Williams Lynne, Frenneaux Michael
Department of Cardiovascular Medicine, University of Birmingham, Edgbaston, Birmingham, West Midlands, UK.
Europace. 2007 Sep;9(9):817-22. doi: 10.1093/europace/eum093. Epub 2007 May 23.
Hypertrophic cardiomyopathy (HCM) is an inherited disease with marked phenotypic variability that includes the extent of hypertrophy, the presence and severity of symptoms, and the natural history of the disease. Symptoms of impaired consciousness (syncope and pre-syncope) occur in approximately 15-25% of patients with hypertrophic cardiomyopathy (HCM). In young patients a history of recurrent syncope is associated with an increased risk of sudden death. Detailed investigations identify a probable mechanism in a minority of these, usually paroxysmal atrial fibrillation or ventricular tachycardia. In the majority, however, no likely mechanism is found despite extensive investigation. Although this may be the case, it is still of vital importance to exclude potentially treatable causes of syncope.
肥厚型心肌病(HCM)是一种具有显著表型变异性的遗传性疾病,其表型变异包括肥厚程度、症状的有无及严重程度,以及疾病的自然史。意识障碍症状(晕厥和先兆晕厥)约发生在15%-25%的肥厚型心肌病(HCM)患者中。在年轻患者中,反复晕厥史与猝死风险增加相关。详细检查在少数患者中确定了可能的机制,通常为阵发性心房颤动或室性心动过速。然而,在大多数患者中,尽管进行了广泛检查,仍未发现可能的机制。尽管可能如此,但排除晕厥的潜在可治疗原因仍然至关重要。