Aus dem Siepen Fabian, Hein Selina, Hofmann Eva, Nagel Christian, Schwarting Stéphanie K, Hegenbart Ute, Schönland Stefan O, Weiler Markus, Frey Norbert, Kristen Arnt V
Department of Cardiology, Angiology and Respiratory Medicine, University Hospital Heidelberg, Im Neuenheimer Feld 410, 69120 Heidelberg, Germany.
Department of Hematology, University Hospital Heidelberg, 69120 Heidelberg, Germany.
J Clin Med. 2024 Apr 12;13(8):2257. doi: 10.3390/jcm13082257.
: Cardiac transthyretin amyloidosis (ATTR) is a progressive, fatal disease leading to heart failure due to accumulation of amyloid fibrils in the interstitial space and may occur as a hereditary (ATTRv) or wild-type (ATTRwt) form. Guidelines recommend the use of ACE inhibitors (ACEis) and beta-blockers (BBs) as heart failure therapy (HFT) in all patients with symptomatic heart failure and reduced ejection fraction, independent of the underlying etiology. However, the prognostic benefit of ACEis and BBs in ATTR has not been elucidated in detail yet. We thus sought to retrospectively investigate the outcome of patients with ATTRwt or ATTRv under HFT. Medical records of 403 patients with cardiac ATTR (ATTRwt: n = 268, ATTRv: n = 135) were screened for long-term medication as well as clinical, laboratory, electrocardiographic and echocardiographic data. Patients were assessed between 2005 and 2020 at the University Hospital Heidelberg. Kaplan-Meier analysis was used to analyze potential differences in survival among different subgroups. The mean follow-up was 28 months. In total, 43 patients (32%) with ATTRv and 140 patients (52%) with ATTRwt received HFT. Survival was significantly shorter in patients receiving HFT in ATTRv (46 vs. 83 months, = 0.0007) vs. non-HFT. A significantly better survival was observed in patients with comorbidities (coronary artery disease, arterial hypertension) and HFT among ATTRwt patients ( = 0.004). No significant differences in survival were observed in the other subgroups. Survival analysis revealed a potential benefit of HFT in patients with ATTRwt and cardiac comorbidities such as coronary artery disease and/or arterial hypertension. In contrast, HFT should be used with caution in patients with ATTRv.
心脏转甲状腺素蛋白淀粉样变性（ATTR）是一种进行性致命疾病，由于淀粉样纤维在间质空间积聚导致心力衰竭，可表现为遗传性（ATTRv）或野生型（ATTRwt）形式。指南建议，对于所有有症状性心力衰竭且射血分数降低的患者，无论潜在病因如何，均应使用血管紧张素转换酶抑制剂（ACEi）和β受体阻滞剂（BB）作为心力衰竭治疗（HFT）。然而，ACEi和BB在ATTR中的预后益处尚未得到详细阐明。因此，我们试图回顾性研究接受HFT的ATTRwt或ATTRv患者的结局。对403例心脏ATTR患者（ATTRwt：n = 268，ATTRv：n = 135）的病历进行筛查，以获取长期用药以及临床、实验室、心电图和超声心动图数据。2005年至2020年期间在海德堡大学医院对患者进行评估。采用Kaplan-Meier分析来分析不同亚组之间生存的潜在差异。平均随访时间为28个月。总共有43例（32%）ATTRv患者和140例（52%）ATTRwt患者接受了HFT。接受HFT的ATTRv患者的生存期明显短于未接受HFT的患者（46个月对83个月， = 0.0007）。在ATTRwt患者中，合并症（冠状动脉疾病、动脉高血压）且接受HFT的患者生存期明显更好（ = 0.004）。在其他亚组中未观察到生存的显著差异。生存分析显示，HFT对患有ATTRwt且合并心脏疾病如冠状动脉疾病和/或动脉高血压的患者有潜在益处。相比之下,对于ATTRv患者，应谨慎使用HFT。
