As a second extension of available knowledge, our patient has been followed up for 10 years on various vasoactive medications, including nifedipine, epoprostenol and, most recently, IV treprostinil, and has remained clinically stable with good functional status (New York Heart Association class II), stable exercise capacity, and freedom from the need for supplemental oxygen. Indeed, to our knowledge, she represents only the second such patient followed up for an extended interval. Schlag et al10 described the 10-year follow-up of a 49-year old woman with HHT and PH (pulmonary artery pressures at right-heart catheterization, 74/33 mm Hg; mean, 51 mm Hg; Fick cardiac output, 6.4 L/min) who was treated initially with calcium-channel blockers and then inhaled iloprost, beraprost, and finally bosentan. Over the course of her follow-up, her mean pulmonary artery pressure remained stable at approximately 50 mm Hg. To our knowledge, our patient represents only the second patient with HHT and PH to be described with long-term follow-up after treatment with a variety of vasoactive medications. Taken together, these two reports demonstrate the possibility of decade-long survival, clinical stability and, in our patient, even functional and physiologic improvement over the course of treatment. Clearly, longer follow-up of larger cohorts is required before definitive conclusions are possible, but this early experience provides strong rationale for aggressive therapy, most certainly in this special subset of patients with both HHT and PH.