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Pulmonary Endarterectomy for Chronic Thromboembolic Pulmonary Hypertension in a 5-year-old Girl with No Risk Factors - A Case Report

Received: 13 May 2022    Accepted: 31 May 2022    Published: 16 June 2022
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Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) in pediatric population is rare. We present a case of five-year-old female child, diagnosed as CTEPH on warfarin and home oxygen for 6 months. Imaging showed extensive thrombus in distal right pulmonary artery (RPA) than left pulmonary artery (LPA). She clinically deterioration inspite of maximal medical management and was referred for surgical option. Considering lack of literature evidence on surgical outcomes in pediatric CTEPH, decision to perform surgery was made after multi-disciplinary team discussion between adult and paediatric pulmonary hypertension and cardiothoracic surgical teams across two units in UK. We performed pulmonary thromboendarterectomy (PEA) using cardiopulmonary bypass under deep hypothermic circulatory arrest (DHCA). The surgical procedure was uneventful with good clinical recovery. Our report highlights the need for earlier and prompt diagnosis with a need for referral to a specialized surgical center at an earlier stage before intractable vascular resistance ensues. Surgery can be safely performed for CTEPH in pediatric populations.

Published in American Journal of Pediatrics (Volume 8, Issue 2)
DOI 10.11648/j.ajp.20220802.24
Page(s) 132-136
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

Pulmonary Endarterectomy, Pulmonary Hypertension, Chronic Thromboembolism

References
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[2] Nazzareno Galiè, Vallerie V. McLaughlin, Lewis J. Rubin, Gerald Simonneau: An overview of the 6th World Symposium on Pulmonary Hypertension. European Respiratory Journal 2019 53: 1802148; DOI: 10.1183/13993003.02148-2018.
[3] Jingi AM, Amougou SN, Jemea B, Ouankou CN, Foutko A, Ateba NA, Nkoke C. Low-pitch peripheral systolic murmur associated with pulmonary embolism in the acute phase: a report of two cases. Clinical Case Reports. 2018; 6 (4): 621–5. https://doi.org/10.1002/ccr3.141.
[4] Mirza H, Hashmi MF. Lung Ventilation Perfusion Scan (VQ Scan) [Updated 2021 Oct 21]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan. https://www.ncbi.nlm.nih.gov/books/NBK564428/
[5] Riedel M Diagnosing pulmonary embolism Postgraduate Medical Journal 2004; 80: 309-319.
[6] D'Souza, D., Bell, D. Pulmonary embolism. Reference article, Radiopaedia.org. https://doi.org/10.53347/rID-1937
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[8] Kim NH, Delcroix M, Jenkins DP, et al: Chronic thromboembolic pulmonary hypertension. J Am Coll Cardiol. 2013 Dec 24; 62 (25 Suppl): D92-9. doi: 10.1016/j.jacc.2013.10.024. PMID: 24355646.
[9] Fedullo P, Kerr KM, Kim NH, Auger WR. Chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med. 2011 Jun 15; 183 (12): 1605-13. doi: 10.1164/rccm.201011-1854CI. Epub 2011 Feb 17. PMID: 21330453.
[10] Andrew M, David M, Adams M, Ali K, Anderson R, Barnard D, et al. Venous thromboembolic complications (VTE) in children: first analyses of the Canadian Registry of VTE. Blood (1994) 83: 1251–7. 10.1002/jbmr.5650090615.
[11] Biss TT, Brandão LR, Kahr WH, Chan AK, Williams S. Clinical features and outcome of pulmonary embolism in children. Br J Haematol (2008) 142: 808–18. 10.1111/j.1365-2141.2008.07243.x.
[12] Van Ommen CH, Heijboer H, Büller HR, Hirasing RA, Heijmans HS, Peters M. Venous thromboembolism in childhood: a prospective two-year registry in the Netherlands. J Pediatr (2001) 139: 676–81. 10.1067/mpd.2001.118192.
[13] Stein PD, Kayali F, Olson RE. Incidence of venous thromboembolism in infants and children: data from the National Hospital Discharge Survey. J Pediatr (2004) 145: 563–5. 10.1016/j.jpeds.2004.06.021.
[14] Dijk FN, Curtin J, Lord D, Fitzgerald DA. Pulmonary embolism in children. Paediatr Respir Rev (2012) 13: 112–22. 10.1016/j.prrv.2011.09.002.
[15] Nagaya Noritoshi, Sasaki Noriko, Ando Motomi, Sakamaki Fumio et al. Prostacyclin Therapy Before Pulmonary Thromboendarterectomy in Patients With Chronic Thromboembolic Pulmonary Hypertension. Clinical Investigations Pulmonary Circulation| Volume 123, Issue 2, P338-343, February 01, 2003. https://doi.org/10.1378/chest.123.2.338
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  • APA Style

    Sudivya Sharma, Adeel Rahman, Arun Beeman, Shahin Moledina, Alex Robertson, et al. (2022). Pulmonary Endarterectomy for Chronic Thromboembolic Pulmonary Hypertension in a 5-year-old Girl with No Risk Factors - A Case Report. American Journal of Pediatrics, 8(2), 132-136. https://doi.org/10.11648/j.ajp.20220802.24

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    ACS Style

    Sudivya Sharma; Adeel Rahman; Arun Beeman; Shahin Moledina; Alex Robertson, et al. Pulmonary Endarterectomy for Chronic Thromboembolic Pulmonary Hypertension in a 5-year-old Girl with No Risk Factors - A Case Report. Am. J. Pediatr. 2022, 8(2), 132-136. doi: 10.11648/j.ajp.20220802.24

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    AMA Style

    Sudivya Sharma, Adeel Rahman, Arun Beeman, Shahin Moledina, Alex Robertson, et al. Pulmonary Endarterectomy for Chronic Thromboembolic Pulmonary Hypertension in a 5-year-old Girl with No Risk Factors - A Case Report. Am J Pediatr. 2022;8(2):132-136. doi: 10.11648/j.ajp.20220802.24

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  • @article{10.11648/j.ajp.20220802.24,
      author = {Sudivya Sharma and Adeel Rahman and Arun Beeman and Shahin Moledina and Alex Robertson and David Jenkins and Mirjana Cvetkovic and Nagarajan Muthialu},
      title = {Pulmonary Endarterectomy for Chronic Thromboembolic Pulmonary Hypertension in a 5-year-old Girl with No Risk Factors - A Case Report},
      journal = {American Journal of Pediatrics},
      volume = {8},
      number = {2},
      pages = {132-136},
      doi = {10.11648/j.ajp.20220802.24},
      url = {https://doi.org/10.11648/j.ajp.20220802.24},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajp.20220802.24},
      abstract = {Chronic thromboembolic pulmonary hypertension (CTEPH) in pediatric population is rare. We present a case of five-year-old female child, diagnosed as CTEPH on warfarin and home oxygen for 6 months. Imaging showed extensive thrombus in distal right pulmonary artery (RPA) than left pulmonary artery (LPA). She clinically deterioration inspite of maximal medical management and was referred for surgical option. Considering lack of literature evidence on surgical outcomes in pediatric CTEPH, decision to perform surgery was made after multi-disciplinary team discussion between adult and paediatric pulmonary hypertension and cardiothoracic surgical teams across two units in UK. We performed pulmonary thromboendarterectomy (PEA) using cardiopulmonary bypass under deep hypothermic circulatory arrest (DHCA). The surgical procedure was uneventful with good clinical recovery. Our report highlights the need for earlier and prompt diagnosis with a need for referral to a specialized surgical center at an earlier stage before intractable vascular resistance ensues. Surgery can be safely performed for CTEPH in pediatric populations.},
     year = {2022}
    }
    

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    T1  - Pulmonary Endarterectomy for Chronic Thromboembolic Pulmonary Hypertension in a 5-year-old Girl with No Risk Factors - A Case Report
    AU  - Sudivya Sharma
    AU  - Adeel Rahman
    AU  - Arun Beeman
    AU  - Shahin Moledina
    AU  - Alex Robertson
    AU  - David Jenkins
    AU  - Mirjana Cvetkovic
    AU  - Nagarajan Muthialu
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    N1  - https://doi.org/10.11648/j.ajp.20220802.24
    DO  - 10.11648/j.ajp.20220802.24
    T2  - American Journal of Pediatrics
    JF  - American Journal of Pediatrics
    JO  - American Journal of Pediatrics
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    EP  - 136
    PB  - Science Publishing Group
    SN  - 2472-0909
    UR  - https://doi.org/10.11648/j.ajp.20220802.24
    AB  - Chronic thromboembolic pulmonary hypertension (CTEPH) in pediatric population is rare. We present a case of five-year-old female child, diagnosed as CTEPH on warfarin and home oxygen for 6 months. Imaging showed extensive thrombus in distal right pulmonary artery (RPA) than left pulmonary artery (LPA). She clinically deterioration inspite of maximal medical management and was referred for surgical option. Considering lack of literature evidence on surgical outcomes in pediatric CTEPH, decision to perform surgery was made after multi-disciplinary team discussion between adult and paediatric pulmonary hypertension and cardiothoracic surgical teams across two units in UK. We performed pulmonary thromboendarterectomy (PEA) using cardiopulmonary bypass under deep hypothermic circulatory arrest (DHCA). The surgical procedure was uneventful with good clinical recovery. Our report highlights the need for earlier and prompt diagnosis with a need for referral to a specialized surgical center at an earlier stage before intractable vascular resistance ensues. Surgery can be safely performed for CTEPH in pediatric populations.
    VL  - 8
    IS  - 2
    ER  - 

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Author Information
  • Department of Cardiothoracic Surgery and Cardiac Intensive Care Unit, Great Ormond Street Hospital for Children, London, United Kingdom

  • Department of Cardiothoracic Surgery and Cardiac Intensive Care Unit, Great Ormond Street Hospital for Children, London, United Kingdom

  • Department of Cardiothoracic Surgery and Cardiac Intensive Care Unit, Great Ormond Street Hospital for Children, London, United Kingdom

  • Department of Cardiothoracic Surgery and Cardiac Intensive Care Unit, Great Ormond Street Hospital for Children, London, United Kingdom

  • Department of Cardiothoracic Surgery and Cardiac Intensive Care Unit, Great Ormond Street Hospital for Children, London, United Kingdom

  • Department of Cardiothoracic Surgery, Royal Papworth Hospital NHS Foundation Trust, Cambridge, United Kingdom

  • Department of Cardiothoracic Surgery and Cardiac Intensive Care Unit, Great Ormond Street Hospital for Children, London, United Kingdom

  • Department of Cardiothoracic Surgery and Cardiac Intensive Care Unit, Great Ormond Street Hospital for Children, London, United Kingdom

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