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Nikaidoh手術的臨床應用

目的 總結Nikaidoh手術治療右心室雙出口（DORV）和大動脈轉位（TGA）患者的臨床經驗，以提高手術療效。 方法 3例先天性心臟病患者中DORV 1例，TGA 2例（平均年齡11.6歲），均行Nikaidoh手術，術中行Lecompte操作，Gore-tex人工血管補片修補室間隔缺損并重建左心室流出道，18＃帶單瓣牛心包片補片重建肺動脈及右室流出道。 結果 術后無早期死亡，平均住院時間13.6d。發生低心排血量綜合征1例，肝功能損害2例，二次開胸止血1例，均經相應的處理治愈。隨訪3例，隨訪時間3～5個月，復查超聲心動圖未發現左、右心室流出道梗阻；其中2例發現輕度至中度主動脈瓣關閉不全，但定期隨訪未發現繼續加重。 結論 采用Nikaidoh手術治療DORV和TGA患者，術后可獲得良好的血流動力學效果，早期臨床結果滿意。

Application of 3D printing technology in the personalized surgery of right ventricular double outlet

ObjectiveTo evaluate the clinical value of three-dimensional (3D) printing model in accurate and minimally invasive treatment of double outlet right ventricle (DORV).MethodsFrom August 2018 to August 2019, 35 patients (22 males and 13 females) with DORV aged from 5 months to 17 years were included in the study. Their mean weight was 21.35±8.48 kg. Ten patients who received operations guided by 3D printing model were allocated to a 3D printing model group, and the other 25 patients who received operations without guidance by 3D printing model were allocated to a non-3D printing model group. Preoperative transthoracic echocardiography and CT angiography were performed to observe the location and diameter of ventricular septal defect (VSD), and to confirm the relationship between VSD and double arteries.ResultsThe McGoon index of patients in the 3D printing model group was 1.91±0.70. There was no statistical difference in the size of VSD (13.20±4.57 mm vs. 13.40±5.04 mm, t=?0.612, P=0.555), diameter of the ascending aorta (17.10±2.92 mm vs. 16.90±3.51 mm, t=0.514, P=0.619) or diameter of pulmonary trunk (12.50±5.23 mm vs. 12.90±4.63 mm, t=?1.246, P=0.244) between CT and 3D printing model measurements. The Pearson correlation coefficients were 0.982, 0.943 and 0.975, respectively. The operation time, endotracheal intubation time, ICU stay time and hospital stay time in the 3D printing model group were all shorter than those in the non-3D printing model group (P<0.05).ConclusionThe relationship between VSD and aorta and pulmonary artery can be observed from a 3D perspective by 3D printing technology, which can guide the preoperative surgical plans, assist physicians to make reasonable and effective decisions, shorten intraoperative exploration time and operation time, and decrease the surgery-related risks.

Ebstein畸形的外科治療

目的 總結15例先天性三尖瓣下移（Ebstein）畸形的手術治療經驗，以提高手術療效。 方法 對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疊術，其中8例隔瓣后瓣發育不全或缺如的患者采用自體心包矯正。 結果 全組無死亡。術后1例發生低心排血量綜合征，經使用正性肌力藥物(洋地黃)和利尿劑控制心力衰竭，術后第3d好轉；其余患者恢復良好，心功能有明顯改善。隨訪13例，2例失訪，隨訪時間1～42個月，其中11例患者心功能恢復至Ⅰ級，2例心功能恢復至Ⅱ級；紫紺和心臟雜音消失；復查超聲心動圖提示：12例三尖瓣水平反流消失，1例仍有輕度至中度反流。 結論 對右心室病理改變的正確認識，完善的三尖瓣功能修復和房化右心室折疊是手術成功的關鍵；自體心包三尖瓣隔瓣后瓣再造，保持了右心室幾何形態和功能，減少了并發癥的發生，能提高手術成功率。

左心轉流時右心室收縮期末壓、容積關系的變化

目的 在左心轉流時應用右心室收縮期末壓、容積關系(ESPVR,以Emax表示)作為評估右心室心肌固有收縮力的指標,從而排除負荷的影響.方法 采用鉗夾肺動脈以產生等容收縮,同步持續記錄肺動脈流量,此流量的積分與相應的右心室射血壓相互構成右心室壓力容積環,此環的左上角即為收縮期末點,自等容收縮壓的峰值起至收縮期末點或與之相切作一直線,其斜率為Emax.6條正常心臟及5條右心室前壁缺血綿羊以心排血量的50%、75%和90%分別作左心轉流各15分鐘,計算其Emax.結果 隨左心轉流量的遞增,全部右心室Emax均有低落,統計學分析差異無顯著性(Pgt;0.05).結論 高左心轉流量在一定程度上可使右心室心肌固有收縮力低落,對其總功能的評估尚須視其舒張順應性與肺動脈阻力而定.

Evaluation of Right Ventricular Function by the Tei Index in Patients with Atrial Septal Defect without Complication of Pulmonary Hypertension

目的　探討組織多普勒（TDI）Tei指數評價房間隔缺損（ASD）無并發肺動脈高壓患者右室功能的改變。 方法　選取2012年3月－10月無并發肺動脈高壓的繼發孔型房間隔缺損患者31例（ASD組）及健康體檢者30例（對照組），應用TDI成像獲取三尖瓣環運動頻譜，測定頻譜圖上Aa 峰至S峰的時間（等容收縮時間，ICT），S峰的持續時間（射血時間，ET）和S峰至Ea峰的時間（等容舒張時間，IRT），計算右室Tei指數。房缺組Tei指數及相關參數與缺損大小和患者年齡作相關性研究。 結果　ASD損無并發肺動脈高壓患者右室等容收縮時間（ICT）低于正常對照[（45 ± 7）、（59 ± 8）ms，P＜0.001]，右室等容舒張時間（IRT）高于正常對照[（85 ± 5）、（78 ± 14）ms，P＜0.01]，房缺組Tei指數低于正常對照（0.48 ± 0.03、0.52 ± 0.05，P＜0.001）。房缺組的缺損大小與Tei指數、ICT呈負相關（r=?0.61，P＜0.01；r=?0.44，P＜0.05），而患者年齡與Tei指數、ICT及IRT呈正相關（r=0.69，P＜0.001；r=0.75，P＜0.001；r=0.63，P＜0.001）。 結論　TDI Tei指數是無創、有效和快捷評價右室功能的方法；ASD無并發肺動脈高壓患者的ICT縮短，Tei指數減低，右室收縮和整體功能增強，而IRT延長，舒張功能受損；缺損越大，右室收縮和整體功能越強；年齡越大，右室整體功能相對減低。

復雜先天性心臟病右心室流出道重建困難的處理

目的　探討復雜先天性心臟病在糾治過程中右心室流出道（RVOT）重建困難的處理方法和其適應證、手術方法、應用中的注意要點。 方法　回顧性分析2008年1月至2010年12月廣州市婦女兒童醫療中心26例復雜先天性心臟病患者行手術治療的臨床資料。其中男14例，女12例；年齡（2.9±2.0）歲；體重（12.2±6.0） kg。所有患者在糾治過程中RVOT重建困難，分別采用以下方法進行處理： （1）肺動脈從根部切斷與主動脈交叉后吻合于右心室切口； （2）肺動脈從根部切斷直接下拉吻合于右心室切口； （3） 肺動脈切斷后遠心端與右心室切口間的后壁用左心耳或右心耳做后壁，人工補片做前壁，重建RVOT； （4）肺動脈與右心室切口間用自體心包做成的心包卷管道連接。　結果　無手術死亡，無Ⅲ°房室傳導阻滯。10例患者因尿少行腹膜透析治療，心臟壓塞和/或胸腔出血開胸止血4例。所有患者隨訪0.5～3.0年復查：應用肺動脈從根部切斷與主動脈交叉后吻合于右心室切口和肺動脈從根部切斷直接下拉吻合于右心室切口兩種方法的7例患者中肺動脈血流速度加快3例，2.5～3.0 m/s；殘留中度以上肺動脈高壓5例；中至重度肺動脈反流6例，其中肺動脈血流速度增快2例，殘留中度肺動脈高壓4例。　結論　以上4種RVOT重建方法在復雜先天性心臟病RVOT重建中應用得當有良好的手術效果；前2種方法有肺動脈血流速度增快的可能性；肺動脈瓣反流與術后肺動脈高壓或肺動脈梗阻有關。

Right Ventricular Outflow Tract Reconstruction with Valved Bovine Jugular Vein Patch in Patients with Complex　Congenital Heart Disease

Objective　To evaluate early results of valved bovine jugular vein patch for reconstruction of the right ventricular outflow tract （RVOT）.?Methods　From May 2009 to March 2010， a total of 60 patients with complex　congenital heart diseases underwent reconstruction of RVOT with valved bovine jugular vein patch in Wuhan Asia Heart Hospital. There were 42 males and 18 females with their mean age of 6.2±8.9 years （ranging from 5 months to 33 years） and mean body weight of 27.5±24.0 kg， and 34 patients were less than 1 year. Preoperative clinical diagnosis included tetralogy of Fallot （n=38） and double outlet of right ventricle with pulmonary stenosis （n=22）. All the patients underwent one-stage surgical repair. Before operation， 4 patients underwent catheter intervention for their major aortopulmonary　collaterals. The diameters of pulmonary arterial ring of all the patients were 2 standard deviation less than normal range， and trans-annular patch was chosen for RVOT reconstruction. All the patients were postoperatively followed up for 18 to 26 months （mean 21.2±4.6 months）.?Results　There was no in-hospital death. And no second surgical intervention was needed for conspicuous RVOT stenosis or pulmonary regurgitation. Three patients needed reintubation for lung edema after extubation as a result of major aortopulmonary collaterals. Four patients underwent reexploration for postoperative bleeding. And all the other patients were discharged uneventfully. Mean cardiopulmonary bypass time was 84.0±22.0 min， and mean aortic cross-clamping time was 42.0±12.0 min. Mean RVOT gradient right after surgery was 18.0±4.5 mm Hg， which was not statistically different from mean RVOT gradient of 19.2±5.4 mm Hg measured by transthoracic echocardiography at their last postoperative follow-up（P＞0.05）. The degree of pulmonary regurgitation right after surgery was trivial in 32 patients（1＋）， mild in 28 patients（2＋）， which were not statistically different from the degree of pulmonary regurgitation at their last postoperative follow-up: trivial in 28 patients （1＋）， mild in 27 patients（2＋）， and moderate in 5 patients（3＋）. Calcification was not observed on the valved bovine jugular vein patch and valve cusp， and the valve cusp motioned well. No thrombosis or endocarditis was observed on the valved bovine jugular vein.?Conclusions　For patients with tetralogy of Fallot or double outlet of right ventricle （DORV） and pulmonary stenosis， valved bovine jugular vein patch is a good choice for trans-annular reconstruction of RVOT. There is no severe postoperative complication related to bovine jugular vein， the RVOT pressure gradient does not increase significantly， and anti-regurgitation result is satisfactory in short-term follow-up. Further follow-up is required to evaluate its long-term outcome.

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.

經皮椎體成形術后右心和下腔靜脈骨水泥占位一例

Outcome of Short and Middle Term of Right Ventricular Outlet Tract Reconstruction with Gore-Tex Monocusp Valve

Abstract: Objective To evaluate the outcome of reconstruction of right ventricular outlet tract (RVOT) with 0.1 mm Gore-Tex monocusp valve for short and middle term. Methods Between June 2002 to July 2006, 48 patients underwent reconstruction of RVOT with Gore-Tex monocusp valve to correct cardiac anomalies, including 33 patients with tetralogy of Fallot (TOF) and pulmonary stenosis, 8 patients with TOF and pulmonary atresia, 3 patients with TOF and absent pulmonary valve, 2 patients with double outlet of right ventricle and pulmonary stenosis, 1 patient with truncus arterious and 1 patient with complete transposition of great artery, ventricular septal defect and pulmonary stenosis. Results There was no operative death. The postoperative blood oxygen saturation was up to 1.00. The ratioes of right ventricular systolic pressure and left ventricular systolic pressure were between 0.22 to 0.65.The gradient between right ventricle and left or right pulmonary artery was less than 10 mmHg. All patients were followed up including echocardiography ranged from 3 to 48 months. There were no late death and complication. Trivial and mild pulmonary insufficiency was detected in 18 patients and valvular motion remained competent in 40 patients. Conclusion The results suggest that the reconstruction of RVOT with Gore-Tex monocusp valve can achieve excellent outcome for short and middle term.