perinatologyРоссийский вестник перинатологии и педиатрииRossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)1027-40652500-2228Ltd. “The National Academy of Pediatric Science and Innovation”10.21508/1027-4065-2025-70-4-16-21perinatology-2226Research ArticleОБЗОРЫ ЛИТЕРАТУРЫLITERATURE REVIEWSСтенозы легочных вен у недоношенных детей и младенцевPulmonary vein stenosis in preterm and newborn infantshttps://orcid.org/0000-0002-2003-7252ЗинченкоМ. К.ZinchenkoM. K.

Зинченко Максим Константинович — врачдетский кардиолог отделения кардиохирургии и интенсивной терапии 

123001, Москва, Садовая-Кудринская ул., 15

123001, Moscow

https://orcid.org/0009-0008-5367-0290НикитинаЕ. К.NikitinaE. K.

Никитина Екатерина Константиновна — врач анестезиолог-реаниматолог, заведующий отделением реанимации и интенсивной терапии для новорожденных и недоношенных отделения кардиохирургии и интенсивной терапии

123001, Москва, Садовая-Кудринская ул., 15

123001, Moscow

https://orcid.org/0000-0002-6334-907XГордейчукЕ. Д.GordeychukE. D.

Гордейчук Елизавета Дмитриевна (автор, ответственный за переписку) — врач-детский кардиолог, медицинский советник-специалист по коммуникациям 

101000, г. Москва, Милютинский переулок, 18А, офис 20А 

101000, Moscow

https://orcid.org/0000-0001-8195-5682КовалевИ. А.KovalyovI. A.

Ковалев Игорь Александрович — д.м.н., проф., главный внештатный специалист детский кардиолог Минздрава России, советник директора 

121552, Москва, Рублевское шоссе, 135

121552, Moscow

https://orcid.org/0000-0003-4162-068XИльинВ. Н.IlyinV. N.

Ильин Владимир Николаевич — д.м.н., проф., зав. отделением кардиохирургии детской кардиологии и интенсивной терапии 

123001, Москва, Садовая-Кудринская ул., 15

123001, Moscow

ГБУЗ «Детская городская клиническая больница имени Н.Ф. Филатова ДЗМ»РоссияFilatov Children’s City HospitalRussian FederationАссоциация «Евразийская Ассоциация Терапевтов»РоссияEurasian Association of Internal MedicineRussian FederationФГБУ «Национальный медицинский исследовательский центр сердечно-сосудистой хирургии им. А.Н. Бакулева» Минздрава РоссииРоссияBakulev National Medical Research Center for Cardiovascular SurgeryRussian Federation20252808202570416–21Copyright © Ltd. “The National Academy of Pediatric Science and Innovation”, 20252025Ltd. “The National Academy of Pediatric Science and Innovation”Ltd. “The National Academy of Pediatric Science and Innovation”https://www.ped-perinatology.ru/jour/about/submissions#copyrightNoticehttps://www.ped-perinatology.ru/jour/article/view/2226

В статье подробно рассмотрены патогенетические механизмы, лежащие в основе стеноза легочных вен у недоношенных детей, включая влияние бронхолегочной дисплазии и легочной гипертензии. Особое внимание уделено изучению распространенности этого заболевания в данной группе пациентов, анализу факторов риска, таких как недоношенность, низкий вес при рождении, длительная искусственная вентиляция легких и кислородотерапия, а также их влиянию на клинический прогноз. Описаны основные морфологические изменения, в том числе ремоделирование сосудистой стенки, упрощение альвеолярных структур и воспалительные процессы, которые обуславливают развитие стеноза. Подчеркивается важность раннего выявления заболевания, поскольку несвоевременная диагностика может привести к прогрессирующему ухудшению состояния пациентов, включая развитие правожелудочковой недостаточности и значительное повышение риска летального исхода. В статье также обсуждаются современные методы диагностики. Отмечается ведущая роль трансторакальной эхокардиографии как метода выбора, ее преимущества и ограничения. При сложных случаях предлагается использование дополнительных методов, таких как компьютерная и магнитно-резонансная томография или катетеризация сердца для уточнения степени стеноза и оценки состояния легочных вен.

The article provides a detailed examination of the pathogenic mechanisms underlying pulmonary vein stenosis in premature infants, including the influence of bronchopulmonary dysplasia and pulmonary hypertension. Special attention is given to the prevalence of the disease in this patient group and the analysis of risk factors such as prematurity, low birth weight, prolonged mechanical ventilation, and oxygen therapy, as well as their impact on clinical outcomes. The key morphological changes are described, including vascular wall remodeling, alveolar simplification, and inflammatory processes, all of which underlie the development of pulmonary vein stenosis. The importance of early disease detection is emphasized, as delayed diagnosis can lead to progressive deterioration in the patient's condition, including right ventricular failure and a significantly increased risk of mortality. The article also discusses modern diagnostic methods, highlighting the primary role of transthoracic echocardiography as the method of choice, along with its advantages and limitations. For complex cases, the use of additional methods, such as computed tomography, magnetic resonance imaging, or cardiac catheterization, is recommended to clarify the severity of stenosis and assess the condition of the pulmonary veins.

недоношенные детистенозы легочных венбронхолегочная дисплазиялегочная гипертензиясердечно-сосудистая патологияpremature infantspulmonary vein stenosisbronchopulmonary dysplasiapulmonary hypertensioncardiovascular pathologyReferencesNasr V.G., Callahan R., Wichner Z., Odegard K.C., DiNardo J.A. Intraluminal Pulmonary Vein Stenosis in Children. Anesth. Analg. 2019; 129: 27–40. DOI: 10.1213/ANE.0000000000003924Nasr V.G., Callahan R., Wichner Z., Odegard K.C., DiNardo J.A. Intraluminal Pulmonary Vein Stenosis in Children. Anesth. Analg. 2019; 129: 27–40. DOI: 10.1213/ANE.0000000000003924Backes C.H., Nealon E., Armstrong A.K., Cua C.L., Mitchell C., Krishnan U., et al. Pulmonary Vein Stenosis in Infants: A Systematic Review, Meta-Analysis, and MetaRegression. J. Pediatr. 2018; 198: 36–45.e3. DOI: 10.1016/j.jpeds.2018.02.030Backes C.H., Nealon E., Armstrong A.K., Cua C.L., Mitchell C., Krishnan U., et al. Pulmonary Vein Stenosis in Infants: A Systematic Review, Meta-Analysis, and MetaRegression. J. Pediatr. 2018; 198: 36–45.e3. DOI: 10.1016/j.jpeds.2018.02.030Jadcherla A.V., Backes C.H., Cua C.L., Smith C.V., Levy P.T., Ball M.K. Primary Pulmonary Vein Stenosis: A New Look at a Rare but Challenging Disease. Neoreviews. 2021; 22:e296–e308. DOI: 10.1542/neo.22–5–e296Jadcherla A.V., Backes C.H., Cua C.L., Smith C.V., Levy P.T., Ball M.K. Primary Pulmonary Vein Stenosis: A New Look at a Rare but Challenging Disease. Neoreviews. 2021; 22:e296–e308. DOI: 10.1542/neo.22–5–e296Kalfa D., Belli E., Bacha E., Lambert V., di Carlo D., Kostolny M., et al. Outcomes and prognostic factors for postsurgical pulmonary vein stenosis in the current era. J. Thorac. Cardiovasc. Surg. 2018; 156: 278–286. DOI: 10.1016/j.jtcvs.2018.02.038Kalfa D., Belli E., Bacha E., Lambert V., di Carlo D., Kostolny M., et al. Outcomes and prognostic factors for postsurgical pulmonary vein stenosis in the current era. J. Thorac. Cardiovasc. Surg. 2018; 156: 278–286. DOI: 10.1016/j.jtcvs.2018.02.038Sadr I.M., Tan P.E., Kieran M.W., Jenkins K.J. Mechanism of pulmonary vein stenosis in infants with normally connected veins. Am. J. Cardiol. 2000; 86: 577–579. DOI: 10.1016/S0002–9149(00)01022–5Sadr I.M., Tan P.E., Kieran M.W., Jenkins K.J. Mechanism of pulmonary vein stenosis in infants with normally connected veins. Am. J. Cardiol. 2000; 86: 577–579. DOI: 10.1016/S0002–9149(00)01022–5Frank D.B., Levy P.T., Stiver C.A., Boe B.A., Baird C.W., Callahan R.M., et al. Primary pulmonary vein stenosis during infancy: State of the art review. J. Perinatol. 2021; 41: 1528–1539. DOI: 10.1038/s41372–021–01008–7Frank D.B., Levy P.T., Stiver C.A., Boe B.A., Baird C.W., Callahan R.M., et al. Primary pulmonary vein stenosis during infancy: State of the art review. J. Perinatol. 2021; 41: 1528–1539. DOI: 10.1038/s41372–021–01008–7Mahgoub L., Kaddoura T., Kameny A.R., Lopez Ortego P., Vanderlaan R.D., Kakadekar A., et al. Pulmonary vein stenosis of ex-premature infants with pulmonary hypertension and bronchopulmonary dysplasia, epidemiology, and survival from a multicenter cohort. Pediatr. Pulmonol. 2017; 52: 1063–1070. DOI: 10.1002/ppul.23679Mahgoub L., Kaddoura T., Kameny A.R., Lopez Ortego P., Vanderlaan R.D., Kakadekar A., et al. Pulmonary vein stenosis of ex-premature infants with pulmonary hypertension and bronchopulmonary dysplasia, epidemiology, and survival from a multicenter cohort. Pediatr. Pulmonol. 2017; 52: 1063–1070. DOI: 10.1002/ppul.23679Jensen E.A., Dysart K., Gantz M.G., McDonald S., Bamat N.A., Keszler M., et al. The Diagnosis of Bronchopulmonary Dysplasia in Very Preterm Infants. An Evidence-based Approach. Am. J. Respir. Crit. Care Med. 2019; 200: 751–759. DOI: 10.1164/rccm.201812–2348OCJensen E.A., Dysart K., Gantz M.G., McDonald S., Bamat N.A., Keszler M., et al. The Diagnosis of Bronchopulmonary Dysplasia in Very Preterm Infants. An Evidence-based Approach. Am. J. Respir. Crit. Care Med. 2019; 200: 751–759. DOI: 10.1164/rccm.201812–2348OCDiLorenzo M.P., Santo A., Rome J.J., Zhang H., Faerber J.A., Mercer-Rosa L., Hopper R.K. Pulmonary Vein Stenosis: Outcomes in Children With Congenital Heart Disease and Prematurity. Semin. Thorac. Cardiovasc. Surg. 2019; 31: 266–273. DOI: 10.1053/j.semtcvs.2018.09.027DiLorenzo M.P., Santo A., Rome J.J., Zhang H., Faerber J.A., Mercer-Rosa L., Hopper R.K. Pulmonary Vein Stenosis: Outcomes in Children With Congenital Heart Disease and Prematurity. Semin. Thorac. Cardiovasc. Surg. 2019; 31: 266–273. DOI: 10.1053/j.semtcvs.2018.09.027Levy P.T., Jain A., Nawaytou H., Teitel D., Keller R.L., Fineman J., et al: Pediatric Pulmonary Hypertension Network (PPHNet). Risk Assessment and Monitoring of Chronic Pulmonary Hypertension in Premature Infants. J. Pediatr. 2019; 217: 199–209.e4. DOI: 10.1016/j.jpeds.2019.10.034Levy P.T., Jain A., Nawaytou H., Teitel D., Keller R.L., Fineman J., et al: Pediatric Pulmonary Hypertension Network (PPHNet). Risk Assessment and Monitoring of Chronic Pulmonary Hypertension in Premature Infants. J. Pediatr. 2019; 217: 199–209.e4. DOI: 10.1016/j.jpeds.2019.10.034Laux D., Rocchisani M.-A., Boudjemline Y., Gouton M., Bonnet D., Ovaert C. Pulmonary Hypertension in the Preterm Infant with Chronic Lung Disease can be Caused by Pulmonary Vein Stenosis: A MustKnow Entity. Pediatr. Cardiol. 2016; 37: 313–321. DOI: 10.1007/s00246–015–1279–1Laux D., Rocchisani M.-A., Boudjemline Y., Gouton M., Bonnet D., Ovaert C. Pulmonary Hypertension in the Preterm Infant with Chronic Lung Disease can be Caused by Pulmonary Vein Stenosis: A MustKnow Entity. Pediatr. Cardiol. 2016; 37: 313–321. DOI: 10.1007/s00246–015–1279–1Heching H.J., Turner M., Farkouh-Karoleski C., Krishnan U. Pulmonary vein stenosis and necrotising enterocolitis: Is there a possible link with necrotising enterocolitis? Arch. Dis. Child. Fetal Neonatal Ed. 2014; 99:F282–F285. DOI: 10.1136/archdischild-2013–304740Heching H.J., Turner M., Farkouh-Karoleski C., Krishnan U. Pulmonary vein stenosis and necrotising enterocolitis: Is there a possible link with necrotising enterocolitis? Arch. Dis. Child. Fetal Neonatal Ed. 2014; 99:F282–F285. DOI: 10.1136/archdischild-2013–304740Benjamin J.T., Hamm C.R., Zayek M., Eyal F.G., Carlson S., Manci E. Acquired left-sided pulmonary vein stenosis in an extremely premature infant: A new entity? J. Pediatr. 2009; 154: 459–459.e1. DOI: 10.1016/j.jpeds.2008.08.022Benjamin J.T., Hamm C.R., Zayek M., Eyal F.G., Carlson S., Manci E. Acquired left-sided pulmonary vein stenosis in an extremely premature infant: A new entity? J. Pediatr. 2009; 154: 459–459.e1. DOI: 10.1016/j.jpeds.2008.08.022Swier N.L., Richards B., Cua C.L., Lynch S.K., Yin H., Nelin L.D., et al. Pulmonary Vein Stenosis in Neonates with Severe Bronchopulmonary Dysplasia. Am. J. Perinatol. 2016; 33: 671–677. DOI: 10.1055/s-0035–1571201Swier N.L., Richards B., Cua C.L., Lynch S.K., Yin H., Nelin L.D., et al. Pulmonary Vein Stenosis in Neonates with Severe Bronchopulmonary Dysplasia. Am. J. Perinatol. 2016; 33: 671–677. DOI: 10.1055/s-0035–1571201Malloy K.W., Austin E.D. Pulmonary hypertension in the child with bronchopulmonary dysplasia. Pediatr. Pulmonol. 2021; 56: 3546–3556. DOI: 10.1002/ppul.25602Malloy K.W., Austin E.D. Pulmonary hypertension in the child with bronchopulmonary dysplasia. Pediatr. Pulmonol. 2021; 56: 3546–3556. DOI: 10.1002/ppul.25602de Wijs-Meijler D.P., Duncker D.J., Tibboel D., Schermuly R.T., Weissmann N., Merkus D., Reiss I.K.M. Oxidative injury of the pulmonary circulation in the perinatal period: Short- and long-term consequences for the human cardiopulmonary system. Pulm. Circ. 2017; 7: 55–66. DOI: 10.1086/689748de Wijs-Meijler D.P., Duncker D.J., Tibboel D., Schermuly R.T., Weissmann N., Merkus D., Reiss I.K.M. Oxidative injury of the pulmonary circulation in the perinatal period: Short- and long-term consequences for the human cardiopulmonary system. Pulm. Circ. 2017; 7: 55–66. DOI: 10.1086/689748Altit G., Bhombal S., Hopper R.K., Tacy T.A., Feinstein J. Death or resolution: The “natural history” of pulmonary hypertension in bronchopulmonary dysplasia. J. Perinatol. 2019; 39: 415–425. DOI: 10.1038/s41372–018–0303–8Altit G., Bhombal S., Hopper R.K., Tacy T.A., Feinstein J. Death or resolution: The “natural history” of pulmonary hypertension in bronchopulmonary dysplasia. J. Perinatol. 2019; 39: 415–425. DOI: 10.1038/s41372–018–0303–8Zettler E., Rivera B.K., Stiver C., Boe B., Cua C., Ball M.K., et al. Primary pulmonary vein stenosis among premature infants with single-vessel disease. J. Perinatol. 2021; 41: 1621–1626. DOI: 10.1038/s41372–020–00830–9Zettler E., Rivera B.K., Stiver C., Boe B., Cua C., Ball M.K., et al. Primary pulmonary vein stenosis among premature infants with single-vessel disease. J. Perinatol. 2021; 41: 1621–1626. DOI: 10.1038/s41372–020–00830–9Drossner D.M., Kim D.W., Maher K.O., Mahle W.T. Pulmonary Vein Stenosis: Prematurity and Associated Conditions. Pediatrics. 2008; 122:e656–e661. DOI: 10.1542/peds.2008–0075Drossner D.M., Kim D.W., Maher K.O., Mahle W.T. Pulmonary Vein Stenosis: Prematurity and Associated Conditions. Pediatrics. 2008; 122:e656–e661. DOI: 10.1542/peds.2008–0075Zaidi A.H., Yamada J.M., Miller D.T., McEnaney K., Ireland C., Roberts A.E., et al. Clinical Syndromic Phenotypes and the Potential Role of Genetics in Pulmonary Vein Stenosis. Children. 2021; 8: 128. DOI: 10.3390/children8020128Zaidi A.H., Yamada J.M., Miller D.T., McEnaney K., Ireland C., Roberts A.E., et al. Clinical Syndromic Phenotypes and the Potential Role of Genetics in Pulmonary Vein Stenosis. Children. 2021; 8: 128. DOI: 10.3390/children8020128Seale A.N., Webber S.A., Uemura H., Partridge J., Roughton M., Ho S.Y., et al. Pulmonary vein stenosis: The UK, Ireland and Sweden collaborative study. Heart. 2009; 95: 1944–1949. DOI: 10.1136/hrt.2008.161356Seale A.N., Webber S.A., Uemura H., Partridge J., Roughton M., Ho S.Y., et al. Pulmonary vein stenosis: The UK, Ireland and Sweden collaborative study. Heart. 2009; 95: 1944–1949. DOI: 10.1136/hrt.2008.161356Quinonez L.G., Gauvreau K., Borisuk M., Ireland C., Marshall A.M., Mayer J.E., et al. Outcomes of surgery for young children with multivessel pulmonary vein stenosis. J. Thorac. Cardiovasc. Surg. 2015; 150: 911–917. DOI: 10.1016/j.jtcvs.2015.06.050Quinonez L.G., Gauvreau K., Borisuk M., Ireland C., Marshall A.M., Mayer J.E., et al. Outcomes of surgery for young children with multivessel pulmonary vein stenosis. J. Thorac. Cardiovasc. Surg. 2015; 150: 911–917. DOI: 10.1016/j.jtcvs.2015.06.050

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