QAV was a rare congenital heart valve dysplasia with an incidence of approximately 0.0003%-0.017%, slightly more in males than females[1][2], according to Hurwitz and Roberts, quadricuspid aortic valves can be divided into seven types, of which type B (three equal large leaflets and one small valve) are the most common, and progressive aortic valve closure insufficiency is the main hemodynamic alteration and the reason for the need for surgical treatment at a later stage; simple aortic stenosis or dilatation of the ascending aorta is relatively uncommon[3][4]. Patients with QAV have unequal size and position of the four aortic valve cusps, leading to an uneven distribution of hemodynamic stresses and incomplete or misaligned diastolic leaflets. This is thought to be an important reason for progressive aortic valve insufficiency. When the disease progresses and the leaflets are severely degenerated, surgical intervention is required, with aortic valve replacement being the most common approach[5]. Some studies report that aortic valvuloplasty is feasible, including the removal of malfunctioning valve leaflets and the "trileaflet valve" or "bicuspid valve" aortic valvuloplasty[6]. Idrees[7] described the surgical experience of 31 QAV patients, with a median follow-up time of 38 months and showing a favorable prognosis, and the other literature reports that QAV patients underwent aortic valve replacement surgery, with good cardiac function recovery and no late death[8][9]. For elderly and high-risk patients with QAV malformations, traditional surgical aortic valve replacement may not be suitable. TAVI is considered an optional and effective surgical method[10][11][12].
QAV usually combined with other congenital defects, the most common being coronary artery opening malformation and dislocation, and abnormal coronary artery opening position is a high-risk factor for coronary artery obstruction during TAVI[13]. In patients with aortic valve regurgitation, the degree of valve calcification is relatively mild. The lack of leaflet calcification markers and insufficient annulus anchoring during TAVI surgery can significantly increase the risk of interventional valve displacement, perivalve leakage, and coronary artery occlusion.
The patient had a rare QAV malformation with a low position of the left and right coronary openings (both below 10 mm). The complex anatomical structure, uneven size, and shape of the aortic valve may result in inconsistent distribution of force on each valve, resulting in easy misalignment and dislocation after TAVI. It is difficult to fully visualize the four valve cusps in the two-dimensional view under DSA, which requires high requirements for optimal positioning of the valve implantation. This increases the risk of coronary ostium obstruction, for which the surgeon chose the transapical approach for J-Valve valve implantation. Compared with the transfemoral approach, the transapical approach is the only compliant TAVI route with a shorter distance to the aortic valve, providing a stable platform for TAVI and facilitating the valve crossing, determination of optimal position, and stability after valve implantation[14].
The J-Valve is an interventional valve for the treatment of aortic regurgitation. It consists of a retaining ring and a support frame with three anatomically positioned U-shaped "grippers", which are uniquely designed to allow for precise anatomical positioning and secure anchorage during TAVI, as well as a movable interconnection between the retaining ring and the support frame. This unique design allows precise anatomical positioning and secure anchoring during TAVI, and the ring is interconnected with the support frame, the position of the valve can be adjusted up and down even after the ring is positioned in the aortic sinus, thus effectively preventing the leaflets obstructing the coronary ostium and lowering the risk of coronary artery obstruction and myocardial infarction.
There is a high incidence of comorbid CAD in patients undergoing TAVI, TAVI combined with PCI has a higher survival rate than PCI without in these patients[15]. Depending on the time of PCI, there are three different strategies: pre-TAVI, intra-TAVI, and post-TAVI. Each of the three modalitie has its own advantages and disadvantages, and the optimal modality is still unclear. TAVI combined with PCI one-stop treatment has the following advantages: 1. No additional vascular access is required; 2. Reduces the risk of vascular complications; 3. Reducing patient's pain; 4. Rational use of medical resources. However, its radiation time and the contrast dosage are higher in one-stop treatment compared with staged strategy, which may increased risk of postoperative acute kidney insufficiency, increase the complexity of surgical operation as well as the radiation time of the surgeon[16]. In this case, the patient was treated with one-stop treatment, the time was not significantly different from the TAVI procedure alone, no postoperative acute kidney insufficiency, and the patient recovered and was discharged from the hospital on the fourth day after the procedure.
Since Cribier[17] completed the world's first TAVI in 2002, TAVI has become an effective therapeutic option for patients with high risk of aortic valve, and the indications for the procedure are expanding as the technology continues to evolve[18], however, due to the rarity of QAV, the application of TAVI in the treatment of QAV rarely reported, and lack of experience. In this study, we report this case of a patient with QAV in combination with CAD, underwent one-stop treatment of TAVI and PCI, the results were satisfactory with a 12-months postoperative follow-up.