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ACCF/AHA 2009 Expert Consensus Document on
Pulmonary Hypertension
by , Vallerie V. McLaughlin, Stephen L. Archer, David B. Badesch, Robyn J. Barst,
Harrison W. Farber, Jonathan R. Lindner, Michael A. Mathier, Michael D. McGoon,
Myung H. Park, Robert S. Rosenson, Lewis J. Rubin, Victor F. Tapson, and John
Varga
Circulation
Volume 119(16):2250-2294
April 28, 2009
Copyright © American Heart Association, Inc. All rights reserved.
Figure 1. Schematic depicting the potential “hits” involved in the development of PAH. A rise in
[Ca2+]cyt in PASMCs (due to decreased Kv channel activity and membrane depolarization,
which opens VDCCs; upregulated TRPC channels that participate in forming receptor- and storeoperated Ca2+ channels ; and upregulated membrane receptors [e.g., serotonin, endothelin, or
leukotriene receptors] ; and their downstream signaling cascades) causes pulmonary
vasoconstriction, stimulates PASMC proliferation, and inhibits the BMP-signaling pathway that
leads to antiproliferative and proapoptotic effects on PASMCs. Dysfunction of BMP signaling
due to BMP-RII mutation and BMP-RII/BMP-RI downregulation and inhibition of Kv channel
function and expression attenuate PASMC apoptosis and promote PASMC proliferation.
Writing Committee Members et al. Circulation.
2009;119:2250-2294
Copyright © American Heart Association, Inc. All rights reserved.
Figure 2. Mean Survival of Patients With PAH Based on Etiology.
Writing Committee Members et al. Circulation.
2009;119:2250-2294
Copyright © American Heart Association, Inc. All rights reserved.
Figure 3. Diagnostic Approach to PAH. General guidelines for the evaluation of pulmonary
hypertension.
Writing Committee Members et al. Circulation.
2009;119:2250-2294
Copyright © American Heart Association, Inc. All rights reserved.
Figure 4. CXR and ECG in PAH. Postero-anterior and lateral CXR (top) shows decreased
peripheral lung vascular markings, hilar pulmonary artery prominence, and right ventricular
enlargement of a patient with idiopathic PAH. ECG (bottom) of the same patient reveals right
atrial enlargement, right ventricular hypertrophy and strain, and right axis deviation of the QRS
complex.
Writing Committee Members et al. Circulation.
2009;119:2250-2294
Copyright © American Heart Association, Inc. All rights reserved.
Figure 5. Echocardiographic Features of PAH. (A) Parasternal short axis view.
Writing Committee Members et al. Circulation.
2009;119:2250-2294
Copyright © American Heart Association, Inc. All rights reserved.
Figure 6. Treatment Algorithm for PAH. Background therapies include warfarin anticoagulation,
which is recommended in all patients with IPAH without contraindication.
Writing Committee Members et al. Circulation.
2009;119:2250-2294
Copyright © American Heart Association, Inc. All rights reserved.