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      Early detection and management of pulmonary arterial hypertension.

      European respiratory review : an official journal of the European Respiratory Society

      therapeutic use, Vasodilator Agents, Sulfones, Sulfonamides, complications, Scleroderma, Systemic, Risk Factors, Respiratory Function Tests, Purines, Practice Guidelines as Topic, Piperazines, analysis, Peptide Fragments, Natriuretic Peptide, Brain, Mass Screening, drug therapy, diagnosis, classification, Hypertension, Pulmonary, Humans, Exercise Test, Echocardiography, Doppler, Early Diagnosis, Antihypertensive Agents, Anemia, Sickle Cell

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          Abstract

          The long-term prognosis for patients with pulmonary arterial hypertension (PAH) remains poor, despite advances in treatment options that have been made in the past few decades. Recent evidence suggests that World Health Organization functional class I or II patients have significantly better long-term survival rates than patients in higher functional classes, thus providing a rationale for earlier diagnosis and treatment of PAH. However, early diagnosis is challenging and there is frequently a delay between symptom onset and diagnosis. Screening programmes play an important role in PAH detection and expert opinion favours echocardiographic screening of asymptomatic patients who may be predisposed to the development of PAH (i.e. those with systemic sclerosis or sickle cell disease), although current guidelines only recommend annual echocardiographic screening in symptomatic patients. This article reviews the currently available screening programmes, including their limitations, and describes alternative screening approaches that may identify more effectively those patients who require right heart catheterisation for a definitive PAH diagnosis.

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          Journal
          10.1183/09059180.00005112
          23204118

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