The Starr-Edwards ball valve prosthesis was successfully introduced in 1961–62 and largely used for aortic and mitral valve replacement [4, 5]. It is one of the artificial valves with very long-term results [9]. Godje et al. reported a 30-year survival rate respectively of 19.9% in patients with aortic valve replacement and 22.6% in patients with mitral valve replacement [3]. Thromboembolism is a major factor contributing to the overall mortality and morbidity of patients who underwent valve replacement. In this setting, the necessity of long-term anticoagulation is controversial. Godje et al. found, in their patient’s population, that only 65.7% of patients with Starr-Edwards aortic valves received anticoagulation therapy, whereas patients with mitral valve replacement were treated with anticoagulant therapy in 90.7% of cases [3]. McGoon et al. found in a cohort of 336 patients that an adequate status of anticoagulation significantly reduced the risk of systemic embolism only in patients with a mitral valve prosthesis, suggesting a difference in the pathogenesis of emboli originating from mitral and aortic valve prostheses [5]. Nowadays, all surviving patients are treated with oral anticoagulation. Even if Starr-Edwards valves have been widely replaced in clinical practice by other mechanical valves, they define a standard concerning long-term durability. Abad et al. reported a case of a patient who lived almost 50 years after aortic valve replacement with a Starr-Edwards Caged-Ball Valve [10]. Yalcinkaya et al. described a still functioning Starr-Edwards mitral valve implanted 41 years before [11]. Starr et al. described that their longest-lived survivor had an original ball-valve prosthesis in the aortic position for 51.7 years, whereas the longest-functioning mitral valve lasted for 44.4 years [12]. Our patient has been living for 46 years with both Starr-Edwards mitral and aortic valves, without showing any sign of prosthetic dysfunction, embolism, infection, periprosthetic leaks or hemolysis.
The case presented is unique since our patient also shows a RCA anomalously originating from the left coronary sinus and coursing between the aortic root and the main pulmonary artery (malignant variant). Congenital anomalies of the coronary arteries are relatively uncommon, occurring in 0.2 to 1.2% of the population. These anatomic variants could be asymptomatic, but sometimes could represent an important cause of chest pain, determining hemodynamically significant abnormalities, until in worst cases sudden cardiac death. The anomalous right coronary origin may have inter-arterial (the most common one), retro-aortic, prepulmonic or septal (subpulmonic) course. The incidence of this variant is unknown. In particular, Yamanaka et al. reported an incidence of 0.1% in their population, who underwent coronary angiography [13]. Erol et al. reported a prevalence rate for RCA branching from the left coronary sinus of 0.43% in patients undergoing CCT [14]. The incidence of clinical events related to RCA abnormal origin, such as angina pectoris, myocardial infarction, or sudden cardiac death, is not known either and it is generally estimated from necropsy patients [15, 16]. Several explanations have been proposed for the association of this anatomic variant and clinical events including acute angulation at the origin, compression of the vessel between the aorta and pulmonary artery, slit like ostium and intramural proximal intussusception of the ectopic artery at the aortic root [17]. Nevertheless, the choice between surgical treatment or a conservative strategy is controversial [18,19,20]. Proposed treatment options include revascularization, translocation of the RCA to the aorta, ostioplasty and bypass grafting [21]. Since our patient did not show any cardiac symptoms a conservative strategy was adopted.
In conclusion, we reported the longest lasting durability (46 years) described in the English literature of a double mitral-aortic Starr-Edwards silastic ball valve in association with an anomalous malignant origin of the RCA, coursing between the aortic root and the main pulmonary artery.
