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Introduction and objectives Myotonic dystrophy type 1 is characterized by muscle damage and systemic manifestations, including cardiac involvement. Our aim was to document the frequency and severity of cardiac involvement (left ventricular dysfunction and arrhythmia or conduction disorders), the need for a pacemaker, implantable cardioverter-defibrillator, or electrophysiological study, and the development of sudden death during follow-up. Methods Retrospective observational study of myotonic dystrophy type 1 patients referred to a specialized cardiac unit. Patients received clinical, electrocardiographic (Holter monitoring), and echocardiographic follow-up. Results We included 81 patients (51.9% men; mean age, 29.9 [14.8] years). The mean follow-up was 5.7 (3.9) years (range: 1-20 years). During this period sinus bradycardia was documented in 48.8%, PR interval≥220 ms in 31.3%, long corrected QT interval in 5%, and QRS interval≥120 ms in 7.5%. A total of 13.8% of patients developed sinus node dysfunction, 10% of patients had supraventricular arrhythmias, 5% had ventricular tachycardia, and 8.8% developed second- or third- degree atrioventricular block. Only 1 patient had severe ventricular dysfunction. During the follow-up, 15 pacemakers and 2 implantable cardioverter-defibrillators were implanted and 5 electrophysiological studies were performed (mainly due to ventricular tachycardia). There was only 1 sudden death. Conclusions Arrhythmia or conduction disorders are frequent during the course of myotonic dystrophy type 1 patients. A significant percentage of patients require electrophysiological study and the use of a device (pacemaker or implantable cardioverter-defibrillator). In our experience, systolic dysfunction and sudden death are rare.

Resumen Fundamento y objetivo La cocaina esta directamente relacionada con la aparicion de sindrome coronario agudo (SCA). Analizamos las caracteristicas diferenciales de los consumidores que presentan un SCA en nuestro medio. Material y metodos Estudio retrospectivo de 914 pacientes con SCA que ingresaron de forma consecutiva en nuestro hospital. Resultados Un total de 34 pacientes refirieron ser consumidores (3,7%, intervalo de confianza del 95% [IC del 95%], 2,7–5,2), con edad media (desviacion estandar [DE]) de 40,1 (9,1) anos, significativamente menor que en los no consumidores (63,6 [12,15] anos; p Conclusiones El enfermo con SCA y consumidor de cocaina es generalmente un varon joven, que con frecuencia consume otras drogas y con una menor prevalencia de factores de riesgo clasico, datos a tener en cuenta a la hora de tomar medidas preventivas y terapeuticas.

Fundamento y objetivo El fragmento N-terminal del propeptido natriuretico cerebral o tipo B (NT-proBNP) se utiliza para el diagnostico de pacientes con sospecha de insuficiencia cardiaca (IC). Los valores de NT-proBNP inferiores a 300 pg/ml indican un diagnostico improbable de IC. Nuestro objetivo ha sido determinar la validez de los puntos de corte del NT-proBNP segun la edad, propuestos en la bibliografia medica (inclusion/exclusion), en los pacientes ingresados en nuestro servicio. Pacientes y metodo Hemos recogido de forma prospectiva los datos de 90 pacientes que ingresaron en nuestro servicio con diagnostico de IC (n = 37) y de otras enfermedades (n = 39), y en quienes se determino la concentracion de NT-proBNP. Se estudiaron la sensibilidad, la especificidad y los valores predictivos de este analisis, asi como la relacion de los valores de dicho marcador con las variables clinicas y ecocardiograficas. Resultados En los pacientes con IC el diametro ventricular era mayor y la fraccion de eyeccion, menor. La sensibilidad para el diagnostico de exclusion fue del 97% y el valor predictivo negativo, del 94%. Conclusiones El punto de corte de exclusion ropuesto (300 pg/ml) posee una alta sensibilidad y valor predictivo negativo para el diagnostico de IC en los pacientes ingresados en nuestro servicio.

A 51-year-old female patient presented to the emergency room with a 3-month history of increasing dyspnoea, and 5 kg weight loss. Her medical history was not relevant. Physical examination revealed 100/90 mm Hg blood pressure, 98 bpm, orthostatic hypotension, a soft grade 2/6 systolic murmur at the left sternal border and bilaterally pulmonary rates. ECG showed normal sinus …

A 14-year-old asymptomatic girl without relevant medical history was referred to our department for heart murmur evaluation. The echocardiogram showed cardiac chambers with normal size and function. Noteworthy was the presence of an apparently fibrous tissue joining the ventricular surfaces of the aortic non-coronary and right coronary leaflets with the anterior mitral leaflet. Both valves were slightly thickened and there was a mild anterior mitral valve 'billowing' causing an eccentric mild-to-moderate regurgitant jet. During systole, tethering of this tissue caused the incomplete opening of both mentioned aortic leaflets, causing a turbulent flow with no significant gradient across the valve. During diastole, moderate eccentric aortic regurgitation jet was noted, probably related to incomplete coaptation at the insertion point of this anomalous tissue. We speculate that this finding may represent the remnant of some tissue during heart development that abnormally persisted in this young lady.