Idiopathic pulmonary hypertension (autopsy observation)

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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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作者简介

Olesya Vasyukova

B.V. Petrovsky Russian Research Center of Surgery

编辑信件的主要联系方式.
Email: o.vas.93@gmail.com
ORCID iD: 0000-0001-6068-7009

junior researcher at the Laboratory of clinical morphology, Scientific Research Institute of Human Morphology named after academician A.P. Avtsyn

俄罗斯联邦, 117418, Moscow, 3 Tsyurupy Str.

Natalya Tsareva

Research Institute of Pulmonology of FMBA of Russia; I.M. Sechenov First Moscow State Medical University of the Ministry of Healthcare of Russia (Sechenov university)

Email: tsareva_n_a@staff.sechenov.ru
ORCID iD: 0000-0001-9357-4924

phd in medical sciences, head of the laboratory of intensive care and respiratory failure, associate professor of the department of pulmonology

俄罗斯联邦, 115682 Moscow, 28 Orekhovy Boulevard; Moscow

Andrey Chernyaev

B.V. Petrovsky Russian Research Center of Surgery; Research Institute of Pulmonology of FMBA of Russia; Central Research Institute of Tuberculosis; N.I. Pirogov Russian National Research Medical University of the Ministry of Healthcare of Russia

Email: cheral12@gmail.com
ORCID iD: 0000-0003-0973-9250

md, professor, head of the department of fundamental pulmonology, leading researcher at the Laboratory of clinical morphology, professor of the department of pathological anatomy and clinical pathological anatomical anatomy of the faculty of general medicine, pathologist, Scientific Research Institute of Human Morphology named after academician A.P. Avtsyn

俄罗斯联邦, 115682, Moscow, 28 Orekhovy Boulevard; Moscow; Moscow; Moscow

Lyudmila Mikhaleva

B.V. Petrovsky Russian Research Center of Surgery

Email: mikhalevaLM@yandex.ru
ORCID iD: 0000-0003-2052-914X

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 

俄罗斯联邦, 117418, Moscow, 3 Tsyurupy Str.

Maria Samsonova

Research Institute of Pulmonology of FMBA of Russia; Central Research Institute of Tuberculosis; A.S. Loginov Moscow Clinical Research Center of the Department of Healthcare of Moscow

Email: sama-ry@mail.ru
ORCID iD: 0000-0001-8170-1260

md, head of the laboratory of pathological anatomy, senior researcher 

俄罗斯联邦, 115682, Moscow, 28 Orekhovy Boulevard; Moscow; Moscow

参考

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1. JATS XML
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2. Рис. 1. Макроскопическая картина ткани легкого пациента М.: сегментарные и субсегментарные ветви легочных артерий выступают над поверхностью разрезов легких в виде «пеньков»

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3. Рис. 2. Плексус в артериоле, окруженный расширенными тонкостенными полнокровными сосудами (окраска гематоксилином и эозином, х2ОО)

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4. Рис. 3. Плексус по периферии ветви легочной артерии с расширенными полнокровными тонкостенными сосудами вокруг (окраска гематоксилином и эозином, х2ОО)

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5. Рис. 4. Два плексуса, окруженные тонкостенными полнокровными сосудами (окраска по Верхоффу ван-Гизону, х2ОО)

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6. Рис. 5. Частичный гиалиноз стенки артериолы с плексусом в стенке и адвентиции (окраска гематоксилином и эозином, х2ОО)

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7. Рис. 6. Гиалиноз стенок легочных вен с организующимся тромбом в просвете одной из них, полнокровие расширенных тонкостенных сосудов по периферии (окраска по Верхоффу ван-Гизону, х2ОО)

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8. Рис. 7. Фиброэластоз интимы венулы, обтурирующий организованный тромб в ветви легочной артерии, полнокровие расширенных тонкостенных сосудов по периферии (окраска по Верхоффу ван-Гизону, х2ОО)

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