Figures
- Figure 1
Diagnostic algorithm. PH: pulmonary hypertension; group: clinical group; TTE: transthoracic echocardiography; PFT: pulmonary function test; HRCT: high-resolution computed tomography; V/Q: ventilation/perfusion; CTEPH: chronic thromboembolic pulmonary hypertension; PVOD: pulmonary veno-occlusive disease; PCH: pulmonary capillary haemangiomatosis; RHC: right heart catheterisation; PAH: pulmonary arterial hypertension; mPAP: mean pulmonary arterial pressure; PWP: pulmonary wedge pressure; ANA: anti-nuclear antibodies; TEE: trans-oesophageal echocardiography; CMR: cardiac magnetic resonance; US: ultrasonography; LFT: liver function test; CTD: connective tissue disease; CHD: congenital heart disease; BMPR2: bone morphogenetic protein receptor 2; ALK1: activin receptor like kinase; HHT: hereditary haemorrhagic telangiectasia. Reproduced from [8] with permission from the publisher.
- Figure 2
Treatment algorithm. IPAH: idiopathic pulmonary arterial hypertension; APAH: associated pulmonary arterial hypertension; WHO-FC: World Health Organization functional class; CCB: calcium channel blockers; FDA: US Food and Drug Administration; CHMP: Committee for Medicinal Products for Human Use; EMA: European Medicines Agency; s.c.: subcutaneous; ERA: endothelin receptor antagonist; PDE-5i: phosphodiesterase type 5 inhibitor; sGCS: soluble guanylate cyclase stimulators; BAS: balloon atrial septostomy. Reproduced from [14] with permission from the publisher.
Tables
- Table 1 Updated clinical classification of pulmonary hypertension#
1. PAH 1.1. Idiopathic PAH 1.2. Heritable PAH 1.2.1. BMPR2 1.2.2. ALK1, ENG, SMAD9, CAV1, KCNK3 1.2.3. Unknown 1.3. Drug and toxin induced 1.4. Associated with: 1.4.1. Connective tissue disease 1.4.2. HIV infection 1.4.3. Portal hypertension 1.4.4. Congenital heart diseases 1.4.5. Schistosomiasis 1′ Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis 1″ Persistent PH of the newborn 2. PH due to left heart disease 2.1. Left ventricular systolic dysfunction 2.2. Left ventricular diastolic dysfunction 2.3. Valvular disease 2.4 Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies 3. PH due to lung diseases and/or hypoxia 3.1. COPD 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 lung diseases 4. CTEPH 5. PH with unclear multifactorial mechanisms 5.1. Haematological disorders: chronic haemolytic anaemia, myeloproliferative disorders, splenectomy 5.2. Systemic disorders: sarcoidosis, pulmonary histiocytosis, lymphangioleiomyomatosis 5.3. Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders 5.4. Others: tumoural obstruction, fibrosing mediastinitis, chronic renal failure, segmental PH #: from the Fifth World Symposium on Pulmonary Hypertension (Nice, France, 2013). Reproduced from [7] with permission from the publisher.
- Table 2 World Health Organization functional assessment classification
Class I: Patients with PH but without resulting limitation of physical activity. Ordinary physical activity does not cause undue dyspnoea or fatigue, chest pain or near syncope. Class II: Patients with PH resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity causes undue dyspnoea or fatigue, chest pain or near syncope. Class III: Patients with PH resulting in marked limitation of physical activity. They are comfortable at rest. Less than ordinary activity causes undue dyspnoea or fatigue, chest pain or near syncope. Class IV: Patients with PH with inability to carry out any physical activity without symptoms. These patients manifest signs of right heart failure. Dyspnoea and/or fatigue may even be present at rest. Discomfort is increased by any physical activity. Reproduced and modified from [9] with permission from the publisher.
