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* From the Department of Pathology (Dr. Tucker), The Johns Hopkins University, Baltimore, MD; and the Division of Pulmonary Sciences and Critical Care Medicine (Drs. Voelkel and Fagan), University of Colorado Health Sciences Center, Denver, CO.
Correspondence to: Norbert F. Voelkel, MD, Division of Pulmonary Sciences and Critical Care Medicine, 4200 E. Ninth Ave, University of Colorado Health Sciences Center, Denver, CO 80262; e-mail norbert.voelkel{at}uchsc.edu.
The challenge is to find a cure for severe pulmonary hypertension—nothing less. Vascular reactivity is a blessing that is not being bestowed on most of our patients. We still face the harsh reality that the diagnosis of severe pulmonary hypertension is usually a late diagnosis of an already established disease. Our treatments are based on the presumption that they work because they achieve pulmonary vasodilation, and we hope that they may affect the altered vascular cell phenotype.
We know now that the transforming growth factor-ß family of molecules and signaling is involved in the spectrum of diseases. Pulmonary circulation has now entered the realm of vascular biology in which a gene is related to the vascular phenotype. In pulmonary hypertension, the first gene to be discovered was BMPRII.12 Although hailed as the prelude of major discoveries on how the disease develops, we face the reality that we still do not understand how it affects pulmonary hypertension. This task continues to challenge us. BMPRII mutations occur in only 50% of the patients with familial idiopathic pulmonary hypertension. They are germline mutations, and approximately 20% of those carrying the gene acquire the disease. Nevertheless, how can we relate alterations in transforming growth factor-ß family signaling with the alterations seen in pulmonary precapillary vessels of patients with severe pulmonary hypertension? The only vascular alteration that is unique to patients with severe and fatal disease is intravascular angioproliferation, which recapitulates several of the molecular mechanisms involved in cancer, such as loss of tumor suppression genes and expression of antiapoptotic proteins. We may have to deal with severe angioproliferative pulmonary hypertension. Comparing neoplastic growth and the growth of cells in the complex vascular lesions in pulmonary hypertension may lead us to new insights into therapies and biomarkers. Our patients are more aware and knowledgeable than ever, since the Internet provides them with the latest information, and they hope for a cure. This is not the time for complacency. Lives and hopes hinge on advancements that are incumbent on us to develop.
This has been the fifth Aspen Lung Conference dedicated to the topic of pulmonary hypertension/circulation, and the first to focus on angiogenesis, cell-cell interactions, gene expression patterns, and altered cell phenotypes. Since the fourth Aspen Pulmonary Hypertension Conference (1997), the interest in this group of diseases has increased worldwide, with increased research into its mechanisms and novel drugs for treatment. The concerted effort of many have led to a European working group for pulmonary hypertension research, a working group for pulmonary hypertension in children, and a World Health Organization-sponsored international meeting held in Venice, Italy, in June of 2003.3
The state-of-the-art speakers again opened new vistas on vascular biology. Although there is a growing recognition of the participation of the pulmonary vessels in many complex chronic lung diseases, such as interstitial lung diseases and COPD, the pathobiology of lung vessels in these disorders has been even less explored than in the severe angioproliferative forms of pulmonary hypertension. Although this conference dealt, to a large measure, with the severe idiopathic form of pulmonary hypertension, including the rare, nonplexogenic pulmonary hemangiomatosis, several presentations addressed fundamental aspects of the biology of the pulmonary circulation.
The superb organization and logistics of this meeting were masterfully handled by Ms. Jeanne Cleary, who continues to be the heart and soul of these conferences. The organizers and the Steering Committee of the Thomas L. Petty Aspen Lung Conference thank this year’s summarizer, Dr. Marlene Rabinovitch, for her stellar performance. The job of the summarizer gets harder every year, as our knowledge expands, and as young investigators with new ideas and technologies join us (Fig 1 ).
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Thank you to all of the forward-looking state-of-the-art speakers. Their talks, which kept the audience on the edge of their chairs, mesmerized and almost suspended time. The excitement over research was contagious. We quote Reuben Cherniack, who said, "I wish I were 20 years younger. I would work in Dr. So and So’s lab."
Thanks for all the great short presentations and the great effort that went into the preparation and presentations of the posters. It looks like the poster, wine, and food sessions are firmly established, and are now as much a tradition as the Thursday family picnic at the foot of the Maroon Bells. This conference, again, was an international meeting with participants from England, France, Germany, Holland, Japan, South Korea, and Canada, and was simply a great success.
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