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Resumen Introduccion y objetivos La distrofia miotonica tipo 1 se caracteriza por afeccion muscular y manifestaciones sistemicas, entre ellas las cardiacas. Nuestro objetivo es documentar la frecuencia y la gravedad de la afeccion cardiovascular (aparicion de disfuncion ventricular izquierda y trastornos del ritmo o conduccion), la necesidad de implantar marcapasos o desfibrilador o de realizar estudio electrofisiologico y la aparicion de muerte subita durante el seguimiento. Metodos Estudio observacional retrospectivo de los pacientes con distrofia miotonica tipo 1 remitidos a una consulta monografica de cardiologia y sometidos a seguimiento clinico, electrocardiografico (con registro Holter) y ecocardiografico. Resultados Se incluyo a 81 pacientes (el 51,9% varones; media de edad, 29,9 ± 14,8 anos). El seguimiento medio fue de 5,7 ± 3,9 (1-20) anos y se documento bradicardia sinusal en el 48,8%, disfuncion sinusal en el 13,8%, arritmias supraventriculares en el 10%, intervalo PR ≥ 220 ms en el 31,3%, taquicardia ventricular el 5%, intervalo QT corregido largo en el 5%, bloqueo auriculoventricular de segundo o tercer grado en el 8,8% e intervalo QRS ≥ 120 ms en el 7,5%. Solo 1 paciente presento disfuncion ventricular grave. Durante el seguimiento se implantaron 15 marcapasos y 2 desfibribladores y se realizaron 5 estudios electrofisiologicos, la mayoria por taquicardia ventricular. Solo se produjo 1 muerte subita. Conclusiones Los trastornos de conduccion y del ritmo son frecuentes durante la evolucion de dichos pacientes, y un porcentaje considerable requiere estudio electrofisiologico e implante de dispositivos (marcapasos o desfibrilador). La disfuncion sistolica y la muerte subita son excepcionales en nuestra experiencia.

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.

The N-terminal brain natriuretic peptide (NT-proBNP) is a new tool for the diagnosis of patients with evidence of heart failure (HF). The diagnosis of HF is improbable in patients with a level of NT-proBNP300 pg/ml. Our objective is to determine the validity of the cut-off points proposed by literature (inclusion/exclusion) regarding the age for NT-proBNP in patients admitted to our service.We gathered consecutively the data of 76 patients admitted to our service with the diagnosis of HF (n=37) and with other diseases (n=39), taking a sample of plasma to determine NT-proBNP. We studied the sensitivity, specificity and predictive values of this test, as well as the relation between the levels of that marker and the clinical and echocardiographic variables.The ventricular diameter was larger and the ejection fraction was lower in patients with HF. Sensitivity for the diagnosis of exclusion reached a value of 97% and the negative predictive value was 94%.The cut-off point proposed for exclusion (300 pg/ml) shows high sensitivity and negative predictive value in the diagnosis of HF in patients admitted to our service.

Cocaine is directly related to the occurrence of acute coronary syndromes (ACS). We analyzed the differential characteristics of consumers who suffer an ACS in our environment.A retrospective study of 914 patients with ACS who entered consecutively to our hospital.Thirty four patients were consumers (3.7%, CI95%: 2.7-5.2), with an average age of 40.1+/-9.1 years, significantly lower than non-consumers (63.6+/-12.15 years, P.001). The prevalence increased progressively in younger groups. Most were men (82.4%), with higher consumption of tobacco (88.2% vs. 34.2%, P.001) and other illegal drugs (35.3% vs 0.9%, P.001). By contrast, they had less frequently hypertension (26.5 vs. 60.5%, P.001) or diabetes (11.7 vs. 39.8%, P.001). There was single vessel disease in 44.1%, and a coronary angiography without significant lesions in 20.6% cases.The typical patient with ACS who consumes cocaine is usually a young man, who often takes other drugs and with a lower prevalence of classic risk factors. These data have to be taken into account when considering preventive and therapeutic manoeuvres.

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.

Secondary or metastatic cardiac tumors are about 20-40 times more common than primary benign or malignant cardiac tumors. Cardiac metastases are more frequent in carcinomas, they usually appear in patients over 50 years and the involvement by gender is similar. Any tumor can cause cardiac or pericardial metastasis, although isolated or combined tumor invasion of the pericardium is more common. Transthoracic echocardiography (TTE) is the primary diagnostic procedure, followed by computed tomography (CT) and cardiac magnetic resonance imaging (MRI) to verify positive findings and to analyze other structures of the mediastinum and chest. Treatment of such tumors is generally associated with poor results and unfavorable prognosis. We report the case of an unusual cardiac metastasis.