While pulmonary hypertension is readily diagnosed at cardiac catheterisation, delayed recognition and targeting treatment to the underlying cause can be challenging. Modern classification distinguishes between pulmonary hypertension secondary to cardiac disease, hypoxia with or without lung disease, thromboembolic pulmonary vascular disease, pulmonary hypertension associated with systemic diseases, and idiopathic forms. The physiological consequences and the use of various imaging modalities in refining diagnosis are discussed. For patients with idiopathic pulmonary hypertension, novel pharmacological approaches, either singly or in combination, have been more rigorously evaluated, and the use of surgery for chronic thromboembolic pulmonary hypertension is now well established.
KeywordsDefinition and classification Diagnostic strategies Investigation Imaging Cardiac catheterisation Pregnancy Anaesthesia Sepsis Pulmonary arterial hypertension Left heart disease Chronic lung disease Chronic thromboembolic disease Pulmonary endarterectomy Targeted treatments
- 1.Hatano S, Strasser T. Primary pulmonary hypertension. In: Report on a WHO meeting, Geneva, 15–17 October 1973. Geneva: World Health Organization; 1975.Google Scholar
- 5.Ling Y, Johnson MK, Kiely DG, Condliffe R, Elliot CA, Gibbs JS, et al. Changing demographics, epidemiology, and survival of incident pulmonary arterial hypertension: results from the pulmonary hypertension registry of the United Kingdom and Ireland. Am J Respir Crit Care Med. 2012;186(8):790–6.CrossRefGoogle Scholar
- 7.Swift AJ, Rajaram S, Condliffe R, Capener D, Hurdman J, Elliot CA, et al. Diagnostic accuracy of cardiovascular magnetic resonance imaging of right ventricular morphology and function in the assessment of suspected pulmonary hypertension results from the ASPIRE registry. J Cardiovasc Magn Reson. 2012;14:40.CrossRefPubMedPubMedCentralGoogle Scholar
- 25.Galie N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, et al. ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J. 2015;46(4):903–75.CrossRefPubMedGoogle Scholar