ABSTRACT
BACKGROUND: Junctional epidermolysis bullosa is a rare skin and mucosal disorder characterized by blister formation in response to minor trauma and extracutaneous manifestations. There have been no reports of cardiac surgery and prognostication in patients with epidermolysis bullosa due to skin and mucosal fragility. CASE PRESENTATION: A 55-year-old man presented with congenital junctional epidermolysis bullosa, hypertension, and vasospastic angina. He complained of dyspnea on exertion, and transthoracic echocardiography revealed severe aortic valve regurgitation, moderate aortic valve stenosis (tricuspid valve), and severe mitral valve regurgitation. Considering that the skin condition in the right chest wall was relatively healthy, the right thoracotomy approach was preferred and totally endoscopic concomitant mitral valve repair and aortic valve replacement were performed using a sutureless bioprosthetic valve (Perceval™ (Corcym, Group, Milan, Italy)). Polyurethane and silicon dressing foams were used to protect the skin at the site of contact with the bag valve mask, arterial pressure catheter, intravenous catheter, and the tracheal intubation tube. Vertical mattress sutures were used for the skin sutures. The postoperative course was uneventful, and the patient was discharged nine days after the operation. There was no indication for reoperation until three years follow-up period. CONCLUSIONS: The totally endoscopic concomitant aortic and mitral valve surgery using Perceval™ prosthesis can be performed safely in patients with junctional epidermolysis bullosa by adequate protection of the skin and mucosa.
Subject(s)
Cardiac Surgical Procedures , Epidermolysis Bullosa, Junctional , Mitral Valve Insufficiency , Male , Humans , Middle Aged , Epidermolysis Bullosa, Junctional/complications , Mitral Valve/surgery , Mitral Valve Insufficiency/surgery , Blister , Aortic Valve/surgeryABSTRACT
Cardiac perforation by a transvenous pacemaker lead is an uncommon, but serious complication. Management strategies in pacemaker lead cardiac perforation depend on the symptoms, the presence of pericardial effusion, hemodynamic status, and injured neighboring organs. A 70-year-old man was admitted due to suspicious right atrial perforation with pneumothorax secondary to a transvenous pacemaker lead. Right atrial perforation was confirmed on computed tomography (CT). A large laryngopharyngeal hemangioma compressing the trachea was also observed. Although he was hemodynamically stable, we chose a surgical removal of a transvenous pacemaker lead considering his large laryngopharyngeal hemangioma. A tracheotomy followed by lower hemisternotomy were performed. A perforated pacemaker lead was observed on the right atrium. The lead was pulled out, and a hole in the right atrium was fixed. His postoperative course was uneventful.
