Treatment and Outcome of Patients With Coronary Artery Ectasia: Current Evidence and Novel Opportunities for an Old Dilemma

Abstract

Coronary artery ectasia (CAE) is defined as a diffuse or focal dilation of an epicardial coronary artery, which diameter exceeds by at least 1. 5 times the normal adjacent segment. The term ectasia refers to a diffuse dilation, involving more than 50% of the length of the vessel, while the term aneurysm defines a focal vessel dilation. CAE is a relatively uncommon angiographic finding and its prevalence ranges between 0.3 and 5% of patients undergoing coronary angiography. Although its pathophysiology is still unclear, atherosclerosis seems to be the underlying mechanism in most cases. The prognostic role of CAE is also controversial, but previous studies reported a high risk of cardiovascular events and mortality in these patients after percutaneous coronary intervention. Despite the availability of different options for the interventional management of patients with CAE, including covered stent implantation and stent-assisted coil embolization, there is no one standard approach, as therapy is tailored to the individual patient. The abnormal coronary dilation, often associated with high thrombus burden in the setting of acute coronary syndromes, makes the interventional treatment of CAE patients challenging and often complicated by distal thrombus embolization and stent malapposition. Moreover, the optimal antithrombotic therapy is debated and includes dual antiplatelet therapy, anticoagulation, or a combination of them. In this review we aimed to provide an overview of the pathophysiology, classification, clinical presentation, natural history, and management of patients with CAE, with a focus on the challenges for both clinical and interventional cardiologists in daily clinical practice.

Keywords: acute coronary syndrome; antithrombotic therapy; coronary artery disease; coronary artery ectasia; percutaneous coronary intervention.

Figure 1

Anatomical definition of CAE according to Markis classification.

Figure 2

Main etiopathogenetic mechanisms of CAE. DES, drug-eluting stent; ECM, extracellular matrix; MMP, metalloproteinases; NO, nitric oxide; PCI, percutaneous coronary intervention.

Figure 3

Mechanisms of ACS in patients with CAE. ACS in patients with CAE can occur through different pathophysiological mechanisms. Catastrophic plaque rupture resulting in acute vessel occlusion due to a huge thrombotic burden untreatable despite a timely primary PCI (A). Relevant filling defect due to endoluminal thrombus without significant underlying atherosclerotic plaque, to be ascribed to flow disturbances in an entirely ectatic RCA (B). Abrupt flow occlusion of the distal segment in a marginal branch (arrow) due to the embolization of clot fragments coming from a proximal saccular CAA of the LCx (C). Ectatic LAD showing images of advanced flow disturbances angiographical pattern, that suggest the relationship between impaired blood progression and myocardial ischemia (D). ACS, Acute Coronary Syndrome; CAA, coronary artery aneurysm; CAE, Coronary Artery Ectasia; LAD, left anterior descending; LCx, left circumflex; PCI, percutaneous coronary intervention; RCA, right coronary artery.

Figure 4

Multimodality imaging approach for diagnosis and PCI guidance in a STEMI patient with angiographic evidence of CAE. 77-year-old man admitted for STEMI, who underwent emergent coronary angiography. Coronary angiography showed an occlusion of the mid RCA due to stent thrombosis (A) and a CAA distal to the occlusion site, which was visible after guidewire crossing and thrombectomy (B). IVUS evaluation showed the previously implanted stents at the proximal (C) and distal (D) necks of the CAA. The PCI strategy consisted of 48 mm EES implantation bridging the proximal and distal CAA necks to create a supporting platform for the deployment of two overlapping covered stents. (E) and (F) show the proximal and distal edges of the EES assessed by IVUS. Coronary angiography showed an optimal sealing of the CAA after the implantation of two overlapping 3.5 × 24 mm single-layer PTFE covered stents (BeGraft, Bentley InnoMed, Hechingen, Germany) (G), which was confirmed by CCTA after the procedure (H). ^*Courtesy of Dr. Iacopo Muraca. CAA, coronary artery aneurysm; CCTA, coronary computed tomography angiography; EES, everolimus-eluting stent; IVUS, intravascular ultrasound; PTFE, polytetrafluoroethylene; RCA, right coronary artery; STEMI, ST-segment elevation myocardial infarction.

Figure 5

Clinical management of patients with CAE. APT, antiplatelet therapy; CAAs, coronary artery aneurysms; CABG, coronary artery bypass grafting; CAE, coronary artery ectasia; CV, cardiovascular; DES, drug-eluting stent; OAC, oral anticoagulation; PCI, percutaneous coronary intervention.

Figure 6

Example of exclusion of a saccular CAA in the distal RCA with high thrombus burden by means of covered stent implantation. Severe ISR of a previously implanted DES in the distal segment of the RCA continuing in a saccular CAA with high thrombus burden (A). A 3.5 × 15 mm PK Papyrus covered stent is advanced to precisely seal the inlet and outlet of the CAA (B). A DES Synergy (Boston Scientific, Marlborough, Massachusetts) 4 x 24 mm is then implanted proximally to the covered stent to treat the severe ISR (C). Final result showing the exclusion of the saccular CAA (D). CAA, coronary artery aneurysm; DES, drug-eluting stent; ISR, in-stent restenosis; RCA, right coronary artery.

Figure 7

Hybrid approach for combined treatment with stent-assisted coil embolization and covered stent implantation in a dual-chamber iatrogenic pseudoaneurysm following PCI of the proximal LAD. The upper panel shows the angiographical view of an iatrogenic pseudoaneurysm of the proximal LAD due to a previous PCI with DES implantation; the lower panel shows the IVUS images of the pseudoaneurysm and its relationship with the previously implanted DES (A). Rendered volume CCTA image showing a large dual-chamber pseudoaneurysm (B). Multiple coils are advanced through a microcatheter to wrap around the old DES until completely filling both cavities of the pseudoaneurysm (C). A BeGraft covered stent (Bentley InnoMed, Hechingen, Germany) 3 x 24 mm has been used to secure the coils correct positioning (D). Final result (E). ^*Courtesy of Dr. Fabio Felice Tarantino. CCTA, coronary computed tomography angiography; DES, drug-eluting stent; IVUS, intravascular ultrasound; LAD, left anterior descending; PCI, percutaneous coronary intervention.