Silveira João Bruno Piantino Dias Moura, Turquetto Aida Luiza Ribeiro, Amato Luciana Patrick, Agostinho Daniela Regina, Caneo Luiz Fernando, Binotto Maria Angelica, da Costa Soares Lopes Maria Isabel, Rodrigues Joaquim Carlos, Santos Marcus Vinicius Barbosa, Oliveira Patricia Alves, TanaKa Ana Cristina Sayuri, Jatene Marcelo Biscegli
Pediatric Cardiac Surgery Unit, Instituto do Coração do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (InCor-FMUSP), São Paulo, Brazil.
Pulmonary Function Laboratory, Instituto da Criança do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (ICr-FMUSP), São Paulo, Brazil.
Pediatr Cardiol. 2024 Oct 21. doi: 10.1007/s00246-024-03666-8.
Studies have shown that respiratory muscle training enhances functional capacity and pulmonary function in Fontan patients. However, diaphragm muscle characteristics in Fontan children have not been fully elucidated. The aim of this study was to compare respiratory function, maximal and submaximal functional capacities, and quality of life, as well as to assess diaphragm mobility and thickness, between Fontan patients aged 8 to 12 years and healthy individuals. This cross-sectional study included 45 children-27 Fontan patients, Fontan Group (FG) and 18 healthy control Group (CG) subjects. Different examinations were performed: spirometry was used to analyze pulmonary volume and capacity; manovacuometry was used to determine respiratory muscle strength; chest ultrasound was used to determine diaphragm muscle characteristics; cardiopulmonary exercise and the six-minute walk test (6MWT) were used to determine functional capacity; the AUQEI questionnaire was used to determine quality of life. Descriptive analysis and intergroup comparisons were performed for all the data. Compared with the CG, the FG exhibited impaired pulmonary function and reduced functional capacity. Significant differences in median values were noted for forced expiratory volume in one second (FEV): 2.39 L/min, p = 0.002; forced vital capacity (FVC): 1.73 vs. 3.06 L/min, p = 0.002; maximal inspiratory pressure: - 73 vs. - 117 cmHO, p = 0.007; absolute peak VO: 1.09 vs. 1.51 L/min, p < 0.001; relative peak VO: 31.9 vs. 42.5 mL/kg/min, p = 0.003; and 6MWT distance: 420 vs. 586 m, p < 0.001. Diaphragmatic thickness, mobility and quality of life were similar between the groups. Despite the reduced functional capacity, impaired pulmonary volume and capacity, and respiratory muscle weakness of the FG compared to those of the CG, the diaphragm characteristics and quality of life were similar between the groups.
研究表明，呼吸肌训练可增强Fontan手术患者的功能能力和肺功能。然而，Fontan儿童的膈肌特征尚未完全阐明。本研究的目的是比较8至12岁Fontan患者与健康个体之间的呼吸功能、最大和次最大功能能力以及生活质量，并评估膈肌的活动度和厚度。这项横断面研究纳入了45名儿童，其中27名Fontan手术患者为Fontan组（FG），18名健康对照者为对照组（CG）。进行了不同的检查：使用肺活量测定法分析肺容积和容量；使用压力真空肺活量测定法确定呼吸肌力量；使用胸部超声确定膈肌特征；使用心肺运动试验和六分钟步行试验（6MWT）确定功能能力；使用AUQEI问卷确定生活质量。对所有数据进行描述性分析和组间比较。与CG相比，FG的肺功能受损且功能能力下降。一秒用力呼气量（FEV）的中位数存在显著差异：2.39 L/min，p = 0.002；用力肺活量（FVC）：1.73 vs. 3.06 L/min，p = 0.002；最大吸气压力：-73 vs. -117 cmH₂O，p = 0.007；绝对峰值VO：1.09 vs. 1.51 L/min，p < 0.001；相对峰值VO：31.9 vs. 42.5 mL/kg/min，p = 0.003；以及6MWT距离：420 vs. 586 m，p < 0.001。两组之间的膈肌厚度、活动度和生活质量相似。尽管与CG相比，FG的功能能力下降、肺容积和容量受损以及呼吸肌无力，但两组之间的膈肌特征和生活质量相似。
