Sweer Joszi, Germund Ingo, Khalil Markus, Apitz Christian, Ten Dam Kim, Wendt Stefanie, Sreeram Narayanswami, Udink Ten Cate Floris E A
Department of Pediatric Cardiology, Academic Center for Congenital Heart Disease, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, the Netherlands.
Department of Pediatric Cardiology, Heart Center Cologne, University Hospital Cologne, Cologne, Germany.
Int J Cardiol Congenit Heart Dis. 2023 Sep 12;13:100475. doi: 10.1016/j.ijcchd.2023.100475. eCollection 2023 Sep.
Thyroid dysfunction may have adverse effects on Fontan hemodynamics. Data on thyroid function in pediatric Fontan patients with or without protein-losing enteropathy (PLE) are limited.
This retrospective multicenter study included 67 Fontan patients (median age 10.9 years; 35.8% female; 28.4% PLE) in whom thyroid function testing was performed.
Subclinical hypothyroidism (SHT) was present in 16 (23.9%) patients. Subjects with SHT had significantly lower systolic blood pressure (p = 0.014) and body weight z-score (p = 0.006), were in a worse New York Heart Association (NYHA) functional class (p = 0.004), were more often pacing dependent (p = 0.007), and were more likely to have PLE (p = 0.033, 8/19 (42.1%) patients). Serum thyroid stimulating hormone (TSH) levels were significantly higher in patients with NYHA class ≥ II (p = 0.005), significant atrioventricular valve regurgitation (p = 0.023), elevated serum natriuretic peptides (p = 0.031), and in those with PLE (p = 0.002). Patients with active PLE had significantly higher TSH levels than those in remission (p = 0.003). A strong inverse relationship was found between lower free triiodothyronine (fT3) levels and natriuretic peptides (r: -0.599, p = 0.040). Using binary logistic regression analysis we found that worse NYHA class was an independent predictor of SHT (OR 4.2; 95% CI 1.1-16.1, p = 0.036).
Subclinical thyroid dysfunction is common in Fontan, particularly in patients with hemodynamic derangements and PLE. Future studies are needed to address the prognostic implications of thyroid dysfunction in the Fontan population.
甲状腺功能障碍可能对Fontan循环血流动力学产生不利影响。关于患有或未患有蛋白丢失性肠病（PLE）的小儿Fontan患者甲状腺功能的数据有限。
这项回顾性多中心研究纳入了67例接受甲状腺功能检测的Fontan患者（中位年龄10.9岁；35.8%为女性；28.4%患有PLE）。
16例（23.9%）患者存在亚临床甲状腺功能减退（SHT）。患有SHT的患者收缩压显著降低（p = 0.014），体重z评分降低（p = 0.006），纽约心脏协会（NYHA）心功能分级更差（p = 0.004），更常依赖起搏器（p = 0.007），且更有可能患有PLE（p = 0.033，8/19例（42.1%）患者）。NYHA分级≥II级的患者、存在显著房室瓣反流的患者（p = 0.023）、血清利钠肽升高的患者（p = 0.031）以及患有PLE的患者（p = 0.002），其血清促甲状腺激素（TSH）水平显著更高。活动性PLE患者的TSH水平显著高于缓解期患者（p = 0.003）。游离三碘甲状腺原氨酸（fT3）水平降低与利钠肽之间存在强烈的负相关关系（r：-0.
