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Vol. 151. Issue 10.
Pages 397-399 (November 2018)
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Vol. 151. Issue 10.
Pages 397-399 (November 2018)
Brief report
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
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Verónica Hernández Jiméneza,
Corresponding author
veronicahernandezz@hotmail.com

Corresponding author.
, Jesús Saavedra Falerob, María Teresa Alberca Velab, Rebeca Mata Caballerob, José Antonio Rosado Sierrac, Isabel Pavón de Pazc
a Servicio de Cardiología, Hospital Universitario Rey Juan Carlos, Móstoles, Madrid, Spain
b Servicio de Cardiología, Hospital Universitario de Getafe, Getafe, Madrid, Spain
c Servicio de Endocrinología y Nutrición, Hospital Universitario de Getafe, Getafe, Madrid, Spain
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Tables (3)
Table 1. Baseline characteristics of patients with osteogenesis imperfecta and control group.
Table 2. Echocardiographic parameters related to the structure and dimension of the heart in both groups.
Table 3. Functional echocardiographic parameters in both groups.
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Abstract
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.

Keywords:
Osteogenesis imperfecta
Collagen
Echocardiography
Valvulopathy
Aortic root
Resumen
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.

Palabras clave:
Osteogénesis imperfecta
Colágeno
Ecocardiografía
Valvulopatías
Raíz aórtica

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