Contemporary Outcomes of Heart Transplantation in Children with Heterotaxy Syndrome: Sub-Optimal Pre-Transplant Optimization Translates into Early Post-Transplant Mortality.

Publisher: Springer Verlag Country of Publication: United States NLM ID: 8003849 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1432-1971 (Electronic) Linking ISSN: 01720643 NLM ISO Abbreviation: Pediatr Cardiol Subsets: MEDLINE

Publication: New York Ny : Springer Verlag
Original Publication: [New York, Springer-Verlag]

Heart Transplantation* ; Heterotaxy Syndrome*/surgery ; Heterotaxy Syndrome*/mortality; Humans ; Male ; Female ; Child ; Child, Preschool ; Infant ; Adolescent ; Heart Defects, Congenital/surgery ; Heart Defects, Congenital/mortality ; Retrospective Studies ; Treatment Outcome ; Cardiomyopathies/surgery ; Cardiomyopathies/mortality ; Survival Rate

Patients with heterotaxy syndrome and congenital heart disease (CHD) experience inferior cardiac surgical outcomes. Heart transplantation outcomes are understudied, however, particularly compared to non-CHD patients. Data from UNOS and PHIS were used to identify 4803 children (< 18 years) undergoing first-time heart transplant between 2003 and 2022 with diagnoses of heterotaxy (n = 278), other-CHD (n = 2236), and non-CHD cardiomyopathy (n = 2289). Heterotaxy patients were older (median 5 yr) and heavier (median 17 kg) at transplant than other-CHD (median 2 yr and 12 kg), and younger and lighter than cardiomyopathy (median 7 yr and 24 kg) (all p < 0.001). UNOS status 1A/1 at listing was not different between groups (65-67%; p = 0.683). At transplant, heterotaxy and other-CHD patients had similar rates of renal dysfunction (12 and 17%), inotropes (10% and 11%), and ventilator-dependence (19 and 18%). Compared to cardiomyopathy, heterotaxy patients had comparable renal dysfunction (9%, p = 0.058) and inotropes (46%, p = 0.097) but more hepatic dysfunction (17%, p < 0.001) and ventilator-dependence (12%, p = 0.003). Rates of ventricular assist device (VAD) were: heterotaxy-10%, other-CHD-11% (p = 0.839 vs. heterotaxy), cardiomyopathy-37% (p < 0.001 vs. heterotaxy). The 1-year incidence of acute rejection post-transplant was comparable between heterotaxy and others (p > 0.05). While overall post-transplant survival was significantly worse for heterotaxy than others (p < 0.05 vs. both), conditional 1-year survival was comparable (p > 0.3 vs. both). Children with heterotaxy syndrome experience inferior post-heart transplant survival, although early mortality appears to influence this trend, with 1-year survivors having equivalent outcomes. Given similar pre-transplant clinical status to others, heterotaxy patients are potentially under risk-stratified. Increased VAD utilization and pre-transplant end-organ function optimization may portend improved outcomes.
(© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Loomba RS, Morales DLS, Redington A (2019) Critical heart disease in infants and children. 796–803.e3. https://doi.org/10.1016/b978-1-4557-0760-7.00067-x.
Khan A, Pahl E, Koehl DA, Cantor RS, Kirklin JK, Rusconi P, Barnes AP, Azeka E, Everitt MD (2021) Improved heart transplant survival for children with congenital heart disease and heterotaxy syndrome in the current era: an analysis from the pediatric heart transplant society. J Hear Lung Transplant. https://doi.org/10.1016/j.healun.2021.07.008. (PMID: 10.1016/j.healun.2021.07.008)
McGovern E, Kelleher E, Potts JE, O’Brien J, Walsh K, Nolke L, McMahon CJ (2016) Predictors of poor outcome among children with heterotaxy syndrome: a retrospective review. Open Hear 3:e000328. https://doi.org/10.1136/openhrt-2015-000328. (PMID: 10.1136/openhrt-2015-000328)
Landis BJ, Cooper DS, Hinton RB (2016) CHD associated with syndromic diagnoses: peri-operative risk factors and early outcomes. Cardiol Young 26:30–52. https://doi.org/10.1017/s1047951115001389. (PMID: 10.1017/s104795111500138926345374)
Jonas RA (2010) Surgical management of the neonate with heterotaxy and long-term outcomes of heterotaxy. World J Pediatric Congenit Hear Surg 2:264–274. https://doi.org/10.1177/2150135110396908. (PMID: 10.1177/2150135110396908)
Alongi AM, Kirklin JK, Deng L, Padilla L, Pavnica J, Romp RL, Mauchley DC, Cleveland DC, Dabal RJ (2019) Surgical management of heterotaxy syndrome: current challenges and opportunities. World J Pediatric Congenit Hear Surg 11:166–176. https://doi.org/10.1177/2150135119893650. (PMID: 10.1177/2150135119893650)
Jacobs JP, Pasquali SK, Morales DLS, Jacobs ML, Mavroudis C, Chai PJ, Tchervenkov CI, Lacour-Gayet FG, Walters H, Quintessenza JA (2010) Heterotaxy. World J Pediatric Congenit Hear Surg 2:278–286. https://doi.org/10.1177/2150135110397670. (PMID: 10.1177/2150135110397670)
Kulkarni A, Patel N, Singh TP, Mossialos E, Mehra MR (2019) Risk factors for death or heart transplantation in single-ventricle physiology (tricuspid atresia, pulmonary atresia, and heterotaxy): a systematic review and meta-analysis. J Hear Lung Transplant 38:739–747. https://doi.org/10.1016/j.healun.2019.04.001. (PMID: 10.1016/j.healun.2019.04.001)
Duong SQ, Godown J, Soslow JH, Thurm C, Hall M, Sainathan S, Morell VO, Dodd DA, Feingold B (2019) Increased mortality, morbidities, and costs after heart transplantation in heterotaxy syndrome and other complex situs arrangements. J Thorac Cardiovasc Surg 157:730-740.e11. https://doi.org/10.1016/j.jtcvs.2018.11.022. (PMID: 10.1016/j.jtcvs.2018.11.02230669235)
Jacobs JP, Asante-Korang A, O’Brien SM, Chai PJ, Dadlani GH, Rodriguez-Fazzi GL, Vu D, McCormack J, McKenna DE, Boucek RJ, Cooper DS, Saltiel A, Carapellucci J, van Gelder HM, Daicoff GR, Quintessenza JA (2011) Lessons learned from 119 consecutive cardiac transplants for pediatric and congenital heart disease. Ann Thorac Surg 91:1248–1255. https://doi.org/10.1016/j.athoracsur.2010.10.083. (PMID: 10.1016/j.athoracsur.2010.10.08321440154)
Larsen RL, Eguchi JH, Mulla NF, Johnston JK, Fitts J, Kuhn MA, Razzouk AJ, Chinnock RE, Bailey LL (2002) Usefulness of cardiac transplantation in children with visceral heterotaxy (asplenic and polysplenic syndromes and single right-sided spleen with levocardia) and comparison of results with cardiac transplantation in children with dilated cardiomyopathy. Am J Cardiol 89:1275–1279. https://doi.org/10.1016/s0002-9149(02)02325-1. (PMID: 10.1016/s0002-9149(02)02325-112031727)
Staples A, LeBlond R, Watkins S, Wong C, Brandt J (2010) Validation of the revised Schwartz estimating equation in a predominantly non-CKD population. Pediatr Nephrol 25:2321–2326. https://doi.org/10.1007/s00467-010-1598-7. (PMID: 10.1007/s00467-010-1598-720652327)
Riggs KW, Broderick JT, Price N, Chin C, Zafar F, Morales DLS (2021) Transplantation for congenital heart disease: focus on the impact of functionally univentricular versus biventricular circulation. World J Pediatric Congenit Hear Surg 12:352–359. https://doi.org/10.1177/2150135121990650. (PMID: 10.1177/2150135121990650)
Swisher M, Jonas R, Tian X, Lee ES, Lo CW, Leatherbury L (2011) Increased postoperative and respiratory complications in patients with congenital heart disease associated with heterotaxy. J Thorac Cardiovasc Surg 141:637-644.e3. https://doi.org/10.1016/j.jtcvs.2010.07.082. (PMID: 10.1016/j.jtcvs.2010.07.08220884020)
Alsoufi B, Al-Halees Z, Manlhiot C, Awan A, Al-Ahmadi M, McCrindle BW, Al-Joufan M, Canver CC (2010) Intermediate results following complex biventricular repair of left ventricular outflow tract obstruction in neonates and infants. Eur J Cardio-thorac 38:431–438. https://doi.org/10.1016/j.ejcts.2010.02.035. (PMID: 10.1016/j.ejcts.2010.02.035)
Basgoze S, Temur B, Guvenc O, Aydin S, Guzelmeric F, Onalan MA, Erek E (2021) Analysis of outcomes in patients with abnormal laterality undergoing congenital heart surgery. Cardiol Young. https://doi.org/10.1017/s1047951121003899. (PMID: 10.1017/s104795112100389934593080)
Edelson JB, Huang Y, Griffis H, Huang J, Mascio CE, Chen JM, Maeda K, Burstein DS, Wittlieb-Weber C, Lin KY, O’Connor MJ, Rossano JW (2021) The influence of mechanical circulatory support on post-transplant outcomes in pediatric patients: a multicenter study from the international society for heart and lung transplantation (ISHLT) registry. J Hear Lung Transplant. https://doi.org/10.1016/j.healun.2021.06.003. (PMID: 10.1016/j.healun.2021.06.003)
Sutcliffe DL, Pruitt E, Cantor RS, Godown J, Lane J, Turrentine MW, Law SP, Lantz JL, Kirklin JK, Bernstein D, Blume ED (2018) Post-transplant outcomes in pediatric ventricular assist device patients: A PediMACS–pediatric heart transplant study linkage analysis. J Hear Lung Transplant 37:715–722. https://doi.org/10.1016/j.healun.2017.12.004. (PMID: 10.1016/j.healun.2017.12.004)
Lorts A, Smyth L, Gajarski RJ, VanderPluym CJ, Mehegan M, Villa CR, Murray JM, Niebler RA, Almond CS, Thrush P, O’Connor MJ, Conway J, Sutcliffe DL, Lantz JE, Zafar F, Morales DLS, Peng DM, Rosenthal DN (2020) The creation of a pediatric health care learning network: the ACTION quality improvement collaborative. Asaio J 66:441–446. https://doi.org/10.1097/mat.0000000000001133. (PMID: 10.1097/mat.000000000000113332224822)
Zafar F, Castleberry C, Khan MS, Mehta V, Bryant R, Lorts A, Wilmot I, Jefferies JL, Chin C, Morales DLS (2015) Pediatric heart transplant waiting list mortality in the era of ventricular assist devices. J Hear Lung Transplant 34:82–88. https://doi.org/10.1016/j.healun.2014.09.018. (PMID: 10.1016/j.healun.2014.09.018)
Rossano JW, VanderPluym CJ, Peng DM, Hollander SA, Maeda K, Adachi I, Davies RR, Simpson KE, Fynn-Thompson F, Conway J, Law SP, Cantor R, Koehl D, Jacobs JP, Amdani S, Kirklin JK, Morales DLS, Investigators P (2021) Fifth annual pediatric interagency registry for mechanical circulatory support (pedimacs) report. Ann Thorac Surg 112:1763–1774. https://doi.org/10.1016/j.athoracsur.2021.10.001. (PMID: 10.1016/j.athoracsur.2021.10.00134648810)
Tsiouris A, Paone G, Nemeh HW, Borgi J, Williams CT, Lanfear DE, Morgan JA (2015) Short and long term outcomes of 200 patients supported by continuous-flow left ventricular assist devices. World J Cardiol 7:792–800. https://doi.org/10.4330/wjc.v7.i11.792. (PMID: 10.4330/wjc.v7.i11.792266359274660474)
Han JJ, Acker MA, Atluri P (2018) Left ventricular assist devices. Circulation 138:2841–2851. https://doi.org/10.1161/circulationaha.118.035566. (PMID: 10.1161/circulationaha.118.03556630565993)
Colvin M, Smith JM, Ahn Y, Skeans MA, Messick E, Bradbrook K, Gauntt K, Israni AK, Snyder JJ, Kasiske BL (2022) OPTN/SRTR 2020 annual data report: heart. Am J Transplant 22:350–437. https://doi.org/10.1111/ajt.16977. (PMID: 10.1111/ajt.1697735266620)
Shugh SB, Riggs KW, Morales DLS (2019) Mechanical circulatory support in children: past, present and future. Transl Pediatrics 8:269–277. https://doi.org/10.21037/tp.2019.07.14. (PMID: 10.21037/tp.2019.07.14)
Miller JR, Lancaster TS, Callahan C, Abarbanell AM, Eghtesady P (2018) An overview of mechanical circulatory support in single-ventricle patients. Transl Pediatrics 7:151–161. https://doi.org/10.21037/tp.2018.03.03. (PMID: 10.21037/tp.2018.03.03)

Keywords: Atrial isomerism; Congenital heart disease; Heart transplantation; Heterotaxy syndrome; Ventricular assist device

Date Created: 20230222 Date Completed: 20240716 Latest Revision: 20240716

20240716

10.1007/s00246-023-03122-z

36811659