Search

This paper presents a 21 years-old-male without structural heart disease who was reanimated from a sudden cardiac death event. His familial history included two siblings suddenly dead in their first year of life. The patient had 10 episodes of ventricular tachycardia (some of them were registered). Electrocardiographically abnormalities were an intermittent short QT interval (280 ms), short QTc (320 ms) and a short ST segment. QT interval subsequently returned to a normal range (360 ms or more), while occasionally a length of 335 ms was recorded. The electrophysiological study findings (AH and HV intervals, refractory periods) were normal. The patient refused the implantable cardioverter defibrillator and he is receiving oral amiodarone (200 mg/day). The evolution has been satisfactory along four years. Sequential electrocardiograms are very important to identify patients with an intermittent short QT interval. Shortening of the interval J wave-Tpeak is also relevant. Related arrhythmias could be ventricular tachycardia or fibrillation.

Arrhythmic sudden cardiac death due to electrical causes is an important clinical and public health problem, which is not yet solved. Ventricular fibrillation is the first cause of the event. It does not adjust to a single model, being a family with diverse electrocardiographic patterns that reveal different disorganization degrees. Many of these deaths happen without witness. We present a patient who was being studied after a first medical visit because of a previous syncope with spontaneous recovery, coursing with left bundle branch block. He was not receiving any antiarrhythmic drug and was asymptomatic at the time. He was wearing a long-term ambulatory electrocardiographic recorder (Holter), which became the sole witness of his death that occurred while he was alone at home. The recording revealed various malignant ventricular arrhythmia (torsades des pointes, ventricular flutter, ventricular fibrillation), immediately preceding events were an increased heart rate, extra-systoles, and left bundle branch block. This is the first recording of the whole sequence of malignant ventricular arrhythmias leading to death in a patient wearing a Hotter device obtained by our department, which has collected 750 Hotter ambulatory records per year for more than 20 years. The literature on the subject is reviewed.

Throughout the 13 years of recognizing the Brugada syndrome as a separate entity, there has been a search for invasive and non-invasive markers for detecting risk of life-threatening arrhythmic events, particularly for asymptomatic individuals in whom the first manifestation may be sudden cardiac death. Hence, the preclinical diagnosis is pivotal for adequate and timely preventive measures. The objective of this study was to compare various non-invasive markers to characterize and stratify patients at risk. Late potentials, QT interval, QT dispersion, and heart variability were analyzed over a two-year period, in 20 patients (17 men and 3 women) with the Brugada syndrome (symptomatic and asymptomatic) and compared with 20 normal individuals similar in age and gender (control group). Late potentials were present in 80% of patients versus 5% in the control group (p < 0.0001); all of these with recurrent episodes had late potentials. In conclusion, this is the most important non-invasive marker for risk stratification, recurrences and inducibility of malignant arrhythmias during electrophysiological testing. Markers, invasive and non-invasive, should be considered integrally, for a better diagnostic and prognostic approach to reality.

The Brugada syndrome is the most frequent electrical cause of sudden death in patients with structurally normal heart. It is characterized by ST segment elevation in V1-V3 leads, pseudo right bundle branch block and proneness for episodes of life-threatening ventricular cardiac arrhythmias. The aim of this study was to characterize patients with Brugada syndrome identified in Cuba between 1995 and 2001. Fourteen patients with these electrical signs (13 male and 1 female, mean age 42.8) were studied. Seven of them were symptomatic and seven were not. The pharmacological test with sodium channel blockers was very useful (it was positive for all the patients). HV interval was normal. Programmed ventricular stimulation was performed. Ventricular arrhythmia was inducible in 3 symptomatic patients and 2 asymptomatic. All the symptomatic patients and those asymptomatic in whom ventricular arrhythmia was inducible received automatic defibrillators. One clinical recurrence of ventricular arrhythmias was observed. Antiarrhythmic drugs were only used rarely. Clinical history and electrocardiographic tracings were very important to diagnose this syndrome. There were light, transient or masked electrical signs. The treatment of choice is the implantable cardioverter-defibrillator.