RESUMO
Objective To explore clinical characteristics of J wave syndromes. Methods Clinical data was retrospectively analyzed in a child with fainting and showing big J wave in ECG. Meanwhile, related literatures were reviewed. Results A 10-year-old boy was admitted to hospital with fainting. ECG showed J point up, reflecting Osbron wave. There was no abnormality in EEG and cranial CT. Ultrasound cardiograph showed approximately normal heart structure and left ventricular systolic function. Fainting was frequently occurred during follow-up and accompanied by malignant arrhythmia. Conclusions Patients with J wave syndromes has high risks of malignant arrhythmia and sudden cardiac death.
RESUMO
La hemocromatosis primaria es la enfermedad genética más común de Occidente (1 de cada 300 a 400 personas). El compromiso cardíaco durante sus estadios iniciales no se detecta por las técnicas de diagnóstico por imágenes y es durante este período cuando sobrevienen arritmias potencialmente letales. A través de la resonancia magnética cardíaca (RMC) con su secuencia de T2 estrella es posible detectar precozmente la afectación cardíaca y permite estratificar el riesgo y monitorizar la evolución del tratamiento de los pacientes afectados.
The primary hemochromatosis is the most common genetic disease in Western regions (1 out of 300/400 individuals). The cardiac involvement during its early stages is not detected with the diagnostic imaging techniques, and it is during these stages when potentially life-threatening arrhythmias occur. Through the CMRI, with its T2 star sequence, it is possible to early detect the cardiac involvement and to stratify the risk and monitor the evolution of treatment in affected patients.
RESUMO
La muerte súbita cardíaca de causa eléctrica en sujetos con corazón "sano", constituye un problema clínico y de salud pública, aún no resuelto. Los objetivos del trabajo fueron: caracterizar pacientes reanimados de un evento de muerte súbita de causa eléctrica y conocer su evolución intervenida en tres años; y estudiar los signos eléctricos premonitorios de muerte súbita. Se estudiaron 42 sujetos, 30 hombres y 12 mujeres, edad promedio 37.7 años, con corazón "sano", por métodos clínicos y paraclínicos. Se consideraron 9 subpoblaciones, en mayor número los síndromes de Brugada y de QT largo y la fibrilación ventricular idiopática. Las arritmias responsables del evento fueron, en primer lugar, la fibrilación ventricular y la torsión de puntas. Existieron signos premonitorios en el 92.8% de los pacientes. Fueron frecuentes las recidivas de las arritmias malignas (71.4%) aunque en la estimulación eléctrica programada, sólo se logró reproducirlas en 4 de 18 pacientes. La fibrilación auricular predominó como arritmia coexistente (19%). En resumen, son frecuentes los signos premonitorios (en especial la fibrilación auricular) y las recidivas de las arritmias malignas aunque su inducibilidad en el laboratorio es pobre. La estratificación de riesgo es muy difícil, por el bajo valor predictivo de los métodos diagnósticos.
Sudden cardiac death due to electrical causes in individuals with no evidence of structural heart disease is an important clinical and public health problem, and it is not yet solved. The objectives of this study were: to characterize patients reanimated from a sudden death event of electrical cause; to know the mediated evolution during a period of three years and to study premonitory electrical signs. 42 individuals were studied, 30 were male and 12 female, mean age 37.7 years, healthy heart, by clinic and paraclinic methods. Nine subpopulations were studied, being Brugada syndrome, long QT syndrome and idiopathic ventricular fibrillation the most frequent. Ventricular fibrillation and twisting of the points were the arrhythmias responsible for most death events. There were premonitory signs in 92.8% and clinical recurrences of life-threatening events in 71.4% but they were induced during programmed electrical stimulation only in 4 of 18 patients. Atrial fibrillation was the most frequent coexistent arrhythmia (19%). In summary, there are frequent premonitory signs (particularly atrial fibrillation), and also malignant arrhythmic recurrences but a poor inducibility at the electrophysiology laboratory. It is very difficult to stratify the risk because of the low predictive value of diagnostic methods.