1. Updated Clinical Classification of Pulmonary Hypertension
- Author
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Michael A. Gatzoulis, Rogiero Souza, Ardeschir Ghofrani, Miguel Angel Gomez Sanchez, Gérald Simonneau, Horst Olschewski, Ian Adatia, Michael J. Landzberg, Roberto F. Machado, R. Krishna Kumar, Ivan M. Robbins, Christopher P. Denton, and David S. Celermajer
- Subjects
Heart Defects, Congenital ,Anemia, Hemolytic ,medicine.medical_specialty ,Hypertension, Pulmonary ,Riociguat ,chronic thromboembolic pulmonary hypertension ,Internal medicine ,pulmonary arterial hypertension ,pulmonary hypertension ,medicine ,Animals ,Humans ,Cambridge Pulmonary Hypertension Outcome Review ,Connective Tissue Diseases ,Associated Pulmonary Arterial Hypertension ,Portopulmonary hypertension ,business.industry ,PH due to chronic lung diseases ,PH due to left heart disease ,medicine.disease ,Pulmonary hypertension ,Surgery ,Pulmonary venoocclusive disease ,Cardiology ,Pulmonary venous hypertension ,Pulmonary Veno-Occlusive Disease ,Cardiology and Cardiovascular Medicine ,business ,medicine.drug - 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 (YPPH) 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. (J Am Coll Cardiol 2013;62:D34-41) a 2013 by the American College of Cardiology Foundation.
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