Abstract
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare precapillary form of pulmonary hypertension that differs from all other forms of PH. Timely diagnosis of CTEPH in children is difficult because clinical symptoms are nonspecific or absent in the early stages of CTEPH. Because the etiology of PH is very diverse, a comprehensive approach is needed to achieve an accurate diagnosis and treatment plan. Patients should be carefully screened for underlying hypercoagulable conditions. A multidisciplinary team, including pediatric cardiologists specializing in pulmonary hypertension, hematologists, cardiac surgeons, and interventionalists, is needed in the diagnosis and treatment of CTEPH.
References
- Wilkens H., Konstantinides S., Lang I.M., Bunck A.C., Gerges M., Gerhardt F. et al. Chronic thromboembolic pulmonary hypertension (CTEPH): Updated Recommendations from the Cologne Consensus Conference 2018. Int. J. Cardiol. 2018; 272S: 69–78. DOI: 10.1016/j.ijcard.2018.08.079
- Madani M.M., Wittine L.M., Auger W.R., Fedullo P.F., Kerr K.M., Kim N.H. Chronic thromboembolic pulmonary hypertension in pediatric patients. J. Thorac. Cardiovasc. Surg. 2011; 624–30. DOI: 10.1016/j.jtcvs.2010.07.010
- Verbelen T., Cools B., Fejzic Z., Van Den Eynde R., Maleux G., Delcroix M., Meyns B. Pulmonary endarterectomy in a 12-year-old boy with multiple comorbidities. Pulmonary Circulation. 2019; 9 (4) 1–4. DOI: 10.1177/2045894019886249
- Ma F., Zhou K., Hua Y., Liu X., Duan H., Li Y., Wang C. Chronic thromboembolic pulmonary hypertension as the first manifestation of nephrotic syndrome in a 12-year-old child. Medicine (Baltimore). 2018; 97 (41): e12349. DOI: 10.1097/MD.0000000000012349
- Rosenzweig E.B., Abman S.H., Adatia I., Beghetti M., Bonnet D., Haworth S. et al. Paediatric pulmonary arterial hypertension: Updates on definition, classification, diagnostics and management. Eur. Respir. J. 2019; 53. DOI: 10.1183/13993003.01916-2018
- Klok F.A., Dzikowska-Diduch O., Kostrubiec M., Vliegen H.W., Pruszczyk P., Hasenfuß G. et al., Derivation of a clinical prediction score for chronic thromboembolic pulmonary hypertension after acute pulmonary embolism. J. Thromb. Haemost. 2016; 14 (1): 121–8. DOI: 10.1111/jth.13175
- Galié N., Humbert M., Vachiery J.L., Gibbs S., Lang I., Torbicki A. et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur. Heart J. 2016; 37 (1): 67–119. DOI: 10.1093/eurheartj/ehv317
- Konstantinides S.V., Meyer G., Becattini C., Bueno H., Geersing G.J., Harjola V.P. et al. ESC Scientific Document Group. 2019 ESC Guidelines for the diagnosis and management of acute pulmonary embolism developed in collaboration with the European Respiratory Society (ERS). Eur. Heart J. 2020; 41 (4): 543–603. DOI: 10.1093/eurheartj/ehz405
- Kim N.H., Delcroix M., Jais X., Madani M.M., Matsubara H., Mayer E. et al. Chronic thromboembolic pulmonary hypertension. Eur. Respir. J. 2019; 53: 1801915. DOI: 10.1183/13993003.01915-2018
- Beghetti M., Berger R.M., Schulze-Neick I., Day R.W., Pulido T., Feinstein J. et al. Diagnostic evaluation of paediatric pulmonary hypertension in current clinical practice. Eur. Respir. J. 2013; 42: 689–700. DOI: 10.1183/09031936.00140112
- Hansmann G., Koestenberger M., Alastalo T.P., Apitz C., Austin E.D., Bonnet D. et al. 2019 updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: The European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT. J. Heart Lung Transplant. 2019; 38 (9): 879–901. DOI: 10.1016/j.healun.2019.06.022
- Berger R.M., Beghetti M., Humpl T., Raskob G.E., Ivy D.D., Jing Z.C. et al. Clinical features of paediatric pulmonary hypertension: A registry study. Lancet. 2012; 379: 537–46.
- Zotova I.V., Zateyshchikov D.A. Inherited thrombophilia and venous thromboembolism: testing rules in clinical practice. Russian Journal of Cardiology. 2020; 25 (3S): 4024 (in Russ.). DOI: 10.15829/1560-4071-2020-4024
- Bokeria L.A., Gorbachevskiy S.V., Shkol’nikova M.A. Guidelines on pulmonary hypertension in children. Moscow, 2013 (in Russ.).
- Skvortsov V.V., Mabudzada Ch.K. Hereditary thrombophilia due to congenital antithrombin III deficiency. Etiology, pathogenesis, diagnosis and treatment. Medical facility laboratory. 2018; 13: 7–15 (in Russ.).
- Hansmann G. Pulmonary Hypertension in Infants, Children, and Young Adults. J. Am. Coll. Cardiol. 2017; 69 (20): 2551–69. DOI: 10.1016/j.jacc.2017.03.575
- Olguntürk F.R. An update on the diagnosis and treatment of pediatric pulmonary hypertension. Expert. Opin. Pharmacother. 2020; 21 (10): 1253–68. DOI: 10.1080/14656566. 2020.1757071
- Beghetti M., Gorenflo M., Ivy D.D., Moledina S., Bonnet D. Treatment of pediatric pulmonary arterial hypertension: A focus on the NO-sGC-cGMP pathway. Pediatr. Pulmonol. 2019; 54 (10): 1516–26. DOI: 10.1002/ppul.24442
- Lang I., Meyer B.C., Ogo T., Matsubara H., Kurzyna M., Ghofrani H.-A., et al. Balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension. Eur. Respir. Rev. 2017; 26: 160119. DOI: 10.1183/16000617.0119-2016
About the authors
- Dinara I. Sadykova, Dr. Med. Sci., Professor, Head of the Department; ORCID
- Dina R. Sabirova, Cand. Med. Sci., Associate Professor; ORCID
- Ilsiia R. Gataullina, Resident Physician; ORCID
