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Vol. 2. Núm. S3.Noviembre 2006
Esclerosis sistémica
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Vol. 2. Núm. S3.Noviembre 2006
Esclerosis sistémica
Páginas S1-S45
Esclerosis sistémica
DOI: 10.1016/S1699-258X(06)73105-9
Acceso a texto completo
Afección cardíaca en la esclerosis sistémica
Cardiac involvement in systemic sclerosis
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Luis H. Silveira-Torre??
Autor para correspondencia
luis_hsil@yahoo.com

Correspondencia: Dr. L.H. Silveira-Torre. Departamento de Bioquímica. Instituto Nacional de Cardiología Ignacio Chávez. Juan Badiano, 1. Colonia Sección XVI. 14080 México DF. México.
Departamento de Bioquímica. Instituto Nacional de Cardiología Ignacio Chávez. México DF. México
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Resumen

La esclerosis sistémica (ES) puede afectar al pericardio, el miocardio, las arterias coronarias, el sistema de conducción y las válvulas cardíacas. La presencia de síntomas y signos es de mal pronóstico. Las manifestaciones incluyen disnea, palpitaciones, dolor precordial, síncope y datos de insuficiencia cardíaca derecha.

La prevalencia de la enfermedad pericárdica con manifestaciones clínicas varía entre el 5 y el 16%; con ecocardiografía, es del 5,4-41% y en estudios de necropsia, es del 33-77,5%. La afección miocárdica característica de la ES es la fibrosis en parches. El hallazgo patológico típico es la “necrosis en banda de contracción”. Las consecuencias importantes de la fibrosis incluyen hipertrofia ventricular izquierda, así como disfunción tanto sistólica como diastólica en ambos ventrículos. La detección en fases tempranas de estas alteraciones es muy importante, sobre todo de la disfunción diastólica, que ocurre mucho antes que la sistólica, por lo que podría predecir el desarrollo de daño cardíaco importante. Se ha descrito la asociación de miositis esquelética y cardíaca. La miopatía esquelética aumenta el riesgo de insuficiencia cardíaca congestiva, arritmias sintomáticas sostenidas y muerte súbita de origen cardíaco. Los vasos pequeños miocárdicos están afectados en la ES; las arterias coronarias epicárdicas no se afectan. Se presentan arritmias, principalmente extrasístoles auriculares y ventriculares, así como alteraciones de la conducción. La afección de las válvulas cardíacas es mínima, y la mitral es la que se afecta con mayor frecuencia. Otras alteraciones que se ha comunicado en relación con la ES son la rigidez de las arterias periféricas de gran calibre y la afección secundaria del corazón por hipertensión arterial pulmonar y sistémica. La afección cardíaca es causa importante de morbimortalidad en la ES.

Palabras clave:
Corazón
Esclerosis sistémica
Pericardio
Miocardio
Válvulas cardíacas
Cardiopatía isquémica

Systemic sclerosis (SS) can involve the pericardium, myocardium, conduction system, and cardiac valves. The presence of overt clinical signs of cardiac disease is a poor prognostic sign. Clinical manifestations include dyspnea, palpitations, chest pain, syncope, and symptoms of right heart failure.

Prevalence of clinically symptomatic pericardial disease is 5-16%. However, ecocardiographic prevalence is 5.4- 41% and at autopsy is 33-77.5%. Patchy fibrosis is the characteristic myocardial finding in SS. Contraction band necrosis is the typical pathological finding. Important complications of fibrosis include left ventricular hypertrophy, as well as systolic and diastolic dysfunction of both ventricles. Early detection of these abnormalities is very important, mainly of the diastolic dysfunction, since it occurs before the systolic dysfunction and can predict important cardiac damage. Association of skeletal myositis with myocardial disease has been described. Patients with skeletal myositis are more likely to develop congestive heart failure, sustained symptomatic arrythmias, and cardiac sudden death. Coronary arteries are normal in systemic sclerosis, but there is no endomyocardial vessel involvement. There is an increased prevalence of arrhytmias, mainly premature atrial and ventricular contractions, as well as conduction system disease. Cardiac valvular involvement is minor in systemic sclerosis; mitral valve is the most frequently affected. Other abnormalities described in this disease include peripheral large vessels stiffness and secondary cardiac involvement due to pulmonary and systemic arterial hypertension. Cardiac involvement confers a high morbi-mortality risk in systemic sclerosis.

Key words:
Heart
Systemic sclerosis
Pericardium
Myocardium
Cardiac valves
Ischemic cardiopathy
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