The Effect of Cardiac Surgery in Children with Trisomy 13 And Trisomy 18: A Systematic Review and Meta-Analysis
Pharmaceutical Science-Medicine and surgery
DOI:
https://doi.org/10.22376/ijlpr.2023.13.1.P113-128Keywords:
Cardiac Surgery, Trisomy 13, Trisomy 18, Systematic Review, Meta-AnalysisAbstract
Infants with trisomy 13 or trisomy 18 frequently suffer from cardiac anomalies. Our study aims to look at the impact of using cardiac surgery on mortality and functional outcome in patients with trisomy 13 and trisomy 18. A search for articles was done on PubMed, Scopus, Science Direct and CENTRAL databases; from inception until 30th October 2022. Quantitative analysis was done using Meta XL and Review Manager 5.4. A p=-value of 0.05 was adopted as the significance threshold. The database search yielded 1,127 articles but only 17 were included in this systematic review. This paper reports a total of 2,551 trisomy cases. 672 cases were T13, 1478 cases were T18 and 401 cases were undefined. The calculated Odds Ratio (OR) was 4.20 95%CI, 2.70 - 6.52], with a p-value < 0.00001. There was also the finding that cardiac surgery had a more positive impact on mortality and discharge outcomes for T18 patients than T13 patients. The difference, however was statistically insignificant. In conclusion, the use of cardiac surgery to manage T13 and T18 patients is plausible, but other factors like post-operative complications should be taken into account.
References
Chen M, Yeh GP, Shih JC, Wang BT. Trisomy 13 mosaicism: study of serial cytogenetic changes in a case from early pregnancy to infancy. Prenat Diagn. 2004 Feb;24(2):137-43. doi: 10.1002/pd.814, PMID 14974123.
Di Giacomo MC, Susca FC, Resta N, Bukvic N, Vimercati A, Guanti G. Trisomy 13 mosaicism in a phenotypically normal child: description of cytogenetic and clinical findings from early pregnancy beyond 2 years of age. Am J Med Genet A. 2007;143A(5):518-20. doi: 10.1002/ajmg.a.31515, PMID 17163529.
Pont SJ, Robbins JM, Bird TM, Gibson JB, Cleves MA, Tilford JM, et al. Congenital malformations among liveborn infants with trisomies 18 and 13. Am J Med Genet A. 2006;140(16):1749-56. doi: 10.1002/ajmg.a.31382, PMID 16835915.
Milligan MCP, Jackson LE, Maurer SH. Clinical Course for Patients With Trisomy 13 and 18 Pursuing Life-Prolonging Therapies Versus Comfort-Directed Care. Am J Hosp Palliat Care. 2021;38(10):1225-9. doi: 10.1177/1049909120985210. PMID 33375814.
Bartholin-Patau syndrome (Thomas Bartholin) [internet]. Available from: http://www.whonamedit.com. Available from: http://www.whonamedit.com/synd.cfm/1024.html [cited 3/12/2022].
Patau K, Smith DW, Therman E, Inhorn SL, Wagner HP. Multiple congenital anomaly caused by an extra autosome. Lancet. 1960 Apr 9;1(7128):790-3. doi: 10.1016/s0140-6736(60)90676-0, PMID 14430807.
Crawford D, Dearmun A. Edwards’ syndrome. Nurs Child Young People. 2016 Dec 8;28(10):17-7. doi: 10.7748/ncyp.28.10.17.s19, PMID 27927129.
Roberts W, Zurada A, Zurada-ZieliŃSka A, Gielecki J, Loukas M. Anatomy of trisomy 18. Clin Anat. 2016 May 3;29(5):628-32. doi: 10.1002/ca.22725, PMID 27087248.
Lin HY, Lin SP, Chen YJ, Hung HY, Kao HA, Hsu CH, et al. Clinical characteristics and survival of trisomy 18 in a medical center in Taipei, 1988-2004. Am J Med Genet A. 2006 May 1;140(9):945-51. doi: 10.1002/ajmg.a.31173, PMID 16528742.
Medline. Trisomy 18: MedlinePlus medical encyclopedia [internet]; 2016. Medlineplus.gov. Available from: https://medlineplus.gov/ency/article/001661.htm [cited 3/12/2022].
Edwards JH, Harnden DG, Cameron AH, Crosse VM, Wolff OH. A new trisomic syndrome. Lancet. 1960 Apr 9;1(7128):787-90. doi: 10.1016/s0140-6736(60)90675-9, PMID 13819419.
Smith DW, Patau K, Therman E, Inhorn SL. A new autosomal trisomy syndrome: multiple congenital anomalies caused by an extra chromosome. J Pediatr. 1960 Sep;57(3):338-45. doi: 10.1016/s0022-3476(60)80241-7, PMID 13831938.
Patau K, Therman E, Smith DW, Demars RI. Trisomy for chromosome no. 18 in man. Chromosoma. 1961;12:280-5. doi: 10.1007/BF00328924, PMID 13733243.
Noriega MA, Siddik AB. Trisomy 13 [internet]. Treasure Island, (FL): StatPearls Publishing; 2020. Available from: https://www.ncbi.nlm.nih.gov/books/NBK559091/ [cited 3/12/2022]. PMID PubMed.
Support Organization For Trisomy. ABOUT trisomy 13: What do we know. Now Publishers [internet]. SOFT – Support Organization For Trisomy. Available from: https://trisomy.org/about-trisomy/trisomy-13/ [cited 3/12/2022].
Vol. 17. Interesting trisomy 13 statistics [internet]. Hudson River Foundation; 2015. Available from: https://healthresearchfunding.org/17-interesting-trisomy-13-statistics/ [cited 3/12/2022].
Satgé D, Nishi M, Sirvent N, Vekemans M, Chenard MP, Barnes A. A tumor profile in Patau syndrome (trisomy 13). Am J Med Genet A. 2017 May 25;173(8):2088-96. doi: 10.1002/ajmg.a.38294, PMID 28544599.
Support Organization for Trisomy. Trisomy 18 [internet]. SOFT – Support Organization For Trisomy [cited Oct 29 2022]. Available from: https://trisomy.org/about-trisomy/trisomy-18/.
Zoler ML. Trisomy 13 survival can exceed 1 year [internet] [web]. Available from: archive.org. Life Health Library; 2012 [cited Oct 29 2022]. Available from: https://web.archive.org/web/20120221034010/http://findarticles.com/p/articles/mi_m0CYD/is_5_38/ai_98830102.
Nelson KE, Rosella LC, Mahant S, Guttmann A. Survival and surgical interventions for children with trisomy 13 and 18. JAMA. 2016 Jul 26;316(4):420-8. doi: 10.1001/jama.2016.9819, PMID 27458947.
Pallotto I, Lantos JD. Treatment decisions for babies with trisomy 13 and 18. HEC Forum. 2017 Apr 1;29(3):213-22. doi: 10.1007/s10730-017-9319-2, PMID 28365826.
Janvier A, Farlow B, Barrington K. Cardiac surgery for children with trisomies 13 and 18: where are we now? Semin Perinatol. 2016 Jun;40(4):254-60. doi: 10.1053/j.semperi.2015.12.015, PMID 26847083.
Carvajal HG, Callahan CP, Miller JR, Rensink BL, Eghtesady P. Cardiac surgery in trisomy 13 and 18: A guide to clinical decision-making. Pediatr Cardiol. 2020 Sep 14;41(7):1319-33. doi: 10.1007/s00246-020-02444-6, PMID 32924070.
Muneuchi J, Yamamoto J, Takahashi Y, Watanabe M, Yuge T, Ohno T, et al. Outcomes of cardiac surgery in trisomy 18 patients. Cardiol Young. 2011 Apr 1;21(2):209-15. doi: 10.1017/S1047951110001848, PMID 21205409.
Page MJ, McKenzie JE, Bossuyt PM, Boutron I, Hoffmann TC, Mulrow CD, et al. The PRISMA 2020 statement: an Updated Guideline for Reporting Systematic Reviews. BMJ. 2021 Mar 29;372(71):n71. doi: 10.1136/bmj.n71, PMID 33782057.
Costello JP, Weiderhold A, Louis C, Shaughnessy C, Peer SM, Zurakowski D, et al. A contemporary, single-institutional experience of surgical versus expectant management of congenital heart disease in trisomy 13 and 18 patients. Pediatr Cardiol. 2015 Jan 23;36(5):987-92. doi: 10.1007/s00246-015-1109-5, PMID 25612784.
Bruns DA, Martinez A. An analysis of cardiac defects and surgical interventions in 84 cases with full trisomy 18. Am J Med Genet A. 2016;170A(2):337-43. doi: 10.1002/ajmg.a.37427. PMID 26453443.
Kwak JH, Song J. Kang I-Seok, Huh J, Yang J-H, Jun T-G. Cardiac Repair in Patients with Trisomy 18: Total or Palliative? Iran J Pediatr. 2021 Mar 13;31(2).
Rosenblum JM, Kanter KR, Shashidharan S, Shaw FR, Chai PJ. Cardiac surgery in children with trisomy 13 or trisomy 18: How safe is it? JTCVS Open. 2022 Sep. doi: 10.1016/j.xjon.2022.09.005.
Cooper DS, Riggs KW, Zafar F, Jacobs JP, Hill KD, Pasquali SK, et al. Cardiac surgery in patients with trisomy 13 and 18: an analysis of the Society of Thoracic Surgeons congenital heart surgery database. J Am Heart Assoc. 2019 Jul 2;8(13). doi: 10.1161/JAHA.119.012349.
Kaneko Y, Kobayashi J, Achiwa I, Yoda H, Tsuchiya K, Nakajima Y, et al. Cardiac surgery in patients with trisomy 18. Pediatr Cardiol. 2009 Apr 2;30(6):729-34. doi: 10.1007/s00246-009-9427-0, PMID 19340475.
Kosiv KA, Gossett JM, Bai S, Collins RT. Congenital heart surgery on in-hospital mortality in trisomy 13 and 18. Pediatrics. 2017 Oct 18;140(5):e20170772. doi: 10.1542/peds.2017-0772, PMID 29046387.
Nakai Y, Asano M, Nomura N, Matsumae H, Mishima A. Effectiveness of cardiac surgery in patients with trisomy 18: a single-institutional experience. Cardiol Young. 2016 Jan 28;26(7):1391-6. doi: 10.1017/S1047951115002723, PMID 26821376.
Graham EM, Bradley SM, Shirali GS, Hills CB, Atz AM, Pediatric Cardiac Care Consortium. Effectiveness of cardiac surgery in trisomies 13 and 18 (from the Pediatric Cardiac Care Consortium). Am J Cardiol. 2004 Mar;93(6):801-3. doi: 10.1016/j.amjcard.2003.12.012, PMID 15019900.
Peterson R, Calamur N, Fiore A, Huddleston C, Spence K. Factors influencing outcomes after cardiac intervention in infants with trisomy 13 and 18. Pediatr Cardiol. 2018;39(1):140-7. doi: 10.1007/s00246-017-1738-y, PMID 28948390.
Swanson SK, Schumacher KR, Ohye RG, Zampi JD. Impact of trisomy 13 and 18 on airway anomalies and pulmonary complications after cardiac surgery. J Thorac Cardiovasc Surg. 2021 Jul;162(1):241-9. doi: 10.1016/j.jtcvs.2020.08.082, PMID 32981701.
Kaneko Y, Kobayashi J, Yamamoto Y, Yoda H, Kanetaka Y, Nakajima Y, et al. Intensive cardiac management in patients with trisomy 13 or trisomy 18. Am J Med Genet A. 2008;146A(11):1372-80. doi: 10.1002/ajmg.a.32311, PMID 18412275.
Weaver MS, Birge N, Hsu H, Woell C, Robinson JE, Wichman C, et al. Mixed method study of quality of life for children with trisomy 18 and 13 after cardiac surgery. Cardiol Young. 2020 Jan 22;30(2):231-7. doi: 10.1017/S1047951120000013, PMID 31964456.
Domingo L, Carey JC, Eckhauser A, Wilkes J, Menon SC. Mortality and resource use following cardiac interventions in children with trisomy 13 and trisomy 18 and congenital heart disease. Pediatr Cardiol. 2019;40(2):349-56. doi: 10.1007/s00246-018-2001-x, PMID 30291384.
Kobayashi J, Kaneko Y, Yamamoto Y, Yoda H, Tsuchiya K. Radical surgery for a ventricular septal defect associated with trisomy 18. Gen Thorac Cardiovasc Surg. 2010 May;58(5):223-7. doi: 10.1007/s11748-009-0431-3, PMID 20449712.
Maeda J, Yamagishi H, Furutani Y, Kamisago M, Waragai T, Oana S, et al. The impact of cardiac surgery in patients with trisomy 18 and trisomy 13 in Japan. Am J Med Genet A. 2011 Oct 11;155A(11):2641-6. doi: 10.1002/ajmg.a.34285, PMID 21990245.
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Copyright (c) 2022 Dr. Samer A. Alzahrani, Dr. Mohannad A. Alghamdi, Mohammed F. Bin Muammar, Dr. Abdulhakeem Mahmoud A Khan, Dr. Raghda Khaled Tayeb
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