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Vol. 38. Issue 12.
Pages 599-602 (December 2002)
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Vol. 38. Issue 12.
Pages 599-602 (December 2002)
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Enfermedad de Rendu-Osler-Weber. Fístulas arteriovenosas
Rendu-Osler-Weber syndrome. Pulmonary arteriovenous fistulas. A report of three cases
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R.A. Gómez Tejada
Corresponding author
ragomtej@mail.retina.ar
jgene@intramed.net.ar

Correspondencia: División de Neumonología. Hospital de Clínicas José de San Martín. Avda. Córdoba, 2351, 7.° piso. 1120 Capital Federal. Argentina
, R.J. Gené, C. Faure, S. Rossi, V. Vollberg, K. Rébora
División de Neumonología. División de Cardiología (Sección de Ecocardiografía). Servicio de Diagnóstico por Imágenes. Hospital de Clínicas José de San Martín. Universidad de Buenos Aires. Argentina
Article information

La telangiectasia hemorrágica heredofamiliar (THH) o síndrome de Rendu-Osler-Weber es un trastorno sistémico infrecuente asociado con malformaciones vasculares pulmonares (MPAV). Aunque el diagnóstico clínico puede fundamentarse en las manifestaciones cutaneomucosas, de vías aéreas altas o en los múltiples episodios de sangrado, la presencia de un compromiso pulmonar suele cursar inaparente y definir el pronóstico.

Se presentan tres pacientes con una THH y con MPAV en los que la medición del shunt con O2 al 100%, la ecocardiografía con contraste y la tomografía helicoidal sustentan el enfoque diagnóstico no invasor. En uno de los pacientes, la arteriografía confirmó la sospecha de MPAV única. Recibió emboloterapia quirúrgica con un coil y se obtuvo definida mejoría clínica y funcional.

Palabras clave:
Malformaciones vasculares pulmonares
Telangiectasia hemorrágica heredofamiliar

Hereditary hemorrhagic telangiectasia (HHT), or Rendu-Osler-Weber syndrome, is a rare disorder that is closely linked to the development of pulmonary arteriovenous malformations (PAVM). Diagnosis can be based on clinical signs such as upper respiratory tract changes or recurrent hemorrhagic events. Nevertheless, pulmonary involvement, a prognostic factor, may remain undetected.

In the three HTT cases with PAVM we report, the following diagnostic information was obtained non-invasively: shunt fraction measurements (breathing 100% oxygen), echocardiographic contrast studies, and three-dimensional helical computed tomographs. Arteriography demonstrated a single PAVM in one case and the patient underwent successful coil embolization, with clinical and functional improvement.

Keywords:
Pulmonary arteriovenous malformations
Hereditary hemorrhagic telangiectasia
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Copyright © 2002. Sociedad Española de Neumología y Cirugía Torácica
Archivos de Bronconeumología

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