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Diagnosis and differential assessment of pulmonary arterial hypertension

Robyn J. Barst, MD^*,*, Michael McGoon, MD, Adam Torbicki, MD, Olivier Sitbon, MD, Michael J. Krowka, MD, Horst Olschewski, MD^|| and Sean Gaine, MD^¶

^* Columbia University College of Physicians and Surgeons, New York, New York, USA
Mayo Clinic, Rochester, Minnesota, USA
Institute of Tuberculosis and Lung Diseases, Warsaw, Poland
Hôpital Antoine Béclère, Clamart, France
^|| Medical Clinic II, University Clinic, Giessen, Germany
^¶ Mater Misericordiae Hospital, University College, Dublin, Ireland

View larger version (24K): [in a new window]   Figure 1 Guidelines for evaluating pulmonary hypertension. Abbreviations: BNP = brain natriuretic peptide; CBC = complete blood count; CT = computerized tomography; CTD = connective tissue disease; EBCT = electron beam computerized tomography; HIV = human immunodeficiency virus; HRCT = high-resolution computerized tomography; LFTs = liver function tests; PH = pulmonary hypertension; RHC = right heart catheterization; SaO2 = systemic arterial oxygen saturation; TEE = transesophageal echocardiography; VD = vasodilator; / = ventilation/perfusion.

View larger version (18K): [in a new window]   Figure 2 (A) Long-term (7-year) survival in patients with idiopathic pulmonary arterial hypertension (IPAH) based on functional class (III vs. IV) at the time of epoprostenol initiation. p = 0.0001 by log-rank test (30). (B) Survival in patients with IPAH treated with intravenous epoprostenol according to New York Heart Association (NYHA) functional class. Estimated percentages of survival for patients in NYHA functional class IV at baseline (dashed line) were 76%, 60%, and 47% at one, two, and three years, respectively, as compared with 90%, 76%, and 71% for patients in NYHA functional class III at baseline (solid line) (p < 0.001 by the Cox-Mantel log-rank test) (32).

View larger version (20K): [in a new window]   Figure 3 (A) Subsequent survival in patients with idiopathic pulmonary arterial hypertension (IPAH) stratified by functional class after 1 year epoprostenal treatment 1: p < 0.001 for functional class III vs. functional class IV and for functional class III vs. functional class I and functional class II (30). (B) Survival in patients with IPAH treated with intravenous epoprostenol according to New York Heart Association (NYHA) functional class. After three months of treatment with epoprostenol, survival rates for patients reclassified in NYHA functional class I or II (solid line) were 100%, 93%, and 88% at one, two, and three years, respectively, as compared with 77%, 46%, and 33% for patients persisting in NYHA functional class III or IV (dashed line) (p < 0.001 by the Cox-Mantel log-rank test) (32).

View larger version (18K): [in a new window]   Figure 4 (A) Kaplan-Meier survival estimates in 156 patients with idiopathic pulmonary arterial hypertension (IPAH), according to the results of the 6-min walk (6MW) test performed after three months of epoprostenol therapy. Survival rates for patients walking >380 m during the 6MW test (corresponding to the median value) (solid line) were 99%, 88%, and 81% at one, two, and three years, respectively, as compared with 86%, 64%, and 56% for patients walking <380 m (dashed line) (p = 0.0005 by the Cox-Mantel log-rank test). (B) Kaplan-Meier survival estimates in 156 patients with IPAH, according to the change in the 6MWT between baseline and three months of epoprostenol therapy. No difference in survival was demonstrated in patients who improved their 6-min walk distance to >112 m (corresponding to the median value) (solid line), as compared with those who did not (dashed line) (p = 0.86 by the Cox-Mantel log-rank test) (32).