Idiopathic pulmonary hypertension in an adult with patent foramen ovale
https://doi.org/10.21518/ms2024-426
Abstract
The article is devoted to the clinical case of combined idiopathic pulmonary hypertension with an open foramen ovale of the atrial septum in a young man of 21 years old with progressive decrease in lung ventilation function. The relationship of pulmonary arterial hypertension with an open foramen ovale of the atrial septum is nearly not described in the literature, as it is rarely found in clinical practice. According to the classification, pulmonary hypertension is divided into idiopathic (primary), secondary and pulmonary hypertension caused by other causes. In the pathogenesis of any PAH, an important role belongs to a complex of vascular changes. Verification of vascular changes is carried out histologically. Variants of idiopathic pulmonary hypertension are plexiform and thrombotic arteriopathy, veno-occlusive disease, capillary hemangiomatosis. An open foramen ovale as a structural anomaly of the adult heart (a vestige of embryonic blood circulation) causes a variety of hemodynamic disorders that can affect the pathogenesis of pulmonary hypertension. Because of this, preclinical observation of a combination of plexiform and thrombotic arteriopathy with a patent foramen ovale of the interatrial septum in a young man of 21 years old and an attempt to determine the role of an open oval window in the progression of pulmonary hypertension and its effect on treatment is of interest. The diagnosis of idiopathic pulmonary hypertension was made based on the clinical picture, laboratory parameters, echocardiography and histological examination of pulmonary vessels. The article discusses the importance of a patent foramen ovale in the pathogenesis of idiopathic pulmonary hypertension.
About the Authors
A. A. ShumilinRussian Federation
Aleksey A. Shumilin, Pulmonologist, Head of the 15th Pulmonology Department
1, Novy Village, Moscow Region, Krasnogorsk, 143420
A. I. Pavlov
Russian Federation
Alexandr I. Pavlov, Dr. Sci. (Med.), Professor, Honored Doctor of the Russian Federation, Colonel of the Medical Service, Deputy Head of the Medical Department, Professor of the Department of Therapy with a Course in Pharmacology and Pharmacy
1, Novy Village, Moscow Region, Krasnogorsk, 143420
A. A. Prokhorchik
Russian Federation
Alexander A. Prokhorchik, Cand. Sci. (Med.), Honored Doctor of the Russian Federation, Chief Therapist
1, Novy Village, Moscow Region, Krasnogorsk, 143420
A. L. Cherniaev
Russian Federation
Andrey L. Cherniaev, Dr. Sci. (Med.), Head of the Division of Fundamental Medicine, Leading Scientist, Professor of Pathology Department
28, Orekhovy Boulevard, Moscow, 115682
M. V. Samsonova
Russian Federation
Maria V. Samsonova, Dr. Sci. (Med.), Head of the Pathology Department Research Institute for Pulmonology of the Federal Medical Biological Agency; Senior Research Associate, Innovative Pathomorphology Laboratory, Loginov Moscow Clinical Scientific Center
28, Orekhovy Boulevard, Moscow, 115682,
1, Novogireevskaya St., Moscow, 111123
M. A. Slavetskya
Russian Federation
Marina A. Slavetskya, Resident of the Department of Pathological Anatomy
5, 2nd Botkinsky Proezd, Moscow, 125284
References
1. Pietra GG, Capron F, Stewart S, Leone O, Humbert M, Robbins IM et al. Pathologic assessment of vasculopathies in pulmonary hypertension. J Am Coll Cardiol. 2004;43(12 Suppl. S):25S–32S. https://doi.org/10.1016/j.jacc.2004.02.033.
2. Voelkel NF, Cool C. Pathology of pulmonary hypertension. Cardiol Clin. 2004;22(3):343–351. https://doi.org/10.1016/j.ccl.2004.04.010.
3. Simonneau G, Gatzoulis MA, Adatia I, Celermajer D, Denton C, Ghofrani A et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25 Suppl.):D34–D41. https://doi.org/10.1016/j.jacc.2013.10.029.
4. Foshat M, Boroumand N. The Evolving Classification of Pulmonary Hypertension. Arch Pathol Lab Med. 2017;141(5):696–703. https://doi.org/10.5858/arpa.2016-0035-ra.
5. Kosanovic D, Avdeev SN, Milovanov AP, Chernyaev AL. Northern Pulmonary Hypertension: A Forgotten Kind of Pulmonary Circulation Pathology. Life (Basel). 2024;14(7):875. https://doi.org/10.3390/life14070875.
6. Есипова ИК. Патологическая анатомия легких. М.: Медицина; 1976. 183 с. Режим доступа: https://search.rsl.ru/ru/record/01006933092?ysclid=m1krdaei13320449297.
7. Heath D, Edwards JE. The pathology of hypertensive pulmonary vascular disease; a description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects. Circulation. 1958;18(4 Part 1):533–547. https://doi.org/10.1161/01.cir.18.4.533.
8. Katzenstein A-LA. Diagnostic Atlas of Non-Neoplastic Lung Disease. A Practical Guide for Surgical Pathologists. 1st ed., NY: Demos Medical Publishing, LLC/Springer Publishing Company; 2016, 352 р. Available at: https://www.semanticscholar.org/paper/Diagnostic-Atlas-of-Non-NeoplasticLung-Disease-Katzenstein/ace69a92af0c81aa63a93d36202033a3a3b63f61.
9. Wagenvoort CA. Grading of pulmonary vascular lesions – a reappraisal. Histopathology. 1981;5(6):595–598. https://doi.org/10.1111/j.1365-2559.1981.tb01826.x.
10. Caslin AW, Heath D, Madden B, Yacoub M, Gosney JR, Smith P. The histopathology of 36 cases of plexogenic pulmonary arteriopathy. Histopathology. 1990;16(1):9–19. https://doi.org/10.1111/j.1365-2559.1990.tb01054.x.
11. Bjornsson J, Edwards WD. Primary pulmonary hypertension: a histopathologic study of 80 cases. Mayo Clin Proc. 1985;60(1):16–25. https://doi.org/10.1016/s0025-6196(12)65277-x.
12. Sakao S, Tatsumi K, Voelkel NF. Endothelial cells and pulmonary arterial hypertension: apoptosis, proliferation, interaction and transdifferentiation. Respir Res. 2009;10(1):95. https://doi.org/10.1186/1465-9921-10-95.
13. Badesch DB, Raskob GE, Elliott CG, Krichman AM, Farber HW, Frost AE et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest. 2010;137(2):376–387. https://doi.org/10.1378/chest.09-1140.
14. Rubin LJ. Primary pulmonary hypertension. N Engl J Med. 1997;336(2):111–117. https://doi.org/10.1056/nejm199701093360207.
15. Burke AP, Farb A, Virmani R. The pathology of primary pulmonary hypertension. Mod Pathol. 1991;4(2):269–282. Available at: https://pubmed.ncbi.nlm.nih.gov/2047387/.
16. Chazova I, Robbins I, Loyd J, Newman J, Tapson V, Zhdaov V, Meyrick B. Venous and arterial changes in pulmonary veno-occlusive disease, mitral stenosis and fibrosing mediastinitis. Eur Respir J. 2000;15(1):116–122. https://doi.org/10.1183/09031936.00.15111600.
17. Nossent EJ, Smits JA, Seegers C, Meijboom LJ, Boonstra A, Aman J et al. Clinical Correlates of a Nonplexiform Vasculopathy in Patients With a Diagnosis of Idiopathic Pulmonary Arterial Hypertension. Chest. 2024;166(1):190–200. https://doi.org/10.1016/j.chest.2024.02.046.
18. Wagenvoort CA. Lung biopsy specimens in the evaluation of pulmonary vascular disease. Chest. 1980;77(5):614–625. https://doi.org/10.1378/chest.77.5.614.
19. Pietra GG, Edwards WD, Kay JM, Rich S, Kernis J, Schloo B et al. Histopathology of primary pulmonary hypertension. A qualitative and quantitative study of pulmonary blood vessels from 58 patients in the National Heart, Lung, and Blood Institute, Primary Pulmonary Hypertension Registry. Circulation. 1989;80(5):1198–1206. https://doi.org/10.1161/01.cir.80.5.1198.
20. Rudoy AS, Bova AA, Nekhaichik TA. Open oval window and associated clinical conditions. Clinical Medicine (Russian Journal). 2017;95(7):607–612. Available at: https://elibrary.ru/zhrjjr.
21. Davison P, Clift PF, Steeds RP. The role of echocardiography in diagnosis, monitoring closure and post-procedural assessment of patent foramen ovale. Eur J Echocardiogr. 2010;11(10):i27–34. https://doi.org/10.1093/ejechocard/jeq120.
22. Шарыкин АС. Врожденные пороки сердца. 2-е изд. М.: БИНОМ; 2009. 111–122 с. Режим доступа: https://djvu.online/file/Zt0nUUh9VXjLv.
23. Calvert PA, Rana BS, Kydd AC, Shapiro LM. Patent foramen ovale: anatomy, outcomes, and closure. Nat Rev Cardiol. 2011;8(3):148–160. https://doi.org/10.1038/nrcardio.2010.224.
24. Sun YP, Homma S. Patent Foramen Ovale and Stroke. Circ J. 2016;80(8):1665–1673. https://doi.org/10.1253/circj.cj-16-0534.
25. Meissner I, Khandheria BK, Heit JA, Petty GW, Sheps SG, Schwartz GL et al. Patent foramen ovale: innocent or guilty? Evidence from a prospective population-based study. J Am Coll Cardiol. 2006;47(2):440–445. https://doi.org/10.1016/j.jacc.2005.10.044.
26. Chazova IE, Zhdanov VS, Veselova SP, Mareev VYu. Pathology of primary pulmonary hypertension. Archives of Pathology. 1993;(3):52–55. (In Russ.)
27. Andersen S, Nielsen-Kudsk JE, Vonk Noordegraaf A, de Man FS. Right Ventricular Fibrosis. Circulation. 2019;139(2):269–285. https://doi.org/10.1161/circulationaha.118.035326.
28. Bradlow WM, Assomull RG, Williams F, Taegtmeyeret A, Gin-sing W, Strange J et al. Cardiovascular magnetic resonance measured right ventricular mass influences prognosis in pulmonary arterial hypertension. Eur Heart J. 2010;31:8. Available at: https://www.researchgate.net/publication/295116646_Cardiovascular_magnetic_resonance_measured_right_ventricular_mass_influences_prognosis_in_pulmonary_arterial_hypertension.
29. Bustamante-Labarta M, Perrone S, De La Fuente RL, Stutzbach P, De La Hoz RP, Torino A, Favaloro R. Right atrial size and tricuspid regurgitation severity predict mortality or transplantation in primary pulmonary hypertension. J Am Soc Echocardiogr. 2002;15(10):1160–1164. https://doi.org/10.1067/mje.2002.123962.
30. van Wolferen SA, Marcus JT, Boonstra A, Marques KM, Bronzwaer JG, Spreeuwenberg MD et al. Prognostic value of right ventricular mass, volume, and function in idiopathic pulmonary arterial hypertension. Eur Heart J. 2007;28(10):1250–1257. https://doi.org/10.1093/eurheartj/ehl477.
Review
For citations:
Shumilin AA, Pavlov AI, Prokhorchik AA, Cherniaev AL, Samsonova MV, Slavetskya MA. Idiopathic pulmonary hypertension in an adult with patent foramen ovale. Meditsinskiy sovet = Medical Council. 2024;(16):38-45. (In Russ.) https://doi.org/10.21518/ms2024-426