Fayssoil Abdallah, Ogna Adam, Chaffaut Cendrine, Lamothe Laure, Ambrosi Xavier, Nardi Olivier, Prigent Helene, Clair Bernard, Lofaso Frederic, Chevret Sylvie, Orlikowski David, Annane Djillali
Service de Réanimation Médicale et Unité de Ventilation à Domicile, CHU Raymond Poincaré, APHP, Université de Versailles Saint Quentin en Yvelines Centre d'Investigation Clinique et Innovation Technologique CIC 14.29, INSERM, Garches Institut de Myologie, CHU Pitié Salpetrière, Centre de Référence Neuro Musculaire Paris Est SBIM, CHU Saint Louis, APHP, Université Paris Diderot, Paris Service de Physiologie-Explorations Fonctionnelles, CHU Raymond Poincaré, APHP, Université de Versailles Saint Quentin en Yvelines, Garches, France.
Medicine (Baltimore). 2018 Jul;97(27):e11381. doi: 10.1097/MD.0000000000011381.
Heart impairment is classical in dystrophinopathies and its management relies on medical drugs. Mechanical ventilation is used to treat respiratory failure, but can affect cardiac function. We aimed to investigate the natural history of cardiac function in patients with Duchenne (DMD) and Becker (BMD) muscular dystrophies on home mechanical ventilation (HMV).We reviewed the chart of DMD and BMD patients, followed in our institution, to obtain ventilation setting at HMV initiation and echocardiographic data at baseline and end follow up, as well as onset cardiac events and thoracic mechanical complication. We analyzed cumulative incidence of cardiac events as well as echocardiographic parameters evolution and its association with ventilation settings.We included 111 patients (101 DMD and 10 BMD). Median age was 21 years [18-26], median pulmonary vital capacity (VC) 15% of predicted [10-24]. All patients were on HMV and 46% ventilated using tracheostomy. After a median follow up of 6.3 years, we found a slight decrease of the left ventricular ejection fraction (LVEF) (45% at end follow up vs 50% at baseline P = .019) and a stabilization of the LV end diastolic diameter indexed (LVEDD indexed 29.4 mm/m vs 30.7 mm/m at end follow up, P = .17). Tidal volume (VT) level was inversely associated with the annual rate of the LVEF decline (r = -0.29, P = .025). Left atrium (LA) diameter decreased with mechanical ventilation (24 mm vs 20 mm, P = .039) and we found a reduction of systolic pulmonary pressure (35 mm Hg vs 25 mm Hg, P = .011). The cumulative incidence of cardiac events was 12.6%. Pneumothorax occurred in 4% of patients. Hypoxic arrest secondary to the presence of tracheal plugin occurred in 4% of patients with invasive ventilation.HMV is not harmful, decreases pulmonary pressure and may protect heart in dystrophinopathies, in addition with cardioprotective drugs. In patients with DMD and BMD on HMV, cumulative incidence of cardiac events remains moderate and incidence of pneumothorax is rare.
在肌营养不良症中，心脏损害很常见，其治疗依赖于药物。机械通气用于治疗呼吸衰竭，但可能影响心脏功能。我们旨在研究接受家庭机械通气（HMV）的杜氏（DMD）和贝克氏（BMD）肌营养不良症患者心脏功能的自然病史。我们回顾了在我们机构随访的DMD和BMD患者的病历，以获取HMV开始时的通气设置以及基线和随访结束时的超声心动图数据，以及心脏事件的发生情况和胸部机械并发症。我们分析了心脏事件的累积发生率以及超声心动图参数的演变及其与通气设置的关联。我们纳入了111例患者（101例DMD和10例BMD）。中位年龄为21岁[18 - 26岁]，中位肺肺活量（VC）为预测值的15%[10 - 24%]。所有患者均接受HMV治疗，46%通过气管切开进行通气。中位随访6.3年后，我们发现左心室射血分数（LVEF）略有下降（随访结束时为45%，基线时为50%，P = 0.019），左心室舒张末期内径指数（LVEDD指数）稳定（随访结束时为29.4 mm/m，基线时为30.7 mm/m，P = 0.17）。潮气量（VT）水平与LVEF下降的年速率呈负相关（r = -0.29，P = 0.025）。左心房（LA）直径随着机械通气而减小（24 mm对20 mm，P = 0.039），并且我们发现收缩期肺动脉压降低（35 mmHg对25 mmHg，P = 0.011）。心脏事件的累积发生率为12.6%。4%的患者发生气胸。4%接受有创通气的患者因气管插管出现低氧性心跳骤停。HMV无害，可降低肺动脉压，并且在肌营养不良症中可能保护心脏，此外还有心脏保护药物。在接受HMV的DMD和BMD患者中，心脏事件的累积发生率仍然适中，气胸的发生率很低。
