Kocot Krzysztof, Barański Kamil, Gondko Daniel, Smolarek-Kurasz Olga, Kusa Jacek
Department of Pediatric Cardiology, Faculty of Medical Sciences in Katowice, Medical University of Silesia in Katowice, 40-752 Katowice, Poland.
Department of Epidemiology, Faculty of Medical Sciences in Katowice, Medical University of Silesia in Katowice, 40-752 Katowice, Poland.
Children (Basel). 2025 Apr 24;12(5):548. doi: 10.3390/children12050548.
: Management of complex congenital heart defects with functionally single ventricle remains one of the greatest challenges of pediatric cardiology. The multistage surgical treatment completed with Fontan procedure is related to multiple complications. Due to non-pulsatile continuous pulmonary flow and chronic hypoxia, Fontan circulation may induce pulmonary endothelial dysfunction. However, the impact of Fontan physiology on respiratory system function is not well studied. The aim of the research was to assess respiratory function in Fontan pediatric patients with hypoplastic left heart syndrome (HLHS) and other morphologies of functionally single ventricle. The article presents the preliminary results drawn from the pilot study, focusing on Fontan patients, without a healthy children control group. : A cross-sectional study involved Fontan patients hospitalized in the Pediatric Cardiology Clinic of the Medical University of Silesia in Katowice between August 2023 and November 2024. The exclusion criteria were lack of parental and/or patient's consent, age < 6 years old, decompensated heart failure, asthma, atopy, respiratory infection within two weeks before the hospitalization, or significant psychomotor disability. Respiratory function assessment involved spirometry and fractional exhaled nitric oxide (FeNO) measurement. : A total of 32 patients who met inclusion criteria performed respiratory measurements. The mean age was 12.9 years old; there were 12 females. A total of 12 patients had HLHS and 20 patients had other morphologies of univentricular heart. FeNO values were relatively high with a mean of 30 ppb. Spirometry showed restrictive or mixed restrictive and obstructive ventilatory pattern. The mean forced vital capacity (FVC) levels were 79.2 ± 12.3% of predicted value (%pv) and forced expiratory volume in the first second (FEV1) 77.3 ± 13.8%pv. Children with HLHS presented statistically significantly lower percentages of predicted value of FEV1. There were statistically significant negative correlations between NT-proBNP concentrations and FEV1, FEV1%pv, MEF25-75 and MEF25-75%pv. : Fontan pediatric patients present a restrictive or mixed restrictive and obstructive ventilatory pattern and relatively high FeNO levels. Patients with HLHS have worse pulmonary function than patients with other univentricular heart morphologies. This may be related to worse ventricular function in patients with HLHS.
功能性单心室复杂先天性心脏病的管理仍然是小儿心脏病学面临的最大挑战之一。采用Fontan手术完成的多阶段手术治疗与多种并发症相关。由于非搏动性持续肺血流和慢性缺氧，Fontan循环可能导致肺内皮功能障碍。然而，Fontan生理学对呼吸系统功能的影响尚未得到充分研究。本研究的目的是评估患有左心发育不全综合征（HLHS）和其他功能性单心室形态的Fontan患儿的呼吸功能。本文介绍了一项初步研究的结果，该研究聚焦于Fontan患者，未设置健康儿童对照组。
一项横断面研究纳入了2023年8月至2024年11月期间在卡托维兹西里西亚医科大学儿科心脏病诊所住院的Fontan患者。排除标准包括缺乏父母和/或患者的同意、年龄<6岁、失代偿性心力衰竭、哮喘、特应性、住院前两周内的呼吸道感染或严重精神运动障碍。呼吸功能评估包括肺活量测定和呼出一氧化氮分数（FeNO）测量。
共有32名符合纳入标准的患者进行了呼吸测量。平均年龄为12.9岁；有12名女性。共有12名患者患有HLHS，20名患者患有其他单心室心脏形态。FeNO值相对较高，平均为30 ppb。肺活量测定显示为限制性或混合性限制性和阻塞性通气模式。平均用力肺活量（FVC）水平为预测值的79.2±12.3%（%pv），第一秒用力呼气量（FEV1）为77.3±13.8%pv。HLHS患儿的FEV1预测值百分比在统计学上显著较低。NT-proBNP浓度与FEV1、FEV1%pv、MEF25-75和MEF25-75%pv之间存在统计学上的显著负相关。
Fontan患儿呈现限制性或混合性限制性和阻塞性通气模式以及相对较高的FeNO水平。HLHS患者的肺功能比其他单心室心脏形态的患者更差。这可能与HLHS患者较差的心室功能有关。
