title: Early detection and management of pulmonary arterial hypertension.
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.
authors.affiliation: Université Paris-Sud, AP-HP, Service de Pneumologie, Hôpital Bicêtre, Inserm U999, Le Kremlin Bicêtre, France.
authors.forename: Marc
authors.initials: M
authors.lastname: Humbert
authors.forename: J
authors.initials: J
authors.lastname: Gerry Coghlan
authors.forename: Dinesh
authors.initials: D
authors.lastname: Khanna
mesh_terms: D000755:Anemia, Sickle Cell / Q000150:complications; D000959:Antihypertensive Agents / Q000627:therapeutic use; D000077300:Bosentan; D042241:Early Diagnosis; D015150:Echocardiography, Doppler; D005080:Exercise Test; D006801:Humans; D006976:Hypertension, Pulmonary / Q000145:classification / Q000175:diagnosis* / Q000188:drug therapy*; D008403:Mass Screening; D020097:Natriuretic Peptide, Brain / Q000032:analysis; D010446:Peptide Fragments / Q000032:analysis; D010879:Piperazines / Q000627:therapeutic use; D017410:Practice Guidelines as Topic; D011687:Purines / Q000627:therapeutic use; D012129:Respiratory Function Tests; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000150:complications; D000068677:Sildenafil Citrate; D013449:Sulfonamides / Q000627:therapeutic use; D013450:Sulfones / Q000627:therapeutic use; D014665:Vasodilator Agents / Q000627:therapeutic use
chemical_list: D000959:Antihypertensive Agents; D010446:Peptide Fragments; D010879:Piperazines; D011687:Purines; D013449:Sulfonamides; D013450:Sulfones; D014665:Vasodilator Agents; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain; D000068677:Sildenafil Citrate; D000077300:Bosentan