Idiopathic pulmonary hypertension (autopsy observation)


DOI: https://dx.doi.org/10.18565/therapy.2023.6.82-88

Vasyukova O.A., Tsareva N.A., Chernyaev A.L, Mikhaleva L.M., Samsonova M.V.

1) Scientific Research Institute of Human Morphology named after academician A.P. Avtsyn of B.V. Petrovsky Russian Research Center of Surgery, Moscow; 2) Research Institute of Pulmonology of FMBA of Russia, Moscow; 3) I.M. Sechenov First Moscow State Medical University of the Ministry of Healthcare of Russia (Sechenov university); 4) Central Research Institute of Tuberculosis, Moscow; 5) N.I. Pirogov Russian National Research Medical University of the Ministry of Healthcare of Russia, Moscow; 6) A.S. Loginov Moscow Clinical Research Center of the Department of Healthcare of Moscow
Abstract. The article presents a description of idiopathic pulmonary arterial hypertension (IPAH) autopsy observation. The diagnosis was made in a 51-year-old patient four years ago and based on the results of an invasive measurement of mean pulmonary artery pressure during right heart catheterization, the index of which was 36 mmHg. During dynamic observation, according to echocardiography, the values of systolic pressure in the pulmonary artery ranged from 66 to 107 mm Hg. Observed patient received treatment with macitentan and spironolactone for 4 years, died with symptoms of pulmonary heart disease. At autopsy, idiopathic plexiform arteriopathy was diagnosed in combination with Heth–Edwards class 4–5 thrombotic arteriopathy with right ventricular failure development.

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About the Autors


Olesya A. Vasyukova, junior researcher at the Laboratory of clinical morphology, Scientific Research Institute of Human Morphology named after academician A.P. Avtsyn of B.V. Petrovsky Russian Research Center Of Surgery. Address: 117418, Moscow, 3 Tsyurupy Str.
E-mail: o.vas.93@gmail.com
ORCID: https://orcid.org/0000-0001-6068-7009
Natalya A. Tsareva, PhD in Medical Sciences, head of the Laboratory of intensive care and respiratory failure, Research Institute of Pulmonology of FMBA of Russia, associate professor of the Department of pulmonology, I.M. Sechenov First Moscow State Medical University of the Ministry of Healthcare of Russia (Sechenov university).
Address: 115682 Moscow, 28 Orekhovy Boulevard.
E-mail: tsareva_n_a@staff.sechenov.ru
ORCID: https://orcid.org/0000-0001-9357-4924
Andrey L. Chernyaev, MD, professor, head of the Department of fundamental pulmonology, Research Institute of Pulmonology of FMBA of Russia, leading researcher at the Laboratory of clinical morphology, Scientific Research Institute of Human Morphology, professor of the Department of pathological anatomy and clinical pathological anatomical anatomy of the Faculty of general medicine, N.I. Pirogov Russian National Research Medical University of the Ministry of Healthcare of Russia, pathologist at Central Research Institute of Tuberculosis.
Address: 115682, Moscow, 28 Orekhovy Boulevard.
E-mail: cheral12@gmail.com.
ORCID: https://orcid.org/0000-0003-0973-9250
Lyudmila M. Mikhaleva, MD, professor, corresponding member of the Russian Academy of Sciences, director, head of the Laboratory of clinical morphology, Scientific Research Institute of Human Morphology named after academician A.P. Avtsyn of B.V. Petrovsky Russian Research Center of Surgery. Address: 117418, Moscow, 3 Tsyurupy Str.
E-mail: mikhalevaLM@yandex.ru
ORCID: https://orcid.org/0000-0003-2052-914X
Maria V. Samsonova, MD, head of the laboratory of pathological anatomy, Research Institute of Pulmonology of FMBA of Russia, senior researcher at A.S. Loginov Moscow Clinical Research Center of the Department of Healthcare of Moscow. Address: 115682, Moscow, 28 Orekhovy Boulevard.
E-mail: sama-ry@mail.ru
ORCID: https://orcid.org/0000-0001-8170-1260


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