Drug-induced pulmonary arterial hypertension: a recent outbreak

Table 1. Updated clinical classification of pulmonary hypertension

1. Pulmonary arterial hypertension

1.1 Idiopathic pulmonary arterial hypertension

1.2 Heritable

1.2.1. BMPR2

1.2.2. ALK1, endoglin (with or without hereditary haemorrhagic telangiectasia)

1.2.3 Unknown

1.3 Drug- and toxin-induced

1.4 Associated with:

1.4.1 Connective tissue diseases

1.4.2 HIV infection

1.4.3 Portal hypertension

1.4.4 Congenital heart diseases

1.4.5 Schistosomiasis

1.4.6 Chronic haemolytic anaemia

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 Systolic dysfunction

2.2 Diastolic dysfunction

2.3 Valvular disease

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

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 Haematological 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: tumoural obstruction, fibrosing mediastinitis, chronic renal failure on dialysis

BMPR2: bone morphogenetic protein receptor, type II; ALK1: activin receptor-like kinase 1 gene. Reproduced from [2] with permission from the publisher.

Table 2. Risk factors for and associated conditions of pulmonary arterial hypertension

Definite	Possible
Aminorex	Cocaine
Fenfluramine	Phenylpropanolamine
Dexfenfluramine	St John's Wort
Toxic rapeseed oil	Chemotherapeutic agents
Benfluorex	Selective serotonin reuptake inhibitors
Dasatinib	Pergolide
Likely	Unlikely
Amphetamines	Oral contraceptives
L-Tryptophan	Oestrogen
Methamphetamines	Cigarette smoking