial therapy with either quinidine or amiodarone.  In patients with an ICD who have recurrent arrhythmias resulting in ICD shocks, therapy with amiodarone, although quinidine is also an option for these patients.

 In contrast to patients with the Brugada syndrome, patients with only the Brugada ECG pattern do not require any specific therapy.

Thank you