Table 7.
Clinicopathologic Differential Diagnosis of Pulmonary Arteriopathy in Children
| Pulmonary arterial hypertension |
| Idiopathic pulmonary arterial hypertension |
| Familial pulmonary arterial hypertension |
| BMPR2 gene mutation-associated, other |
| Associated with systemic disease |
| Congenital systemic to pulmonary shunts |
| Congenital heart disease with Eisenmenger's syndrome. Examples: atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus, other |
| Systemic arteriovenous shunt (vein of Galen malformation) |
| Portal (portopulmonary) hypertension |
| Collagen vascular disease |
| Human immunodeficiency virus infection |
| Drugs and toxins |
| Other systemic disease |
| Sickle cell disease/hemoglobinopathies |
| Metabolic storage diseases (glycogen storage disease, Gaucher's disease, other) |
| Hereditary hemorrhagic telangiectasia |
| Langerhans cell histiocytosis |
| Myeloproliferative disorders |
| Splenectomy |
| Associated with significant intrapulmonary venous or capillary involvement: |
| Alveolar capillary dysplasia with misalignment of pulmonary veins |
| Pulmonary veno-occlusive disease |
| Pulmonary capillary hemangiomatosis |
| Pulmonary venous hypertension |
| Pulmonary vein obstruction |
| Pulmonary vein stenosis |
| Anomalous pulmonary venous connection with obstructive physiology |
| Left atrial or ventricular disease heart disease |
| Cardiomyopathy |
| Other left ventricular failure |
| Left-sided valvular heart disease |
| Mitral stenosis |
| Aortic stenosis |
| Pulmonary arterial hypertension associated with hypoxemia |
| Persistent pulmonary hypertension of the newborn |
| Alveolar growth abnormalities (prematurity, hypoplasia, other) |
| Interstitial lung disease |
| Chronic obstructive pulmonary disease (cystic fibrosis, other) |
| Other |
| Sleep-disordered breathing |
| Alveolar hypoventilation disorders |
| Chronic exposure to high altitude |
| Pulmonary hypertension due to chronic thrombotic and/or embolic disease |
| Pulmonary arterial thromboembolism, proximal or distal |
| Other embolism |
| Examples: tumor, parasites, foreign material/intravenous drug abuse |
| Miscellaneous |
| Mediastinal/hilar pulmonary vascular compression, lymphangiomatosis, sarcoidosis |
Modified from 2003 revised World Health Organization classification of pulmonary hypertension, Proceedings of the 3rd World Symposium on Pulmonary Arterial Hypertension, Venice, Italy, June 23–25, 2003. J Am Coll Cardiol 2004; 43(Suppl. 12):1S–90S.