Nuevo modelo de consulta externa de cardiología integrado con atención primaria / New model of integration between primary health care and specialized cardiology care

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Debut of an unusual liver cirrhosis.

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.

Debut de cirrosis hepática poco habitual / Debut of an unusual liver cirrhosis

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Cardiac Relapse of Acute Myeloid Leukemia after Allogeneic Hematopoietic Stem Cell Transplantation.

Secondary or metastatic cardiac tumors are much more common than primary benign or malignant cardiac tumors. Any tumor can cause myocardial or pericardial metastasis, although isolated or combined tumor invasion of the pericardium is more common. Types of neoplasia with the highest rates of cardiac or pericardial involvement are melanoma, lung cancer, and breast and mediastinal carcinomas. Acute myeloid leukemia (AML) is the most common type of acute leukemia in adults. Initial treatment involves chemotherapy followed by consolidation treatment to reduce the risk of relapse. In high-risk patients, the treatment of choice for consolidation is hematopoietic stem cell transplantation (HSCT). Relapse of AML is the most common cause of HSCT failure. Extramedullary relapse is rare. The organs most frequently affected, called "sanctuaries," are the testes, ovaries, and central nervous system. We present a case with extramedullary relapse in the form of a solid cardiac mass.

Aortic valve tethering: embryonic remnant of a common origin?

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.

Manifestaciones cardiacas en los pacientes con distrofia miotónica tipo 1 seguidos de forma protocolizada en una consulta monográfica / Cardiac manifestations in myotonic dystrophy type 1 patients followed using a standard protocol in a specialized unit

Introducción y objetivos. La distrofia miotónica tipo 1 se caracteriza por afección muscular y manifestaciones sistémicas, entre ellas las cardiacas. Nuestro objetivo es documentar la frecuencia y la gravedad de la afección cardiovascular (aparición de disfunción ventricular izquierda y trastornos del ritmo o conducción), la necesidad de implantar marcapasos o desfibrilador o de realizar estudio electrofisiológico y la aparición de muerte súbita durante el seguimiento. Métodos. Estudio observacional retrospectivo de los pacientes con distrofia miotónica tipo 1 remitidos a una consulta monográfica de cardiología y sometidos a seguimiento clínico, electrocardiográfico (con registro Holter) y ecocardiográfico. Resultados. Se incluyó a 81 pacientes (el 51,9% varones; media de edad, 29,9 ± 14,8 años). El seguimiento medio fue de 5,7 ± 3,9 (1-20) años y se documentó bradicardia sinusal en el 48,8%, disfunción 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%. Sólo 1 paciente presentó disfunción ventricular grave. Durante el seguimiento se implantaron 15 marcapasos y 2 desfibribladores y se realizaron 5 estudios electrofisiológicos, la mayoría por taquicardia ventricular. Sólo se produjo 1 muerte súbita. Conclusiones. Los trastornos de conducción y del ritmo son frecuentes durante la evolución de dichos pacientes, y un porcentaje considerable requiere estudio electrofisiológico e implante de dispositivos (marcapasos o desfibrilador). La disfunción sistólica y la muerte súbita son excepcionales en nuestra experiencia (AU)

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 (AU)

Cardiac manifestations in myotonic dystrophy type 1 patients followed using a standard protocol in a specialized unit.

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.