Pulmonary Hypertension, Primary

9/22/00 (Schultz)

Group: Friday Interns

RE: A 31 year old female with ASD presenting with dyspnea, orthopnea, leg cramps, and increase in ascites. 

Question: What is the current therapy for primary pulmonary hypertension?

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Unique Identifier: 20207709

Authors: Arroliga AC. Dweik RA. Kaneko FJ. Erzurum SC.

Institution: Department of Pulmonary and Critical Care Medicine, Cleveland Clinic, USA. arrolia@ccf.org

Abstract: Primary pulmonary hypertension is a progressive and fatal disease that chiefly affects young adults. Although its etiology is a mystery, recent research has uncovered several biochemical derangements in this disorder, and new therapies are prolonging survival. [References: 50]

Link Directly to Fulltext Article at Science Direct

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Unique Identifier: 98397221

Authors: Gaine SP. Rubin LJ.

Institution: Division of Pulmonary and Critical Care Medicine, University of Maryland School of Medicine, Baltimore 21201, USA.

Abstract: Primary pulmonary hypertension (PPH) is a progressive disease characterised by raised pulmonary vascular resistance, which results in diminished right-heart function due to increased right ventricular afterload. PPH occurs most commonly in young and middle-aged women; mean survival from onset of symptoms is 2-3 years. The aetiology of PPH is unknown, although familial disease accounts for roughly 10% of cases, which suggests a genetic predisposition. Current theories on pathogenesis focus on abnormalities in interaction between endothelial and smooth-muscle cells. Endothelia-cell injury may result in an imbalance in endothelium-derived mediators, favouring vasoconstriction. Defects in ion-channel activity in smooth-muscle cells in the pulmonary artery may contribute to vasoconstriction and vascular proliferation. Diagnostic testing primarily excludes secondary causes. Catheterisation is necessary to assess haemodynamics and to evaluate vasoreactivity during acute drug challenge. Decrease in pulmonary vascular resistance in response to acute vasodilator challenge occurs in about 30% of patients, and predicts a good response to chronic therapy with oral calcium-channel blockers. For patients unresponsive during acute testing, continuous intravenous epoprostenol (prostacyclin, PGI2) improves haemodynamics and exercise tolerance, and prolongs survival in severe PPH (NYHA functional class III-IV). Thoracic transplantation is reserved for patients who fail medical therapy. We review the progress made in diagnosis and treatment of PPH over the past 20 years. [References: 39]

Link Directly to Fulltext Article at Publishe

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Unique Identifier: 97198996

Authors: Badesch DB.

Institution: University of Colorado Health Sciences Center, Denver 80262, USA. David.Badesch@uchsc.edu

Abstract: Progress in treatment of pulmonary hypertension has been impaired by the lack of formal clinical trials. This is now beginning to change, and the impact on our approach to treating patients with pulmonary hypertension in substantial. As with other relatively uncommon medical disorders, randomized, controlled, multi-center trials are needed to assess the safety and efficacy of potential therapeutic modalities. Treatments showing promise at the level of small pilot studies within a single center should be studied more rigorously. [References: 53]

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Unique Identifier: 97102851

Authors: Kneussl MP. Lang IM. Brenot FP.

Institution: Dept of Internal Medicine IV (Pulmonary Medicine), Pulmonary Hypertension Clinic of the Vienna General Hospital, University of Vienna, Austria.

Abstract: Primary pulmonary hypertension (PPH) is a poorly understood, progressive disease that is characterized by elevation of pulmonary artery pressure and vascular resistance, leading to right ventricular failure and death within 2-3 yrs after diagnosis. Based upon the concept that vasoconstriction and thrombotic occlusion of resistance vessels precipitate this process, vasodilator therapy and anticoagulation have become the main strategies for improving survival in these patients. Whereas, a few years ago, medical therapy of primary pulmonary hypertension was perceived as a bridging therapy to lung or heart lung transplantation, modes of therapy are being clinically tested at this time to offer an alternative to the surgical treatment of this disease. However, no selective pulmonary vasodilator is yet available. Therefore, and because of the potential hazards of vasodilator treatment, standardized haemodynamic testing is performed prior to initiation of vasodilator treatment. In this update, the currently available compounds both for haemodynamic testing and chronic therapy, their mode of action, method of administration and efficacy are reviewed. [References: 104]