We describe a case of an adult patient with mitral valve regurgitation and the anomalous origin and course of the left circumflex coronary artery. Use of a ringless procedure or a microinvasive approach, such as transapical neochordae implantation, would have possibly avoided a life-threatening post-operative complication. ( Level of Difficulty: Advanced. )

We present the unusual case of an AMI STEMI, complicated by ventricular fibrillation, in a 26 years old caucasic male patient, affected by necrotizing and granulomatous aortitis, in the context of a primary immunodeficiency. Initially the patient was referred to the Cardiology Unit for worsening dyspnea and fatigue. In his medical history he has primary immunodeficiency complicated by multiple opportunistic infections. We performed an echocardiography that revealed an aneurismatic ascending aorta (58 mm) with associated significant aortic valvular regurgitation. He was later referred to heart surgery OR where Bentall-De Bono operation was performed. Two years later he developed a lateral STEMI, complicated by ventricular fibrillation. The coronary angiography, performed in foreign country, showed total occlusion of the circumflex coronary artery with diffuse aneurismatic coronary disease, granulomatosis and ulcerative lesions. A percutaneous revascularization approach was tried, but ineffective. Actually the patient, during follow up, presents dilatative post-ischemic cardiopathy with moderate ejection fraction reduction, initial aortic bioprosthesis failure. We noted at follow up echocardiograms early dilatation of the native aortic arch (35 mm), suggesting a progressive disease. In conclusion, AMI's etiology includes many non atherosclerotic mechanisms that should be considered alongside common causes. This case shows a rare cause of STEMI in a very young male patient, and teaches us that aortitis is a progressive and complex pathology, often involving multiple arteriosus districts, that needs close follow-up and integrated management.

No abstract

In this case report, we describe a giant coronary aneurysm (CAAs) of the right coronary artery occasionally found in a patient that underwent Bentall procedure 18 years earlier for acute aortic dissection. A 77–year–old man, former smoker, with type II diabetes, hypertension, dyslipidaemia, and obesity, was referred to our echo lab for echocardiography in December 2020. His medical history included anteroseptal non–Q–wave myocardial infarction (1983), type A acute aortic dissection treated with Bentall procedure and implantation of mechanical aortic valve (2002), and reparation of abdominal aortic aneurysm (2003). During the echocardiogram a paracardiac mass was incidentally found, in close contact to the right atrium and with a diameter of 60 mm in A4Ch–view, with the appearance of a cystic formation (Figure A). After a few days, a CT angiography was performed, with evidence of 61.6 mm–sized giant aneurysm located in the proximal right coronary artery, characterised by calcified walls, eccentric thrombosis, and compression of the right atrium (Figure B). The patient was subsequently evaluated by the Cardiac Surgery Division. Conservatively management with regular follow–up was performed, because of the high surgical risk of redo and the lack of symptoms. In November 2022 we performed a follow–up echocardiography with evidence of stability of the size of coronary artery aneurysm (Figure C). CAAs are defined as giant when the diameter of the vessel is greater than 20 mm. These pathological entities are extremely rare, with an incidence rate of 0.02%, and aetiologies are various. The most frequent aetiologies comprehend atherosclerosis (about 50% of cases), congenital (17%), arteritis (e.g., Kawasaki disease), connective tissue disease. Our case is characterised by the occasional diagnosis of CAA 18 years after Bentall Surgery, in absence of inflammatory vessels disease. To the best of our knowledge, It’s the largest giant CAA described in literature after Bentall cardiac surgery. Therefore, cardiologists should consider these rare entities when approaching an incidental echocardiographic finding of paracardiac mass, especially in patients with high burden of atherosclerotic disease. This case report underlines furthermore the importance of an integrated approach in the differential diagnosis of a paracardiac mass.

We present the unusual case of an AMI STEMI, complicated by ventricular fibrillation, in a 26 years old caucasic male patient, affected by necrotizing and granulomatous aortitis, in the context of a primary immunodeficiency. Initially the patient was referred to the Cardiology Unit for worsening dyspnea and fatigue. In his medical history he has primary immunodeficiency complicated by multiple opportunistic infections. We performed an echocardiography that revealed an aneurismatic ascending aorta (58 mm) with associated significant aortic valvular regurgitation. He was later referred to heart surgery OR where Bentall–De Bono operation was performed. Two years later he developed a lateral STEMI, complicated by ventricular fibrillation. The coronary angiography, performed in foreign country, showed total occlusion of the circumflex coronary artery with diffuse aneurismatic coronary disease, granulomatosis and ulcerative lesions. A percutaneous revascularization approach was tried, but ineffective. Actually the patient, during follow up, presents dilatative post–ischemic cardiopathy with moderate ejection fraction reduction, initial aortic bioprosthesis failure. We noted at follow up echocardiograms early dilatation of the native aortic arch (35 mm), suggesting a progressive disease. In conclusion, AMI‘s etiology includes many non atherosclerotic mechanisms that should be considered alongside common causes. This case shows a rare cause of STEMI in a very young male patient, and teaches us that aortitis is a progressive and complex pathology, often involving multiple arteriosus districts, that needs close follow–up and integrated management.