Aleksander Kempny1,2, MD; Stephen J. Wort1,2, MD, BA, MBBS, FRCP, PhD; Konstantinos Dimopoulos1,2*, MD, MSc, PhD, FESC
1. Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, United Kingdom; 2. National Heart and Lung Institute, Imperial College School of Medicine, London, United Kingdom
Chronic thromboembolic pulmonary hypertension (CTEPH) is an insidious disease and is associated with poor survival with medical therapy only1. The cumulative incidence of CTEPH following pulmonary embolism (PE) is 1% after six months, 3.1% after 12 months and 3.8% after two years after PE in anticoagulated patients2. While the extent of perfusion abnormalities following PE is a major determinant for the development of CTEPH, numerous studies have demonstrated that even large perfusion defects that do not resolve over time do not cause pulmonary hypertension, either at rest or on effort, in the majority of patients3. Only a subset of patients with large perfusion ...