Cambios estructurales y funcionales en el corazón de pacientes adultos con osteogénesis imperfecta: estudio de casos y controles / Structural and functional changes in the heart of adult patients with osteogenesis imperfecta: Case-control study

Introducción y objetivos: La osteogénesis imperfecta (OI) es una enfermedad del tejido conectivo que se caracteriza por una síntesis anómala del colágeno tipo i. Se sabe poco sobre la afectación cardiovascular que presentan estos pacientes. Las descritas con más frecuencia son la disfunción valvular y la dilatación de la aorta. Hemos querido analizar los cambios estructurales y funcionales del corazón en pacientes adultos con OI. Métodos: Estudiamos prospectivamente 82 pacientes con OI y los comparamos con 60 sujetos sanos emparejados por edad y sexo. A todos se les realizó un estudio ecocardiográfico. Resultados: No encontramos diferencias en la incidencia de valvulopatías respecto al grupo control. Nuestros pacientes tenían un mayor tamaño de la aurícula izquierda y mayores diámetros del ventrículo izquierdo (VI) ajustados por superficie corporal. La raíz de la aorta era significativamente mayor. La fracción de eyección del VI en los pacientes con OI era menor y tenían una presión sistólica de la arteria pulmonar mayor. Conclusiones: Los pacientes con OI presentan cambios estructurales y funcionales en el corazón. Sería recomendable un seguimiento para ver la evolución de estos cambios

Introduction and objectives: Osteogenesis imperfecta (OI) is a connective tissue disease characterised by an anomalous synthesis of type i collagen. Little is known about the cardiovascular affectation suffered by these patients. The most frequently described are valvular dysfunction and dilatation of the aorta. We wanted to analyse the structural and functional changes of the heart in adult patients with OI. Methods: We prospectively studied 82 patients with OI and compared them with 60 healthy subjects matched for age and sex. All of them underwent an echocardiographic study. Results: We found no differences in the incidence of valvulopathies compared to the control group. Our patients had a larger left atrium and larger left ventricle (LV) diameters adjusted for body surface area. Aortic root was significantly higher. LV ejection fraction in patients with OI was lower and they had higher pulmonary artery systolic pressure. Conclusions: Patients with OI suffer structural and functional changes in the heart. A follow-up is recommended to observe the evolution of these changes

Structural and functional changes in the heart of adult patients with osteogenesis imperfecta: Case-control study. / Cambios estructurales y funcionales en el corazón de pacientes adultos con osteogénesis imperfecta: estudio de casos y controles.

INTRODUCTION AND OBJECTIVES: Osteogenesis imperfecta (OI) is a connective tissue disease characterised by an anomalous synthesis of type i collagen. Little is known about the cardiovascular affectation suffered by these patients. The most frequently described are valvular dysfunction and dilatation of the aorta. We wanted to analyse the structural and functional changes of the heart in adult patients with OI. METHODS: We prospectively studied 82 patients with OI and compared them with 60 healthy subjects matched for age and sex. All of them underwent an echocardiographic study. RESULTS: We found no differences in the incidence of valvulopathies compared to the control group. Our patients had a larger left atrium and larger left ventricle (LV) diameters adjusted for body surface area. Aortic root was significantly higher. LV ejection fraction in patients with OI was lower and they had higher pulmonary artery systolic pressure. CONCLUSIONS: Patients with OI suffer structural and functional changes in the heart. A follow-up is recommended to observe the evolution of these changes.

Infarto agudo miocárdico-embólico asociado a embolia Pulmonar / Embolic acute myocardial infarction associated with pulmonary embolism

Resumen El infarto agudo de miocardio de origen embólico es poco frecuente y suele ocurrir en pacientes con la fibrilación auricular o las valvulopatías. Suele afectar a la arteria descendente anterior. La presentación simultánea de un embolismo pulmonar y un infarto agudo de miocardio también es rara y difícil de diagnosticar porque ambas entidades producen síntomas parecidos y alteraciones electrocardiográficas a veces superponibles. Se presenta el caso de un paciente con embolia pulmonar y un infarto agudo de miocardio simultáneos, en probable relación con una embolia paradójica.

Abstract Acute myocardial infarction of embolic origin is rare and usually develops in patients with atrial fibrillation or valvular heart diseases. It affects the anterior descending artery. Simultaneous presentation of a pulmonary embolism and acute myocardial infarction is also rare and difficult to diagnose because both entities produce similar symptoms and sometimes overlapping electrocardiographic alterations. The case of a patient with simultaneous pulmonary embolism and acute myocardial infarction is presented, probably related to a paradoxical embolism.

Impacto de la cirugía bariátrica a medio plazo en la estructura y la función del corazón / Mid-term impact of bariatric surgery on cardiac structure and function

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Alteraciones cardiacas en la osteogénesis imperfecta. Estudio ecocardiográfico de casos y controles / Cardiac abnormalities in osteogenesis imperfecta. Case-control echocardiographic study

Fundamento y objetivos: La osteogénesis imperfecta (OI) se caracteriza por una síntesis anómala del colágeno tipo i, que ocasiona fragilidad ósea y otras manifestaciones extraesqueléticas. Se han descrito diversas manifestaciones cardiovasculares, incluyendo la disfunción valvular (en especial el prolapso mitral) y la dilatación de la raíz de la aorta (AO). Nuestro objetivo es evaluar la prevalencia de anomalías cardiacas en los pacientes con OI en comparación con un grupo control de individuos sanos de la misma edad y sexo. Pacientes y método:Se estudió de forma prospectiva a 26 pacientes diagnosticados de OI y los comparamos con 25 pacientes sanos. Se llevó a cabo en todos ellos un estudio ecocardiográfico en modo M, 2D y Doppler color. Resultados:No encontramos en nuestro estudio diferencias significativas entre ambos grupos en cuanto a la función sistólica y diastólica del ventrículo izquierdo (VI) ni en la incidencia de valvulopatías. Las dimensiones de la AO, la aurícula izquierda y el VI únicamente al ajustarlas a la superficie corporal eran significativamente mayores en los pacientes con osteogénesis frente al grupo control, probablemente debido a la menor superficie corporal de estos pacientes. Sin embargo, existía una diferencia significativa al calcular la proporción AO/diámetro telediastólico del VI, independiente de la superficie corporal, que era mayor en los pacientes con OI.Conclusiones: No encontramos una mayor incidencia de valvulopatías respecto al grupo control y sí encontramos una raíz de la AO de mayor tamaño en relación con el tamaño del VI (AU)

Background and objectives: Osteogenesis imperfecta is a rare disease with abnormal synthesis of type 1 collagen that affects diverse extra-skeletal tissues. Aortic root dilatation and valvular dysfunction have been described. Our purpose was to evaluate the prevalence of cardiac abnormalities in patients with osteogenesis imperfecta in comparison with an age and sex-matched control group of healthy people. Patients and methods: We prospectively studied 26 patients with osteogenesis imperfecta and compared them with 25 healthy people. All patients underwent a transthoracic standard M-mode, 2D and colour Doppler study. Results: We did not find significant differences between both groups in the left ventricular diastolic and systolic function and the incidence of valvulopathies. The dimensions of the aortic root, left atrium and left ventricle when indexed by body surface area were significantly larger in patients with osteogenesis imperfecta compared with the control group, which was probably due to the reduced body surface of these patients. However there was a significant difference in the aortic root/telediastolic diameter of left ventricle ratio independent of the body surface, which was higher in patients with osteogenesis imperfecta.Conclusions: The incidence of valvular disease in osteogenesis imperfecta is similar to that of the normal population. However aortic root is larger in the former patients and is related to the left ventricular diastolic diameter (AU)

[Cardiac abnormalities in osteogenesis imperfecta. Case-control echocardiographic study]. / Alteraciones cardiacas en la osteogénesis imperfecta. Estudio ecocardiográfico de casos y controles.

BACKGROUND AND OBJECTIVES: Osteogenesis imperfecta is a rare disease with abnormal synthesis of type 1 collagen that affects diverse extra-skeletal tissues. Aortic root dilatation and valvular dysfunction have been described. Our purpose was to evaluate the prevalence of cardiac abnormalities in patients with osteogenesis imperfecta in comparison with an age and sex-matched control group of healthy people. PATIENTS AND METHODS: We prospectively studied 26 patients with osteogenesis imperfecta and compared them with 25 healthy people. All patients underwent a transthoracic standard M-mode, 2D and colour Doppler study. RESULTS: We did not find significant differences between both groups in the left ventricular diastolic and systolic function and the incidence of valvulopathies. The dimensions of the aortic root, left atrium and left ventricle when indexed by body surface area were significantly larger in patients with osteogenesis imperfecta compared with the control group, which was probably due to the reduced body surface of these patients. However there was a significant difference in the aortic root/telediastolic diameter of left ventricle ratio independent of the body surface, which was higher in patients with osteogenesis imperfecta. CONCLUSIONS: The incidence of valvular disease in osteogenesis imperfecta is similar to that of the normal population. However aortic root is larger in the former patients and is related to the left ventricular diastolic diameter.

Miocardiopatía restrictiva reversible secundaria a cloroquina / Reversible restrictive cardiomyopathy secondary to chloroquine

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