Pulmonary hypertension (differential)

Pulmonary hypertension (differential)

Pulmonary hypertension has many causes, and these can be divided in many ways. A simple and systematic approach is to proceed along the cardiopulmonary pulmonary circulation, as causes are found at each site (for a more official classification system see 2003 third world symposium on pulmonary arterial hypertension classification).

For further discussion of this condition, see pulmonary arterial hypertension.

Causes

• right heart
  • Eisenmenger phenomenon: chronic left to right shunts
    • atrial septal defect (ASD)
    • ventricular septal defect (VSD)
    • patent ductus arteriosus (PDA)
• pulmonary arteries
  • pulmonary artery stenosis
    • hypertension will be confined to the right heart and artery proximal to the stenosis
  • chronic/repeated pulmonary emboli including
    • fat embolism
    • tumor emboli, e.g. trophoblastic disease
  • arteritis
    • polyarteritis nodosa (PAN)
    • systemic lupus erythematosus (SLE)
    • Takayasu arteritis
    • granulomatosis with polyangiitis
• pulmonary capillaries and pulmonary parenchyma
  • asthma
  • bronchiectasis, including
    • cystic fibrosis (CF)
  • chronic bronchitis
  • emphysema including
    • alpha-1-antitrypsin deficiency
  • lymphangiomyomatosis (LAM)
  • Langerhans cell histiocytosis (LCH/histiocytosis X)
  • pulmonary fibrosis: any cause, including
    • connective tissue disorders
      • systemic sclerosis
      • dermatomyositis
      • rheumatoid lung
      • systemic lupus erythematosus (SLE)
    • idiopathic pulmonary fibrosis
    • pneumoconiosis
    • sarcoidosis
• pulmonary veins
  • pulmonary veno occlusive disease (PVOD)
  • venolobar syndrome
  • pulmonary vein atresias
    • unilateral pulmonary vein atresia
  • pulmonary vein stenosis
  • partial anomalous pulmonary venous return 
• left heart
  • chronic left heart failure
  • mitral valve stenosis
  • hypoplastic left heart syndrome
• chest wall and upper airway resulting in chronic hypoxia
  • kyphoscoliosis
  • obesity without or with hypoventilation syndrome, e.g. Pickwickian syndrome
  • fibrothorax
• others
  • chronic renal failure
  • pulmonary tumoral thrombotic microangiopathy
  • fibrosing mediastinitis
  • hematological disorders
    • chronic hemolytic anemia
    • myeloproliferative disorders
    • splenectomy
  • metabolic disorders
    • glycogen storage disease
    • Gaucher disease

Siehe auch:

• Polyarteriitis nodosa
• Bronchiektasen
• Lungenarterienembolie
• Persistierender Ductus arteriosus
• Atriumseptumdefekt
• Lungenfibrose
• Lymphangioleiomyomatose
• Granulomatose mit Polyangiitis
• Dermatomyositis
• systemischer Lupus Erythematodes
• zystische Fibrose
• Pneumonokoniose
• Takayasu-Arteriitis
• Fibrothorax
• Alpha-1-Antitrypsin-Mangel
• pulmonale Hypertonie
• Histiozytose X
• hypoplastic left heart syndrome
• pulmonale venookklusive Erkrankung
• idiopathische Lungenfibrose
• Pulmonary hypertension (2003 classification)
• rheumatoid lung
• Eisenmenger-Reaktion
• Ventrikelseptumdefekt
• Emphysem
• trophoblastic disease
• venolobar syndrome

und weiter:

• secondary pulmonary arterial hypertension