Online ISSN: 3007-0244,
Print ISSN:  2410-4280
CONGENITAL HEART DISEASES IN CHILDREN COMPLICATED BY PULMONARY HYPERTENSION. DIAGNOSIS AND TREATMENT. LITERATURE REVIEW

Introduction. Congenital heart diseases (CHD) occupy one of the leading places in the structure of the incidence of children and adolescents. The study of congenital heart anomalies, methods of diagnosis and treatment began in the 30s of the XX century. And to date, this problem is relevant for further study, since there are still no algorythm of early diagnostic of the CHD. The most frequent and severe complication of many CHD is pulmonary hypertension, which, when untimely surgical treatment leads to disability and early infant mortality.

Purpose: To study and analyze the literature data on the diagnosis and treatment of children with congenital heart disease complicated by pulmonary hypertension.

Search strategy: we conducted a search and analysis of scientific publications in the databases Pubmed, Medline, Web of Knowledge, Scientific electronic library. The depth of the search was 41 years, from 1975 to 2016.

The criteria for inclusion of the source in the review were: reports on randomized and cohort studies conducted on large populations; meta-analyzes and systematic reviews, full versions of articles, protocols for the diagnosis and treatment of children with CHD for the last 5 years have also been studied. Literature in Russian and English.

Criteria for exclusion were: articles describing single cases, summaries of reports, personal reports and newspaper publications.

Results. A study of the literature on this topic shows that late diagnosis and untimely treatment of most of the CHD is complicated by pulmonary hypertension. This worsens the prognosis and postoperative course, affects the survival rate of children, increases the risk of disability and mortality.

Conclusions. The most important tasks of pediatric cardiology are optimization of diagnosis, prognosis and treatment of congenital heart defects complicated by pulmonary hypertension before and after surgical correction, which leads to an improvement in the quality of life of children and a decreasing of infant and child mortality.

Yelena I. Khagay 1, http://orcid.org/0000-0001-5323-4001

Guzyal D. Abilmazhinova 2, http://orcid.org/0000-0003-0007-9890

Pavlodar branch of Semey State Medical University

1 Master's Degree 2 course,

2 Department of Internal Medicine

Pavlodar city, Republic of Kazakhstan.

Agaipov L.I. Diagnostika i lechenie legochnoi arterial'noi gipertenzii u detei [Diagnosis and treatment of pulmonary arterial hypertension in children]. Lechashhii vrach, 2 chast' [Therapist], 2014. 4. p. 50-53, [in Russian]

2.         Belozerov Ju.M. Detskaya kardiologiya [Pediatric Cardiology]. M.: Med-press inform, 2004. - 600 p., [in Russian]

3.         Belokon' N.A., Kuberger M.B. Bolezni serdtsa i sosudov u detei [Diseases of the heart and blood vessels in children]. Tom 1, Moskva, 1987. - p. 216-240, [in Russian]

4.         Burakovskii V.I., Bokeriya L.A. Serdechno-sosudistaya khirurgiya: rukovodstvo. [Cardiovascular surgery]: guideline – M.: Medicina, 1989. – 752 p, [in Russian]

5.         Burakovskii V.I., Bukharin V.A., Plotnikova L.R. Legochnaya gipertenziya pri vrozhdennykh porokakh serdtsa. [Pulmonary hypertension in congenital heart disease] M.: Medicina; 1975. p. 247, [in Russian]

6.         Gorbachevskii S.V. Problema legochnoi gipertenzii v khirurgii VPS u detei rannego vozrasta: [The problem of pulmonary hypertension in surgery of CHD in children of early age.] Abstract. dis. … Doctor of Medical Sciences. M.; 1995. p.121, [in Russian]

7.         Duisenbaeva B.S., Avtoref. dis. … «Epidemiologicheskie aspekty i sravnitel'naya otsenka tomograficheskikh metodov diagnostiki pri vrozhdennykh porokakh serdechno-sosudistoi sistemy v Kazakhstane» [Abstract. dis. Epidemiological aspects and comparative evaluation of tomographic diagnostic methods for congenital malformations of the cardiovascular system in Kazakhstan]. Astana 2013, p. 22-26, [in Russian]

8.         Zin'kovskii M.F. Vrozhdennye poroki serdtsa / Pod red. A.F. Vozianova. [Congenital heart disease edited by A.F. Vozianova] — K.: Kniga plyus, [Book plus] 2008. – 1168 p, [in Russian]

9.         Kamalov I.I., Galyavich A.S., Molokovich G.N. Sovremennye aspekty luchevoi diagnostiki vrozhdennykh porokov serdtsa u novorozhdennykh i detei rannego vozrasta, [Modern aspects of radiation diagnosis of congenital heart disease in newborns and young children]. Kazanskii meditsinskii zhurnal, [Kazan Medical Journal]. 2006, T87, №1. p. 7-9, [in Russian]

10.       Legochnaya gipertenziya. [Pulmonary hypertension] Koncensus ACC/AHA 4 (9) (2009), http://www.medreview.com.ua/ [in Russian]

11.       Martynyuk T.V., Konosova I.D., Chazova I.E. Sovremennye podkhody k medikamentoznomu lecheniyu legochnoy gipertenzii [Modern approaches to drug treatment of pulmonary hypertension]. Consilium medicum. 2003; 5: p.1–4, [in Russian]

12.       Moshhich P.S., Sidel'nikov V.M., Krivchenya D.Yu. Kardiologija detskogo vozrasta, [Cardiology of childhood]. Kiev, 1986. p. 144-149, [in Russian]

13.       Mutaf'yan O.A., Detskaya kardiologiya. [Pediatric cardiology] – M.: GJeOTAR- Media, 2009. – p.170-177, [in Russian]

14.       Nikolaeva T.N. Gemomikrotsirkulyatsiya: patologija pri vrozhdennyh porokah serdca. [Hemocirculation: pathology in congenital heart disease] - M., 1996. 179 p, [in Russian]

15.       Orlov V.N. Rukovodstvo po elektrokardiografii. [Guide by ECG] M.: OOO «Meditsinskoye informatsionnoe agentstvo», [Medical news agency]. 1997, p.528, [in Russian]

16.       Prakhov A.V., Neonatal'naya kardiologiya. [Neonatal Cardiology] – N.Novgorod, 2008. – 388p, [in Russian]

17.       Shabalov N.P., Detskie bolezni, [Childhood diseases] tom 2, Piter, 2007, 928p, [in Russian]

18.       Shipulin V.M., Merunko A.A., Ivanov S.N. i dr. Voprosy patologii serdtsa u detei. [Questions of the heart pathology in children] - Tomsk: Krasnoe znamya [Red flag]. 2000. - 114 p. [in Russian]

19.       Shheglova K.T., Gornostaev A.A., Chernogrivov A.E., Bazylev V.V., Pervyi opyt primeneniya Bozentana v sostave kombinirovannoi terapii legochnoi gipertenzii v rannem posle operatsionnom periode u detei pervogo goda zhizni posle radikal'noi korrektsii vrozhdennykh porokov serdtsa s izbytochnym legochnym krovotokom. [The first drug use of Bosentan in the combination therapy of pulmonary hypertension in the early postoperative period in children of the first year of life after radical correction of congenital heart defects with excessive pulmonary circulation]. Detskie bolezni serdtsa i sosudov [Children's diseases of the heart and blood vessels]. 2015 №3; p.57-64, [in Russian]

20.       Ayhan Pektas, Rana Olgunturk, Ayhan Cevik, Semiha Terlemez, Emre Kasar and Yusuf Ali Oner. Texas Heart Institute Journal, Magnetic Resonance Imaging in Pediatric Pulmonary Hypertension 2015 Jun; 42 (3), p.209-215. [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4473612/] (дата обращения: 03.08.2017).

21.       Aleha.n D., Yildirim I., Sahin M.et al. Long-term inhaled iloprost use in children with pulmonary arterial hypertension, Cardiol Young. 2012; 22: p. 396–403.

22.       Astrid E. Lammers, Franz Freudenthal, Ian Adatia, et al. Functional classification of pulmonary hypertension in children: Report from the PVRI pediatric taskforce, Panama 2011. Pulmonary Circulation 2011; Vol 1: No 2; p. 280-285 [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3161406/]

23.       Barst R. J., McGoon M. D., Elliott C. G., Foreman A. J., Miller D. P.et al. Survival in childhood pulmonary arterial hypertension: insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management, Circulation. 2012; 125: p. 113–122. [https://www.ncbi.nlm.nih.gov/pubmed/22086881] (дата обращения 03.08.2017).

24.       Barst R. J., McGoon M., Torbicki A. et al. Diagnosis and differential assessment of pulmonary arterial hypertension, J Am Coll Cardiol. 2004; 43: р40–47. [http://www.sciencedirect.com/science/article/pii/S0735109704004413?via%3Dihub] (дата обращения 04.08.2017)

25.       Beghetti M. Bosentan in pediatric patients with pulmonary arterial hypertension, Curr Vasc Pharmacol. 2009; 7: p. 225–233. [https://www.ncbi.nlm.nih.gov/pubmed/?term] (дата обращения 21.08.2017).

26.       Beghetti M., Haworth S. G., Bonnet D. et al. Pharmacokinetic and clinical profile of a novel formulation of bosentan in children with pulmonary arterial hypertension: the FUTURE-1 study, Br J Clin Pharmacol. 2009; 68: p. 948–955. [https://www.ncbi.nlm.nih.gov/pubmed/?term] (дата обращения 21.08.2017)

27.       Beghetti M., Tissot C. Pulmonary Hypertension in Congenital Shunts. Rev. Esp. Cardiol.           2010;             63:       1179–93. [https://www.ncbi.nlm.nih.gov/pubmed/?term]. (дата обращения 21.08.2017)

28.       Dan-Chen Wu, Hong-Da Zhang, Zhi-Cheng Jing. Pediatric Pulmonary Arterial Hypertension, Curr Hypertens Rep. 2013 Oct 27. [https://link.springer.com/journal/11906]. (дата обращения 21.08.2017)

29.       David B. Frank and Brian D. Hanna. Pulmonary Arterial Hypertension Associated with Congenital Heart Disease and Eisenmenger Syndrome: Current Practice in Pediatrics. Minerva Pediatrica Journal, 2015 Apr, 67 (2): 169-185.

30.       ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension 2015.

31.       Galiè N., Hoeper M., Humbert M. Guidelines for the diagnosis and treatment of pulmonary hypertension, European Heart Journal. 2009; 30 (20): 2493–2537 [https://academic.oup.com/eurheartj/article-lookup/doi/10.1093/eurheartj/ehp297]. (дата обращения 21.08.2017)

32.       Heiner Latus, Titus Kuehne, Philipp Beerbaum, Christian Apitz, Georg Hansmann, Vivek Muthurangu, Shahin Moledina Cardiac MR and CT imaging in children with suspected or confirmed pulmonary hypertension/pulmonary hypertensive vascular disease. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK // Pulmonary vascular disease. Original article. 2016. №102. P. 31-34. [http://heart.bmj.com/content/102/Suppl_2/ii30]. (дата обращения 21.08.2017).

33.       Hislop A. A., Moledina S., Foster H. et al. Long-term efficacy of bosentan in treatment of pulmonary arterial hypertension in children, Eur Respir J. 2011; 38: p. 70–77.

34.       Hoffman J.I., Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002; p.1890-1900. [https://ac.els-cdn.com/S0735109702018867/1-s2.0-S0735109702018867-main.pdf]. (дата обращения 21.08.2017).

35.       Hoffman J.I., Kaplan S., Liberthson R.R. Prevalence of congenital heart disease. Am Heart J 2004; p.425-439

36.       Ivy D. D., Claussen L., Doran A. Transition of stable pediatric patients with pulmonary arterial hypertension from intravenous epoprostenol to intravenous treprostinil, Am J Cardiol. 2007; 99: p. 696–698.

37.       Ivy D. D., Rosenzweig E. B., Lemarie J. C.et al. Long-term outcomes in children with pulmonary arterial hypertension treated with bosentan in real-world clinical settings, Am J Cardiol. 2010; 106: p.1332–1338.

38.       Jonh F. Keane, James E. Lock, Donald C. Fyler, NADAS’ Pediatric Cardiology, second edition, 2006, p.43-49.

39.       Kouchoukos Nicholas T., Kirklin. James K., Blackstone Eugene H.., Hanley Frank L., Kirklin/Barrat-Boyes Cardiac Surgery. – 4-th ed., 2013. – p.2054

40.       Gabor Kovacs, Alexander Avian, Michael Pienn, Robert Naeije, and Horst Olschewski. Reading pulmonary vascular pressure tracings. How to handle the problems of zero leveling and respiratory swings. Am J Respir Crit Care Med 2014; p.252–257 [http://www.atsjournals.org/doi/full/10.1164/rccm.201402-0269PP]. (дата обращения 21.08.2017).

41.       Krishnan U., Takatsuki S., Ivy D. D. et al. Effectiveness and safety of inhaled treprostinil for the treatment of pulmonary arterial hypertension in children, Am J Cardiol. 2012; 110: p. 1704–1709. [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3508003/]. (дата обращения 21.08.2017).

42.       Laas E., Lelong N., Thieulin A.C., et al. Preterm birth and Congenital Heart Defects: A population based study. Pediatrics 2012; p.829-837. [http://pediatrics.aappublications.org/content/130/4/e829.full] (дата обращения 03.09.2017).

43.       Lammers A. E., Hislop A. A., Flynn Y., Haworth S. G. Epoprostenol treatment in children with severe pulmonary hypertension, Heart. 2007; 93(6). p. 739–743. [http://pubmedcentralcanada.ca/ptpicrender.fcgi?aid=1605607&blobtype=html&lang=en-ca] (дата обращения 03.09.2017)

44.       Marelli A. J. Mackie AS, Ionescu-Ittu R, Rahme E, Andrew S et al. Congenital heart disease in the general population: changing prevalence and age distribution // Circulation. 2007. P 163-172.

45.       Mavrodis C. Pediatric Cardiac Surgery. – 3-d ed. – Mosby, 2003. – p.875.

46.       Mereles D., Ehlken N., Kreuscher S., Ghofrani S., Hoeper M.M. et al. Exercise and respiratory training improve exercise capacity and quality of life in patients with severe chronic pulmonary hypertension, Circulation. 2006; 114: p.1482–1489. [http://circ.ahajournals.org/content/114/14/1482] (дата обращения 29.08.2017)

47.       Nagel E., Rossem A.C., Fleck E Bauer W, Sechtem U, Cardiovascular Magnetic Resonance. – Springer, 2003. – p. 270

48.       Rich S., Dantzker D. R., Ayres S., M. Bergofsky E.H., Brundage B.H. et al. Primary pulmonary hypertension. A national prospective study, Ann Intern Med. 1987; 107: p.16–223.

49.       Richard A. Jonas Comprehensive Surgical Management of Congenital Heart Disease, 2004. - p.544

50.       Rubin L.J. Primary pulmonary hypertension. N. Engl. J. Med. 1997; 336: 111–17.

51.       Sandoval J., Aguirre J. S., Pulido T. et al. Nocturnal oxygen therapy in patients with the Eisenmenger syndrome, Am J Respir Crit Care Med. 2001; 164: p. 1682–1687. [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3612812/] (дата обращения 29.08.2017)

52.       Stark J. Surgery for congenital heart defects. – 3-d ed. – John Wiley&Sons, 2006. – p.574.

53.       Van der Linde D., Konnings E., Slager M. et al. Birth prevalence of congenital heart disease wordwide: a systemic review and meta-analysis. J Am Cardiol 2011; p. 2241-2247.

54.       Wei-Zie Zeng, Xian-Ling Lu, Chang-Ming Xiong, Guang-Liang Shan, Zhi-Hong Liu et al The efficacy and safety of Sildenafil in patients with pulmonary arterial hypertension associated with the different types of congenital heart disease. Clinical Cardiology J. 2011, 34.8. p.513-518 [http://onlinelibrary.wiley.com] (дата обращения 29.08.2017)

55.       Widlitz A., Barst R.J. Pulmonary arterial hypertension in children. Eur. Respir. J. 2003; 21: 155–76. [http://erj.ersjournals.com/content/21/1/155.long]. (дата обращения 28.08.2017)

Number of Views: 1092


Category of articles: Reviews

Bibliography link

Khagay Ye.I., Abilmazhinova G.D. Congenital heart diseases in children complicated by pulmonary hypertension. Diagnosis and treatment. Literature review. Nauka i Zdravookhranenie [Science & Healthcare]. 2017, 5, pp. 129-144


Авторизируйтесь для отправки комментариев