Pulmonary hypertension associated with portal hypertension and pulmonary hypertension in sarcoididosis of breathing organs: complex pathogenetic relationships

 In the literature review, modern views on the features of pathogenesis and diagnosis of pulmonary hypertension associated with portal hypertension and sarcoidosis of respiratory organs are presented. A variety of views is due to the lack of a convincing evidence base underlying the algorithm for diagnosis and treatment of this pathology. Pulmonary hypertension is one of the most complex cardiological problems and represents a pathological condition that is based on increasing resistance to blood flow in a small circle of blood circulation at any of site. Unlike the systemic arteries, there is no direct access to the pulmonary artery for measuring blood pressure by non-invasive methods, so the diagnosis of pulmonary hypertension in the early stages, before the formation of a lesion in the target organ, which is the right ventricle of the heart, is practically impossible. In actual clinical practice, pulmonary hypertension is at best diagnosed at the stage of latent right ventricular dysfunction, which is manifested by dilatation of its cavity and / or hypertrophy of its walls, at worst at the stage of right ventricular heart failure. At present, there has been a trend towards an improvement in the diagnosis of pulmonary hypertension (LH) in various diseases. This is the reason for the special interest in this interdisciplinary problem.

1. Krahmalova E.O. Echocardiography in the diagnosis of pulmonary hypertension. Kardiologija. Aspekty diagnostiki. 2011; 69–70 (in Russ.).

2. Avdeyev S.N. Approaches to the therapy of pulmonary arterial hypertension: the role of antagonist prescriptions of endothelium bosentan. Terapevticheskiy arkhiv. 2015; 9: 64–71 (in Russ.). DOI: 10.17116/terarkh201587964-71.

3. Baranova O.P., Perley V.E., Zolotnitskaya V.P., Speranskaya A.A., Il’kovich M.M. Pulmonary hypertension in respiratory sarcoidosis. Meditsinskiy sovet. 2014; 12– 16 (in Russ.). DOI: 10.21518/2079-701X-2014-4-12-16.

4. Vizel’ A.A., Vizel’ I.Yu. Sarcoidosis: international conciliation recommendations. Russkiy meditsinskiy zhurnal. 2014; 5: 356–359 (in Russ.).

5. Galiи N., Humbert M., Vachiery J.-L., Gibbs S., Lang L., Torbicki A., Simonneaua G., Peacocka A., Noordegraafa A., Beghettib M., Ghofrania A. ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. 2015. DOI: 10.1183/13993003.01032-2015.

6. Chuchalin A.G., Vizel’ A.A., Il’kovich M.M. Diagnosis and treatment of sarcoidosis: a summary of federal conciliation clinical recommendations (Part II: Diagnosis, treatment, prognosis). Vestnik sovremennoy klinicheskoy meditsiny. 2014; 7 (5): 73–81 (in Russ.).

7. Chazova I.E., Arkhipova O.A., Valieva Z.S., Nakonechnikov S.N., Martynyuk T.V. Pulmonary hypertension in Russia: first results of the national registry. Terapevticheskiy arkhiv. 2014; 9: 56–64 (in Russ.). DOI: 10.17116/terarkh20168844-8.

8. Sadykova D.I. Modern approaches to the diagnosis and treatment of pulmonary hypertension. Pediatriya. 2012; 7 (62): 21–26 (in Russ.).

9. Yakovleva L.N. Pulmonary arterial hypertension. Diabet i serdtse. 2011; 10 (156): 62–29 (in Russ.).

10. Avdeyev S.N. Pulmonary hypertension in sarcoidosis. Pul’monologija. 2016; 26 (6): 725–735 (in Russ.). DOI: 10.18093/0869-0189-2016-26-6-725-735.

11. Shorr A.F., Helman D.L., Davies D.B., Nathan S.D. Pulmonary hypertension in advanced sarcoidosis: epidemiology and clinical characteristics. Eur. Respir. J. 2005; 25: 783–788. DOI:10.1007/s00408-011-9361-9.

12. Perley V.E., Lebedeva E.V., Amosov V.I., Yalfimov A.N., Il’kovich M.M. Diagnosis of heart damage in respiratory sarcoidosis. Ukrainskiy pul’monologicheskiy zhurnal. 2005; 2: 50–53 (in Russ.).

13. ESC/ERS recommendations on the diagnosis and treatment of pulmonary hypertension 2015. Rossiyskiy kardiologicheskiy zhurnal. 2016; 5: 5–64 (in Russ.). DOI: 10.15829/1560-4071-2016-5-5-64.

14. Sarcoidosis. Clinical recommendations; under the editorship of the member of RAMS Mukhin N.A. Sarkoidoz. Klinicheskie rekomendatsii; рod red. akad. RAMN N.A. Mukhina. M.: IMA-PRESS Publ.; 2009: 128 (in Russ.).

15. Shigemitsu H., Nagai S., Sharma O.P. Pulmonary hypertension and granulomatous vasculitis in sarcoidosis. Curr. Opin. Pulm. Med. 2007; 13: 434–438.

16. Battesti J.P., Georges R., Basset F., Saumon G. Chronic cor pulmonale in pulmonary sarcoidosis. Thorax. 1978; 33: 76–84.

17. Rizzato G., Pezzano A., Sala G., Merlini R., Ladelli L., Tansini G., Montanari G., Bertoli L. Right heart impairment in sarcoidosis: haemodynamic and echocardiographic study. Eur. J Respir. Dis. 1983; 64: 121–128.

18. Mayock R.L., Bertrand P., Morrison C.E., Scott J.H. Manifestations of sarcoidosis. Analysis of 145 patients with a review of nine seriesselected from the literature. Am. J. Med. 1963; 35: 67–89.

19. Pacherskikh F.N. Pulmonary arterial hypertension: clinical picture, diagnosis, treatment: A manual for students. Irkutsk: IGMU Publ., 2015 (in Russ.).

20. Garbuzenko D.V. The pathogenesis of portal hypertension. Rossiyskiy zhurnal gstroenterologii, gepatologii i koloproktologii. 2002; 12 (5): 23–28 (in Russ.).

21. Kalacheva T.P., Beloborodova E.V., Beloborodova E.I., Chernyavskaya G.M., Ostanko V.L., Ivanova T.V., Konaplev D.I., Petrova L.A. A view of the problem of portopulmonary hypertension. Klinicheskie perspektivy gastroenterologii, gepatologii. 2016; 1 (56): 41–47 (in Russ.).

22. Kalacheva T.P., Chernyavskaya G.M., Beloborodova E.I., Ivanova T.V., Konaplev D.I. Evaluation of respiratory function of the lungs, structural and functional parameters of the right heart and pulmonary artery blood flow in comparison with the clinical course and indices of portal hemodynamics in liver cirrhosis. Pul’monologiya. 2011; 3: 90–95 (in Russ.).

23. Kalacheva T.P., Chernyavskaya G.M., Beloborodova E.I. Ultrasonice valuation and prognostic value of pulmonary-cardia chemodynamics in case of liver cirrhosis. Byulleten’ SO RAMN. 2013; 33 (4): 56–64 (in Russ.).

24. Hoeper M.M., Krowka M.J., Strassburg C.P. Portopulmonary hypertension and hepatopulmonary syndrome. Lancet. 2004; 363 (9419): 1461–1468. DOI: 10.1016/S0140-6736(04)16107-2.

25. Garbuzenko D.V. Aspects of pathogenetic pharmacotherapy of portal hypertension in liver cirrhosis. Terapevticheskiy arkhiv. 2016; 2 (88): 101–108 (in Russ.). DOI: 10.17116/terarkh2016888101-108.

26. Berzigotti A., Bosch J. Pharmacologic Management of Portal Hypertension. Clin. Liver Dis. 2014; 18 (2): 303– 317. DOI: 10.1016/j.cld.2013.12.003.

27. Sampaio F., Pimenta J., Bettencourt N., Fontes-Carvalho R., Silva A.P., Valente J., Bettencourt P., Fraga J., Gama V. Systolic and diastolic dysfunction in cirrhosis: A tissue-Doppler and speckle tracking echocardiography study. Liver Int. 2013; 33: 1158–1165. DOI: 10.1111/liv.12187.

28. Porres-Aguilar M., Gallegos-Orozco J.F., Garcia H., Aguirre J., Macias-Rodriguez R.U., Torre-Delgadillo Pulmonary vascular complications in portal hypertension and liver disease: A concise review. Rev. Gastroenterol. Mex. 2013; 78 (1): 35–44. DOI: 10.1016/j.rgmx.2012.10.004.