Heart Transplantation in Children with Turner Syndrome: Analysis of a Linked Dataset

被引:14
作者
Chew, Joshua D. [1 ]
Soslow, Jonathan H. [1 ]
Thurm, Cary [2 ]
Hall, Matt [2 ]
Dodd, Debra A. [1 ]
Feingold, Brian [3 ]
Simmons, Jill [4 ]
Godown, Justin [1 ]
机构
[1] Vanderbilt Univ, Monroe Carell Jr Childrens Hosp Vanderbilt, Dept Pediat Cardiol, 2200 Childrens Way,Suite 5230 DOT, Nashville, TN 37232 USA
[2] Childrens Hosp Assoc, Lenexa, KS USA
[3] Univ Pittsburgh, Sch Med, Pediat & Clin & Translat Sci, Pittsburgh, PA USA
[4] Monroe Carell Jr Childrens Hosp, Pediat Endocrinol, Nashville, TN USA
基金
美国国家卫生研究院;
关键词
Heart transplant; Pediatric; Turner syndrome; Outcomes; Hypoplastic left heart syndrome; HYPOPLASTIC LEFT-HEART; INTERNATIONAL SOCIETY; FOLLOW-UP; REGISTRY; DISEASE; RECIPIENTS; WOMEN; DYSFUNCTION; PREVALENCE; AUTOIMMUNE;
D O I
10.1007/s00246-017-1801-8
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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.
引用
收藏
页码:610 / 616
页数:7
相关论文
共 50 条
[41]   Outcomes of heart transplantation in children with previously palliated hypoplastic left heart syndrome [J].
Sizemore, Johnna ;
Furlong-Dillard, Jamie ;
Wilkens, Sarah ;
Kozik, Deborah ;
Deshpande, Shriprasad ;
Trivedi, Jaimin ;
Alsoufi, Bahaaldin .
EUROPEAN JOURNAL OF CARDIO-THORACIC SURGERY, 2024, 66 (01)
[42]   Ear and hearing problems in relation to karyotype in children with Turner syndrome [J].
Verver, E. J. J. ;
Freriks, K. ;
Thomeer, H. G. X. M. ;
Huygen, P. L. M. ;
Pennings, R. J. E. ;
Alfen-van der Velden, A. A. E. M. ;
Timmers, H. J. ;
Otten, B. J. ;
Cremers, C. W. R. J. ;
Kunst, H. P. M. .
HEARING RESEARCH, 2011, 275 (1-2) :81-88
[43]   Characteristics and progression of hearing loss in children with turner's syndrome [J].
Hamberis, Alexandra O. ;
Mehta, Charmee H. ;
Dornhoffer, James R. ;
Meyer, Ted Albert .
LARYNGOSCOPE, 2020, 130 (06) :1540-1546
[44]   Turner Syndrome and Associated Problems in Turkish Children: A Multicenter Study [J].
Yesilkaya, Ediz ;
Bereket, Abdullah ;
Darendeliler, Feyza ;
Bas, Firdevs ;
Poyrazoglu, Sukran ;
Aydin, Banu Kucukemre ;
Darcan, Sukran ;
Dundar, Bumin ;
Buyukinan, Muammer ;
Kara, Cengiz ;
Sari, Erkan ;
Adal, Erdal ;
Akinci, Aysehan ;
Atabek, Mehmet Emre ;
Demirel, Fatma ;
Celik, Nurullah ;
Ozkan, Behzat ;
Ozhan, Bayram ;
Orbak, Zerrin ;
Ersoy, Betul ;
Dogan, Murat ;
Atas, Ali ;
Turan, Serap ;
Goksen, Damla ;
Tarim, Omer ;
Yuksel, Bilgin ;
Ercan, Oya ;
Hatun, Sukru ;
Simsek, Enver ;
Okten, Aysenur ;
Abaci, Ayhan ;
Doneray, Hakan ;
Ozbek, Mehmet Nuri ;
Keskin, Mehmet ;
Onal, Hasan ;
Akyurek, Nesibe ;
Bulan, Kezban ;
Tepe, Derya ;
Emeksiz, Hamdi Cihan ;
Demir, Korcan ;
Kizilay, Deniz ;
Topaloglu, Ali Kemal ;
Eren, Erdal ;
Ozen, Samim ;
Abali, Saygin ;
Akin, Leyla ;
Eklioglu, Beray Selver ;
Kaba, Sultan ;
Anik, Ahmet ;
Bas, Serpil .
JOURNAL OF CLINICAL RESEARCH IN PEDIATRIC ENDOCRINOLOGY, 2015, 7 (01) :27-36
[45]   Distribution and Outcomes of Congenital Heart Surgery in Turner Syndrome Using the Society of Thoracic Surgeons Congenital Heart Surgery Database. [J].
Chew, Joshua D. ;
Hill, Kevin D. ;
Jacobs, Marshall L. ;
Jacobs, Jeffrey P. ;
Killen, Stacy A. ;
Godown, Justin ;
Wallace, Amelia S. ;
Chiswell, Karen ;
Bichell, David P. ;
Soslow, Jonathan H. .
CIRCULATION, 2018, 138
[46]   New-onset diabetes mellitus after heart transplantation in children - Incidence and risk factors [J].
Sehgal, Swati ;
Bock, Matthew J. ;
Palac, Hannah Louks ;
Brickman, Wendy J. ;
Gossett, Jeffrey G. ;
Marino, Bradley S. ;
Backer, Carl L. ;
Pahl, Elfriede .
PEDIATRIC TRANSPLANTATION, 2016, 20 (07) :963-969
[47]   A Risk-Prediction Model for In-hospital Mortality After Heart Transplantation in US Children [J].
Almond, C. S. ;
Gauvreau, K. ;
Canter, C. E. ;
Rajagopal, S. K. ;
Piercey, G. E. ;
Singh, T. P. .
AMERICAN JOURNAL OF TRANSPLANTATION, 2012, 12 (05) :1240-1248
[48]   Hearts transplanted after circulatory death in children: Analysis of the International Society for Heart and Lung Transplantation registry [J].
Kleinmahon, Jake A. ;
Patel, Sonali S. ;
Auerbach, Scott R. ;
Rossano, Joseph ;
Everitt, Melanie D. .
PEDIATRIC TRANSPLANTATION, 2017, 21 (08)
[49]   Epidemiological analysis of turner syndrome in children aged 0-14 years: global, regional, and national perspectives (1990-2021) [J].
Ding, Fang ;
Xu, Jiaoli ;
Xiong, Jingxuan ;
Li, Qinhong ;
Cheng, Zugen ;
Deng, Lili .
FRONTIERS IN ENDOCRINOLOGY, 2025, 16
[50]   Heart Transplantation in Heterotaxy Syndrome is Associated With Reduced Survival and Increased Cost of Care: A Multicenter Linked Registry Analysis [J].
Duong, Son Q. ;
Godown, Justin ;
Soslow, Jonathan H. ;
Thurm, Cary ;
Hall, Matt ;
Dodd, Debra A. ;
Feingold, Brian .
CIRCULATION, 2017, 136