PDF icon Download PDF
Semin Respir Crit Care Med 2017; 38(05): 596-605
DOI: 10.1055/s-0037-1606202
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Screening of Pulmonary Arterial Hypertension

Edmund M. T. Lau
1   Department of Respiratory and Sleep Medicine, Royal Prince Alfred Hospital, Sydney Medical School, University of Sydney, Camperdown, Australia
,
David S. Celermajer
2   Department of Cardiology, Royal Prince Alfred Hospital, Sydney Medical School, University of Sydney, Camperdown, Australia
,
Anne Keogh
3   Heart Transplant Unit, St Vincent's Hospital, Darlinghurst, Australia
4   St. Vincent's Clinical School, University of New South Wales, Darlinghurst, Australia
,
Vivek Thakkar
5   Department of Rheumatology, Liverpool Hospital, Sydney, New South Wales, Australia
6   School of Medicine, Western Sydney University, Campbelltown, New South Wales, Australia
7   South Western Sydney Clinical School, University of New South Wales, Liverpool, Australia
8   Arthritis Research Unit of the Ingham Institute, Liverpool, Australia
› Author Affiliations
Further Information

Publication History

Publication Date:
15 October 2017 (online)

   Permissions and Reprints
Preview

Abstract

The majority of patients with pulmonary arterial hypertension (PAH) present in advanced stages of the disease, when therapeutic interventions may potentially be less effective. Early diagnosis is associated with improved outcomes, supporting best practice recommendations that asymptomatic patients in high-risk populations such as systemic sclerosis or carriers of a PAH-causing mutation should be offered periodic screening for PAH. An evidence-based algorithm for screening systemic sclerosis-associated PAH is now available based on the DETECT study. A multimodal approach using Doppler echocardiography, lung function testing, and B-type natriuretic peptides improves screening accuracy but at the expense of higher health care costs. Patients with abnormal hemodynamics but who do not meet definition for diagnosis of pulmonary hypertension (such as mean pulmonary artery pressure between 21 and 24 mm Hg) should be closely followed up given their increased risk of progression to PAH. This review summarizes the current approach to screening for PAH and discusses areas of uncertainties that require further research.

Keywords

pulmonary arterial hypertension - systemic sclerosis - screening - early detection