Rare clinical case of the diagnosis of idiopathic pulmonary artery hypertension

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Abstract

In this work, a rather rare (morbidity of about 1–2 cases per 1 million of adult population per year) and interesting clinical case of the diagnosis of idiopathic pulmonary artery hypertension (IPAH) was described. This case was also known as Ayerza disease or Ayerza–Arilago syndrome. At the initial stage, this pathology was characterized by an asymptomatic course that caused certain difficulties in making a correct diagnosis. In general, this disease had a poor prognosis. The main cause of death was decompensated right ventricular failure. The occurrence of complications could be significantly reduced and the life quality of such patients could be improved by the timely diagnosis and correct approach.

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About the authors

Grigory A. Ignatenko

M. Gorky Donetsk National Medical University

Email: gai-1959@mail.ru
ORCID iD: 0000-0002-1155-563X

MD, Dr.Sci.(Med.), Professor, Corresponding Member of the Ukraine National Academy of Medical Sciences, Honored Scientist and Technician of Ukraine, Rector, Head of the Propaedeutic and Internal Medicine Department

Ukraine, Donetsk

Ilya S. Grekov

M. Gorky Donetsk National Medical University

Email: ilya.grekov.1998@gmail.com
ORCID iD: 0000-0002-6140-5760

6th year student

Ukraine, Donetsk

Marina V. Grushina

M. Gorky Donetsk National Medical University

Author for correspondence.
Email: grushinamarina@inbox.ru
ORCID iD: 0000-0003-3670-3376

MD, Cand.Sci.(Med.), , Associate Professor of the Propaedeutic and Internal Medicine Department

Ukraine, Donetsk

Anna V. Dubovyk

M. Gorky Donetsk National Medical University

Email: dubovyk-anna@mail.ru
ORCID iD: 0000-0002-8753-3824

MD, Cand.Sci.(Med.), , Associate Professor of the Propaedeutic and Internal Medicine Department

Ukraine, Donetsk

References

  1. Chazova IE, Martynyuk TV, Valieva ZS, et al. Eurasian clinical guidelines on diagnosis and treatment of pulmonary hypertension. Eurasian Heart Journal. 2020;(1):78-122. (In Russ.) doi: 10.38109/2225-1685-2020-1-78-122
  2. Galiè N, Humbert M, Vachiery J-L, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. European Respiratory Journal. 2015;46(4):903-75. doi: 10.1183/13993003.01032-2015
  3. Kochetov VE. Aerza-Arrilaga syndrome: pathogenesis and pathophysiology. Bulletin of Emergency Medicine. 2016; 9(1):107-11. (In Russ).
  4. Tsareva NA. Updated classification and diagnosis of pulmonary hypertension. Consilium Medicum. 2017;19(3):66-71. (In Russ).
  5. Martyniuk TV, Chazova IE. Strategy of medical treatment of pulmonary arterial hypertension in the current international recommendations. Systemic Hypertension. 2016;13(2):46-64. (In Russ).

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Copyright (c) 2021 Ignatenko G.A., Grekov I.S., Grushina M.V., Dubovyk A.V.


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