Mejren Ali Hussein Jaber, Fensman Sie Kronborg, Poulsen Steen Hvitfeldt
Department of Cardiology, Aarhus University Hospital, Palle Juul-Jensens Boulevard 99, Aarhus N 8200, Denmark.
Eur Heart J Case Rep. 2025 May 12;9(5):ytaf233. doi: 10.1093/ehjcr/ytaf233. eCollection 2025 May.
Transthyretin amyloid cardiomyopathy (ATTR-CM) is a restrictive cardiomyopathy caused by amyloid deposition in the myocardium. Its phenotypical overlap with hypertrophic cardiomyopathy, particularly in cases involving left ventricular outflow tract obstruction (LVOTO), challenges accurate diagnosis. Medical management of LVOTO in ATTR-CM is challenged by the opposing effects of beta-blockers and diuretics.
A 79-year-old male with left ventricular hypertrophy and LVOTO presented with worsening dyspnoea. Full diagnostic work-up confirmed wild-type ATTR-CM. A conservative medical approach with diuretics and beta-blockers proved challenging. Alcohol septal ablation was successfully performed without major complication, resolving the LVOTO and improving symptoms.
The diagnosis and management of ATTR-CM with LVOTO are complex. A thorough diagnostic approach is needed to avoid mismanagement. Diuretics and beta-blockers must be carefully balanced to achieve optimal clinical results. Alcohol septal ablation may be considered in patients with persistent symptoms and high LVOT gradients despite optimal medical therapy.
转甲状腺素蛋白淀粉样变心肌病（ATTR-CM）是一种由淀粉样物质沉积于心肌引起的限制性心肌病。它与肥厚型心肌病在表型上存在重叠，尤其是在涉及左心室流出道梗阻（LVOTO）的病例中，这对准确诊断构成挑战。在ATTR-CM中，LVOTO的药物治疗受到β受体阻滞剂和利尿剂相反作用的影响。
一名患有左心室肥厚和LVOTO的79岁男性出现呼吸困难加重。全面的诊断检查确诊为野生型ATTR-CM。事实证明，使用利尿剂和β受体阻滞剂的保守药物治疗方法具有挑战性。成功进行了酒精间隔消融术，无重大并发症，解除了LVOTO并改善了症状。
伴有LVOTO的ATTR-CM的诊断和治疗很复杂。需要采用全面的诊断方法以避免管理不当。必须仔细平衡利尿剂和β受体阻滞剂以取得最佳临床效果。对于尽管接受了最佳药物治疗仍有持续症状和高LVOT梯度的患者，可考虑进行酒精间隔消融术。
