Updated clinical classification of pulmonary hypertension

Abstract

In 1998, a clinical classification of pulmonary hypertension (PH) was established, categorizing PH into groups which share similar pathological and hemodynamic characteristics and therapeutic approaches. During the 5th World Symposium held in Nice, France, in 2013, the consensus was reached to maintain the general scheme of previous clinical classifications. However, modifications and updates especially for Group 1 patients (pulmonary arterial hypertension [PAH]) were proposed. The main change was to withdraw persistent pulmonary hypertension of the newborn (PPHN) from Group 1 because this entity carries more differences than similarities with other PAH subgroups. In the current classification, PPHN is now designated number 1. Pulmonary hypertension associated with chronic hemolytic anemia has been moved from Group 1 PAH to Group 5, unclear/multifactorial mechanism. In addition, it was decided to add specific items related to pediatric pulmonary hypertension in order to create a comprehensive, common classification for both adults and children. Therefore, congenital or acquired left-heart inflow/outflow obstructive lesions and congenital cardiomyopathies have been added to Group 2, and segmental pulmonary hypertension has been added to Group 5. Last, there were no changes for Groups 2, 3, and 4.

Keywords: CHD; HAART; HIV; IFN; PAH; PAP; PH; PH due to chronic lung diseases; PH due to left heart disease; POPH; PPHN; PVR; SCD; Sch-PAH; TGF; TKI; chronic thromboembolic pulmonary hypertension; congenital heart disease; highly active antiretroviral therapy; human immunodeficiency virus; interferon; persistent pulmonary hypertension of the newborn; portopulmonary hypertension; pulmonary arterial hypertension; pulmonary arterial pressure; pulmonary hypertension; pulmonary vascular resistance; schistosomiasis-associated PAH; sickle cell disease; tumor growth factor; tyrosine kinase inhibitor.