Heart transplantation in children with Turner syndrome: analysis of a linked dataset
Open accessAbstract
Turner syndrome (TS) patients with hypoplastic left heart syndrome (HLHS) have poor single ventricle palliation outcomes; therefore, consideration of other potential management strategies is important. Little is known about heart transplantation (HTx) in this group, as standard HTx databases do not allow for identification of TS. This study describes experiences and outcomes of HTx in TS using a unique linkage between the Scientific Registry of Transplant Recipients and the Pediatric Health Information System databases. All pediatric HTx recipients (2002-2016) with TS were identified in the database using ICD-9 code 758.6 (gonadal dysgenesis) in conjunction with female sex. Patient characteristics and outcomes were described. Fourteen patients with TS were identified who underwent 16 HTx procedures at eight centers. For initial HTx, HLHS was the most common indication (10/14) with a median age of 10 months (IQR 3-73 months). Median transplant-free survival following initial HTx was 4.1 years (IQR 16 days-10.5 years), with all deaths occurring in the first year post-HTx. For patients that survived past 1 year (8/14), follow-up ranged from 4.1 to 10.9 years (median 8.0 years) with no deaths observed. Our cohort demonstrates that while there is a clear risk for early mortality, there is the potential for favorable outcomes following HTx in patients with TS. Therefore, TS should not be viewed as an absolute contraindication to HTx, but careful assessment of candidate risk is needed. Primary palliation with HTx for HLHS and TS may be a reasonable consideration given the poor outcomes of single ventricle palliation in this group. Further research is needed to fully delineate the outcomes and characteristics of this unique population.
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Operative management can be more challenging given the medical complexity of TS.
“Operative management can be more challenging given the medical complexity of TS.410-413”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [412]
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Cites
Nielsen, J., & Wohlert, M. (1991) · Human Genetics · doi:10.1007/bf01213097 · Cited by 1,193 · see citing works
Care of girls and women with Turner syndrome: a guideline of the Turner syndrome study group
Bondy, C. A. (2007) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2006-1374 · Cited by 1,005 · see citing works
Lund, L. H., Edwards, L. B., Dipchand, A. I., Goldfarb, S., Kucheryavaya, A. Y., Levvey, B. J., Meiser, B., Rossano, J. W., Yusen, R. D., & Stehlik, J. (2016) · The Journal of Heart and Lung Transplantation · doi:10.1016/j.healun.2016.08.017 · Cited by 760 · see citing works
Gravholt, C. H., Juul, S., Naeraa, R. W., & Hansen, J. (1998) · Journal of Clinical Epidemiology · doi:10.1016/s0895-4356(97)00237-0 · Cited by 548 · see citing works
Leppke, S. N., Leighton, T., Zaun, D. A., Chen, S., Skeans, M. A., Israni, A. K., Snyder, J. J., & Kasiske, B. L. (2013) · Transplantation Reviews · doi:10.1016/j.trre.2013.01.002 · Cited by 309 · see citing works
Incidence of chromosome aberrations among 11 148 newborn children
Nielsen, J., & Sillesen, I. (1975) · Human Genetics · doi:10.1007/bf00273626 · Cited by 252 · see citing works
Nielsen, J., & Wohlert, M. (1990) · PubMed · Cited by 247 · see citing works
Prevalence and Clinical Picture of Celiac Disease in Turner Syndrome
Bonamico, M., Pasquino, A. M., Mariani, P., Danesi, H. M., Culasso, F., Mazzanti, L., Petri, A., & Bona, G. (2002) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2002-020855 · Cited by 184 · see citing works
Hypothyroidism is common in Turner syndrome: results of a five-year follow-up
El-Mansoury, M., Bryman, I., Berntorp, K., Hanson, C., Wilhelmsen, L., & Landin-Wilhelmsen, K. (2005) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2004-1262 · Cited by 166 · see citing works
Rossano, J. W., Dipchand, A. I., Edwards, L. B., Goldfarb, S., Kucheryavaya, A. Y., Levvey, B. J., Lund, L. H., Meiser, B., Yusen, R. D., & Stehlik, J. (2016) · The Journal of Heart and Lung Transplantation · doi:10.1016/j.healun.2016.08.018 · Cited by 165 · see citing works
Rossano, J. W., Cherikh, W. S., Chambers, D. C., et al. (2017) · The Journal of Heart and Lung Transplantation · doi:10.1016/j.healun.2017.07.018 · Cited by 149 · see citing works
Vascular involvement of the liver in Turner's syndrome
Roulot, D., Degott, C., Chazouillères, O., Oberti, F., Calès, P., Carbonell, N., Benferhat, S., Bresson-Hadni, S., & Valla, D. (2004) · Hepatology · doi:10.1002/hep.20026 · Cited by 143 · see citing works
Freriks, K., Timmermans, J., Beerendonk, C. C. M., Verhaak, C. M., Netea-Maier, R. T., Otten, B. J., Braat, D. D. M., Smeets, D. F. C. M., Kunst, D. H. P. M., Hermus, A. R. M. M., et al. (2011) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2011-0346 · Cited by 120 · see citing works
Congenital Cardiovascular Disease in Turner Syndrome
Bondy, C. A. (2008) · Congenital Heart Disease · doi:10.1111/j.1747-0803.2007.00163.x · Cited by 117 · see citing works
Livadas, S., Xekouki, P., Fouka, F., Kanaka‐Gantenbein, C., Kaloumenou, I., Mavrou, A., Constantinidou, N., & Dacou‐Voutetakis, C. (2005) · Thyroid · doi:10.1089/thy.2005.15.1061 · Cited by 114 · see citing works
A high incidence of chronic inflammatory bowel disease in patients with Turner's syndrome.
Price, W. H. (1979) · Journal of Medical Genetics · doi:10.1136/jmg.16.4.263 · Cited by 85 · see citing works
Cramer, J. W., Bartz, P. J., Simpson, P. M., & Zangwill, S. D. (2013) · Pediatric Cardiology · doi:10.1007/s00246-013-0766-5 · Cited by 62 · see citing works
Insulin Resistance Is an Intrinsic Defect Independent of Fat Mass in Women with Turner’s Syndrome
Salgin, B., Amin, R., Yuen, K. C. J., Williams, R. M., Murgatroyd, P. R., & Dunger, D. B. (2006) · Hormone Research in Paediatrics · doi:10.1159/000090907 · Cited by 60 · see citing works
Feingold, B., Zheng, J., Law, Y. M., et al. (2011) · Pediatric Transplantation · doi:10.1111/j.1399-3046.2011.01564.x · Cited by 59 · see citing works
Self-citationRisk Factors for Development of New-Onset Diabetes Mellitus in Adult Heart Transplant Recipients
Ye, X., Kuo, H., Sampaio, M., Jiang, Y., Reddy, P. N., & Bunnapradist, S. (2010) · Transplantation · doi:10.1097/tp.0b013e3181dd6bd9 · Cited by 58 · see citing works
Hathout, E. H., Chinnock, R., Johnston, J. K., et al. (2003) · American Journal of Transplantation · doi:10.1034/j.1600-6143.2003.00186.x · Cited by 51 · see citing works
Lara, D. A., Ethen, M. K., Canfield, M. A., Nembhard, W. N., & Morris, S. A. (2017) · Congenital Heart Disease · doi:10.1111/chd.12413 · Cited by 47 · see citing works
Outcome of infants with hypoplastic left heart and turner syndromes
Reis, P. (1999) · Obstetrics and Gynecology · doi:10.1016/s0029-7844(98)00462-1 · Cited by 40 · see citing works
Infectious, Malignant, and Autoimmune Complications in Pediatric Heart Transplant Recipients
Kulikowska, A., Boslaugh, S., Huddleston, C. B., et al. (2007) · The Journal of Pediatrics · doi:10.1016/j.jpeds.2007.10.018 · Cited by 36 · see citing works
Frequency and outcomes of cardiac operations and catheter interventions in Turner syndrome
Madriago, E., Nguyen, T., McFerson, M., Larson, E. V., Airhart, N., Moller, J. H., & Silberbach, M. (2012) · The American Journal of Cardiology · doi:10.1016/j.amjcard.2012.04.036 · Cited by 34 · see citing works
Godown, J. A., Thurm, C. W., Dodd, D. A., et al. (2017) · American Heart Journal · doi:10.1016/j.ahj.2017.08.014 · Cited by 34 · see citing works
Self-citationInitial Counseling Prior to Palliation for Hypoplastic Left Heart Syndrome
Yates, A. R., Hoffman, T. M., Boettner, B. L., Feltes, T. F., & Cua, C. L. (2011) · Congenital Heart Disease · doi:10.1111/j.1747-0803.2011.00525.x · Cited by 23 · see citing works
Clinical predictors of autoimmune and severe atopic disease in pediatric heart transplant recipients
Mouledoux, J. H., Albers, E. L., Lu, Z., Saville, B. R., Moore, D. J., & Dodd, D. A. (2013) · Pediatric Transplantation · doi:10.1111/petr.12205 · Cited by 20 · see citing works
Self-citationKawabata, S., Sakamoto, S., Honda, M., Hayashida, S., Yamamoto, H., Mikami, Y., & Inomata, Y. (2016) · Surgical Case Reports · doi:10.1186/s40792-016-0194-x · Cited by 14 · see citing works
An Unusual Occurrence of Hepatic Granulomas and Secondary Sitosterolemia in Turner Syndrome
Chintanaboina, J., Shah, P. R., & Riley, T. (2015) · Case Reports in Medicine · doi:10.1155/2015/186718 · Cited by 9 · see citing works
Aucejo, F., Ibrahim, Z., Hashimoto, K., Quintini, C., Kelly, D. M., VOGT, D. P., Eghtesad, B., Fung, J. J., Miller, C. C., & Tuthill, R. J. (2008) · Liver Transplantation · doi:10.1002/lt.21374 · Cited by 6 · see citing works
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- [2]Nielsen J Sillesen I 1975 Incidence of chromosome aberrations among 11148 newborn children Humangenetik 30 1 1 12 1184003
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Clinical practice guidelines for the care of girls and women with Turner syndrome.
Gravholt, C. H., Andersen, N. H., Christin-Maitre, S., Davis, S. M., Duijnhouwer, A., Gawlik, A., Maciel-Guerra, A. T., Gutmark-Little, I., Fleischer, K., Hong, D., et al. (2024) · European Journal of Endocrinology · doi:10.1093/ejendo/lvae050 · Cited by 224 · see citing works
Lara, D. A., Ethen, M. K., Canfield, M. A., Nembhard, W. N., & Morris, S. A. (2017) · Congenital Heart Disease · doi:10.1111/chd.12413 · Cited by 47 · see citing works
- Authors
- Chew, J. D., Soslow, J. H., Thurm, C., Hall, M., Dodd, D. A., Feingold, B., Simmons, J., & Godown, J.
- Affiliations
- Monroe Carell Jr. Children's Hospital (US)
- Children's Hospital Association (US)
- University of Pittsburgh (US)
- Corresponding author
- Chew, Joshua D.
- ORCID
- Venue
- Pediatric Cardiology
- Published
- 2018-01-03
- Volume
- 39
- Issue
- 3
- Pages
- 610-616
- ISSN
- 0172-0643 (ISSN-L)
- 1432-1971
- PMC
- PMC5831528
- OpenAlex
- W2781879891
- Cited by
- 15
- References
- 31
- Citations by year
- 2025: 2 · 2024: 2 · 2023: 2 · 2022: 2 · 2021: 2 · 2020: 1 · 2019: 3 · 2016: 1
- FWCI
- 1.56
- Type
- journal article
- Language
- en
- Open access
- green
- europepmc.org
- Licence
- Locations
- Pediatric Cardiology (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- Funders
- Vanderbilt University (Katherine Dodd Faculty Scholar Program)
- National Heart, Lung, and Blood Institute (K23HL123938)
- Topics
- Health Sciences › Medicine › Surgery › Transplantation: Methods and Outcomes
- Health Sciences › Medicine › Epidemiology › Congenital Heart Disease Studies
- Physical Sciences › Engineering › Biomedical Engineering › Mechanical Circulatory Support Devices
- Keywords
- Turner syndrome, heart transplantation, hypoplastic left heart syndrome, single ventricle palliation, transplant-free survival, early post-transplant mortality, transplant candidate selection, gonadal dysgenesis
- MeSH terms
- Adolescent, Child, Child, Preschool, Female, Follow-Up Studies, Humans, Infant, Infant, Newborn, Palliative Care, Registries, Retrospective Studies, Turner Syndrome, Heart Transplantation, Databases, Factual, Treatment Outcome, Hypoplastic Left Heart Syndrome
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