Articles
Nomenclature and Classification of Pulmonary Arterial Hypertension
Nomenclature and Classification
of Pulmonary Arterial Hypertension
| 1 | Primary Arterial Hypertension | ||||||
| 1.1 | Primary Pulmonary Hypertension | ||||||
| (a) | Sporadic | ||||||
| (b) | Familial | ||||||
| 1.2 | Related to: | ||||||
| (a) | Collagen Vascular Disease | ||||||
| (b) | Congenital Systemic to Pulmonary Shunts | ||||||
| (c) | Portal Hypertension | ||||||
| (d) | HIV Infection | ||||||
| (e) | Drugs/Toxins | ||||||
| (1) | Anorexigens | ||||||
| (2) | Other | ||||||
| (f) | Persistent Pulmonary Arterial Hypertension of the Newborn | ||||||
| (g) | Other | ||||||
| 2 | Pulmonary Venous Hypertension | ||||||
| 2.1 | Left-Sided Atrial or Ventricular Heart Disease | ||||||
| 2.2 | Left-Sided Valvular Heart Disease | ||||||
| 2.3 | Extrinsic Compression of Central Pulmonary Veins | ||||||
| (a) | Fibrosing Mediastinitis | ||||||
| (b) | Adenopathy/Tumors | ||||||
| 2.4 | Pulmonary Veno-Occlusive Disease | ||||||
| 2.5 | Other | ||||||
| 3 | Pulmonary Arterial Hypertension Associated with Disorders of the Respiratory System and/or Hypoxemia | ||||||
| 3.1 | Chronic Obstructive Pulmonary Disease | ||||||
| 3.2 | Interstitial Lung Disease | ||||||
| 3.3 | Sleep Disordered Breathing | ||||||
| 3.4 | Alveolar Hypoventilation Disorders | ||||||
| 3.5 | Chronic Exposure to High Altitude | ||||||
| 3.6 | Neonatal Lung Disease | ||||||
| 3.7 | Alveolar-Capillary Dysplasia | ||||||
| 3.8 | Other | ||||||
| 4 | Pulmonary Arterial Hypertension due to Chronic Thrombotic and/or Embolic Disease | ||||||
| 4.1 | Thromboembolic Obstruction of Proximal Pulmonary Arteries | ||||||
| 4.2 | Obstruction of Distal Pulmonary Arteries | ||||||
| (a) | Pulmonary embolism (Thrombus, Tumor, OVA and/or parasites, Foreign Material) | ||||||
| (b) | In-situ Thrombosis | ||||||
| (c) | Sickle Cell Disease | ||||||
| 5 | Pulmonary Arterial Hypertension due to Disorders Directly Affecting the Pulmonary Vasculature | ||||||
| 5.1 | Inflammatory | ||||||
| (a) | Schistosomiasis | ||||||
| (b) | Sarcoidosis | ||||||
| (c) | Other | ||||||
| 5.2 | Pulmonary Capillary Hemangiomatosis | ||||||
.jpg)