The Outcome of Congenital Cardiac Surgery in Patients with Down Syndrome: Single-Center Experience

Authors

  • Abdulhameed A. Alnajjar Pediatric Cardiac Services, Madinah Cardiac Center MCC, 42210 Madinah, Saudi Arabia
  • Sherif S. Salem Pediatric Cardiac Services, Madinah Cardiac Center MCC, 42210 Madinah, Saudi Arabia; Pediatric Department, Faculty of Medicine, Menoufia University, 32951 Menoufia, Egypt
  • Luna S. Baangood Pediatric Cardiac Services, Madinah Cardiac Center MCC, 42210 Madinah, Saudi Arabia
  • Mansour B. Al-Mutairi Pediatric Cardiac Services, Madinah Cardiac Center MCC, 42210 Madinah, Saudi Arabia
  • Mohamed F. Morsy Pediatric Cardiac Services, Madinah Cardiac Center MCC, 42210 Madinah, Saudi Arabia; Pediatric Department, Faculty of Medicine, Sohag University, 82511 Sohag, Egypt
  • Mustafa Al-Muhaya Pediatric Cardiac Services, Madinah Cardiac Center MCC, 42210 Madinah, Saudi Arabia
  • Alassal A. Alkodami Pediatric Cardiac Services, Madinah Cardiac Center MCC, 42210 Madinah, Saudi Arabia
  • Merhamer L. Sabtirul Pediatric Cardiac Services, Madinah Cardiac Center MCC, 42210 Madinah, Saudi Arabia
  • Mohamed S. Hussein Pediatric Cardiac Services, Madinah Cardiac Center MCC, 42210 Madinah, Saudi Arabia
  • Eman W. Altommeihi Pediatric Cardiac Services, Madinah Cardiac Center MCC, 42210 Madinah, Saudi Arabia
  • Ahmed M. Shaban Pediatric Cardiac Services, Madinah Cardiac Center MCC, 42210 Madinah, Saudi Arabia; Cardiothoracic Surgery Department, Faculty of Medicine, Suez Canal University, 41522 Suez Canal, Egypt
  • Mohamed Alashwal Pediatric Cardiac Services, Madinah Cardiac Center MCC, 42210 Madinah, Saudi Arabia
  • Ayman R. Abdelrehim Pediatric Cardiac Services, Madinah Cardiac Center MCC, 42210 Madinah, Saudi Arabia; Cardiothoracic Surgery Department, Faculty of Medicine, Menoufia University, 32951 Menoufia, Egypt https://orcid.org/0000-0002-1947-2839

DOI:

https://doi.org/10.59958/hsf.5795

Keywords:

cardiac surgery, Down syndrome, congenital heart defects, mortality, outcomes

Abstract

Objectives: This study aimed to describe the outcomes of Down syndrome patients who underwent cardiac surgery for congenital heart defects and to develop risk prediction models for in-hospital mortality, recurrent hospital admission, and the need for catheter intervention among a cohort of patients. Methods: This single-centre retrospective cohort study included consecutive Down syndrome patients who underwent cardiac surgery for congenital heart defects between January 2018 and December 2021. We reviewed the electronic medical records. Two hundred patients fulfilled the eligibility criteria with complete data reporting. The patients' perioperative data and outcomes were recorded. Results: Females constituted 56.5%. Most (78.5%) patients showed accepted recovery. The incidence of all-cause in-hospital mortality was 3.0%. The rates of the need for a second operation, heart failure management, and permanent pacemaker insertion were 3.0%, 2.0%, and 2.5%, respectively. Only 8 (4.0%) patients stayed in the hospital for a long duration after chylothorax or tracheostomy (if intubated more than 2 weeks). The model had an accuracy of 99% and included the intraoperative transesophageal echocardiography (TEE) abnormalities (residual heart lesions) (adjusted odds ratio [AOR]: 26.541, p = 0.033), the duration of mechanical ventilation following the operation (AOR: 1.152, p = 0.009), and the occurrence of postoperative heart block (AOR: 76.447, p = 0.005). Conclusion: Surgical treatment of congenital heart defects in Down syndrome patients had good outcomes with accepted recovery (without intra-hospital or during follow-up mortality or morbidity) of 78.5% and a 3% incidence of in-hospital mortality. Though, the occurrence of chylothorax was considerably high, and resulted in a long hospital stay (more than 10 days). Repair of tetralogy of Fallot and coarctation of the aorta were associated with an increased likelihood of catheter intervention following the operation.

References

Colvin KL, Yeager ME. What people with Down Syndrome can teach us about cardiopulmonary disease. European Respiratory Review. 2017; 26: 160098.

Santoro SL, Steffensen EH. Congenital heart disease in Down syndrome–A review of temporal changes. Journal of Congenital Cardiology. 2021; 5: 1–14.

Al-Aama JY, Bondagji NS, El-Harouni AA. Congenital heart defects in Down syndrome patients from western Saudi Arabia. Saudi Medical Journal. 2012; 33: 1211–1215.

Rehman Y, Wazir HD, Akbar A, Khan AM, Hussain I, Afridi A, et al. Congenital Heart Disease and Its Association in Children With Down Syndrome. Cureus. 2022; 14: e29176.

Abduljawad EM, AlHarthi A, AlMatrafi SA, Hussain M, Shawli A, Waggass R. The Prevalence of Congenital Heart Diseases in Syndromic Children at King Khalid National Guard Hospital from 2005 to 2016. Cureus. 2020; 12: e7891.

Alhuzaimi AN, Alotaibi NM, Alsuhaibani GI, Alanazi RK, Temsah MH. Congenital Heart Defect and Pulmonary Hypertension in Children With Down Syndrome: Clinical Profile Over Two Decades. Cureus. 2021; 13: e13212.

Dimopoulos K, Constantine A, Clift P, Condliffe R, Moledina S, Jansen K, et al. Cardiovascular Complications of Down Syndrome: Scoping Review and Expert Consensus. Circulation. 2023; 147: 425–441.

Alhaddad FA, Alkhushi NA, Alharbi AM, Al Talib SA, Sultan SM, Bahawi YO. Quality of Life Among Down Syndrome Patients With and Without Congenital Heart Disease at King Abdulaziz University Hospital, Jeddah, Saudi Arabia. Cureus. 2023; 15: e33553.

Krishna N, Varma PK. Risk stratification in cardiac surgery. Indian Journal of Thoracic and Cardiovascular Surgery. 2015; 31: 224–233.

Delany DR, Gaydos SS, Romeo DA, Henderson HT, Fogg KL, McKeta AS, et al. Down syndrome and congenital heart disease: perioperative planning and management. Journal of Congenital Cardiology. 2021; 5: 7.

Dhillon GS, Ghanayem NS, Broda CR, Lalani SR, Mery CM, Shekerdemian LS, et al. An Analysis of Hospital Mortality After Cardiac Operations in Children With Down Syndrome. Seminars in Thoracic and Cardiovascular Surgery. 2020; 32: 947–957.

Bergström S, Carr H, Petersson G, Stephansson O, Bonamy AKE, Dahlström A, et al. Trends in Congenital Heart Defects in Infants With Down Syndrome. Pediatrics. 2016; 138: e20160123.

Martin T, Smith A, Breatnach CR, Kent E, Shanahan I, Boyle M, et al. Infants Born with Down Syndrome: Burden of Disease in the Early Neonatal Period. The Journal of Pediatrics. 2018; 193: 21–26.

Pfitzer C, Helm PC, Rosenthal LM, Berger F, Bauer UMM, Schmitt KR. Dynamics in prevalence of Down syndrome in children with congenital heart disease. European Journal of Pediatrics. 2018; 177: 107–115.

Brown KL, Ridout D, Pagel C, Wray J, Anderson D, Barron DJ, et al. Incidence and risk factors for important early morbidities associated with pediatric cardiac surgery in a UK population. The Journal of Thoracic and Cardiovascular Surgery. 2019; 158: 1185–1196.e7.

Alghamdi BA, Alharthi RA, AlShaikh BA, Alosaimi MA, Alghamdi AY, Yusnoraini N, et al. Risk Factors for Post-cardiac Surgery Infections. Cureus. 2022; 14: e31198.

Barker GM, O'Brien SM, Welke KF, Jacobs ML, Jacobs JP, Benjamin DK Jr, et al. Major infection after pediatric cardiac surgery: a risk estimation model. The Annals of Thoracic Surgery. 2010; 89: 843–850.

Ma M, Gauvreau K, Allan CK, Mayer JE Jr, Jenkins KJ. Causes of death after congenital heart surgery. The Annals of Thoracic Surgery. 2007; 83: 1438–1445.

Huggard D, Doherty DG, Molloy EJ. Immune Dysregulation in Children With Down Syndrome. Frontiers in Pediatrics. 2020; 8: 73.

Ram G, Chinen J. Infections and immunodeficiency in Down syndrome. Clinical and Experimental Immunology. 2011; 164: 9–16.

Kwak JH, Lee SW, Cha HR, Huh J, Kang IS, Jun TG, et al. Long-Term Observational Outcomes after Total Correction of Congenital Heart Disease in Korean Patients with Down Syndrome: A National Cohort Study. Children. 2022; 9: 1329.

Lindberg L, Olsson AK, Jögi P, Jonmarker C. How common is severe pulmonary hypertension after pediatric cardiac surgery? The Journal of Thoracic and Cardiovascular Surgery. 2002; 123: 1155–1163.

Fudge JC Jr, Li S, Jaggers J, O'Brien SM, Peterson ED, Jacobs JP, et al. Congenital heart surgery outcomes in Down syndrome: analysis of a national clinical database. Pediatrics. 2010; 126: 315–322.

Wada N, Takahashi Y, Ando M, Park IS, Sasaki T. Single ventricle repair in children with Down's syndrome. General Thoracic and Cardiovascular Surgery. 2008; 56: 104–108.

Alkattan HN, Ardah HI, Arifi AA, Yelbuz TM. The evolving treatment of congenital heart disease in patient with Down syndrome: Current state of knowledge. Journal of Cardiac Surgery. 2022; 37: 3760–3768.

Alsuhaymi ZS SM, Alsuhimi SS, Alharbi HB, Khoshal SQ, Syed AU. Outcome of congenital heart surgery for patients with Down Syndrome. Chirurgia. 2020; 33: 119–122.

Ebrahim MA, Al Balool JA, Al Saegh AH, Menshawi HH, Almeeri NM, Elsayed MA, et al. Surgical Outcomes of Congenital Heart Disease in Down Syndrome: Tertiary Center Experience-Focus on the Electrical Conduction System. Pediatric Cardiology. 2023; 44: 204–209.

Cavalcante CTDMB, de Souza NMG, Pinto VC, Júnior, Branco KMPC, Pompeu RG, Teles ACDO, et al. Analysis of Surgical Mortality for Congenital Heart Defects Using RACHS-1 Risk Score in a Brazilian Single Center. Brazilian Journal of Cardiovascular Surgery. 2016; 31: 219–225.

Jenkins KJ, Gauvreau K. Center-specific differences in mortality: preliminary analyses using the Risk Adjustment in Congenital Heart Surgery (RACHS-1) method. The Journal of Thoracic and Cardiovascular Surgery. 2002; 124: 97–104.

Gritti MN, Farid P, Manlhiot C, Noone D, Sakha S, Ali S, et al. Factors Associated With Acute Kidney Injury After Cardiopulmonary Bypass in Children. CJC Pediatric and Congenital Heart Disease. 2023; 2: 20–29.

Evans JM, Dharmar M, Meierhenry E, Marcin JP, Raff GW. Association between Down syndrome and in-hospital death among children undergoing surgery for congenital heart disease: a US population-based study. Circulation: Cardiovascular Quality and Outcomes. 2014; 7: 445–452.

Tóth R, Szántó P, Prodán Z, Lex DJ, Sápi E, Szatmári A, et al. Down syndrome and postoperative complications after paediatric cardiac surgery: a propensity-matched analysis. Interactive Cardiovascular and Thoracic Surgery. 2013; 17: 691–697.

Published

2023-08-24

How to Cite

Alnajjar, A. A., Salem, S. S., Baangood , L. S., Al-Mutairi, M. B., Morsy, M. . F., Al-Muhaya, M., Alkodami, A. A., Sabtirul, M. L., Hussein, M. S., Altommeihi, E. W., Shaban, A. M., Alashwal, M., & Abdelrehim, A. R. (2023). The Outcome of Congenital Cardiac Surgery in Patients with Down Syndrome: Single-Center Experience. The Heart Surgery Forum, 26(4), E372-E380. https://doi.org/10.59958/hsf.5795

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