A. Guitarte Vidaurre, Y. Dulac, F. Bajanca, M. Langeois, E. Garrigue, B. Chesneau, T. Edouard
{"title":"MARFANPOWER：针对患有马凡氏综合征的儿童和年轻人的家庭心肺和肌肉康复计划的成果","authors":"A. Guitarte Vidaurre, Y. Dulac, F. Bajanca, M. Langeois, E. Garrigue, B. Chesneau, T. Edouard","doi":"10.1016/j.acvd.2024.07.037","DOIUrl":null,"url":null,"abstract":"<div><h3>Introduction</h3><p>Marfan or associated syndromes (MFS) are some rare genetic diseases leading to a multisystem damage related to connective tissue fragility. Chronic fatigue and decreased physical endurance are almost constant complaints of patients with MFS. Muscle mass worsens through adolescence, which could explain the bone-mass deficit observed in this population.</p></div><div><h3>Objective</h3><p>We hypothesize that a personalized exercise rehabilitation program will improve fitness and quality of life (QoL) of these patients.</p></div><div><h3>Methods</h3><p>Self-controlled study with a 6-month home-based cyclo-ergometer and muscular strengthening personalised rehabilitation program based on first ventilatory-threshold. Baseline evaluation was performed 3<!--> <!-->months prior to the start of the rehabilitation program, then at the beginning of the rehabilitation program and at 6<!--> <!-->months, with a mid-term evaluation at 3<!--> <!-->months.</p></div><div><h3>Results</h3><p>We included 28 MFS patients between 7 and 20<!--> <!-->years (mean 12.8<!--> <!-->±<!--> <!-->3.69<!--> <!-->years), of which 11 were females, with a mild aortic dilatation for 50% (mean z-score +2.4), no major valvopathy, no cardiac impairment and a history of pneumothorax for 3 of them; most where under preventive beta-blocker treatment (93%).</p><p>After a 6-month rehabilitation program no progression in aortic diameters was found. Significant improvement in first ventilatory-threshold was achieved (+15.8% of expected value; <em>P</em> <!--><<!--> <!-->0.05), alongside an increase in maximal sustained workload with a mean of +24.5 Watts (17.37–31.63; <em>P</em> <!--><<!--> <!-->0.05) associated to a significant increase in VO<sub>2</sub> (+131.58<!--> <!-->mL.min<sup>−1</sup>; 30.17–232.99; <em>P</em> <!--><<!--> <!-->0.05), whereas maximal heart rate at effort was reduced by 29.95 bpm (16.17–43.73; <em>P</em> <!--><<!--> <!-->0.05). Muscular strength gain was shown both by dominant hand-grip (mean +4.3<!--> <!-->kg; 2.45–5.2; <em>P</em> <!--><<!--> <!-->0.05) and dominant leg strength (+67.33<!--> <!-->Nm; 25.08–109.59; <em>P</em> <!--><<!--> <!-->0.05). Overall, self and proxy-reported QoL of participants improved (<span><span>Fig. 1</span></span>).</p></div><div><h3>Conclusion</h3><p>Young patients with MFS can safely benefit for a home-based rehabilitation program personalised according to their baseline capacities, improving both their overall fitness, muscular strength and cardiovascular health; with a favourable impact in their global QoL.</p></div>","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":null,"pages":null},"PeriodicalIF":2.3000,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"MARFANPOWER: Results of a home-based cardiorespiratory and muscle rehabilitation program for children and young adults with Marfan syndrome\",\"authors\":\"A. Guitarte Vidaurre, Y. Dulac, F. Bajanca, M. Langeois, E. Garrigue, B. Chesneau, T. Edouard\",\"doi\":\"10.1016/j.acvd.2024.07.037\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><h3>Introduction</h3><p>Marfan or associated syndromes (MFS) are some rare genetic diseases leading to a multisystem damage related to connective tissue fragility. Chronic fatigue and decreased physical endurance are almost constant complaints of patients with MFS. Muscle mass worsens through adolescence, which could explain the bone-mass deficit observed in this population.</p></div><div><h3>Objective</h3><p>We hypothesize that a personalized exercise rehabilitation program will improve fitness and quality of life (QoL) of these patients.</p></div><div><h3>Methods</h3><p>Self-controlled study with a 6-month home-based cyclo-ergometer and muscular strengthening personalised rehabilitation program based on first ventilatory-threshold. Baseline evaluation was performed 3<!--> <!-->months prior to the start of the rehabilitation program, then at the beginning of the rehabilitation program and at 6<!--> <!-->months, with a mid-term evaluation at 3<!--> <!-->months.</p></div><div><h3>Results</h3><p>We included 28 MFS patients between 7 and 20<!--> <!-->years (mean 12.8<!--> <!-->±<!--> <!-->3.69<!--> <!-->years), of which 11 were females, with a mild aortic dilatation for 50% (mean z-score +2.4), no major valvopathy, no cardiac impairment and a history of pneumothorax for 3 of them; most where under preventive beta-blocker treatment (93%).</p><p>After a 6-month rehabilitation program no progression in aortic diameters was found. 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引用次数: 0
摘要
导言马凡氏综合征或相关综合征（MFS）是一种罕见的遗传性疾病，会导致与结缔组织脆性有关的多系统损伤。慢性疲劳和身体耐力下降几乎是马凡氏综合征患者的主诉。我们假设，个性化的运动康复计划将改善这些患者的体能和生活质量（QoL）。方法：基于首次通气阈值，进行为期 6 个月的家庭自行车测力计和肌肉强化个性化康复计划的自我对照研究。在康复计划开始前 3 个月进行基线评估，然后在康复计划开始时和 6 个月时进行评估，并在 3 个月时进行中期评估。其中 11 人为女性，50% 的患者有轻度主动脉扩张（平均 Z 值 +2.4），无重大瓣膜病，无心功能损害，其中 3 人有气胸病史；大多数患者接受了β-受体阻滞剂的预防性治疗（93%）。在为期 6 个月的康复计划后，主动脉直径没有出现恶化。首次通气阈值有了明显改善（预期值的 +15.8%；P < 0.05），同时最大持续工作量也有所增加，平均为 +24.5 瓦特（17.37-31.63；P < 0.05）。63; P <0.05），这与 VO2 的显著增加有关（+131.58 mL.min-1; 30.17-232.99; P <0.05），而用力时的最大心率降低了 29.95 bpm (16.17-43.73; P <0.05)。优势手握力（平均 +4.3 公斤；2.45-5.2；P <；0.05）和优势腿力（+67.33 牛米；25.08-109.59；P <；0.05）均显示出肌肉力量的增加。总体而言，参与者的自我和委托人报告的 QoL 均有所改善（图 1）。
MARFANPOWER: Results of a home-based cardiorespiratory and muscle rehabilitation program for children and young adults with Marfan syndrome
Introduction
Marfan or associated syndromes (MFS) are some rare genetic diseases leading to a multisystem damage related to connective tissue fragility. Chronic fatigue and decreased physical endurance are almost constant complaints of patients with MFS. Muscle mass worsens through adolescence, which could explain the bone-mass deficit observed in this population.
Objective
We hypothesize that a personalized exercise rehabilitation program will improve fitness and quality of life (QoL) of these patients.
Methods
Self-controlled study with a 6-month home-based cyclo-ergometer and muscular strengthening personalised rehabilitation program based on first ventilatory-threshold. Baseline evaluation was performed 3 months prior to the start of the rehabilitation program, then at the beginning of the rehabilitation program and at 6 months, with a mid-term evaluation at 3 months.
Results
We included 28 MFS patients between 7 and 20 years (mean 12.8 ± 3.69 years), of which 11 were females, with a mild aortic dilatation for 50% (mean z-score +2.4), no major valvopathy, no cardiac impairment and a history of pneumothorax for 3 of them; most where under preventive beta-blocker treatment (93%).
After a 6-month rehabilitation program no progression in aortic diameters was found. Significant improvement in first ventilatory-threshold was achieved (+15.8% of expected value; P < 0.05), alongside an increase in maximal sustained workload with a mean of +24.5 Watts (17.37–31.63; P < 0.05) associated to a significant increase in VO2 (+131.58 mL.min−1; 30.17–232.99; P < 0.05), whereas maximal heart rate at effort was reduced by 29.95 bpm (16.17–43.73; P < 0.05). Muscular strength gain was shown both by dominant hand-grip (mean +4.3 kg; 2.45–5.2; P < 0.05) and dominant leg strength (+67.33 Nm; 25.08–109.59; P < 0.05). Overall, self and proxy-reported QoL of participants improved (Fig. 1).
Conclusion
Young patients with MFS can safely benefit for a home-based rehabilitation program personalised according to their baseline capacities, improving both their overall fitness, muscular strength and cardiovascular health; with a favourable impact in their global QoL.
期刊介绍：
The Journal publishes original peer-reviewed clinical and research articles, epidemiological studies, new methodological clinical approaches, review articles and editorials. Topics covered include coronary artery and valve diseases, interventional and pediatric cardiology, cardiovascular surgery, cardiomyopathy and heart failure, arrhythmias and stimulation, cardiovascular imaging, vascular medicine and hypertension, epidemiology and risk factors, and large multicenter studies. Archives of Cardiovascular Diseases also publishes abstracts of papers presented at the annual sessions of the Journées Européennes de la Société Française de Cardiologie and the guidelines edited by the French Society of Cardiology.
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