Institute of Life Sciences, Scuola Superiore Sant'Anna, Pisa, Italy.
Institute of Life Sciences, Scuola Superiore Sant'Anna, Pisa, Italy; Cardiology Division, Fondazione Toscana Gabriele Monasterio, Pisa, Italy.
Eur J Intern Med. 2020 Oct;80:66-72. doi: 10.1016/j.ejim.2020.05.015. Epub 2020 May 29.
Drugs for neurohormonal antagonism are usually denied to patients with cardiac amyloidosis (CA) because of safety concerns.
Patients diagnosed with CA at a tertiary referral centre from 2009 to 2019 were enrolled. In the absence of contraindications, beta-blockers, angiotensin converting enzyme inhibitors or angiotensin receptor blockers (ACEi/ARB), and mineralocorticoid receptor antagonists (MRA) were started or up-titrated.
99 patients were evaluated (72% men, age 80 years [72,83], 33% light-chain and 67% transthyretin amyloidosis); 56% were started on or underwent up-titration of a beta-blocker, 25% of ACEi/ARB, and 39% of MRA; beta-blockers were then prescribed to 87% of patients, ACEi/ARB to 75%, and MRA to 63%, with median bisoprolol, ramipril, valsartan, and spironolactone daily equivalent doses of 2.5 mg, 5 mg, 80 mg, and 25 mg, respectively. Patients starting or starting/up-titrating a beta-blocker did not show a higher frequency of hypotension, fatigue, syncope, symptomatic bradycardia, need for pacemaker implantation, or HF hospitalization. Lower stroke volume and cardiac output (CO) predicted HF hospitalization regardless of amyloidosis type; lower left ventricular ejection fraction predicted hypotension, and lower CO and diastolic blood pressure predicted syncope. Patients who had an ACEi/ARB or MRA being started or up-titrated did not experience more adverse events than other patients.
ACEi/ARB and MRA can be safely used in CA, provided that no contraindications are present, treatment is started at a low dose and slowly up-titrated, and patients are monitored quite closely. Beta-blocker therapy is less tolerated in patients with AL amyloidosis and/or worse haemodynamic function.
由于安全性问题，神经激素拮抗剂药物通常不用于治疗心脏淀粉样变性（CA）患者。
从 2009 年到 2019 年，在一家三级转诊中心诊断为 CA 的患者被纳入研究。在没有禁忌症的情况下，开始或增加使用β受体阻滞剂、血管紧张素转换酶抑制剂或血管紧张素受体阻滞剂（ACEi/ARB）和盐皮质激素受体拮抗剂（MRA）。
共评估了 99 例患者（72%为男性，年龄 80 岁[72，83]，33%为轻链淀粉样变性，67%为转甲状腺素淀粉样变性）；56%的患者开始或增加使用β受体阻滞剂，25%的患者开始或增加使用 ACEi/ARB，39%的患者开始或增加使用 MRA；随后，87%的患者处方了β受体阻滞剂，75%的患者处方了 ACEi/ARB，63%的患者处方了 MRA，美托洛尔、雷米普利、缬沙坦和螺内酯的日等效剂量分别为 2.5mg、5mg、80mg 和 25mg。开始或开始/增加β受体阻滞剂的患者并未出现更高频率的低血压、疲劳、晕厥、有症状的心动过缓、需要植入起搏器或心力衰竭住院。无论淀粉样变性类型如何，较低的每搏量和心输出量（CO）均预测心力衰竭住院；较低的左心室射血分数预测低血压，较低的 CO 和舒张压预测晕厥。开始或增加 ACEi/ARB 或 MRA 的患者并未经历更多的不良事件。
只要没有禁忌症，以低剂量开始并缓慢增加剂量，并密切监测患者，ACEi/ARB 和 MRA 可安全用于 CA。AL 淀粉样变性和/或更差的血液动力学功能的患者对β受体阻滞剂治疗的耐受性较差。
