International Journal of Anesthesia and Clinical Medicine

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Timely Surgical Intervention for Absent Pulmonary Valve Syndrome with an Intact Ventricular Septum in a Patient with a Long-Term Survival: A Case Report

Received: 31 January 2024    Accepted: 21 February 2024    Published: 29 February 2024
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Abstract

Absent pulmonary valve syndrome with an intact ventricular septum (APVS with IVS) is a rare congenital heart defect that is associated with a high perinatal mortality. Most fetuses can hard to survive without inotropic support or heart transplantation due to the severe respiratory dysfunction, heart failure and serious infections resulting from tracheobronchial compression and right ventricular dilation. Almost one-third of the patients opted for a termination of pregnancy. The etiology of APVS patients is still unknown. Previous research only noted an enhancement in the long-term survival of APVS with tetralogy of Fallot patients over the past decade. However, the timeliness of surgical intervention and survival status in APVS with IVS patients remains unclear. In our case, we described a 27-year-old patient with a rare combination of the rudimentary pulmonary valve, intact ventricular septum and absent superior vena cava who successfully developed biventricular physiology without any medical intervene. And he received a timely pulmonary valve implantation in our centre to prevent further right ventricular dysfunction. Thus, we highlighted that biventricular circulation can be successfully achieved in this rare subtype of APVS with IVS patients. However, more multicentre studies and lifelong follow-up are required to determine the timeliness for pulmonary valve surgery to avoid over-dilatation of the right ventricle in APVS with IVS patients.

DOI 10.11648/j.ijacm.20241201.14
Published in International Journal of Anesthesia and Clinical Medicine (Volume 12, Issue 1, June 2024)
Page(s) 15-19
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

Heart Defects, Congenital, Pulmonary Valve Insufficiency, Tricuspid Valve Insufficiency, Case Report

References
[1] Axt-Fliedner R, Kurkevych A, Slodki M, et al. Absent pulmonary valve syndrome - diagnosis, associations, and outcome in 71 prenatally diagnosed cases. Prenat Diagn. 2017; 37(8): 812-819. https://doi.org/10.1002/pd.5094
[2] Monacci F, Bondi T, Canessa C, Chiappa E. 'Absent' pulmonary valve with intact ventricular septum mimicking tricuspid valve atresia: Prenatal diagnosis and postnatal course. J Obstet Gynaecol Res. 2019; 45(3): 714-718. https://doi.org/10.1111/jog.13878
[3] Babaoğlu K, Doğan Y, Erdem S, Özbarlas N, Başar E, Uzun O. Prenatal Diagnosis, Associations and Outcome for Fetuses with Congenital Absence of the Pulmonary Valve Syndrome. Anatol J Cardiol. 2022; 26(9): 702-709. https://doi.org/10.5152/AnatolJCardiol.2022.1461
[4] Szwast A, Tian Z, McCann M, et al. Anatomic variability and outcome in prenatally diagnosed absent pulmonary valve syndrome. Ann Thorac Surg. 2014; 98(1): 152-158. https://doi.org/10.1016/j.athoracsur.2014.03.002
[5] Favilli S, Lapi E, Pollini I, Calabri GB, Bini RM. Prenatal diagnosis and postnatal outcome in patients with absent pulmonary valve syndrome not associated with tetralogy of Fallot: report of one case and review of the literature. J Cardiovasc Med (Hagerstown). 2008; 9(11): 1127-1129. https://doi.org/10.2459/JCM.0b013e3283100eb1
[6] Recker F, Weber EC, Strizek B, Geipel A, Berg C, Gembruch U. Management and outcome of prenatal absent pulmonary valve syndrome. Arch Gynecol Obstet. 2022; 306(5): 1449-1454. https://doi.org/10.1007/s00404-022-06397-4
[7] Wertaschnigg D, Jaeggi M, Chitayat D, et al. Prenatal diagnosis and outcome of absent pulmonary valve syndrome: contemporary single-center experience and review of the literature. Ultrasound Obstet Gynecol. 2013; 41(2): 162-167. https://doi.org/10.1002/uog.11193
[8] Avdikos V, Johansson Ramgren J, Hanséus K, Malm T, Liuba P. Outcomes following surgical repair of absent pulmonary valve syndrome: 30 years of experience from a Swedish tertiary referral centre. Interact Cardiovasc Thorac Surg. 2022; 35(2): ivac193. https://doi.org/10.1093/icvts/ivac193
[9] Rakha S, Alkhushi N. Fetal diagnosis of isolated absent pulmonary valve with intact interventricular septum: How to counsel the parents?. Ann Pediatr Cardiol. 2020; 13(2): 136-140. https://doi.org/10.4103/apc.APC_101_19
[10] Okajima K, Kawase Y, Hasegawa T, et al. Long-surviving patient with isolated absent pulmonary valve syndrome: A case report. Journal of Cardiology. 2005; 45(4): 165-171. PMID: 15875539.
[11] Yong MS, Yim D, Brizard CP, et al. Long-term outcomes of patients with absent pulmonary valve syndrome: 38 years of experience. Ann Thorac Surg. 2014; 97(5): 1671-1677. https://doi.org/10.1016/j.athoracsur.2014.01.035
[12] Torok K, Brettle E, Desai T, et al. Long-term outcomes in children with absent pulmonary valve syndrome: it is not just fixing the heart. Arch Dis Child. 2021; 106(9): 877-881. https://doi.org/10.1136/archdischild-2020-320219
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  • APA Style

    Zhang, Y., Luo, S., Wang, E. (2024). Timely Surgical Intervention for Absent Pulmonary Valve Syndrome with an Intact Ventricular Septum in a Patient with a Long-Term Survival: A Case Report. International Journal of Anesthesia and Clinical Medicine, 12(1), 15-19. https://doi.org/10.11648/j.ijacm.20241201.14

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    ACS Style

    Zhang, Y.; Luo, S.; Wang, E. Timely Surgical Intervention for Absent Pulmonary Valve Syndrome with an Intact Ventricular Septum in a Patient with a Long-Term Survival: A Case Report. Int. J. Anesth. Clin. Med. 2024, 12(1), 15-19. doi: 10.11648/j.ijacm.20241201.14

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    AMA Style

    Zhang Y, Luo S, Wang E. Timely Surgical Intervention for Absent Pulmonary Valve Syndrome with an Intact Ventricular Septum in a Patient with a Long-Term Survival: A Case Report. Int J Anesth Clin Med. 2024;12(1):15-19. doi: 10.11648/j.ijacm.20241201.14

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  • @article{10.11648/j.ijacm.20241201.14,
      author = {Yunyi Zhang and Shuhua Luo and Enping Wang},
      title = {Timely Surgical Intervention for Absent Pulmonary Valve Syndrome with an Intact Ventricular Septum in a Patient with a Long-Term Survival: A Case Report},
      journal = {International Journal of Anesthesia and Clinical Medicine},
      volume = {12},
      number = {1},
      pages = {15-19},
      doi = {10.11648/j.ijacm.20241201.14},
      url = {https://doi.org/10.11648/j.ijacm.20241201.14},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ijacm.20241201.14},
      abstract = {Absent pulmonary valve syndrome with an intact ventricular septum (APVS with IVS) is a rare congenital heart defect that is associated with a high perinatal mortality. Most fetuses can hard to survive without inotropic support or heart transplantation due to the severe respiratory dysfunction, heart failure and serious infections resulting from tracheobronchial compression and right ventricular dilation. Almost one-third of the patients opted for a termination of pregnancy. The etiology of APVS patients is still unknown. Previous research only noted an enhancement in the long-term survival of APVS with tetralogy of Fallot patients over the past decade. However, the timeliness of surgical intervention and survival status in APVS with IVS patients remains unclear. In our case, we described a 27-year-old patient with a rare combination of the rudimentary pulmonary valve, intact ventricular septum and absent superior vena cava who successfully developed biventricular physiology without any medical intervene. And he received a timely pulmonary valve implantation in our centre to prevent further right ventricular dysfunction. Thus, we highlighted that biventricular circulation can be successfully achieved in this rare subtype of APVS with IVS patients. However, more multicentre studies and lifelong follow-up are required to determine the timeliness for pulmonary valve surgery to avoid over-dilatation of the right ventricle in APVS with IVS patients.
    },
     year = {2024}
    }
    

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    AB  - Absent pulmonary valve syndrome with an intact ventricular septum (APVS with IVS) is a rare congenital heart defect that is associated with a high perinatal mortality. Most fetuses can hard to survive without inotropic support or heart transplantation due to the severe respiratory dysfunction, heart failure and serious infections resulting from tracheobronchial compression and right ventricular dilation. Almost one-third of the patients opted for a termination of pregnancy. The etiology of APVS patients is still unknown. Previous research only noted an enhancement in the long-term survival of APVS with tetralogy of Fallot patients over the past decade. However, the timeliness of surgical intervention and survival status in APVS with IVS patients remains unclear. In our case, we described a 27-year-old patient with a rare combination of the rudimentary pulmonary valve, intact ventricular septum and absent superior vena cava who successfully developed biventricular physiology without any medical intervene. And he received a timely pulmonary valve implantation in our centre to prevent further right ventricular dysfunction. Thus, we highlighted that biventricular circulation can be successfully achieved in this rare subtype of APVS with IVS patients. However, more multicentre studies and lifelong follow-up are required to determine the timeliness for pulmonary valve surgery to avoid over-dilatation of the right ventricle in APVS with IVS patients.
    
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Author Information
  • Department of Anesthesia and Operation Center, West China Hospital of Sichuan University, Chengdu, China

  • Department of Cardiovascular Surgery, West China Hospital of Sichuan University, Chengdu, China

  • Department of Cardiovascular Surgery, West China Hospital of Sichuan University, Chengdu, China

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