Multiple progresses have been achieved in pulmonary vascular diseases in the last decades, including the areas of pulmonary hypertension and pulmonary thromboembolic disease. The increase in knowledge has been accomplished in pathophysiological, clinical and treatment domains and has included as examples the discovery of gene mutations related to the hereditary forms of pulmonary arterial hypertension and the proposals of personalized treatment algorithms in patients with acute pulmonary embolism, chronic thromboembolic pulmonary hypertension and pulmonary arterial hypertension, validated in this specific area by more than 45 randomized controlled trials. The diagnostic processes have been refined, increasing the awareness that appropriate and precise diagnosis is essential for the optimal treatment strategy. The drugs approved for pulmonary arterial hypertension are recommended in this group and in specific patients with chronic thromboembolic pulmonary hypertension but are contraindicated in patients with pulmonary hypertension due to left heart and lung diseases. In pulmonary vascular diseases, the therapy cannot be considered as a simple prescription of medications and interventions but is a complex strategy which includes baseline patients' risk stratification, initial therapy, long-term follow-up and treatment adjustments when required. Today, computed tomography pulmonary artery angiography is the gold standard for diagnosis in both acute pulmonary embolism and chronic thromboembolic pulmonary hypertension. In this last condition, the combination with data derived from the right heart catheterization and the traditional pulmonary artery angiography, allows to a team of experts to decide if the patient is a candidate to surgical pulmonary endarterectomy or to percutaneous pulmonary artery balloon angioplasty which may improve symptoms, quality of life and prognosis.