How to evaluate patients with congenital heart disease-related pulmonary arterial hypertension.
Expert Rev Cardiovasc Ther. 2018 Nov 20;:
Authors: Ntiloudi D, Zanos S, Gatzoulis MA, Karvounis H, Giannakoulas G
INTRODUCTION: Patients with congenital heart disease (CHD), who develop pulmonary arterial hypertension (PAH), live longer and have better quality of life compared to the past due to PAH specific therapy and improved tertiary care. Areas covered: Clinical examination, objective assessment of functional capacity, natriuretic peptide levels, cardiac imaging and haemodynamics all play a pivotal role in the evaluation, general care and management of PAH specific therapy. This review discusses the epidemiology and pathophysiology of PAH-CHD, and provides hints for the optimal evaluation of these patients. Expert commentary: Further research should be performed in the field of PAH-CHD, as there are many of areas lacking evidence that should be addressed in the future. Networking, especially among the tertiary expert centres, could play a key role in this direction.
PMID: 30457398 [PubMed – as supplied by publisher]