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November 2005 EKG Case: A 24 year old Asian male walks into the Emergency Department after an episode of syncope. He has no chest pain. He has no significant past medical history but is concerned because of a family history of sudden cardiac death. Rate: 75 Rhythm: sinus Axis: normal PR: 0.16 QRS: 0.08-0.10, rsR’ in V2 ST: elevated in V1 V2 V3 EKG Diagnosis: Sinus with ST elevations in V1-V3 Clinical Diagnosis: Brugada Syndrome Treatment: Implantable defibrillator The syndrome of right bundle branch block, ST segment elevation in V1 to V3 and sudden death is a clinical-EKG diagnosis based on syncopal or sudden death episodes in patients with a structurally normal heart and a characteristic EKG pattern: The EKG shows ST elevation in V1-V3, with a morphology of the QRS complex resembling a right bundle branch block The episodes of syncope or sudden death are caused by fast polymorphic ventricular tachycardias or ventricular fibrillation. These arrhythmias appear with no warning. There is no prolongation of the QT interval during sinus rhythm. Only in a few cases there is alternation of long-short sequences before the polymorphic V. tach. a finding which is very common in other arrhythmias like “torsade de pointes” in the long QT syndrome. There is no preceding acceleration of the heart rate as is the case of catecholamine dependent polymorphic V. tach. This syndrome is genetically determined. Approximately 60% of patients with syncope with the typical EKG have a family history of sudden death, or have family members with the same EKG abnormalities. There are also sporadic cases who are probably the patients with a de novo mutation in the family. The pattern of transmission is autosomal dominant. There is a predominance of affected males. In Thailand, the disease almost exclusively affects males. This syndrome has a very poor prognosis when left untreated: 1/3 of patients who have suffered from syncopal episodes or resuscitated from near-sudden death with a typical EKG develop a new episode of polymorphic V. tach within 2 years. Unfortunately, prognosis of asymptomatic individuals with a typical EKG is also poor. 1/3 of these patients also have a first episode of polymorphic V.tach or V. fib within 2 years of follow-up. Because anti arrhythmic drugs (amiodarone or beta blockers) do not protect against sudden cardiac death, the only available treatment is the implantable cardioverter-defibrillator. This device effectively recognizes and treats the ventricular arrhythmias. When provided with the implantable defibrillator, total mortality in patients with Brugada syndrome has been 0 % with upto 10 years follow-up. This is not surprising considering that these patients are young and usually devoid of other diseases. Because the heart is structurally normal, and there is no coronary artery disease, these patients do not die from heart failure or complications of ischemic events. Thus, they are the most ideal candidates for treatment with an implantable defibrillator. Symptomatic patients must receive this device.
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