EB is a group of hereditary skin diseases characterized by blistering of the skin, induced by mild trauma. Blistering results in open wounds that predispose to both scarring and infection [1, 3]. The four main types of EB are as follows: epidermolysis bullosa simplex, dystrophic epidermolysis bullosa, JEB, and Kindler syndrome [1]. JEB is the major form of EB, which is a generalized severe form of the condition, with a poor prognosis [1, 2]. Affected individuals experience blistering over large regions of the body from birth or early infancy. Blistering also affects mucous membranes, such as the moist lining of the mouth and digestive tract, which can cause difficulty in eating and digesting food.
The milder form of junctional epidermolysis bullosa is called the JEB generalized intermediate and is typically associated with a better prognosis [2]. Junctional epidermolysis bullosa most commonly results from mutations in the LAMA3, LAMB3, LAMC2, and COL17A1 genes. Mutations in each of these genes can cause JEB-generalized severe or JEB-generalized intermediates [2]. There is currently no specific treatment for EB. There are two reports in the literature on breast cancer treatment in patients with EB [4, 5]; however, there are no reports of cardiac surgery.
Because of skin and mucosal fragility, careful attention should be paid not only to the surgical technique, but also to anesthesia induction [5]. To protect the skin and mucosa, polyurethane and silicon dressing foams should be used at the contact sites, and a large amount of xylocaine jelly is better suited for use in tracheal intubation tubes and TEE probes. Surgical instruments that could potentially tear the skin, such as forceps, should be substituted for skin hooks or use of hands to perform suturing with vertical mattress sutures, and draping should be carefully removed using a remover. The surgical wounds had good healing using our strategy.
Both the aortic and mitral valves were treated. Median sternotomy for aortic and mitral valve surgery is performed classically, but the present case had skin fragility and a large blister in the median chest wall, which was at high risk for mediastinitis such as atopic dermatitis. Endoscopic surgical mitral valve repair is a difficult but established technique [6, 7]. Hence, the main skin incision was placed in the fourth intercostal space outside the nipple, the second skin incision was in the second intercostal space, and the camera port was in the fourth intercostal space and mid-axillary line, considering the skin condition and the running of the aorta. We did not choose the anterolateral large incision in the third or fourth intercostal space because of several erosions [Fig. 4] and decided to use the Perceval™ prosthesis for reliable insertion and implantation. After mitral valve repair, the camera position was changed from the port to the main incision site. These strategies improved the camera view of the aortic valve, and the sizer and prosthesis inserted from the second incision maintained the coaxial axis properly. Additionally, incisions in the skin that are in a relatively good condition contributed to healing without infection.
The American College of Cardiology/American Heart Association guidelines state that the choice of mechanical versus bioprosthetic valve replacement for patients aged between 50 and 65 years should be made in a shared decision-making process that must account for the trade-offs between durability (and the need for reintervention), bleeding, and thromboembolism [8]. The 2021 European Society of Cardiology and European Association for Cardio-Thoracic Surgery Guidelines state that a mechanical prosthesis should be considered in patients aged < 60 years for prostheses in the aortic position [9]. The patient was 55 years old; however, given the fragility of the skin and mucosa, the risk of bleeding due to lifelong anticoagulation therapy is considered higher than in other patients, so the use of a bioprosthetic valve was preferred after thorough informed consent from the patient.
The Perceval sutureless bioprosthesis is constructed from bovine pericardium fixed in a metal cage composed of an alloy of nickel and titanium. Szecel et al. reported that after more than 11 years of continued clinical use of the Perceval sutureless valve, they observed low mortality and stroke rates, and none for structural valve degeneration with good hemodynamic behavior of the valve [10]. A meta-analysis revealed no significant difference in early mortality, perioperative complications, and paravalvular leakage between the Perceval and conventional prosthesis groups. Moreover, a shorter CPB time, aortic cross-clamping time, and higher pacemaker implantation rate were observed in the Perceval group [11]. Some reports revealed that the authors did not experience more failures or PVL when using Perceval in multiple valve procedures, which corroborates recent findings [10, 12]. We decided to use the Perceval prosthesis because the insertion and implantation of the sutureless valve were easier than those of conventional prostheses in total endoscopic surgery. The sutureless technique is advantageous for preventing PVL in totally endoscopic minimally invasive cardiac surgery. In this patient, the postoperative mean pressure gradient of the Perceval prosthesis was higher than the previous reports [10]. There was no reoperation up to three years post-operation; however, close follow-up based on TTE is mandatory to detect prosthesis failure.
In conclusion, totally endoscopic minimally invasive concomitant aortic and mitral valve surgery using Perceval prosthesis can be performed safely for patients with junctional epidermolysis bullosa with adequate protection of the skin and mucosa.