Acute Lower Limb Ischaemia as a Presenting Sign of Atrial Myxoma: Case Report and Scoping Review of the Literature

Abstract

Objective: Cardiac myxomas (CMs) are the most common primary cardiac tumour in adults. They are a rare cause of peripheral embolisation and may present as acute lower limb ischaemia (ALI). A scoping review was undertaken and a case of ALI due to CM embolisation is presented in this paper.

Methods: MEDLINE, Scopus, and Embase were systematically searched for studies reporting data on ALI as a presentation of CM embolisation. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses Extension for Scoping Reviews (PRISMA-ScR) was followed.

Results: A healthy 26 year old female presented to the emergency department with bilateral ALI. Urgent bilateral aorto-iliac embolectomy and distal embolectomy of the left femoropopliteal axis were performed. The retrieved embolic material exhibited a yellowish appearance and jelly like consistency, and histological analysis provided a diagnosis of a myxomatous embolus. Transoesophageal echocardiography confirmed the left atrial origin of a myxomatous tumour, but the residual mass was considered too small for further excision. At a two year clinical follow up, the patient was alive and well without recurrence. Between 1989 and 2023, 59 patients with ALI due to CM embolisation were identified in the literature. An in hospital mortality rate of 12.1% (n = 7) was reported, while the in hospital complication and re-intervention rates were 34.5% (n = 20) and 27.6% (n = 16), respectively. No post-discharge deaths, complications, or re-interventions were reported; fasciotomies were the most reported (n = 10). Post-discharge follow up was reported in 22 (37.3%) patients. Mean follow up was 18.0 ± 18.8 months (range 1-120), and 86.4% of patients (n = 19) were alive and well at last follow up.

Conclusion: This review and the associated case report underline that CM embolisation should be considered in healthy young patients presenting with cryptogenic ALI. Early transoesophageal echocardiography and histological analysis of the retrieved embolus are recommended to minimise misdiagnosis in these populations.

Keywords: Acute limb ischaemia; Atrial myxoma; Embolectomy; Peripheral embolism.

Figure 1

Computed tomography angiography images. (A) Evidence of a saddle embolus of the aortic bifurcation (red arrow) and occlusion of the right iliac axis. (B) Visceral involvement with multiple focal, wedge shaped renal parenchymal defects involving both the cortex and medulla and extending to the capsular surface (green arrows).

Figure 2

Final angiogram displaying successful aorto-iliac recanalisation after surgical embolectomy.

Figure 3

Tumour cell immunoreactivity and identification in the histological analysis of the embolic material. (A) Diffuse immunoreactivity for calretinin and (B) cytokeratin AE1/AE3; (C) focal immunoreactivity for S100 protein; (D) ETS related gene (ERG) highlighting endothelial cells but not perivascular tumour cells (H&E 200x).