A 4-year-old boy with left intralobar pulmonary sequestration associated with left main coronary artery obstruction (LMCAO) and severe mitral regurgitation (MR) was admitted to our hospital. Since the patient presented with dyskinesia of the cardiac apex and increased left ventricular end-diastolic volume (LVEDV), left main coronary artery reconstruction and mitral annuloplasty were performed. The enlargement of the left ventricle was improved after sequential surgeries. There was a risk of deterioration of MR and regrowth of LVEDV due to shunt blood flow; therefore, left lower lobectomy and aberrant artery division were performed. This is a very rare case of a patient with pulmonary sequestration associated with LMCAO and severe MR.

一名 4 岁男孩患有左主干冠状动脉阻塞 (LMCAO) 和严重二尖瓣关闭不全 (MR) 相关的左叶内肺隔离症。由于患者出现心尖部运动障碍和左心室舒张末期容积（LVEDV）增加，进行了左主干冠状动脉重建和二尖瓣瓣环成形术。序贯手术后左心室扩大得到改善。由于分流血流，存在MR恶化和LVEDV再生的风险；因此，进行了左下肺叶切除术和异常动脉分割术。这是与 LMCAO 和严重 MR 相关的肺隔离症患者的罕见病例。