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Pulmonary hypertension encompasses a multifactorial group of disorders characterized by an elevated mean pulmonary artery pressure (mPAP) of >20 mmHg, measured at rest.
https://doi.org/10.1038/s41572-023-00486-7
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Introduction:
Central illustration on pulmonary hypertension BPA, balloon pulmonary angioplasty; CCB, calcium channel blocker; CTEPH, chronic thrombo-embolic pulmonary hypertension; CpCPH, combined post- and pre-capillary pulmonary hypertension; IpcPH, isolated post-capillary pulmonary hypertension; LHD, left heart disease; PAH, pulmonary arterial hypertension; PEA, pulmonary endarterectomy; PH, pulmonary hypertension. aTreatment of heart failure according to the ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure.27 Treatment of left-sided valvular heart disease according to the 2021 ESC/EACTS Guidelines for the management of valvular heart disease.28
Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. European Heart Journal. 2022;43(38):3618-3731. doi:https://doi.org/10.1093/eurheartj/ehac237
https://www.youtube.com/watch?v=Pfj_PXaiSaU
https://www.youtube.com/watch?v=I1Mu7qiS9R8
https://www.youtube.com/watch?v=FdmKV88eNQM
a Overview of pulmonary hypertension hemodynamic profiles. b Precapillary pulmonary hypertension. c Isolated post-capillary pulmonary hypertension. d Combined post- and precapillary pulmonary hypertension.
VC vena cava; RA right atrium; RV right ventricle; PA pulmonary arterial circulation; PC pulmonary capillary network; PV pulmonary venous circulation; LA left atrium; LV left ventricle; Ao aorta; PVR pulmonary vascular resistance; TPG transpulmonary gradient; DPG diastolic pulmonary gradient. De Marco T, Barnett CF, Fang JC, Horn EM, Tedford R. Pulmonary hypertension in left heart disease – systolic, diastolic, valvular. In: Kirklin JK, editor. ISHLT Monograph Series, Volume 9: Pulmonary Hypertension and Right Heart Failure. Birmingham: UAB Printing; 2015. p. 194.
Haemodynamic definitions of pulmonary hypertension: https://doi.org/10.1093/eurheartj/ehac237
| Definition | Haemodynamic characteristics |
|---|---|
| PH | mPAP > 20 mmHg |
| Pre-capillary PH | mPAP > 20 mmHgPAWP ≤ 15 mmHgPVR > 2 WU |
| IpcPH | mPAP > 20 mmHgPAWP > 15 mmHgPVR ≤ 2 WU |
| CpcPH | mPAP > 20 mmHgPAWP > 15 mmHgPVR > 2 WU |
| Exercise PH | mPAP/CO slope between rest and exercise > 3 mmHg/L/min |
CO, cardiac output; CpcPH, combined post- and pre-capillary pulmonary hypertension; IpcPH, isolated post-capillary pulmonary hypertension; mPAP, mean pulmonary arterial pressure; PAWP, pulmonary arterial wedge pressure; PH, pulmonary hypertension; PVR, pulmonary vascular resistance; WU, Wood units.
Etiological classification:
| Group | Description |
|---|---|
| 1. Pulmonary arterial hypertension | This category is broken down further into several subcategories. These categories include: idiopathic, heritable and drug/toxin induced. There is also a category for pulmonary arterial hypertension secondary to certain diseases, including congenital heart disease, connective tissue diseases, HIV infection, portal hypertension and schistosomiasis. |
| Group 1′ includes pulmonary veno-occlusive disease (PVOD) and/or pulmonary capillary haemangiomatosis. | |
| Group 1″ is persistent pulmonary hypertension of the newborn. |
https://www.youtube.com/watch?v=fvMEoc7Voa0 https://www.youtube.com/watch?v=XWtWJO0wUs8 | | 2. Pulmonary hypertension secondary to left heart disease | This category may be caused due to left-heart disease of the valves, systolic dysfunction or diastolic dysfunction. The Nice classification added congenital/acquired left heart inflow/outflow tract obstruction along with congenital cardiomyopathies to this category.
https://doi.org/10.3390/jcm13020625 | | 3. Pulmonary hypertension with lung disease/hypoxaemia | This category may be caused by COPD, interstitial lung diseases, pulmonary diseases with a mixed restrictive and obstructive pattern, sleep-disordered breathing, alveolar hypoventilation disorders, chronic exposure to high altitude and developmental lung diseases. | | 4. Chronic thromboembolic pulmonary hypertension | This category arises in patients who have had chronic thromboembolic disease resulting in reorganization of the pulmonary vasculature. | | 5. Pulmonary hypertension with unclear multifactorial mechanism | This is a broad category that includes pulmonary hypertension secondary to hematological disorders (including chronic hemolytic anemia), systemic disorders (like sarcoidosis and lymphangioleiomyomatosis), metabolic disorders (like glycogen storage diseases or thyroid disorders) and other causes (including chronic renal failure and segmental pulmonary hypertension). |