endosergЭндокринная хирургияEndocrine Surgery2306-35132310-3965Типография «Печатных дел Мастер»10.14341/serg13003endoserg-13003Research ArticleКЛИНИЧЕСКИЕ СЛУЧАИCASE REPORTSФеохромоцитомы и параганглиомы у больных с врожденными пороками сердцаPheochromocytomas and paragangliomas in patients with congenital heart defectshttps://orcid.org/0000-0002-9066-5190БохянВ. Ю.BokhianV. Y.

Бохян Ваган Юрикович, д.м.н.

Москва

Vagan Y. Bokhian, MD, PhD

Moscow

b.vagan@mail.ruhttps://orcid.org/0000-0002-6456-9269ДаниловТ. Ю.DanilovT. Y.

Данилов Тимур Юрьевич, д.м.н.

Москва

Timur Yu. Danilov, MD, PhD

Moscow

timurdanilov@mail.ruhttps://orcid.org/0000-0002-8824-1137ТамразоваМ. Р.TamrazovaM. R.

Тамразова Марьям Романовна 

Москва

Mariam R. Tamrazova

Moscow

maryamtamrazova@yandex.ruhttps://orcid.org/0000-0003-3629-8438ГабраваМ. М.GabravaM. M.

Габрава Мераб Мерабович

Москва

Merab M. Gabrava

Moscow

gabromm@icloud.comhttps://orcid.org/0000-0002-4108-439XЖуликовЯ. А.ZhulikovY. A.

Жуликов Ярослав Андреевич, к.м.н.

Москва

Yaroslav A. Zhulikov, MD

Moscow

 

yarikzhulikov@gmail.comhttps://orcid.org/0000-0002-3595-3472ШинА. Р.ShinA. R.

Шин Александр Родионович, к.м.н. 

Москва

Alexander R. Shin, MD

Moscow

dr.alex.shin@gmail.comhttps://orcid.org/0000-0002-0666-7311МихайловаА. М.MikhaylovaA. M.

Михайлова Анастасия Михайловна

Москва

Anastasia M. Mikhaylova

Moscow

nastya.amm@gmail.comФГБУ «Национальный медицинский исследовательский центр онкологии им. Н.Н. Блохина»РоссияN.N. Blokhin National Medical Research Center of OncologyRussian FederationФГБУ «НМИЦ ССХ им. А.Н. Бакулева» Минздрава РоссииРоссияA.N. Bakulev Center for cardiovascular surgery of the Russian Ministry of HealthRussian Federation2025010220261935462Copyright © Бохян В.Ю., Данилов Т.Ю., Тамразова М.Р., Габрава М.М., Жуликов Я.А., Шин А.Р., Михайлова А.М., 20262026Бохян В.Ю., Данилов Т.Ю., Тамразова М.Р., Габрава М.М., Жуликов Я.А., Шин А.Р., Михайлова А.М.Bokhian V.Y., Danilov T.Y., Tamrazova M.R., Gabrava M.M., Zhulikov Y.A., Shin A.R., Mikhaylova A.M.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.surg-endojournals.ru/jour/article/view/13003

Корреляция феохромоцитом/параганглиом (ФЕО/ПГЛ) и цианотических врожденных пороков сердца встречается редко, но не является случайной. Исследования показывают, что развитие ФЕО/ПГЛ у таких пациентов может объясняться хронической гипоксией, нарушениями в работе симпатической нервной системы и возможной генетической предрасположенностью. Эти факторы могут провоцировать опухолевую трансформацию хромаффинных клеток. Кроме того, диагностика ФЕО/ПГЛ у пациентов с пороками сердца осложнена из-за гемодинамических нарушений, которые маскируют характерные для ФЕО/ПГЛ симпатоадреналовые кризы. Возникновение данных новообразований может усугублять клиническое течение врожденных пороков на фоне повышенной секреции катехоламинов, приводящих к вазоконстрикции и усилению гипоксии. Мы представляем два клинических случая ФЕО/ПГЛ на фоне врожденных пороков сердца, пациенты наблюдались и лечились в НМИЦ онкологии им. Н.Н. Блохина. Эти наблюдения подчеркивают важность настороженности при появлении симптомов тахикардии и неконтролируемой артериальной гипертензии таких пациентов. Несмотря на редкость, необходимо учитывать взаимосвязь между этими патологиями для точной диагностики и выбора оптимального лечения.

The correlation between pheochromocytoma/paraganglioma (PHEO/PGL) and cyanotic congenital heart defects is rare but not coincidental. Research suggests that the development of PHEO/PGL in these patients maybe explained by chronic hypoxia, disruptions in the sympathetic nervous system, and potential genetic predisposition. These factors may provoke the tumor transformation of chromaffin cells. Additionally, diagnosing PHEO/PGL in patients with heart defects is complicated by hemodynamic disturbances that mask the characteristic sympathoadrenal crises of PHEO/PGL. The occurrence of these neoplasms can exacerbate the clinical course of congenital defects due to increased catecholamine secretion, leading to vasoconstriction and worsening hypoxia. We present two clinical cases of PHEO/PGL in the context of congenital heart defects, with patients observed and treated at the N.N. Blokhin National Medical Research Center of Oncology and the A.N. Bakoulev National Medical Research Center of Cardiovascular Surgery. These observations emphasize the importance of vigilance for symptoms of tachycardia and uncontrolled hypertension in such patients. Despite their rarity, the relationship between these pathologies must be considered for accurate diagnosis and optimal treatment selection.

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The authors declare that there are no conflicts of interest present.