Department of Cardiology, The Children's Memorial Health Institute, Al. Dzieci Polskich 20, 04-730, Warsaw, Poland.
Pediatr Cardiol. 2020 Dec;41(8):1725-1729. doi: 10.1007/s00246-020-02434-8. Epub 2020 Aug 9.
Functional status assessed by the WHO-FC scale derived from adults is a known prognostic factor for pulmonary hypertension. Data on the usefulness of the Panama-FC scale in assessing children with pulmonary hypertension are limited. The study was performed to compare functional status results (WHO-FC and Panama-FC) and to assess the usefulness of these scales in various clinical situations. The reliability of the Panama-FC questionnaire method for facilitating patient evaluation was also examined. 26 functional status assessments (7 in disease progression/after treatment intensification) in both scales were analyzed in 19 patients with PAH confirmed in RHC. WHO-FC, Panama-FC scales, and questionnaire-based on Panama-FC were conducted independently by three different physicians. Results of assessments were compared with each other and with 6MWD, NTproBNP level, and echo parameters (TAPSE, RV/LV ratio). The Panama-FC scale results obtained using the medical interview method and questionnaire did not differ. Both WHO-FC and Panama-FC classes well-reflected disease advancement confirmed by non-invasive parameters (NTproBNP, 6MWD, TAPSE, RV/LV ratio). Differences between grading the class in both scales were observed: 5pts were classified to II (Panama-FC) vs I (WHO-FC), 2pts were in lower risk group in WHO-FC (II) vs Panama (IIIa). Worsening or improvement after treatment intensification in functional status in both scales was connected with the significant change of NTproBNP level. The 6-min walking distance did not change. TAPSE, RV/LV ratio changed significantly in 3pts with IPAH, accordingly to change in WHO-FC and Panama-FC. WHO-FC and Panama-FC well reflect the disease advancement. The questionnaire method simplified the use of the Panama-FC scale. The Panama-FC scale appears to be better for assessing functional status during long-term follow-up, while the WHO-FC scale was more useful in short-term treatment monitoring.
采用成人 WHO-FC 量表评估的功能状态是肺动脉高压的已知预后因素。关于巴拿马-FC 量表在评估肺动脉高压儿童中的作用的数据有限。该研究旨在比较功能状态评估结果（WHO-FC 和巴拿马-FC），并评估这些量表在各种临床情况下的用途。还检查了巴拿马-FC 问卷方法在促进患者评估中的可靠性。对 19 例经 RHC 证实的 PAH 患者的 26 项功能状态评估（7 项在疾病进展/治疗强化后）进行了分析，分别采用 WHO-FC 和巴拿马-FC 量表进行评估，由 3 位不同的医生独立进行。将评估结果相互比较，并与 6MWD、NTproBNP 水平和超声参数（TAPSE、RV/LV 比）进行比较。通过医疗访谈方法和问卷获得的巴拿马-FC 量表结果没有差异。WHO-FC 和巴拿马-FC 两类均能很好地反映非侵入性参数（NTproBNP、6MWD、TAPSE、RV/LV 比）证实的疾病进展。在两个量表中，分级差异观察到：5 分被归类为 II（巴拿马-FC）与 I（WHO-FC），2 分在 WHO-FC（II）中属于低风险组与巴拿马（IIIa）。功能状态在两个量表中的治疗强化后恶化或改善与 NTproBNP 水平的显著变化有关。6 分钟步行距离没有变化。TAPSE、RV/LV 比值在 3 例特发性肺动脉高压患者中发生变化，与 WHO-FC 和巴拿马-FC 的变化相对应。WHO-FC 和巴拿马-FC 能很好地反映疾病进展。问卷方法简化了巴拿马-FC 量表的使用。在长期随访中，巴拿马-FC 量表似乎更适合评估功能状态，而 WHO-FC 量表在短期治疗监测中更有用。
