# Totally endoscopic concomitant aortic and mitral valve surgery in junctional epidermolysis bullosa: a case report

## TL;DR

This case report describes a successful heart surgery in a patient with a rare skin condition called junctional epidermolysis bullosa.

## Contribution

The paper presents the first reported case of totally endoscopic aortic and mitral valve surgery in a patient with junctional epidermolysis bullosa.

## Key findings

- Totally endoscopic concomitant aortic and mitral valve surgery was successfully performed in a patient with junctional epidermolysis bullosa.
- Adequate skin and mucosal protection allowed for a safe surgical outcome with no reoperation needed after three years.

## Abstract

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.

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.

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.