Johns Hopkins Bloomberg School of Public Health (J.S., D.L.R.), Johns Hopkins University, Baltimore.
National Human Genome Research Institute (J.S.), National Institutes of Health, Bethesda, MD.
Circ Genom Precis Med. 2023 Dec;16(6):e004133. doi: 10.1161/CIRCGEN.123.004133. Epub 2023 Nov 28.
Limiting high-intensity exercise is recommended for patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) due to its association with penetrance, arrhythmias, and structural progression. Guidelines recommend shared decision-making (SDM) for exercise level, but there is little evidence regarding its impact. Therefore, we sought to evaluate the extent and implications of SDM for exercise, decisional conflict, and decisional regret in patients with ARVC and at-risk relatives.
Adults diagnosed with ARVC or with positive genetic testing enrolled in the Johns Hopkins ARVC Registry were invited to complete a questionnaire that included exercise history and current exercise, SDM (SDM-Q-9), decisional conflict, and decisional regret.
The response rate was 64.8%. Two-thirds of participants (68.0%, n=121) reported clinically significant decisional conflict regarding exercise at diagnosis/genetic testing (DCS [decisional conflict scale]≥25), and half (55.1%, n=98) in the past year. Prevalence of decisional regret was also high with 55.3% (n=99) reporting moderate to severe decisional regret (DRS [decisional regret scale]≥25). The extent of SDM was highly variable ranging from no (0) to perfect (100) SDM (mean, 59.6±25.0). Those diagnosed in adolescence (≤age 21) reported significantly more SDM (=0.013). Importantly, SDM was associated with less decisional conflict (ß=-0.66, R=0.567, <0.01) and decisional regret (ß=-0.37, R=0.180, <0.001) and no difference in vigorous intensity aerobic exercise in the 6 months after diagnosis/genetic testing or the past year (=0.56; =0.34, respectively).
SDM is associated with lower decisional conflict and decisional regret; and no difference in postdiagnosis exercise. Our data thus support SDM as the preferred model for exercise discussions for ARVC.
由于心律失常性右室心肌病（ARVC）与穿透性、心律失常和结构进展有关，因此建议限制高强度运动。指南建议对运动水平进行共同决策（SDM），但关于其影响的证据很少。因此，我们试图评估 ARVC 患者和高危亲属的 SDM 在运动、决策冲突和决策后悔方面的程度和影响。
被诊断患有 ARVC 或基因检测呈阳性的成年人被邀请填写一份问卷，其中包括运动史和当前运动、SDM（SDM-Q-9）、决策冲突和决策后悔。
回复率为 64.8%。三分之二的参与者（68.0%，n=121）报告在诊断/基因检测时对运动有明显的决策冲突（DCS[决策冲突量表]≥25），过去一年中有一半（55.1%，n=98）。决策后悔的发生率也很高，55.3%（n=99）报告中度至重度决策后悔（DRS[决策后悔量表]≥25）。SDM 的程度差异很大，从无（0）到完美（100）SDM（平均 59.6±25.0）。在青春期（≤21 岁）被诊断的患者报告的 SDM 明显更多（=0.013）。重要的是，SDM 与较低的决策冲突（β=-0.66，R=0.567，<0.01）和决策后悔（β=-0.37，R=0.180，<0.001）相关，并且在诊断/基因检测后的 6 个月或过去一年中剧烈强度有氧运动没有差异（=0.56；=0.34，分别）。
SDM 与较低的决策冲突和决策后悔相关，并且在诊断后运动中没有差异。因此，我们的数据支持 SDM 作为 ARVC 运动讨论的首选模式。
