El-Mhadi Samah, Mouine Najat, Benjelloun Halima, Aboudrar Souad, El Bakkali Mustapha
Cardiology A Department, Ibn Sina University Hospital Center, Rabat, Morocco.
Department of Cardiology, Mohammed V Military Hospital, Rabat, Morocco.
Eur Heart J Case Rep. 2024 Feb 5;8(2):ytae073. doi: 10.1093/ehjcr/ytae073. eCollection 2024 Feb.
Primary autonomic failure (PAF) or Bradbury Eggleston syndrome is a neurodegenerative disorder of the autonomic nervous system characterized by orthostatic hypotension.
We report the case of a 76-year-old patient with a history of hypertension, who presented with exercise-induced fatigue. He exhibited systolic hypertension and resting bradycardia in the supine position, with orthostatic hypotension without reactive tachycardia, suggesting dysautonomia. Neurological examination was unremarkable. The patient underwent cardiovascular autonomic testing, revealing evidence of beta-sympathetic deficiency associated with neurogenic orthostatic hypotension. Causes of secondary dysautonomia were excluded. The patient was diagnosed with PAF. Even if managing the combination of supine hypertension and orthostatic hypotension was challenging, significant improvements in functional and haemodynamic status were observed with a personalized management approach.
Throughout this case report, we emphasize the critical need for an evaluation of autonomic function and blood pressure's dynamics in hypertensive patients experiencing orthostatic symptoms, enabling the implementation of tailored therapeutic strategies.
原发性自主神经功能衰竭(PAF)或布拉德伯里 - 埃格尔斯顿综合征是一种以体位性低血压为特征的自主神经系统神经退行性疾病。
我们报告了一例76岁有高血压病史的患者,该患者出现运动诱发的疲劳。他在仰卧位时表现为收缩期高血压和静息性心动过缓,伴有体位性低血压且无反应性心动过速,提示自主神经功能障碍。神经系统检查无异常。患者接受了心血管自主神经测试,结果显示存在与神经源性体位性低血压相关的β-交感神经功能缺陷。继发性自主神经功能障碍的病因被排除。该患者被诊断为PAF。尽管管理仰卧位高血压和体位性低血压的组合具有挑战性,但采用个性化管理方法后,患者的功能和血流动力学状态有了显著改善。
在本病例报告中,我们强调对于有体位性症状的高血压患者,评估自主神经功能和血压动态变化至关重要,这有助于实施针对性的治疗策略。