目的探讨罕见肺动脉瓣缺如综合征（absent pulmonary valve syndrome，APVS）的外科治疗效果。 方法回顾性分析3例APVS患者在低温体外循环下行根治手术，其中女2例、男1例，年龄分别为9个月、14岁、10岁，其中2例合并右室双出口（double outlet right ventricle，DORV）。3例均采用自体心包片重建肺动脉瓣，1例行肺动脉成形术，2例未处理肺动脉。 结果3例均存活，术中食管超声及术后超声复查肺动脉瓣功能良好，随访8～24个月，均恢复良好、生活质量明显改善，复查心脏彩色超声心动图肺动脉瓣功能良好，无明显反流。 结论APVS是一种少见的先天性心脏病，合并DORV或心内其他结构正常的病例非常罕见，手术治疗可获得良好效果。
ObjectiveTo summarize the indication and surgical experiences of valve replacement, high-risk factors and long-term results for aortic regurgitation and large left ventricle. MethodsWe retrospectively analyzed the clinical data of 42 patients with aortic regurgitation and left ventricle end-diastolic diameter (LVEDD) ≥70 mm and left ventricle end-systolic diameter (LVESD) ≥ 50 mm in our hospital from March 2004 through December 2012. There were 38 males and 4 females,aged 16-73 (45.86±14.99) years. The patients underwent aortic valve replacement, who were evaluated by echocardiography at pre-operation, pre-discharge and early follow-up. The follow up period was 12-132 months. ResultsEarly death occurred in one patient. And five patients died during the follow-up. One week after surgery in 41 patients, LVEDD (62.00±13.21 mm), LVESD (50.71±14.02 mm), indexed LVEDD (35.23±8.58 mm/m2), indexed LVESD (28.92±9.08 mm/m2), LVEF (46.41%±12.49%), were significantly smaller than those before the operation (P<0.01). Heart function grades, preoperative EF, LVEDD and indexed LVEDD were the predictors for left ventricular function recovery. One-year, 5-year, 10-year survival rate was 92.9%, 90.2%, 83.8%, respectively. ConclusionMost of patients with aortic regurgitation and large left ventricle still have indications for surgical treatment, but severe left ventricular dysfunction and ventricular arrhythmia are high risk factors for long-term survival.
Objective To summarize the experience with median sternotomy ascending-descending thoracic aortic bypass grafting via median sternotomy for the treatment of complex coarctation of aorta (COA), and to present the intermediate to long-term follow-up outcomes. Methods A retrospective analysis was performed on patients with complex COA who underwent ascending-descending thoracic aortic bypass grafting through a median sternotomy in the First Hospital of Tsinghua University from August 2004 to May 2017. ResultsA total of 7 children were enrolled, including 4 males and 3 females, with an average age of (13.3±4.6) years and an average weight of (40.2±12.2) kg. Six (85.7%) patients had hypertension in the upper limbs. Among them four patients had coarctation associated with intracardiac anomalies, two with recurrent coarctation post-surgery, and one with both recurrent coarctation and intracardiac anomalies. All surgeries were performed under cardiopulmonary bypass, with no operative mortality or severe complications. The systolic pressure gradient between the upper and lower extremities decreased significantly from preoperative (51.4±13.5) mm Hg to postoperative (2.9±2.7) mm Hg (P<0.01). During a follow-up period of (14.9±5.9) years (ranging from 7 to 19 years), there were no late deaths or graft-related complications. Except for one patient who continued to have mild hypertension, the blood pressure of all other patients returned to normal. Conclusion The ascending-descending thoracic aortic bypass grafting via median sternotomy for complex COA is a safe and reliable procedure that effectively reduces upper limb blood pressure and the pressure difference between the upper and lower extremities. It has a low rate of complications and satisfactory intermediate to long-term outcomes.