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doi:10.1378/chest.06-2275
(Chest. 2007; 131:984-987)
© 2007 American College of Chest Physicians
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Long-term Outcome in a Patient With Pulmonary Hypertension and Hereditary Hemorrhagic Telangiectasia*

Omar A. Minai, MD, FCCP; Christina Rigelsky, MS; Charis Eng, MD, PhD; Alejandro C. Arroliga, MD, FCCP and James K. Stoller, MD, MS, FCCP

* From the Department of Pulmonary, Allergy, and Critical Care Medicine (Dr. Minai), Cleveland Clinic, Cleveland, OH; Genomic Medicine Institute (Ms. Rigelsky), Lerner Research Institute, Cleveland, OH; Cleveland Clinic Genomic Medicine Institute (Dr. Eng), Cleveland, OH; Division of Pulmonary, Critical Care, and Sleep Medicine (Dr. Arroliga), Scott and White Clinic, Temple, TX; and Cleveland Clinic Lerner College of Medicine (Dr. Stoller), Cleveland Clinic, Cleveland, OH.

Correspondence to: Omar A. Minai, MD, FCCP, Department of Pulmonary, Allergy, and Critical Care Medicine, A90, Cleveland Clinic, 9500 Euclid Ave, Cleveland, OH 44195; e-mail: minaio{at}ccf.org

Abstract

Hereditary hemorrhagic telangiectasia (HHT) may be associated with pulmonary hypertension (PH). In the context that little attention has been given to long-term follow-up of such individuals, we report a patient with PH associated with HHT with special attention to clinical features and long-term response to therapy. To our knowledge, this case represents only the second with a 10-year follow-up reported and demonstrates that aggressive therapy can lead to long-term improvement in clinical parameters and survival.

Key Words: genetic • hereditary hemorrhagic telangiectasia • pulmonary hypertension • pulmonary vascular disease • shunt






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