Pulmonary Hypertension

Classification
Simonneau G; Galie N; Rubin LJ et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2004; 43(12 Suppl S):5S-12S.  Update to the original 1998 Evian Clinical classification of PH with the 2003 classifications determined during the Third World Symposium on Pulmonary Arterial Hypertension held in Venice, Italy.  http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=15194173

Arcasoy SM, Christie JD, Ferrari VA, et al. Echocardiographic assessment of pulmonary hypertension in patients with advanced lung disease. Am J Respir Crit Care Med 2003; 167:735-40.  The cardiology literature indicates echocardiography-derived estimates of pulmonary artery pressures are accurate. This study found 52% of echo estimates were inaccurate (off by > 10 mmHg) in 166 lung transplant candidates and the difference was > 20 mmHg in 28%.  In patients without hypertension, echo was more likely to overestimate pressures while in patients with pulmonary hypertension; it was as likely to over as underestimate.  Accuracy and ability to obtain an estimate varied with the underlying disease. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=12480614

Evaluation and treatmentt of pulmonary arterial hypertension
Badesch DB, Abman SH, Simonneau G, et al. Medical therapy for pulmonary arterial hypertension: Updated ACCP evidence-based clinical practice guidelines. Chest 2007; 131:1917-28. Treatment options for pulmonary arterial hypertension have increased substantially in recent years. This update includes a summary of the previous 2004 guidelines for diagnosis and treatment and provides new recommendations on medical treatment. Recommendations are based on the quality of evidence as well as the net benefit of therapy. http://www.ncbi.nlm.nih.gov/pubmed/17565025?ordinalpos=2&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum

Rich S, Kaufman E, Levy PS. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N Engl J Med 1992;327:76-81. Study with suboptimal design but convincing hemodynamic data found improved survival and is the basis for use of CCBs in patients with a good response to vasodilators. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=1603139

Sitbon O, Humbert M, Jais X, et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation  2005; 111:3105-3111. This retrospective analysis found that only 54% of the patients who had initial vasoreactivity and were placed on calcium channel blockers had a favorable long-term response.  This finding prompted a revision in the consensus definition of a favorable response to acute vasodilator testing. http://www.ncbi.nlm.nih.gov/pubmed/15939821?ordinalpos=37&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum

Barst RJ, Rubin LJ, Long WA, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) and conventional therapy for primary pulmonary hypertension. N Engl J Med 1996;334:296-301. RCT found the epoprostenol group had improved hemodynamics, quality of life, and survival. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=8532025

Badesch DB, Tapson VF, McGoon MD, et al. Continuous intravenous epoprostenol for pulmonary hypertension due to scleroderma spectrum of disease. Ann Intern Med 2000; 132:425- 34. Noteworthy for showing benefit from prostacyclin in patients with a secondary cause of pulmonary hypertension.  RCT found prostacyclin improved exercise tolerance, modestly reduced PA pressures, and improved dyspnea scores in some patients, but was associated with frequent side effects and more adverse events. No difference in survival, but trial was only 12 weeks duration. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=10733441

Channick RN, Simonneau G, Sitbon O, et al. Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomized placebo-controlled study.  Lancet 2001; 358:1119-23. Early study of bosentan in 32 patients with primary or scleroderma-related pulmonary hypertension.  Over the 12 weeks of the study, bosentan was well-tolerated and improved cardiac index and exercise capacity (70 meter gain in 6-minute walk). Similar results were obtained in a subsequent larger study of 213 patients (Rubin LJ et al. New Engl J Med 2002; 346:896-903). http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=11597664

Galie N, Ghofrani HA, Torbicki A, et al.  Sildenafil citrate therapy for pulmonary arterial hypertension.  N Engl J Med. 2005; 353:2148-57. Double-blind RCT comparing sildenafil (20, 40, or 80 mg; po tid x 12 weeks) and placebo in 278 patients with symptomatic pulmonary arterial hypertension found sildenafil improved exercise capacity, WHO functional class, and hemodynamics. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=16291984&query_hl=4&itool=pubmed_docsum