The University of Sydney, Sydney, New South Wales, Australia; Women's and Children's Hospital, Adelaide, South Australia, Australia.
Woolcock Institute of Medical Research, Sydney, New South Wales, Australia.
Int J Cardiol. 2022 Oct 1;364:38-43. doi: 10.1016/j.ijcard.2022.06.037. Epub 2022 Jun 14.
Fontan patients have abnormal lung function, in particular restrictive lung disease and low diffusing capacity of carbon monoxide (DLCO). We sought to further characterise these abnormalities with detailed pulmonary function testing and examine associations with clinical parameters.
132 Fontan patients across Australia and New Zealand underwent spirometry, with 126 subjects included in final analyses. Measurement of diffusion capacity (DLCO) including its components (alveolar volume (VA) and rate of uptake of CO (KCO)) and oscillometry (reactance (X5) and resistance (R5)) were assessed in a subset of Fontan patients (n = 44) and healthy controls (n = 12). Double diffusion (to assess diffusing capacity of nitric oxide (DLNO), capillary blood volume (Vc), alveolar capillary membrane function (DmCO)) was performed in Fontan patients (n = 18) and healthy controls (n = 12).
FEV and FVC z-scores were low in Fontan subjects (mean - 1.67 ± 1.24 and - 1.61 ± 1.29, respectively) and correlated with exercise capacity. Compared to controls, z-scores for X5, DLCO, KCO, VA and DLNO were significantly lower in Fontan patients. R5, Vc and DmCO z-scores were preserved. X5 was associated with VA (r = 0.41,p = 0.009) and DmCO (r = 0.61,p = 0.008). Older age at Fontan completion was associated with lower z-scores for FEV (r = -0.46,p = 0.002), FVC (r = -0.47,p = 0.002), X5 (r = -0.32,p = 0.033) and VA (r = -0.36,p = 0.022).
Fontan patients have a reduced DLCO which is largely driven by low VA. Lung stiffness (X5) is increased which is associated with VA and DmCO. These parameters negatively correlate with older age of Fontan completion suggesting that earlier Fontan completion may have a beneficial effect on lung function.
Fontan 患者的肺功能异常，特别是限制性肺病和一氧化碳弥散量（DLCO）降低。我们试图通过详细的肺功能测试进一步描述这些异常，并检查其与临床参数的关系。
澳大利亚和新西兰的 132 名 Fontan 患者接受了肺量测定，其中 126 名患者纳入最终分析。在 Fontan 患者亚组（n=44）和健康对照组（n=12）中评估了扩散能力（DLCO）及其组成部分（肺泡容积（VA）和 CO 摄取率（KCO））和振荡测量（电抗（X5）和阻力（R5））。在 Fontan 患者（n=18）和健康对照组（n=12）中进行了双扩散（以评估一氧化氮扩散能力（DLNO）、毛细血管血容量（Vc）、肺泡毛细血管膜功能（DmCO））。
Fontan 患者的 FEV 和 FVC z 分数较低（平均-1.67±1.24 和-1.61±1.29），与运动能力相关。与对照组相比，Fontan 患者的 X5、DLCO、KCO、VA 和 DLNO 的 z 分数明显较低。R5、Vc 和 DmCO z 分数得到保留。X5 与 VA（r=0.41，p=0.009）和 DmCO（r=0.61，p=0.008）相关。Fontan 完成时年龄较大与 FEV（r=-0.46，p=0.002）、FVC（r=-0.47，p=0.002）、X5（r=-0.32，p=0.033）和 VA（r=-0.36，p=0.022）的 z 分数降低有关。
Fontan 患者的 DLCO 降低，主要由 VA 降低引起。肺僵硬（X5）增加，与 VA 和 DmCO 相关。这些参数与 Fontan 完成时的年龄呈负相关，表明较早完成 Fontan 手术可能对肺功能有益。
