Okamoto Nao, Kubo Toru, Nakashima Yasuteru, Ochi Yuri, Takahashi Asa, Baba Yuichi, Hirota Takayoshi, Yamasaki Naohito, Kitaoka Hiroaki
Department of Cardiology and Geriatrics, Kochi Medical School, Kochi University, Japan.
Intern Med. 2020 Feb 1;59(3):339-343. doi: 10.2169/internalmedicine.3675-19. Epub 2019 Oct 7.
Objective Transthyretin amyloidosis, particularly wild-type transthyretin amyloid cardiomyopathy (ATTRwt), has been recognized as an important cause of morbidity and mortality in the aging population. However, it is difficult to manage heart failure itself in patients with cardiac amyloidosis. Methods We herein report the management of heart failure in an elderly patient with severe heart failure due to ATTRwt. We also review the clinical situation in an additional seven patients with cardiac amyloidosis who were administered pimobendan in our hospital. Results We treated a 71-year-old man with refractory heart failure due to ATTRwt. He was expected to be dependent on dobutamine infusion. We administered pimobendan and successfully improved his symptoms and hemodynamic status to allow his discharge from the hospital. An additional retrospective investigation observed that there were eight patients with ATTR amyloidosis who were administered pimobendan. Although all of the patients at the time of administration of pimobendan were NYHA class III or IV with repeated hospitalization for heart failure, pimobendan seemed to be effective for improving symptoms and enabling patients to be discharged and receive outpatient medical care. Furthermore, focusing on the changes in some biomarkers, we found that the brain natriuretic peptide and estimated glomerular filtration rate values improved after the administration of pimobendan in 5 consecutive patients for whom data were available without additional treatment (p=0.018 and 0.051, respectively). Conclusion In clinical practice, pimobendan seems to have beneficial effects in heart failure management for improving physical activities and the quality of life in patients with transthyretin cardiac amyloidosis.
目的 转甲状腺素蛋白淀粉样变性，尤其是野生型转甲状腺素蛋白淀粉样心肌病（ATTRwt），已被公认为老年人群发病和死亡的重要原因。然而，心脏淀粉样变性患者的心力衰竭本身难以处理。方法 我们在此报告一名因ATTRwt导致严重心力衰竭的老年患者的心力衰竭管理情况。我们还回顾了我院另外7例接受匹莫苯丹治疗的心脏淀粉样变性患者的临床情况。结果 我们治疗了一名因ATTRwt导致难治性心力衰竭的71岁男性。预计他将依赖多巴酚丁胺输注。我们给予匹莫苯丹，成功改善了他的症状和血流动力学状态，使其得以出院。另外一项回顾性调查发现，有8例ATTR淀粉样变性患者接受了匹莫苯丹治疗。尽管在给予匹莫苯丹时所有患者均为纽约心脏协会（NYHA）III级或IV级，且因心力衰竭反复住院，但匹莫苯丹似乎对改善症状、使患者出院并接受门诊医疗有效。此外，关注一些生物标志物的变化，我们发现连续5例有可用数据且未接受额外治疗的患者在给予匹莫苯丹后，脑钠肽和估算肾小球滤过率值有所改善（分别为p = 0.018和0.051）。结论 在临床实践中，匹莫苯丹似乎在心力衰竭管理中具有有益作用，可改善转甲状腺素蛋白心脏淀粉样变性患者的身体活动和生活质量。
