Shone’s syndrome is a complex left-sided cardiac anomaly which consists of supravalvular mitral membrane, PMV, subaortic stenosis, and CoA. In the literature, most reports were among the childhood population, while few cases of adult patients had been described. However, the partial Shone’s syndrome, characterized by two or three of the obstructive components had been reported more rarely in adults [4, 5]. In our case, the patient had supravalvular mitral membrane, PMV, CoA and BAV stenosis, without subaortic stenosis. BAV disease is known to coexist with other congenital vascular malformations, the most common of which is CoA. Prior autopsy examination showed the incidence of CoA patients with BAV was 46% [6, 7]. There are a number of syndromes whose cardiac involvement includes BAV and left-sided obstructive lesions: Shone’s syndrome, Williams syndrome with supravalvular stenosis, and Turner syndrome with CoA [8].

Echocardiography is an effective and non-invasive method to diagnose Shone's syndrome. Patients with Shone’s syndrome usually can be diagnosed with careful echocardiographic evaluation during childhood. And as the patient ages, it becomes symptomatic. This patient was delayed in diagnosis and treatment because she lived in undeveloped west areas of China with limited access to echocardiography.

Mitral valve obstruction due to PMV may be the most critical abnormality determining the longterm outcome. The key to successful surgery is the management of the mitral valve [9]. Most of the reasons for the reoperation are mitral valve related. In this case study, the lesion of mitral valve was not very severe. Only the supravalvular mitral membrane was resected and separated from the posterior commissure. The valve admitted a 19-mm Hegar’s dilator. No obvious reflux was observed in the flushing test. TEE demonstrated a valve orifice area of 2.0 cm2 with trivial regurgitation. The freedom of mitral valve cusp mobility deserved no further intervention on the mitral valve.

Management of aortic coarctation in Shone’s syndrome is also vital [10]. Mutiple surgical techniques have been applied for CoA repair. Surgical repair has traditionally been the mainstay of treatment for CoA correction despite advances in endovascular technology with stents allow for minimally invasive approaches in older children and adults with native CoA and complications [11]. The resection and graft interposition technique were first described by Gross in 1951 [12, 13]. A tube graft is sewn into the aorta after the cross-clamping of the aorta and resection of the coarctate segment. It is useful for patients with long-segment CoA. However, longer cross-clamp time is required and the graft cannot grow with the patient thus being not suitable for children. A surgical follow-up study showed that all the interposition grafts dilated up by 50% of their original size for more than a decade [14]. Extra-anatomical bypass technique is an alternative strategy particularly for patients with long-segment coarctation, and concomitant cardiac procedures such as coronary artery bypass grafting or aortic valve replacement [15, 16]. It is performed through median sternotomy in adults and provides additional blood flow to the distal aorta leaving the coarctate region of aorta in situ [17]. In our case of Shone’s syndrome, the procedure is more convenient and safer for surgeons to correct all the cardiac malformations in the same incision, and in a single-stage.

The descending aorta anastomosis is deep behind the heart, and the ideal method is to expose the operative field after extracorporeal circulation. In the case study, this procedure was performed after heart rebeating to shorten the clamping time and reduce the ischemic myocardial damage. However, technically, this approach is more challenging as the partial occlusion clamp on the aorta tends to slip with the resumed heart beat and also because of the risk of fatal bleeding. The procedure needs experienced surgeons to overcome these difficulties and obstacles and achieve the goal.

In conclusion, this case study highlights the importance of echocardiographic evaluation in the diagnosis of Shone’s syndrome. The surgical strategy should be tailored according to both the patient’s profile and the surgeon’s personal surgical experience with better recognition of Shone’s syndrome. Extra-anatomical bypass procedure is an appropriate technique for adult patients with long-segment coarctation and concomitant cardiac lesions. The outcomes of the case study indicate that the primary definitive surgery of Shone’s syndrome is encouraging.