“Clinical phenotypes and outcomes of precapillary pulmonary hypertension of sickle cell disease.” Laurent Savale, Anoosha Habibi, François Lionnet, Bernard Maitre, Vincent Cottin, Xavier Jais, Ari Chaouat, Elise Artaud-Macari, Matthieu Canuet, Grégoire Prevot, Christelle Chantalat-Auger, David Montani, Olivier Sitbon, Fréderic Galacteros, Gérald Simonneau, Florence Parent, Pablo Bartolucci and Marc Humbert. Eur Respir J 2019; 54: 1900585.

Ventilation/perfusion (V/Q) lung scintigraphy and digital subtraction pulmonary angiography (DSA) according to the disease genotype. a) The proportion of patients with normal perfusion, heterogeneous perfusion and segmental perfusion defects on V/Q lung scintigraphy according to the genotype of sickle cell disease. b) A case of homozygous for haemoglobin S (SS) sickle cell disease with heterogeneous perfusion on V/Q lung scintigraphy and diffuse subpleural hypoperfusion on DSA. c) An example of an SC sickle cell disease patient with segmental perfusion defects on V/Q lung scintigraphy and distal chronic thromboembolic pulmonary hypertension on DSA. RAO: right anterior oblique; RPO: right posterior oblique; LAO: left anterior oblique; LPO: left posterior oblique.