Öztürk Ayse-Gül, Dellborg Mikael, Damlin Anna, Giang Kok Wai, Mandalenakis Zacharias, Sörensson Peder
Department of Medicine Solna, Karolinska Institutet, Stockholm, Sweden.
Department of Cardiology, Karolinska University Hospital, Stockholm, Sweden.
Int J Cardiol Congenit Heart Dis. 2024 Feb 18;15:100503. doi: 10.1016/j.ijcchd.2024.100503. eCollection 2024 Mar.
Children with univentricular heart (UVH) have a limited life expectancy without early treatment. Long-term survival in UVH, in an unselected nationwide cohort, is unclear.
To determine long-term survival in patients with UVH including non-operated patients compared with a control population in Sweden.
Patients with UVH born between 1970 and 2017 were identified from the National Registers and were matched for birth year and sex with 10 individuals without congenital heart disease. Follow-up was from birth until death, transplantation, or the end of study. Mortality risk was estimated by Cox proportional regression models and Kaplan-Meier survival analysis.
We included 5075 patients with UVH including 758 (14.9%) patients with hypoplastic left heart syndrome (HLHS), and 50,620 matched controls. Median follow-up time was 13.6 (IQR 0.7; 26.8) years. The hazard ratio for death in patients with UVH was 53.0 (95% confidence interval, 48.0-58.6), and for HLHS, 163.5 (95% CI, 124.3-215.2). In patients with HLHS, 84% of those who were born between 1982 and 1993 died or had transplantation during the first year of life compared with 29% born between 2006 and 2017. In patients with UVH without HLHS, death/transplantation in the first year of life declined from 36% in those born between 1970 and 1981 to 8.7% in those born between 2006 and 2017.
The risk of mortality was >50 times higher in patients with UVH than in controls. The survival rate increased with a later decade of birth but was still <75% in patients born with HLHS.
单心室心脏（UVH）患儿若不接受早期治疗，预期寿命有限。在未经过挑选的全国队列中，UVH患者的长期生存率尚不清楚。
确定UVH患者（包括未接受手术治疗的患者）与瑞典对照人群相比的长期生存率。
从国家登记处识别出1970年至2017年出生的UVH患者，并按出生年份和性别与10名无先天性心脏病的个体进行匹配。随访从出生直至死亡、移植或研究结束。通过Cox比例回归模型和Kaplan-Meier生存分析估计死亡风险。
我们纳入了5075例UVH患者，其中包括758例（14.9%）左心发育不全综合征（HLHS）患者，以及50620名匹配的对照者。中位随访时间为13.6（四分位间距0.7；26.8）年。UVH患者的死亡风险比为53.0（95%置信区间，48.0 - 58.6），HLHS患者为163.5（95%CI，124.3 - 215.2）。在HLHS患者中，1982年至1993年出生的患者中有84%在出生后第一年内死亡或接受了移植，而2006年至2017年出生的患者这一比例为29%。在无HLHS的UVH患者中，出生后第一年内的死亡/移植率从1970年至1981年出生的患者中的36%降至2006年至2017年出生的患者中的8.7%。
UVH患者死亡风险比对照者高50倍以上。生存率随出生年代的后移而提高，但HLHS患者的生存率仍低于75%。
