Cases reported "Bundle-Branch Block"

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1/7. Brugada.

    The brugada syndrome was first described in 1992. Palpitations and/or syncope are usually experienced during rest and increased vagal activity. [table: see text] However, in 15% of patients with brugada syndrome, symptoms occur during physical activity. The syndrome is further characterized by a pattern of RBBB and ST-segment elevation in V1 to V2/V3 (see Table). One major and one minor criterion from the Table can serve to establish a diagnosis of brugada syndrome. The patient presented in this report described symptoms only during physical activity. EPS confirmed the diagnosis of the brugada syndrome, and an ICD was implanted. The clinical importance of the brugada syndrome is that it calls attention to patients at risk for SCD. The syndrome is genetically determined and caused by mutations in the cardiac ion channels. Signal averaging that reveals late potentials can help identify persons who may be at high risk for SCD and who would thus be candidates for EPS, which can identify those at risk of SCD. The ICD is the only therapy known to help prevent SCD in patients with brugada syndrome.
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2/7. Congenital aneurysm of the sinus of valsalva protruding into the left ventricle. review of diagnosis and treatment of the unruptured aneurysm.

    During a routine physical examination, a 34-year-old man, in apparent good health, had an electrocardiogram which revealed left axis deviation. Cardiac evaluation disclosed mild aortic regurgitation and left anterior fascicular block. Nine months later, the patient was admitted to the critical care unit after he had experienced cardiac arrest while jogging. Angiograms demonstrated a large unruptured aneurysm in the right coronary sinus of valsalva protuding into the left ventricle. Echocardiographic manifestations also suggested also suggested a sinus of valsalva aneurysm. The defect was repaired through an aortic approach. The aneurysmal sac was emptied and the neck sutured securely. Twenty-two months postoperatively, the patient continues to be asymptomatic. We anticipate that this will decrease the threat of recurrent arrhythmias..
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3/7. A newly characterized SCN5A mutation underlying brugada syndrome unmasked by hyperthermia.

    Febrile illness has been rarely reported to modulate ST segment elevation in right precordial leads on ECG or even precipitate ventricular fibrillation in patients with brugada syndrome. We report the case of a patient whose Brugada ECG pattern was unmasked by hyperthermia secondary to acute cholangitis. Serial ECGs showed progressive attenuation of ST segment elevation as body temperature gradually returned to normal. Structural heart disease was ruled out. Intravenous flecainide injection reproduced a less remarkable ST segment elevation. Genetic screening demonstrated a single amino acid substitution (H681P) in the SCN5A gene, thus confirming the diagnosis of brugada syndrome. in vitro expression of this newly characterized genetic defect revealed novel biophysical abnormalities consisting of a shift in both steady-state activation and inactivation, resulting in a 60% reduction of sodium window current. Thus, SCN5A-H681P mutation induces a significant loss of transmembrane current and is clinically associated with a pathologic phenotype that is elicited by hyperthermia. overall the observed clinical features are in agreement with previous observations and strongly suggest that fever may be an environmental modifier among brugada syndrome patients with a detrimental (and possibly arrhythmogenic) effect on cardiac repolarization.
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4/7. A 28 year old woman with ventricular tachycardia and an abnormal chest radiograph.

    A 28 year old white woman with no medical history presented to the emergency room with symptomatic non-sustained ventricular tachycardia. She was asymptomatic up to a few days before presentation. Her physical examination was essentially normal and hence did not help with the differential diagnosis of the problem. Bronchoscopic transbronchial biopsy led to the final diagnosis of cardiac sarcoidosis.
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5/7. Electrocardiographic pattern of brugada syndrome disclosed by a febrile illness: clinical and therapeutic implications.

    BACKGROUND: Recent studies have identified a direct link between the ionic mechanisms responsible for the electrocardiographic (ECG) pattern of the Brugada syndrome (BS) and the in vitro experimental temperature, pointing to the possibility that some BS patients may display the ECG phenotype only during a febrile state, being in this setting at risk of lethal arrhythmias. CASE REPORT: A 53-year-old man referred to the emergency room for abdominal pain and fever. The ECG showed dome-shaped ST-segment elevation in V1-V3, as in the typical BS. The personal and family history were unremarkable for syncope and sudden death and physical, laboratory and ultrasound examinations were negative. On day 3, at normal body temperature, the patient's ECG returned to normal and the ECG abnormalities were later reproduced with intravenous flecainide. The patient refused the implantation of a loop recorder and was discharged after 6 days. He has remained asymptomatic during 2 years of follow-up. CONCLUSIONS: The typical ECG phenotype of BS disclosed by a febrile illness confirms the in vitro experimental data that previously established a correlation between ECG pattern of BS and temperature variations. The clinical and therapeutic implications of these findings are discussed.
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6/7. brugada syndrome presenting with sustained monomorphic ventricular tachycardia.

    Typically brugada syndrome presents with either ventricular fibrillation or polymorphic ventricular tachycardia that may result in sudden death or syncope in patients without any structural heart disease. We report the case of a patient with brugada syndrome who presented atypically with recurrent presyncope following physical exertion due to sustained monomorphic ventricular tachycardia, which appeared to be sensitive to both adenosine and catecholamine. He refused ICD implantation but remained asymptomatic on treatment with a beta-blocker.
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7/7. Right bundle branch block, persistent ST-segment elevation in V1-V3 and sudden cardiac death: always a distinct syndrome?

    Right bundle branch block, persistent ST segment elevation in right precordial leads and sudden cardiac death, unexplainable by currently known disease, define a new distinct clinical and electrocardiographic syndrome. This report describe a patient with these features, whose physical examination, echocardiography, chest computed tomography and right ventricular angiography were normal. However, despite the negativity of these examinations, cardiac nuclear magnetic resonance allowed the identification of right ventricular dysplasia. Thus, right ventricular dysplasia should be considered in the differential diagnosis of the syndrome characterized by right bundle branch block, persistent ST segment elevation and sudden cardiac death.
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