Recurrent chest pain and dyspnoea in a patient with pulmonary arterial hypertension. Issue 4 (4th July 2019)

• Record Type: Journal Article
• Authors: Van Tittelboom, Nathalie; Devos, Dan; Thierry, Bové; De Pauw, Michel; Vandecasteele, Els
• Is Part Of: Acta clinica belgica. Volume 74:Issue 4(2019)
• Page Start: 292
• Page End: 295
• Publication Date: 2019-07-04
• Subjects: Pulmonary arterial hypertension -- Transplantation -- Aneurysmectomy -- Left main coronary artery compression -- pulmonary artery aneurysm; Clinical medicine -- Periodicals; Diagnosis, Laboratory -- Periodicals; 616.005
• DOI: 10.1080/17843286.2018.1516180
• Languages: English
• ISSNs: 1784-3286

Abstract

ABSTRACT: Background : Pulmonary arterial hypertension (PAH) is a devastating, life-threatening disease with poor prognosis when left untreated. The long-term prognosis is definitely influenced by the natural progression of PAH but late disease-specific complications may also contribute. Case summary : We present a patient with a long-standing idiopathic PAH in whom progressive dilatation of pulmonary trunk and pulmonary arteries leads to compression of the left main coronary artery and the left atrium with hemodynamic compromise. Conclusion : With the current treatment options, survival in PAH has improved. Guidelines focus on more aggressive treatment with initial combination therapy and earlier referral for transplantation.