Figures
- FIGURE 1
The slope of the mean pulmonary arterial pressure (mPAP)–cardiac output (CO) relationship is different in normal control versus pulmonary arterial hypertension (PAH) subjects. Reproduced and modified from [26] with permission.
- FIGURE 2
Knowledge of the mean pulmonary arterial pressure (mPAP)–cardiac output (CO) relationship does not allow distinction between left heart disease (LHD) and pulmonary vascular disease (PVD) patients; knowledge of exercise pulmonary arterial wedge pressure is also required. TPR: total pulmonary resistance; WU: Wood Units. Reproduced from [29] with permission.
Tables
- TABLE 1
Haemodynamic definitions of pulmonary hypertension (PH)
Definitions Characteristics Clinical groups# Pre-capillary PH mPAP >20 mmHg 1, 3, 4 and 5 PAWP ≤15 mmHg PVR ≥3 WU Isolated post-capillary PH (IpcPH) mPAP >20 mmHg 2 and 5 PAWP >15 mmHg PVR <3 WU Combined pre- and post-capillary PH (CpcPH) mPAP >20 mmHg 2 and 5 PAWP >15 mmHg PVR ≥3 WU mPAP: mean pulmonary arterial pressure; PAWP: pulmonary arterial wedge pressure; PVR: pulmonary vascular resistance; WU: Wood Units. #: group 1: PAH; group 2: PH due to left heart disease; group 3: PH due to lung diseases and/or hypoxia; group 4: PH due to pulmonary artery obstructions; group 5: PH with unclear and/or multifactorial mechanisms.
- TABLE 2 Updated clinical classification of pulmonary hypertension (PH)
1 PAH 1.1 Idiopathic PAH 1.2 Heritable PAH 1.3 Drug- and toxin-induced PAH (table 3) 1.4 PAH associated with: 1.4.1 Connective tissue disease 1.4.2 HIV infection 1.4.3 Portal hypertension 1.4.4 Congenital heart disease 1.4.5 Schistosomiasis 1.5 PAH long-term responders to calcium channel blockers (table 4) 1.6 PAH with overt features of venous/capillaries (PVOD/PCH) involvement (table 5) 1.7 Persistent PH of the newborn syndrome 2 PH due to left heart disease 2.1 PH due to heart failure with preserved LVEF 2.2 PH due to heart failure with reduced LVEF 2.3 Valvular heart disease 2.4 Congenital/acquired cardiovascular conditions leading to post-capillary PH 3 PH due to lung diseases and/or hypoxia 3.1 Obstructive lung disease 3.2 Restrictive lung disease 3.3 Other lung disease with mixed restrictive/obstructive pattern 3.4 Hypoxia without lung disease 3.5 Developmental lung disorders 4 PH due to pulmonary artery obstructions (table 6) 4.1 Chronic thromboembolic PH 4.2 Other pulmonary artery obstructions 5 PH with unclear and/or multifactorial mechanisms (table 7) 5.1 Haematological disorders 5.2 Systemic and metabolic disorders 5.3 Others 5.4 Complex congenital heart disease PAH: pulmonary arterial hypertension; PVOD: pulmonary veno-occlusive disease; PCH: pulmonary capillary haemangiomatosis; LVEF: left ventricular ejection fraction.
- TABLE 3
Updated classification of drugs and toxins associated with PAH
Definite Possible Aminorex Cocaine Fenfluramine Phenylpropanolamine Dexfenfluramine l-tryptophan Benfluorex St John's wort Methamphetamines Amphetamines Dasatinib Interferon-α and -β Toxic rapeseed oil Alkylating agents Bosutinib Direct-acting antiviral agents against hepatitis C virus Leflunomide Indirubin (Chinese herb Qing-Dai) - TABLE 4
Definitions of acute and long-term response
Acute pulmonary vasoreactivity# for patients with idiopathic, hereditable or drug-induced PAH Reduction of mPAP ≥10 mmHg to reach an absolute value of mPAP ≤40 mmHg Increased or unchanged cardiac output Long-term response to CCBs New York Heart Association Functional Class I/II With sustained haemodynamic improvement (same or better than achieved in the acute test) after at least 1 year on CCBs only PAH: pulmonary arterial hypertension; mPAP: mean pulmonary arterial pressure; CCB: calcium channel blocker. #: nitric oxide (10–20 ppm) is recommended for performing vasoreactivity testing, but i.v. epoprostenol, i.v. adenosine or inhaled iloprost can be used as alternatives.
- TABLE 5
Signs evocative of venous and capillary (pulmonary veno-occlusive disease/pulmonary capillary haemangiomatosis) involvement
Pulmonary function tests Decreased DLCO (frequently <50%) Severe hypoxaemia Chest HRCT Septal lines Centrilobular ground-glass opacities/nodules Mediastinal lymph node enlargement Response to PAH therapy Possible pulmonary oedema Genetic background Biallelic EIF2AK4 mutations Occupational exposure Organic solvent (trichloroethylene) DLCO: diffusing capacity of the lung for carbon monoxide; HRCT: high-resolution computed tomography; PAH: pulmonary arterial hypertension.
- TABLE 6
Pulmonary hypertension (PH) due to pulmonary artery obstructions
4.1 Chronic thromboembolic PH 4.2 Other pulmonary artery obstructions 4.2.1 Sarcoma (high or intermediate grade) or angiosarcoma 4.2.2 Other malignant tumours
Renal carcinoma
Uterine carcinoma
Germ cell tumours of the testis
Other tumours4.2.3 Non-malignant tumours
Uterine leiomyoma4.2.4 Arteritis without connective tissue disease 4.2.5 Congenital pulmonary artery stenoses 4.2.6 Parasites Hydatidosis - TABLE 7
Pulmonary hypertension with unclear and/or multifactorial mechanisms
5.1 Haematological disorders Chronic haemolytic anaemia Myeloproliferative disorders 5.2 Systemic and metabolic disorders Pulmonary Langerhans cell histiocytosis Gaucher disease Glycogen storage disease Neurofibromatosis Sarcoidosis 5.3 Others Chronic renal failure with or without haemodialysis Fibrosing mediastinitis 5.4 Complex congenital heart disease See the Task Force article by Rosenzweig et al. [31] in this issue of the European Respiratory Journal
