Case reportLung transplantation in a patient with arteriovenous malformations
Section snippets
Materials and methods
In 1996, a 39 year-old female patient with HHT presented for lung transplantation. She had Class III to IV functional dyspnea, cyanosis and digital clubbing, and telangiectasis was noted in her lips. She had experienced epistaxis since childhood. She also complained of frequent migraine headaches.
The patient was under continuous oxygen therapy. She underwent a left upper lobectomy and closure of an atrioventricular (AV) fistula in the lower left lobe in 1973. Her father died from HHT, and one
Results
The patient underwent a single right lung transplantation on May 30, 1998 at the Hospital Italiano, Buenos Aires, and remained hospitalized for 21 days. She received triple therapy as immunosuppression (corticosteroids, cyclosporine, azathioprine). There were no acute rejection episodes. She suffered only a few mild upper airway infections.
At the time of this report, 2 years and 10 months later, she is still alive and performing normal activities. She has an oxygen saturation of 99%, even
Discussion
Very rarely do AV malformations indicate symptoms at an early age. However, when this occurs, they may be severe and accompanied by heart failure and even by fulminant respiratory failure.5 Usually, symptoms develop between the fourth and sixth decade of life when the size of the fistulas are >2 cm.
Epistaxis is one of the most frequent symptoms, mainly in patients with HHT, followed by dyspnea and digital clubbing, and less commonly there may be migraine activity. Typically, AVF patients have
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