While there are still gaps in understanding pulmonary vasculature, tremendous progress has been made in understanding its functionality, its adaptation to hypoxia, the effects of increased pulmonary vascular resistance on the right ventricular function, and the molecular pathways affected in this process. As a result, there are currently seven therapies for pulmonary arterial hypertension and the field is rapidly moving forward, with several novel molecular targets under development. Therefore, a textbook addressing developmental, cellular and clinical findings about pulmonary vascular disorders is relevant and much needed