Successful Surgical Repair for Adult Ebstein Disease Using Cone Reconstruction Combined with One and a Half Ventricle Repair

• Published in: Kyobu geka. The Japanese journal of thoracic surgery Vol. 75; no. 2; p. 92
• Main Authors: Mizumoto, Masahiro, Uchida, Tetsuro, Kuroda, Yoshinori, Oba, Eiichi, Yamashita, Atsushi, Ishizawa, Ai, Nakai, Shingo, Kobayashi, Kimihiro, Ochiai, Tomonori, Matsuo, Soumei, Arai, Shusuke
• Format: Journal Article
• Language: Japanese
• Published: Japan 01-02-2022
• Subjects: Adult; Aged; Cardiac Surgical Procedures - methods; Ebstein Anomaly - complications; Ebstein Anomaly - diagnostic imaging; Ebstein Anomaly - surgery; Female; Heart Ventricles - diagnostic imaging; Heart Ventricles - surgery; Humans; Treatment Outcome; Tricuspid Valve - diagnostic imaging; Tricuspid Valve - pathology; Tricuspid Valve - surgery
• ISSN: 0021-5252

A 67-year-old woman presented with dyspnea on effort and cyanosis due to massive tricuspid regurgitation and an atrial septal defect with right to left shunt. She was diagnosed with Ebstein disease at the age of 53 when she underwent surgery for varicose veins. Echocardiography showed the severe apical displacement of the septal and posterior leaflet. The anterior leaflet also partially displaced to the apex and demonstrated tethering caused by a dilated right ventricle. Cardiac magnetic resonance imaging showed a dilated right atrium and an enlarged atrialized right ventricle, in addition to marked low cardiac output in the dilated right ventricle. The surgical findings corresponded to Carpentier classification type C. Cone reconstruction was performed. Bidirectional Glenn anastomosis was reguired because of low cardiac output in the remaining functional right ventricle after Cone reconstruction. The patient's postoperative course was uneventful, and tricuspid regurgitation and stenosis remained mild. The patients had no occurrence of right heart failure or arrhythmia for two years after surgery.