Magnusson Peter, Gadler Fredrik, Mörner Stellan
Uppsala Universitet - Centrum för forskning och utveckling, Region Gävleborg/Gävle Uppsala, Sweden Uppsala Universitet - Centrum för forskning och utveckling, Region Gävleborg/Gävle Uppsala, Sweden.
Karolinska Institutet - Inst f Medicin Stockholm, Sweden Karolinska Institutet - Inst f Medicin Stockholm, Sweden.
Lakartidningen. 2018 Jun 11;115:E4XX.
Hypertrophic cardiomyopathy is the most common cardiogenetic disease affecting 1/500-1/1 000 individuals. Dyspnea is common but chest pain, dizziness or fainting may also cause considerable limitation for the patient. The diagnosis can be suspected from ECG. Echocardiography confirms hypertrophy of at least 15 mm, usually in the septum. If the obstruction of the outflow tract is severe, myectomy or alcohol ablation can relieve symptoms. Genetic evaluation of family members is advisable. To reduce symptoms, betablockers are used; verapamil or disopyramide are alternatives. Atrial fibrillation is often prevalent and requires special attention concerning anticoagulation and rhythm or rate control. An end-stage heart failure warrants advanced treatment options such as cardiac resynchronization therapy, ventricular assist devices or heart transplant. Sudden cardiac death is unpredictable and evaluation of risk markers is important to identify potential candidates for an implantable defibrillator.
肥厚型心肌病是最常见的心脏源性疾病,影响着1/500至1/1000的人群。呼吸困难很常见,但胸痛、头晕或昏厥也可能给患者带来相当大的限制。心电图可怀疑诊断。超声心动图可确认至少15毫米的肥厚,通常在室间隔。如果流出道梗阻严重,心肌切除术或酒精消融可缓解症状。建议对家庭成员进行基因评估。为减轻症状,可使用β受体阻滞剂;维拉帕米或丙吡胺是替代药物。心房颤动通常很常见,在抗凝以及心律或心率控制方面需要特别关注。终末期心力衰竭需要先进的治疗方案,如心脏再同步治疗、心室辅助装置或心脏移植。心脏性猝死不可预测,评估风险标志物对于识别植入式除颤器的潜在候选者很重要。