Diego A. Lara1, Mary K. Ethen2, Mark A. Canfield2, Wendy N. Nembhard3, Shaine A. Morris1
Congenital Heart Disease, Vol.12, No.1, pp. 105-112, 2017
Abstract Background: Hypoplastic left heart syndrome (HLHS) is strongly associated with Turner syndrome
(TS); outcome data when these conditions coexist is sparse. We aimed to investigate long-term
survival and causes of death in this population.
Methods: The Texas Birth Defects Registry was queried for all live born infants with HLHS during
1999–2007. We used Kaplan–Meier and Cox regression analyses to compare survival among
patients with HLHS with TS (HLHS/TS＋) to patients who had HLHS without genetic disorders or
extracardiac birth defects (HLHS/TS–).
Results: Of the 542 patients with HLHS, 11 had TS (2.0%), 71 had other extracardiac birth defects
or genetic… More >
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