biomedicines Review Endothelial Dysfunction in Pulmonary Hypertension: Cause or Consequence? Kondababu Kurakula 1,† , Valérie F. E. D. Smolders 2,† , Olga Tura-Ceide 3,4,5, J. Wouter Jukema 6 , Paul H. A. Quax 2 and Marie-José Goumans 1,* 1 Department of Cell and Chemical Biology, Laboratory for CardioVascular Cell Biology, Leiden University Medical Center, 2300 RC Leiden, The Netherlands;
[email protected] 2 Department of Surgery, Einthoven Laboratory for Experimental Vascular Medicine, Leiden University Medical Center, 2300 RC Leiden, The Netherlands;
[email protected] (V.F.E.D.S.);
[email protected] (P.H.A.Q.) 3 Department of Pulmonary Medicine, Hospital Clínic-Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, 08036 Barcelona, Spain;
[email protected] 4 Department of Pulmonary Medicine, Dr. Josep Trueta University Hospital de Girona, Santa Caterina Hospital de Salt and the Girona Biomedical Research Institut (IDIBGI), 17190 Girona, Catalonia, Spain 5 Biomedical Research Networking Centre on Respiratory Diseases (CIBERES), 28029 Madrid, Spain 6 Department of Cardiology, Leiden University Medical Center, 2300 RC Leiden, The Netherlands;
[email protected] * Correspondence:
[email protected] † The authors contributed equally. Abstract: Pulmonary arterial hypertension (PAH) is a rare, complex, and progressive disease that is characterized by the abnormal remodeling of the pulmonary arteries that leads to right ventricular failure and death. Although our understanding of the causes for abnormal vascular remodeling in PAH is limited, accumulating evidence indicates that endothelial cell (EC) dysfunction is one of the first triggers initiating this process.