成人Ebstein病に対するCone手術および1.5心室修復術

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

Ebstein病は先天性心疾患の約0.5％とまれ1）であるが，三尖弁および右室形態の多様性から2心室または1心室修復，さらに両方向性Glenn（BDG）手術を追加する1.5心室修復術など，さまざまな術式が選択される．われわれは，成人Ebstein病に対してCone手術および1.5心室修復術を施行し，良好な結果を得たので報告する．

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.