Cappannoli Luigi, Ciliberti Giuseppe, Restivo Attilio, Palumbo Pierpaolo, D'Alò Francesco, Sanna Tommaso, Crea Filippo, D'Amario Domenico
Department of Cardiovascular Sciences, Catholic University of the Sacred Heart (UCSC), Rome, Italy.
Department of Diagnostic Imaging, area of Cardiovascular and Interventional Imaging, Abruzzo Health Unit 1, L'Aquila, Italy.
Eur Heart J Case Rep. 2022 May 31;6(7):ytac225. doi: 10.1093/ehjcr/ytac225. eCollection 2022 Jul.
Cardiac amyloidosis (CA) is a rapidly progressive infiltrative cardiomyopathy, whose role is emerging as a not-so-rare disorder leading to heart failure (HF). Myocardial bridge (MB) is the most common inborn coronary artery variant, and its clinical relevance is still matter of debate. The exceptional coexistence of these two conditions could accelerate disease progression and worsen the already compromised clinical conditions.
We present the case of a 76-year-old female patient experiencing relapsing HF decompensation and presenting to our centre with dyspnoea at rest and severe peripheral congestion. Echocardiogram showed severe concentric hypertrophy, severe biventricular contractile dysfunction, and third-degree diastolic dysfunction. Coronary angiography excluded epicardial atherosclerotic disease, though displaying a long intramyocardial course of left anterior descending artery. Physiological invasive test was achieved in terms of instantaneous wave-free ratio (iFR), both at baseline and after inotropic and chronotropic stimuli, and attested haemodynamic significance. Concurrently, the diagnostic flow chart for CA was accomplished, by means of both invasive (periumbilical fat biopsy, bone marrow aspiration) and non-invasive tests (Tc-diphosphonate scintigraphy, serum-urine immunofixation) that confirmed the suspect of primary amyloidosis. Acute HF therapy was personalized according to the singularity of the case, avoiding both nitrates and beta-blockers, then first cycle of chemotherapy was started.
Our clinical case shows a unique interaction between infiltrative cardiomyopathy and coronary artery abnormality. Amyloidosis can contribute to the ischaemic burden of the MB and this may, in turn, abbreviate the path to HF decompensation.
心脏淀粉样变性（CA）是一种快速进展的浸润性心肌病，其作为导致心力衰竭（HF）的一种并非罕见的疾病，作用正日益显现。心肌桥（MB）是最常见的先天性冠状动脉变异，其临床相关性仍存在争议。这两种情况的罕见共存可能会加速疾病进展，并使本已受损的临床状况恶化。
我们报告了一例76岁女性患者，经历复发性HF失代偿，因静息时呼吸困难和严重外周充血前来我院就诊。超声心动图显示严重的向心性肥厚、严重的双心室收缩功能障碍和三度舒张功能障碍。冠状动脉造影排除了心外膜动脉粥样硬化疾病，但显示左前降支有一段长的心肌内走行。通过瞬时无波比率（iFR）在基线以及强心和变时刺激后进行了生理侵入性测试，并证实了血流动力学意义。同时，通过侵入性（脐周脂肪活检、骨髓穿刺）和非侵入性测试（锝二膦酸盐闪烁扫描、血清-尿液免疫固定）完成了CA的诊断流程，证实了原发性淀粉样变性的怀疑。根据病例的特殊性对急性HF治疗进行了个体化，避免使用硝酸盐和β受体阻滞剂，然后开始了第一个化疗周期。
我们的临床病例显示了浸润性心肌病与冠状动脉异常之间独特的相互作用。淀粉样变性可加重MB的缺血负担，进而可能缩短HF失代偿的病程。
