Factors associated with the uncorrectable congenital heart disease in children with pulmonary arterial hypertension

  • Handoyo Department of Child Health, Faculty of Medicine, Universitas Udayana, Sanglah Hospital, Bali, Indonesia https://orcid.org/0000-0002-7808-9346
  • Eka Gunawijaya Department of Child Health, Faculty of Medicine, Universitas Udayana, Sanglah Hospital, Bali, Indonesia
  • Ni Putu Veny Kartika Yantie Department of Child Health, Faculty of Medicine, Universitas Udayana, Sanglah Hospital, Bali, Indonesia https://orcid.org/0000-0002-1381-2572
DOI: https://doi.org/10.13181/mji.oa.236582
Keywords: children, congenital heart disease, heart septal defects, pulmonary hypertension
Abstract viewed: 295 times
PDF downloaded: 213 times
HTML downloaded: 86 times
EPUB downloaded: 74 times

Abstract

BACKGROUND Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a common complication of uncorrected left-to-right shunt defects in acyanotic CHD and a frequent type of pulmonary hypertension in youth. The standards for operability in left-to-right shunts with increased pulmonary vascular resistance are not universally agreed upon. This study aimed to identify variables associated with uncorrectable lesion in children with PAH-CHD.

METHODS This retrospective study used a database of all children who underwent cardiac catheterization at Sanglah Hospital, Bali, from May 2009 to April 2021. Pulmonary hypertension was defined as pulmonary artery pressure of >25 mmHg, while correctability was a fall of >20% in the pulmonary arterial resistance index (PARI) with final value of <6 WU/m2 when doing an acute vasoreactivity test using 100% oxygen. The analyses were carried out using SPSS software version 22.0 (IBM Corp., USA).

RESULTS A total of 104 children were included. Cardiac catheterization showed that the uncorrectable group had a higher PARI (14.4 [8.88] WU/m2 versus 8.43 [3.85] WU/m2) and lower flow ratio (1.27 [0.83] versus 1.47 [0.77]) at baseline. In terms of correctability, pre-tricuspid lesions (OR = 0.05; 95% CI = 0.01–0.47; p = 0.01) and younger age group (OR = 0.32; 95% CI = 0.12–0.85; p = 0.01) were protective variables, whilst high baseline PARI (OR = 4.54; 95% CI = 1.64–12.57; p = 0.01) was unfavorable.

CONCLUSIONS High baseline PARI was the most significant variable in predicting uncorrectable left-to-right shunt defects in PAH-CHD.

References

  1. Amedro P, Basquin A, Gressin V, Clerson P, Jais X, Thambo JB, et al. Health-related quality of life of patients with pulmonary arterial hypertension associated with CHD: the multicentre cross-sectional ACHILLE study. Cardiol Young. 2016;26(7):1250-9. https://doi.org/10.1017/S1047951116000056

  2. Bouma BJ, Mulder BJ. Changing landscape of congenital heart disease. Circ Res. 2017;120(6):908-22. https://doi.org/10.1161/CIRCRESAHA.116.309302

  3. Lewis RA, Armstrong I, Bergbaum C, Brewis MJ, Cannon J, Charalampopoulos A, et al. EmPHasis-10 health-related quality of life score predicts outcomes in patients with idiopathic and connective tissue disease-associated pulmonary arterial hypertension: results from a UK multicentre study. Eur Respir J. 2021;57(2):2000124. https://doi.org/10.1183/13993003.00124-2020

  4. Manes A, Palazzini M, Leci E, Bacchi Reggiani ML, Branzi A, Galiè N. Current era survival of patients with pulmonary arterial hypertension associated with congenital heart disease: a comparison between clinical subgroups. Eur Heart J. 2014;35(11):716-24. https://doi.org/10.1093/eurheartj/eht072

  5. Hemnes AR, Beck GJ, Newman JH, Abidov A, Aldred MA, Barnard J, et al. PVDOMICS: a multi-center study to improve understanding of pulmonary vascular disease through phenomics. Circ Res. 2017;121(10):1136-9. https://doi.org/10.1161/CIRCRESAHA.117.311737

  6. Axt-Fliedner R, Schwarze A, Smrcek J, Germer U, Krapp M, Gembruch U. Isolated ventricular septal defects detected by color Doppler imaging: evolution during fetal and first year of postnatal life. Ultrasound Obstet Gynecol. 2006;27(3):266-73. https://doi.org/10.1002/uog.2716

  7. Behjati-Ardakani M, Golshan M, Akhawan-Karbasi S, Hosseini S, Behjati-Ardakani M, Sarebanhassanabadi M. The clinical course of patients with atrial septal defects. Iran J Pediatr. 2016; 26(4):e4649. https://doi.org/10.5812/ijp.4649

  8. Fernando R, Koranne K, Loyalka P, Kar B, Gregoric I. Patent ductus arteriosus closure using an AmplatzerTM ventricular septal defect closure device. Exp Clin Cardiol. 2013;18(1):e50-4.

  9. Chinawa JM, Chukwu BF, Chinawa AT, Duru CO. The effects of ductal size on the severity of pulmonary hypertension in children with patent ductus arteriosus (PDA): a multi‑center study. BMC Pulm Med. 2021;21:79. https://doi.org/10.1186/s12890-021-01449-y

  10. Berger RM, Beghetti M, Humpl T, Raskob GE, Ivy DD, Jing ZC, et al. Clinical features of paediatric pulmonary hypertension: a registry study. Lancet. 2012;379(9815):537-46. https://doi.org/10.1016/S0140-6736(11)61621-8

  11. Bush D, Galambos C, Ivy DD, Abman SH, Wolter-Warmerdam K, Hickey F. Clinical characteristics and risk factors for developing pulmonary hypertension in children with Down syndrome. J Pediatr. 2018;202:212-9.e2. https://doi.org/10.1016/j.jpeds.2018.06.031

  12. Sharma M, Khera S, Sondhi V, Devgan A. A study to determine the prevalence of pulmonary arterial hypertension in children with Down syndrome and congenital heart disease. Med J Armed Forces India. 2013;69(3):241-5. https://doi.org/10.1016/j.mjafi.2012.11.013

  13. Galambos C, Minic AD, Bush D, Nguyen D, Dodson B, Seedorf G, et al. Increased lung expression of anti-angiogenic factors in down syndrome: potential role in abnormal lung vascular growth and the risk for pulmonary hypertension. PLoS One. 2016;11(8):e0159005. https://doi.org/10.1371/journal.pone.0159005

  14. Inrianto W, Murni IK, Safitri I. Predictors of pulmonary hypertension in children with left-to-right shunting in acyanotic congenital heart disease. Paediatr Indones. 2021;61(3):119-24. https://doi.org/10.14238/pi61.3.2021.119-24

  15. Chiu SN, Weng KP, Lin MC, Wang JN, Hwang BT, Dai ZK, et al. Congenital heart disease with pulmonary artery hypertension in an Asian cohort-initial report from TACHYON (TAiwan congenital heart disease associated with pulmonarY arterial hypertension) registry. Int J Cardiol. 2020;317:49-55. https://doi.org/10.1016/j.ijcard.2020.05.086

  16. Sommer RJ, Hijazi ZM, Rhodes JF Jr. Pathophysiology of congenital heart disease in the adult: part I: shunt lesions. Circulation. 2008;117(8):1090-9. https://doi.org/10.1161/CIRCULATIONAHA.107.714402

  17. Backes CH, Hill KD, Shelton EL, Slaughter JL, Lewis TR, Weisz DE, et al. Patent ductus arteriosus: a contemporary perspective for the pediatric and adult cardiac care provider. J Am Heart Assoc. 2022;11(17):e025784. https://doi.org/10.1161/JAHA.122.025784

  18. Ivy DD, Abman SH, Barst RJ, Berger RM, Bonnet D, Fleming TR, et al. Pediatric pulmonary hypertension. J Am Coll Cardiol. 2013;62(25 Suppl):D117-26. https://doi.org/10.1016/j.jacc.2013.10.028

  19. Galiè N, Corris PA, Frost A, Girgis RE, Granton J, Jing ZC, et al. Updated treatment algorithm of pulmonary arterial hypertension. J Am Coll Cardiol. 2013;62(25 Suppl):D60-72. https://doi.org/10.1016/j.jacc.2013.10.031

  20. Beghetti M, Berger RM, Schulze-Neick I, Day RW, Pulido T, Feinstein J, et al. Diagnostic evaluation of paediatric pulmonary hypertension in current clinical practice. Eur Respir J. 2013;42(3):689-700. https://doi.org/10.1183/09031936.00140112

  21. Apitz C, Hansmann G, Schranz D. Hemodynamic assessment and acute pulmonary vasoreactivity testing in the evaluation of children with pulmonary 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. Heart. 2016;102 Suppl 2:ii23-9. https://doi.org/10.1136/heartjnl-2014-307340

  22. Wu Y, Liu HM, Gu L, Li QW, Zhu L. Prostacyclins and pulmonary arterial hypertension in children. Eur Rev Med Pharmacol Sci. 2022;26(1):37-45.

  23. Kuang H, Li Q, Yi Q, Lu T. The efficacy and safety of aerosolized iloprost in pulmonary arterial hypertension: a systematic review and meta-analysis. Am J Cardiovasc Drugs. 2019;19(4):393-401. https://doi.org/10.1007/s40256-018-00324-2

  24. Takatsuki S, Parker DK, Doran AK, Friesen RH, Ivy DD. Acute pulmonary vasodilator testing with inhaled treprostinil in children with pulmonary arterial hypertension. Pediatr Cardiol. 2013;34(4):1006-12. https://doi.org/10.1007/s00246-012-0597-9

  25. Apitz C, Reyes JT, Holtby H, Humpl T, Redington AN. Pharmacokinetic and hemodynamic responses to oral sildenafil during invasive testing in children with pulmonary hypertension. J Am Coll Cardiol. 2010;55(14):1456-62. https://doi.org/10.1016/j.jacc.2009.11.065

  26. Ajami GH, Borzoee M, Radvar M, Amoozgar H. Comparison of the effectiveness of oral sildenafil versus oxygen administration as a test for feasibility of operation for patients with secondary pulmonary arterial hypertension. Pediatr Cardiol. 2008;29(3):552-5. https://doi.org/10.1007/s00246-007-9139-2

  27. Douwes JM, Humpl T, Bonnet D, Beghetti M, Ivy DD, Berger RM; TOPP Investigators. Acute vasodilator response in pediatric pulmonary arterial hypertension: current clinical practice from the TOPP registry. J Am Coll Cardiol. 2016;67(11):1312-23. https://doi.org/10.1016/j.jacc.2016.01.015

  28. Hansmann G, Apitz C. Treatment of children with pulmonary hypertension. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK. Heart. 2016;102 Suppl 2:ii67-85. https://doi.org/10.1136/heartjnl-2015-309103

  29. Kozlik-Feldmann R, Hansmann G, Bonnet D, Schranz D, Apitz C, Michel-Behnke I. Pulmonary hypertension in children with congenital heart disease (PAH-CHD, PPHVD-CHD). Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK. Heart. 2016;102 Suppl 2:ii42-8. https://doi.org/10.1136/heartjnl-2015-308378

  30. Day RW. Improving guidance for the correctability of congenital cardiovascular shunts with increased pulmonary vascular resistance. Int J Cardiol Congenit Heart Dis. 2021;4:100184. https://doi.org/10.1016/j.ijcchd.2021.100184

  31. Jarutach J, Roymanee S, Wongwaitaweewong K. Survival of patients after left-to-right shunt repaired of congenital heart defect: a comparison between baseline pulmonary vascular resistances. J Health Sci Med Res. 2020;39(1):13-21. https://doi.org/10.31584/jhsmr.2020755

  32. Lopes A, Alnajashi K. Saudi guidelines on the diagnosis and treatment of pulmonary hypertension: pulmonary arterial hypertension associated with congenital heart disease. Ann Thorac Med. 2014;9(Suppl 1):S21-5. https://doi.org/10.4103/1817-1737.134015

Published
2023-07-17
How to Cite
1.
Handoyo, Gunawijaya E, Yantie NPVK. Factors associated with the uncorrectable congenital heart disease in children with pulmonary arterial hypertension. Med J Indones [Internet]. 2023Jul.17 [cited 2024May8];32(1):38-4. Available from: http://mji.ui.ac.id/journal/index.php/mji/article/view/6582
Issue
Vol. 32 No. 1 (2023): March
Section
Clinical Research

Copyright (c) 2023 Handoyo, Eka Gunawijaya, Ni Putu Veny Kartika Yantie

Creative Commons License

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

Authors who publish with Medical Journal of Indonesia agree to the following terms:

  1. Authors retain copyright and grant Medical Journal of Indonesia right of first publication with the work simultaneously licensed under a Creative Commons Attribution-NonCommercial License that allows others to remix, adapt, build upon the work non-commercially with an acknowledgment of the work’s authorship and initial publication in Medical Journal of Indonesia.
  2. Authors are permitted to copy and redistribute the journal's published version of the work non-commercially (e.g., post it to an institutional repository or publish it in a book), with an acknowledgment of its initial publication in Medical Journal of Indonesia.