Multiple pulmonary arteriovenous fistulas in hereditary haemorrhagic teleangiectasia
Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Lazarettstr. 36, D-80636 München, Germany
* Corresponding author. E-mail address: hager{at}dhm.mhn.de
A 22-year-old woman was evaluated for multiple round shadows in the lower parts of her lungs at chest X-ray. At childhood she often had nose bleeding, but now she did not reveal any symptoms. Her father died at the age of 46 years from a cerebral abscess.
Clinical examination revealed teleangiectasia on the tongue and on the buccal mucous membranes and a slight cyanosis. At exercise her oxygen saturation measured by pulse oximetry declined from 90 to 80%.
Helical CT scanning (Siemens Somatom Sensation Cardiac, Siemens Medical Solutions, Erlangen, DE) showed multiple pulmonary arteriovenous fistulas of different sizes (Panel A).
At cardiac catheterization a right-to-left shunt of 17% of the systemic circulation could be calculated from oximetry. Five of the most prominent fistulas, three on the left side (Panel B) and two on the right, were transcutanously embolized with Amplatzer duct occluders (AGA Medical Corporation, Golden Valley, MN, USA).
Afterwards, cyanosis improved to a SpO2 of 94% at rest and 91% at maximal exercise performance.
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