Pulmonary Hypertension: Understanding the Underlying Anatomy and Physiology of Pulmonary Circulation
DOI:
https://doi.org/10.36497/jri.v45i2.779Keywords:
hypoxic pulmonary vasoconstriction, pulmonary circulation, pulmonary hypertension, pulmonary vascular resistanceAbstract
The circulatory system has a vital role in fulfilling oxygen demand in tissues and maintaining homeostasis. There are two types of circulatory systems in our body, the systemic and pulmonary circulation. Pulmonary circulation, the critical pathway of blood oxygenation through heart-lung-pulmonary vascular interaction, remains poorly understood despite its central role in the various pulmonary vascular diseases. One of the most prevalent pulmonary vascular diseases is pulmonary hypertension (PH), which is characterized by high mortality and disease progression. A thorough understanding of the physiologic structure and function of pulmonary circulation is essential for diagnosing and treating patients with PH. Pulmonary blood flow is determined by pulmonary vascular pressure (PVP) and pulmonary vascular resistance (PVR). Lung volume, neural factors (sympathetic and parasympathetic nervous systems), humoral factors (vasoconstrictor and vasodilator), and alveolar hypoxia play an important role in regulating PVR. Alveolar hypoxia triggers hypoxic pulmonary vasoconstriction to maintain the ventilation-perfusion ratio balance. High PVR increases pulmonary arterial pressure (Pa), which is the main pathophysiology of PH. In addition, endothelial dysfunction and vasoactive imbalance also contribute significantly to the pathogenesis of the disease.
Downloads
References
1. Haji G, Joseph T, Davies R. Pulmonary vascular disease: pulmonary thromboembolism and pulmonary hypertension. Medicine (Baltimore). 2023;51(12):864–9.
2. Rasmin M, Putra AC, Septiyanti D, Alatas MF. Hipertensi pulmoner. Jakarta: Perhimpunan Dokter Paru Indonesia; 2018.
3. Dalley AF, Agur AM. Moore’s clinically oriented anatomy. 9th ed. Philadelphia: Wolters Kluwer; 2023.
4. Blood flow to the lung. In: Levitzky MG, editors. Pulmonary physiology. 10th ed. New York: McGraw-Hill; 2022. p. 96–127.
5. Ormiston ML. Pathobiology of pulmonary hypertension. In: Peacock AJ, Naejie R, Rubin LJ Pulmonary circulation diseases and their treatment. 4th ed. Boca Raton: CRC Press; 2016. p. 79–95.
6. Rathmann J, Segelmark M, Englund M, Mohammad AJ. Stable incidence but increase in prevalence of ANCA-associated vasculitis in southern Sweden: a 23-year study. RMD Open. 2023;9(1):e002949.
7. Rana MK, Rahman O, O’Brien A. Primary pulmonary angiosarcoma. BMJ Case Rep. 2021 Nov 9;14(11):e244578.
8. Shovlin CL. Pulmonary arteriovenous malformations. Am J Respir Crit Care Med. 2014 Dec 1;190(11):1217–28.
9. Almeida J, Leal C, Figueiredo L. Evaluation of the bronchial arteries: normal findings, hypertrophy and embolization in patients with hemoptysis. Insights Imaging. 2020 May 19;11(1):70.
10. Swisher JW, Weaver E. The evolving management and treatment options for patients with pulmonary hypertension: current evidence and challenges. Vasc Health Risk Manag. 2023;19:103–26.
11. Humbert M, Kovacs G, Hoeper MM, Badagliacca R, Berger RMF, Brida M, et al. 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Jeart K. 2022;43(38):3618–731.
12. Muliawan HS, Hartopo AB, Dinati LK, Irnizarifka, Dewangga MSY, Suciadi LP, et al. Pedoman diagnosis dan tatalaksana hipertensi pulmonal. 1st ed. Jakarta: Perhimpunan Dokter Spesialis Kardiovaskular Indonesia; 2021.
13. Lan NSH, Massam BD, Kulkarni SS, Lang CC. Pulmonary arterial hypertension: pathophysiology and treatment. Diseases. 2018;6(2):38.
14. Dinarti LK, Anggrahini DW, Lilyasari O, Siswanto BB, Hartopo AB. Pulmonary arterial hypertension in Indonesia: current status and local application of international guidelines. Glob Heart. 2021;16(1):23.
15. Lim Y, Low TT, Chan SP, Teo TW, Jang JHJ, Yip N, et al. Pulmonary arterial hypertension in a multi-ethnic Asian population: characteristics, survival and mortality predictors from a 14-year follow-up study. Respirol Carlton Vic. 2019;24(2):162–70.
16. Gelzinis TA. Pulmonary hypertension in 2021: part I—definition, classification, pathophysiology, and presentation. J Cardiothorac Vasc Anesth. 2022;36(6):1552–64.
17. Blanco I, Tura-Ceide O, Peinado VI, Barberà JA. Updated perspectives on pulmonary hypertension in COPD. Int J Chron Obstruct Pulmon Dis. 2020;15:1315–24.
18. Nathan SD, Barbera JA, Gaine SP, Harari S, Martinez FJ, Olschewski H, et al. Pulmonary hypertension in chronic lung disease and hypoxia. Eur Respir J. 2019;53(1):1801914.
19. Ley L, Grimminger F, Richter M, Tello K, Ghofrani A, Bandorski D. The early detection of pulmonary hypertension. Dtsch Ärztebl Int. 2023;120(48):823–30.
20. Frost A, Badesch D, Gibbs JSR, Gopalan D, Khanna D, Manes A, et al. Diagnosis of pulmonary hypertension. Eur Respir J. 2019;53(1):1801904.
21. Sakao S. Chronic obstructive pulmonary disease and the early stage of cor pulmonale: A perspective in treatment with pulmonary arterial hypertension-approved drugs. Respir Investig. 2019;57(4):325–9.
22. Septiyanti D, Sari AP, Aniwidyaningsih W, Antariksa B, Siswanto BB. The prevalence of pulmonary hypertension in destroyed lung due to post pulmonary tuberculosis and its relation to exercise capacity. J Respirologi Indones. 2018;38(4):227–31.
23. Maron BA, Bortman G, De Marco T, Huston JH, Lang IM, Rosenkranz SH, et al. Pulmonary hypertension associated with left heart disease. Eur Respir J. 2024;64(4):2401344.
24. Troncoso JÁ, Abánades CS, Robles-Marhuenda Á, Batres SA, Peña MFV, Valero SJ, et al. Prevalence, risk factors and echocardiographic predictors of pulmonary hypertension in systemic lupus erythematosus: towards a screening protocol. RMD Open. 2024;10(1):e003674.
25. Chang KY, Duval S, Badesch DB, Bull TM, Chakinala MM, De Marco T, et al. Mortality in pulmonary arterial hypertension in the modern era: early insights from the pulmonary hypertension association registry. J Am Heart Assoc. 2022;11(9):e024969.
26. Hendriks PM, Staal DP, van de Groep LD, van den Toorn LM, Chandoesing PP, Kauling RM, et al. The evolution of survival of pulmonary arterial hypertension over 15 years. Pulm Circ. 2022;12(4):e12137.
27. Sharara RS, Hattab Y, Patel K, DiSilvio B, Singh AC, Malik K. Introduction to the anatomy and physiology of pulmonary circulation. Crit Care Nurs Q. 2017;40(3):181–90.
28. Kandathil A, Chamarthy M. Pulmonary vascular anatomy & anatomical variants. Cardiovasc Diagn Ther. 2018;8(3):201–7.
29. Pleura, lungs, trachea, and bronchi. In: Standring S, editor. Gray’s anatomy the anatomical basis of clinical practice. 41st ed. Philadelphia: Elsevier; 2016. p. 953–75.
30. Yoshiya T, Mimae T, Tsubokawa N, Sasada S, Tsutani Y, Kushitani K, et al. The differences in histological changes among pulmonary vessels divided with an energy device. Interact Cardiovasc Thorac Surg. 2018;27(3):372–8.
31. Albertine KH, Ramirez MI, Morty RE. Anatomy. In: Broaddus VC, Ernst JD, King TE, Lazarus SC, Samriento KF, Schnapp LM, et al, editors. Murray & Nadel’s textbook of respiratory medicine. 7th ed. Philadelphia: Elsevier Saunders; 2022. p. 3–23.
32. West JB. Evolution of the pulmonary circulation and the right heart. In: Peacock AJ, Naejie R, Rubin LJ, editors. Pulmonary circulation diseases and their treatment. 4th ed. Boca Raton: CRC Press; 2016. p. 2–10.
33. West JB, Luks AM. Blood flow and metabolism. In: West’s respiratory physiology: the essentials. 11th ed. Philadelphia: Wolters Kluwer; 2020. p. 45–68.
34. McCormack MC, West JB. Ventilation, blood flow, and gas exchange. In: Broaddus VC, Ernst JD, King TE, Lazarus SC, Samriento KF, Schnapp LM, et.al, editors. Murray & Nadel’s textbook of respiratory medicine. 7th ed. Philadelphia: Elsevier Saunders; 2022. p. 116–39.
35. Naeije R. The pulmonary circulation. In: Grippi MA, Antin-Ozerkis DE, Cruz CSD, Kotloff RM, Kotton CN, Pack AI, editors. Fishman’s pulmonary diseases and disorders. 6th ed. New York: McGraw-Hill Education; 2023. p. 193–210.
36. Widrich J, Shetty M. Physiology, Pulmonary Vascular Resistance [Internet]. National Library of Medicine. 2024 [cited 2024 Jun 26]. Available from: http://www.ncbi.nlm.nih.gov/books/NBK554380/
37. Langleben D, Orfanos SE, Fox BD, Messas N, Giovinazzo M, Catravas JD. The paradox of pulmonary vascular resistance: restoration of pulmonary capillary recruitment as a sine qua non for true therapeutic success in pulmonary arterial hypertension. J Clin Med. 2022;11(15):4568.
38. Dunham-Snary KJ, Wu D, Sykes EA, Thakrar A, Parlow LRG, Mewburn JD, et al. Hypoxic pulmonary vasoconstriction: From Molecular Mechanisms to Medicine. Chest. 2017;151(1):181–92.
39. Hannemann J, Böger R. Dysregulation of the nitric oxide/dimethylarginine pathway in hypoxic pulmonary vasoconstriction—molecular mechanisms and clinical significance. Front Med. 2022; 17;9:835481.
40. Redel-Traub G, Sampson KJ, Kass RS, Bohnen MS. Potassium channels as therapeutic targets in pulmonary arterial hypertension. Biomolecules. 2022;12(10):1341.
41. Parreno GM, Cogolludo A, Perez-Vizcaino F. Potassium (K+) channels in the pulmonary vasculature: implications in pulmonary hypertension physiological, pathophysiological and pharmacological regulation. Pharmacol Ther. 2021;225:107835.
42. Kim DB, Mandel J, Smith KA, Yuan J. Pulmonary arterial hypertension. In: Grippi MA, Antin-Ozerkis DE, Cruz CSD, Kotloff RM, Kotton CN, Pack AI, editors. Fishman’s pulmonary diseases and disorders. 6th ed. New York: McGraw-Hill Education; 2023. p. 1211–57.
43. Dhoble S, Patravale V, Weaver E, Lamprou DA, Patravale T. Comprehensive review on novel targets and emerging therapeutic modalities for pulmonary arterial Hypertension. Int J Pharm. 2022;621:121792.
44. Tobal R, Potjewijd J, van Empel VPM, Ysermans R, Schurgers LJ, Reutelingsperger CP, et al. Vascular remodeling in pulmonary arterial hypertension: the potential involvement of innate and adaptive immunity. Front Med. 2021;8:806899.
45. Boyd S, Chatmetahkul T, McNamara P. How to diagnose and treat acute pulmonary hypertension when you have no cardiology support. Early Hum Dev. 2022;174:105668.
Downloads
Published
Issue
Section
License
Copyright (c) 2025 Bagus Radityo Amien, Andika Chandra Putra
This work is licensed under a Creative Commons Attribution-ShareAlike 4.0 International License.
- The authors own the copyright of published articles. Nevertheless, Jurnal Respirologi Indonesia has the first-to-publish license for the publication material.
- Jurnal Respirologi Indonesia has the right to archive, change the format and republish published articles by presenting the authors’ names.
- Articles are published electronically for open access and online for educational, research, and archiving purposes. Jurnal Respirologi Indonesia is not responsible for any copyright issues that might emerge from using any article except for the previous three purposes.
