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Ebstein畸形的外科治疗

目的 总结15例先天性三尖瓣下移（Ebstein）畸形的手术治疗经验，以提高手术疗效。 方法 对2002年4月至2007年8月收治的15例Ebstein畸形患者采用三尖瓣成形和房化右心室折叠术，其中8例隔瓣后瓣发育不全或缺如的患者采用自体心包矫正。 结果 全组无死亡。术后1例发生低心排血量综合征，经使用正性肌力药物(洋地黄)和利尿剂控制心力衰竭，术后第3d好转；其余患者恢复良好，心功能有明显改善。随访13例，2例失访，随访时间1～42个月，其中11例患者心功能恢复至Ⅰ级，2例心功能恢复至Ⅱ级；紫绀和心脏杂音消失；复查超声心动图提示：12例三尖瓣水平反流消失，1例仍有轻度至中度反流。 结论 对右心室病理改变的正确认识，完善的三尖瓣功能修复和房化右心室折叠是手术成功的关键；自体心包三尖瓣隔瓣后瓣再造，保持了右心室几何形态和功能，减少了并发症的发生，能提高手术成功率。

Hemodynamic study of right ventricular outflow tract reconstruction with valved bovine jugular vein conduit in canine model

Objective To investigate the hemodynamic performance of valved bovine jugular vein conduits (BJVC) for right ventricular outflow tract reconstruction in canine model. Methods The BJVC that were treated with the glutaraldehyde were implanted between the pulmonary artery and right ventricle in seven young canines. Right ventricular and pulmonary artery pressures were measured directly before and after the implantation. Hemodynamic evaluations were carried out by echocardiography and cardiac catheterization after the implantation. Results Seven canines were survival one year after the implantation. The pulmonary artery pressures (including systolic pressure, diastolic pressure and mean pressure) had not significantly changed after reconstruction with the conduits. The right ventricular diastolic pressures had not increased after the reconstruction, but the right ventricular systolic pressure and mean pressure had increased. One year later, the echocardiography showed valve motion with no obvious thickening of the leaflets. No graft kinking or obvious regurgitation of the valve was observed. Cardiac catheterization and angiography showed that the pressure gradients between the right ventricle and the conduits varied from 3 to 19mmHg, the diastolic pressures in the conduits were higher than that of right ventricle((Plt;)0.01), and the conduits and pulmonary arteries had no obvious obstruction. Conclusion The glutaraldehyde-fixed bovine jugular vein conduit has good hemodynamic performance in the pulmonary circulation.

Change of Blood Gas and Hemodynamic Status after Palliative Procedurein the Patients with Pulmonary Atresia and Ventricular Septal Defect

Objective Comparing postoperative change of blood gas and hemodynamic status in patients underwent a right ventricletopulmonary artery (RVPA) conduit or a modified BlalockTaussig (mBT) shunt for pulmonary atresia with ventricular septal defect and without major arterial pulmonary collaterals (MAPCAs), to affirm the effect on oxygen supply /demand with different procedure. Methods From July 2006 to October 2007, 38 patients with pulmonary atresia and ventricular septal defect without MAPCAs were divided into two groups according to different procedures: RVPA group (n=25) and mBT group (n=13).Perioperative mortality, blood gas and hemodynamic data during postoperative 48 hours, including heart rate, blood pressure, systemic oxygen saturation, mixed venous oxygen saturation, oxygen excess factor, inotropic score were compared in both groups. Results The difference in the mortality between RVPA group (4.0％，1/25) and mBT group （7.7％，1/13) showed no statistical significance（Pgt;0.05）. The total of 33 patients were followed up, the followup time was from 6 to 18 months.11 patients (4 patients in mBT group, 7 patients in RVPA group) underwent corrected procedures during 9 to 18 months after palliative procedures, one case died of elevated pulmonary vascular resistance and right ventricle failure. The mixed venous oxygen saturation at 24h and 48h after surgery were higher than that at 6h after surgery (Plt;0.01) both in RVPA group and mBT group. The systolic blood pressures at 6h, 24h, 48h after surgery in RVPA group were lower than those in mBT group (P=0.048，0.043， 0.045)，the mean systemic blood pressures in RVPA group were higher than those in mBT group (P=0.048, 0.046, 0.049),the diastolic blood pressures in RVPA group were higher than those in mBT group (P=0.038, 0.034, 0.040)， the inotropic scores in RVPA group were lower than those in mBT group (P=0.035， 0.032，0.047). Conclusion The blood pressures and inotropic scores are found significantly different in RVPA conduit and mBT procedures, while postoperative systemic oxygen delivery areequivalent. Both RVPA and mBT patients decline to nadir in hemodynamic status at 6 h after surgery.

Optimized Surgical Management and Indication of Doubleoutlet Right Ventricle with Atrioventricular Discordance

Objective To optimize the surgical strategies and managements of doubleoutlet right ventricle(DORV)with atrioventricular discordance, the anatomic features, the surgical managements and results were reviewed. Methods From September 1990 to August 2004, 7 cases of DORV with atrioventricular discordance received surgical therapy. The age ranged from 3 years to 7 years. Surgical managements included: ventricular septal defect (VSD )repair and homograft connected the morphologic left ventricle with pulmonary artery in 3 cases, double-switch in 2 cases, modified Fontan in 2 cases. Results The early mortality rate was 14.3% (1/7). The death was because by Homograft infection 2 months after repair. Complete atrioventricular block occurred in one case who received permanent pacemaker later. Obstruction of superior vena cava return and pericardial effusion occurred in one Fontan case who was recovered at the time of discharge. The time of ICU stay was shortest in double-switch patients in 6 days postoperation. Conclusion Double-switch operation is the first choice in correction of DORV with atrioventricular discordance, especially in patients with right ventricular disfunction or tricuspidal regurgitation. If the heart can not be corrected because of combined complicated malformations, the strategy of one ventricular repair can be chosen.

Right Ventricle-pulmonary Anastomosis for Right Ventricle Outflow Reconstruction in Patients with Pulmonary Atresia and Ventricular Septal Defect

ObjectiveTo investigate pulmonary artery growth, valvular regurgitation and right heart function after right ventricle-pulmonary artery (RV-PA) anastomosis for right ventricle outflow (RVOT) reconstruction in patients with different types of pulmonary atresia and ventricle septal defect (PA/VSD). MethodsClinical data of 31 PA/VSD patients who underwent right ventricle-pulmonary anastomosis for RVOT reconstruction from November 2002 to September 2012 in Guangdong General Hospital were retrospectively analyzed. There were 20 male and 11 female patients with their age ranging from 14 days to 14.50 years (47.90±53.84 months). Patients were followed up after discharge to evaluate the anastomasis, pulmonary artery growth, pulmonary artery and tricuspid regurgitation and their relationship with follow-up duration. Right ventricular strain and other echocardiography indexes were used to evaluate right heart function. ResultsThree patients died postoperatively. Twenty-seven patients (27/31, 87.1%)were followed up for 4-129 (35.97±28.24)months. There was no late death during follow-up. There was no anastomotic restenosis in patients who received radical surgery. Four patients who received palliative surgery had comparative anastomotic restenosis. The diameters of RV-PA anastomasis, left and right branches of the pulmonary artery during follow-up were significantly larger than early postoperative diameters (P < 0.05). Echocardiography showed that pulmonary artery and tricuspid regurgitation were aggravated, which was not correlated to follow-up duration. Overall right heart function was good during follow-up. ConclusionsClinical outcomes of RV-PA anastomosis for RVOT reconstruction in patients with PA/VSD are satisfactory. RV-PA anastomasis, left and right branches of the pulmonary artery can keep their growth and development. Mid-term and long-term right heart function is good.

镜面右位心并右室双出口病变一例

双向上腔静脉肺动脉吻合术治疗左心室发育不良的右心室双出口

目的 总结双向上腔静脉肺动脉吻合术治疗合并左心室发育不良的右心室双出口（DORV）的临床经验。方法 2000年1月至2004年12月手术治疗7例患者，均伴有肺动脉狭窄和左心室发育不良，左心室舒张期末容积指数均〈30ml／m2。5例在体外循环下完成手术，2例在非体外循环下完成手术。结果 全组无手术死亡。术后机械通气时间为9.0±7.9h，无严重术后并发症发生，活动能力改善。随访6个月～4年，效果满意。结论 对左心室发育不良的DORV患者，双向上腔静脉肺动脉吻合术可以获得满意的治疗效果。

右心室双通道术治疗右心室流出道狭窄

摘要： 目的 探讨先天性右心室流出道狭窄合并冠状动脉横跨右心室流出道表面患者行右心室双通道术的方法及临床效果。 方法 2006年4月至2008年8月，我中心连续收治6例右心室流出道狭窄合并冠状动脉横跨右心室流出道表面患者， 男4例，女2例；年龄0.8~12.0岁，平均年龄5.5岁。法洛四联症2例，右心室双出口4例。手术在保留原来右心室肺动脉出口的同时，采用自体心包片或国产牛心包片建立右心室的第2个出口。 结果 围术期无死亡患者，术后右心室与肺动脉压差为24.17±6.18 mm Hg。出院前复查心脏超声心动图提示：2个出口均有血液，未见流出道阻塞。随访6例，随访时间3个月～1年，其中4例患者心前区闻及2/6级收缩期杂音，2例闻及3/6级杂音。超声心动图提示：所建的右心室通道通畅，右心室与肺动脉间的压差（24.67±5.32 mm Hg）与术后早期比较变化不大，远期效果尚待进一步观察。 结论 右心室双通道术可在保护横跨右心室流出道表面冠状动脉的情况下疏通右心室流出道。

Percutaneous pulmonary valve replacement with self-expanding valve for a patient with cone-shaped right ventricular outflow tract: a case report

This article reports a 16-year-old patient with severe pulmonary valve regurgitation after corrective surgery for tetralogy of Fallot. The shape of the right ventricular outflow tract to the main pulmonary artery was cone-shaped, which is extremely challenging. After admission, percutaneous pulmonary valve replacement with self-expanding valve was successfully performed. The patient’s condition remained stable during the 2-year follow-up period after surgery. This case aims to provide a reference for percutaneous pulmonary valve replacement in patients with cone-shaped right ventricular outflow tract.

Nikaidoh手术的临床应用

目的 总结Nikaidoh手术治疗右心室双出口（DORV）和大动脉转位（TGA）患者的临床经验，以提高手术疗效。 方法 3例先天性心脏病患者中DORV 1例，TGA 2例（平均年龄11.6岁），均行Nikaidoh手术，术中行Lecompte操作，Gore-tex人工血管补片修补室间隔缺损并重建左心室流出道，18＃带单瓣牛心包片补片重建肺动脉及右室流出道。 结果 术后无早期死亡，平均住院时间13.6d。发生低心排血量综合征1例，肝功能损害2例，二次开胸止血1例，均经相应的处理治愈。随访3例，随访时间3～5个月，复查超声心动图未发现左、右心室流出道梗阻；其中2例发现轻度至中度主动脉瓣关闭不全，但定期随访未发现继续加重。 结论 采用Nikaidoh手术治疗DORV和TGA患者，术后可获得良好的血流动力学效果，早期临床结果满意。