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Enhanced Hypoxic Pulmonary Vasoconstriction in Families of Adults or Children With Idiopathic Pulmonary Arterial Hypertension*

Ekkehard Grünig, MD; Christoph Dehnert, MD; Derliz Mereles, MD; Rolf Koehler, PhD; Horst Olschewski, MD; Peter Bärtsch, MD; Bart Janssen, PhD
Author and Funding Information

Affiliations: *From the Department of Cardiology (Drs. Grünig and Mereles), Institute of Sports Medicine (Drs. Dehnert and Bärtsch), Institute of Human Genetics (Drs. Koehler and Janssen), University of Heidelberg, Heidelberg, Germany; and the Department of Pneumology (Dr. Olschewski), University of Giessen, Giessen, Germany.,  These authors contributed equally to this study.

Correspondence to: Ekkehard Grünig, MD, Department of Cardiology and Pneumology, University Hospital of Heidelberg, INF 410, 69120 Heidelberg, Germany; e-mail: ekkehard_gruenig@med.uni-heidelberg.de



Chest. 2005;128(6_suppl):630S-633S. doi:10.1378/chest.128.6_suppl.630S
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Idiopathic pulmonary arterial hypertension (IPAH) is a rare life-threatening disease that is characterized by the sustained elevation of pulmonary artery pressure without demonstrable cause.1 The disease is chronically progressive and is believed to evolve slowly, with an asymptomatic increase in pulmonary arterial constriction and remodeling for some years. In most patients, signs and symptoms appear when pulmonary artery pressure is markedly elevated.2 Since the identification of mutations of the bone morphogenetic protein receptor II (BMPR2) gene in patients with IPAH,35 methods aimed at the identification of persons at risk for the disease have gained attention. In previous studies67 performed in families with IPAH, we demonstrated that healthy individuals who shared the risk haplotype (RH) with the index patient (the IPAH gene) have an increased pulmonary artery systolic pressure (PASP) during exercise. We hypothesized that healthy carriers of the IPAH trait would not only have an abnormal increase in pulmonary artery pressure during exercise but also during exposure to hypoxia.

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