Abstract
Pulmonary arterial hypertension (PAH) is a devastating vascular complication of a number of CTDs. In patients with SSc, PAH has a dramatic impact on prognosis and survival and is the single most common cause of disease-related death.Yearly echocardiographic screening for PAH is recommended in patients with SSc. If suspected, confirmation of PAH diagnosis by right heart catheterization is necessary. Treatment goals for patients with PAH associated with SSc (PAH-SSc) aim to slow disease progression and improve quality of life. Some measures used to gauge the effect of treatment in patients with PAH-SSc remain to be fully validated; the 6-min walk distance, for example, is a simple and reproducible means of assessing exercise capacity, but there exists a need to understand what constitutes a clinically relevant change in this specific patient population. Currently, pharmacological intervention in PAH-SSc may target one or more of three pathophysiological pathways in PAH. The prostacyclin analogue epoprostenol has been shown to improve exercise capacity and haemodynamics in PAH-SSc patients and similar data are available from smaller studies on trepostinil and iloprost. The dual endothelin receptor antagonist bosentan has been shown to improve exercise capacity and haemodynamics in PAH-SSc, and similar data have been obtained in small numbers of patients treated with the endothelin receptor A antagonists sitaxsentan and ambrisentan. Impaired production of nitric oxide may be addressed by inhibiting phosphodiesterase type-5 with sildenafil or possibly tadalafil. Combinations of multiple targeted therapies may be beneficial to this patient population.
Highlights
Classification of pulmonary hypertensionPulmonary arterial hypertension (PAH), a disease of the pulmonary circulation, is defined as a mean pulmonary arterial pressure of >25 mmHg at rest or >30 mmHg during exercise together with a pulmonary capillary wedge pressure of
We look at the impact of PAH in CTD—focusing in particular on patients with SSc—and discuss how diagnostic and treatment strategies have evolved in recent years
The first placebo-controlled trial to examine the effects of specific PAH therapy in New York Heart Association functional class (NYHA FC) II mildly symptomatic PAH patient population, treatment with bosentan was associated with a significant reduction in pulmonary vascular resistance (PVR) and a trend towards an improvement in exercise capacity (6MWD) as well as a delay in time to clinical worsening, a secondary end point, (77% reduction in risk; P 1⁄4 0.0114) [35]
Summary
Pulmonary arterial hypertension (PAH), a disease of the pulmonary circulation, is defined as a mean pulmonary arterial pressure (mPAP) of >25 mmHg at rest or >30 mmHg during exercise together with a pulmonary capillary wedge pressure of
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