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Table 1 Updated clinical classification of pulmonary hypertension

From: In search of markers of treatment failure and poor prognosis in IPAH - the value of mosaic lung attenuation pattern on thin-section CT scansAlla ricerca di un marker di resistenza al trattamento e prognosi infausta nell’ipertensione polmonare idiopatica (IPAH) – il valore del pattern di attenuazione a mosaico nella TAC torace a sezioni sottili

1. Pulmonary arterial hypertension (PAH)

   1.1 Idiopathic

   1.2 Heritable

1.2.1 BMPR2

1.2.2 ALK1, endoglin (with or without hereditary hemorrhagic teleangiectasia)

1.2.3 Unknown

   1.3 Drugs and toxins induced

   1.4 Associated with (APAH):

1.4.1 Connective tissue diseases

1.4.2 HIV infection

1.4.3 Portal hypertension

1.4.4 Congenital heart disease

1.4.5 Schistosomiasis

1.4.6 Chronic haemolytic anemia

   1.5 Persistent pulmonary hypertension of the newborn

1' Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis

2. Pulmonary hypertension due to left heart disease

   2.1 Sysytolic dysfunction

   2.2 Diastolic dysfunction

   2.3 Valvular disease

3. Pulmonary hypertension due to lung diseases and/or hypoxemia

   3.1 Chronic obstructive pulmonary disease

   3.2 Interstitial lung disease

   3.3 Other pulmonary diseases with mixed restrictive and obstructive pattern

   3.4 Sleep-disordered breathing

   3.5 Alveolar hypoventilation disorders

   3.6 Chronic exposure to high altitude

   3.7 Developmental abnormalities

4. Chronic thromboembolic pulmonary hypertension

5. Pulmonary hypertension with unclear and/or multifactorial mechanisms

   5.1 Hematological disorders: myeloproliferative disorders, splenectomy

   5.2 Systemic disorders: sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, neurofibromatosis, vasculitis

   5.3 Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders

   5.4 Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure on dialysis

  1. From [1].