The Brugada Syndrome

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History

54 years old make, brought to the Emergency Room (ER) at Miguel Server Hospital when he was found semi unconscious. He was seen working a few hours earlier in an apparently normal way; he arrives to the ER in an obnubilate state, with evident right hemiparesis and motor aphasia.

History of Personal Past Illnesses

His family members referred that he had a non-treated diabetes (high blood sugar), hypertension (high blood pressure), dyslipidemia (high cholesterol), habitual alcoholic consumption and he was in the hospital 4 months earlier with a sternal fracture (fracture of the breastbone). There was no past history of syncope and no previous cardiological studies.

Physical Examination

He was in a obnubilate state, reactive, Glasglow 3-4-5 [this means that he opened his eyes when called, he was confused and he was able to localize a produced motion in his body by the doctor], with well hydrated skin and good color pigmentation.

Heart and lung examination was normal.

Neurological examination: Glasgow3-4-5, motor aphasia, right hemiparesis, right plantar reflex in extention (Babinski reflex) [this means there was damage in the motor control pathways leading from the brain]

Complementary Studies

Biochemistry – Urea 22, Glucose (blood sugar) 202, Sodium 135, Potassium 3.5, Creatinine 0.7. (they are all within normal range except blood sugar which is high, normal range is 70-110mg/dL when fasting, and 70-140 mg/dL after eating).

Hemogram – Hem 4, Htc 38, Hb 13, VCM 96, LEU 9.7, Neut 84. (it is all normal).

Cardiac enzymes – At 6:15 pm CK 296, CK-MB 20. At 10: 00 pm CK 306, CK-MB 16 [this means that heart muscles cells were damaged].

CT Scan: Microinfarcts. Left thalamic brain hematoma (blood clot) [this means that he probably hit his head when he fainted and explains all the neurological symptoms, thalamic hemorrhage often presents with contralateral sensory loss, so this is why he has symptoms in his right side)

ECG – Sinus rhythm, 100 beats per min, right bundle branch block, rsR´V, segment elevation in V2 [this all means that it is compatible with Brugada syndrome, see below]

Evolution

This man had a significant improvement of his neurological symptoms, cardiac enzymes were normalized and he was further followed by the neurologist and specially the cardiologist for further treatment.

Brugada Syndrome

The diagnosis in this case is Brugada syndrome which is made by the ECG, which is a print out of the heart’s electrical rhythm. In 1992 an ECG pattern was described in people having syncope episodes and/or sudden death with no previously heart disease. This ECG pattern consists on right bundle branch block with ST segment elevation in the right side precordial leads, resulting in sudden death due to non expected cardiac arrest events.

Here is a normal ECG pattern:

Normal ECG

Here is the ECG of the current case:

ECG Brugada

The association between Brugada syndrome and prevalence of cardiac arrest could be as high as 40 to 60% idiopathic [“unknown cause”] ventricular fibrillation and there seems to be an autosomal dominant expression involved in the gene SCN5A coding protein components in the sodium channels in heart cells. The channel defect causes abnormal electrical conduction in the heart resulting in ventricular arrhythmia which leads to death. The man in this case was the typical presentation of unexplained fainting spells, in fact, many patients diagnosed at some point with Brugada syndrome presented themselves initially with unexplained fainting spells.

In this moment implantation of an ICD (implantable cardiac defibrillator), in order to avoid complications (cardiac arrest, ventricular fibrillation), is the only treatment available for preventing sudden death associated to this Syndrome.

Case reference

http://www.medspain.com/ant/n13_jun00/Brugada.htm

Recommended links

http://www.stuartxchange.com/Bangungot.html
http://en.wikipedia.org/wiki/Brugada_syndrome
http://www.brugada.org/

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