OBJECTIVE:To analyze clinical outcomes and prognosis under different treatment strategies for myocardial bridging (MB) patients, providing evidence to optimize clinical decision-making. METHODS:A retrospective study was conducted on 243 patients diagnosed with MB by coronary angiography at our institution. Of them, 77 patients underwent surgery (group A), and were divided into group A1 (n = 31, compression ≥70%) and group A2 (n = 46, combined with other heart surgeries). The remaining 166 patients underwent medical treatment and were divided into group B1 (n = 125, compression <70%) and group B2 (n = 41, compression ≥70%). The quality of life, mortality, and major adverse cardiac events were analyzed for each group. RESULTS:Mean follow-up was 55.6 months in Group A and 85.3 months in Group B. The10-year cumulative survival rates for group A1, A2, B1, B2 were 95%, 92.1%, 93.4%, and 88.2%, and the 10-year rates of freedom from major adverse cardiac events in these groups were 89.7%, 85.0%, 74.4%, and 67.8%, respectively. For patients with isolated MB and systolic compression ≥70%, the incidence of major adverse cardiac events was significantly higher than in group B2, compared with group A1 (P = .01). Seattle Angina Questionnaire (SAQ) scores improved significantly after treatment across all groups (P < .01). CONCLUSIONS:For patients with isolated MB and systolic coronary compression ≥70%, and refractory to medical therapy, myotomy had better outcomes than medical therapy. In cases with MB coexisting with other cardiac diseases, myotomy with other cardiac surgeries can achieve satisfactory outcomes. For symptomatic patients with isolated MB and systolic coronary compression <70%, medical treatment can yield good outcomes.

BACKGROUND:Ebstein's anomaly (EA) is a rare and complex congenital heart anomaly, and the effect of surgical treatment is not ideal. This study aims to introduce our experience in management strategies, surgical techniques, and operative indications for patients with Ebstein's anomaly.METHODS:A retrospective study of 258 operations was performed in 253 patients by the same cardiac surgeon in The First Hospital of Tsinghua University between March 2004 and January 2020. 32 patients had previously received cardiac surgery in other hospitals. The clinical data including diagnosis, operative indications, techniques, pathological changes, and survival rates were collected and analyzed.RESULTS:Anatomical correction was performed in 203 (78.7%) operations, 1½ ventricle repair in 38 (14.7%) operations, tricuspid valve repair only in four operations (1.6%), tricuspid valve replacement in ten (3.9%), total cavopulmonary connection (TCPC) in two (0.8%), and Glenn operation in one operation (0.4%). Reoperation was performed in five patients (2.0%) during hospitalization. Among them, tricuspid valve replacement was performed in one patient, 1½ ventricle repair in two patients, and tricuspid valve annulus reinforcement in two patients. Five patients died with an early mortality rate of 2.0%. Complete atrioventricular conduction block was complicated in one patient (0.4%). A total of 244 patients was followed up (four in the 253 patients lost) with a duration of 3.0-168.0 (87.6 ± 38.4) months. Cardiac function of 244 patients improved significantly with mean New York Heart Association (NYHA) functional class recovery from 3.5 to 1.1. The mean grade of tricuspid valve regurgitation improved from 3.6 to 1.5. Three late deaths (1.2%) occurred. The survival rates at five and ten years after surgery were 98.6% and 98.2%, respectively. Reoperation was performed in five patients (2.0%) during the follow-up period.CONCLUSION:Based on our management strategies and operative principles and techniques, anatomical correction of EA is capable of achieving excellent long-term results, and low rates of TCPC, 1½ ventricle repair and valvular replacement.

Background Myocardial bridges are congenital coronary artery anomalies. There are still many controversies surrounding surgical treatment strategies for myocardial bridges combined with other heart disorders. The purpose of this study was to evaluate the surgical treatment strategies and outcomes in patients with these conditions. Methods Between March 2004 and October 2021, our institution witnessed 77 patients diagnosed with myocardial bridging who underwent surgical intervention. According to the myocardial bridge and combined heart disorder, four groups were identified: 1. isolated LAD supra-arterial myotomy group, 2. LAD CABG and(or not) myotomy group, 3. LAD supra-arterial myotomy and grafting of other branches group, and 4. LAD supra-arterial myotomy and other cardiac surgery group. The perioperative outcomes, symptoms, life quality, mortality, and major adverse cardiac events (MACEs) were analyzed. Results There were no deaths during hospitalization and no rethoractomy for postoperative bleeding or major adverse cardiac events (MACEs). The follow-up period ranged from 2 months to 199.2 months (55.61 ± 10.21) months, the 10-year cumulative survival rates for the four groups of patients were 95.0%, 100%, 100% and 74.1%, and the 10-year freedom rates from the MACEs were 83.9%, 92.0%, 87.5% and 76.2%, respectively. Conclusions Supra-arterial myotomy is preferred in patients with isolated myocardial bridge, and acceptable results can be achieved by choosing supra-arterial myotomy in combination with CABG or other cardiac surgery simultaneously for patients with myocardial bridges and other heart disorders.

INTRODUCTION:Myocardial bridging (MB) is a common and usually benign inborn coronary abnormality that may lead to anginal symptoms, acute coronary syndrome, arrhythmias, and rarely sudden cardiac death. MB are most commonly localized in the middle segment of the left anterior descending coronary artery (LAD). The treatment of LAD-MB is still challenging. Our objective was to assess the short- and long-term results of surgical procedures in patients with LAD-MB who had chest pain refractory to medical therapy.METHODS:Between March 2005 and January 2020, 26 patients (19 males and 7 females; mean 55.8 ± 12.4 years) with MB underwent surgery. All MB was located in the mid-segment of the LAD with a mean length of 4.2 ± 1.7 cm. Coronary angiography before surgery demonstrated LAD-MB with systolic compression more than or equal to 70% in all patients.RESULTS:Twenty-five patients underwent myotomy and one patient underwent coronary artery bypass grafting (CABG). All patients survived and recovered uneventfully. Neither hospital or late death nor major complications occurred. Follow-up time was 3-173 months (mean 55.7 months). Follow-up of coronary angiography or computed tomography scan performed in 16 patients demonstrated restoration of coronary blood flow and myocardial perfusion without significant residual compression of the artery. All patients were symptom-free and are currently in NYHA Class I.CONCLUSION:The symptomatic LAD-MB patients who are refractory to medication should actively undergo the surgical intervention such as myotomy and CABG to eliminate the clinical symptoms and achieve satisfactory results by follow-up findings. Myotomy is a preferred procedure because of its safety and satisfactory results.

Lecithin is a natural mixture of phospholipids and neutral lipids, and plays a vital role in the maintenance of cell-membrane integrity for all of the basic biological processes.This study aimed to evaluate the adhesion and growth of bone marrow-derived mesenchymal stem cells (BMSCs) on the three-dimensional (3D) electrospun lecithin-containing poly (L-lactic acid) (PLLA) fibrous scaffolds. In this study, PLLA and 0-15% of lecithin (wt.%) were dissolved in methylene chloride and thoroughly mixed to fabricate fibrous scaffolds by electrospinning technique. Surface morphology and hydrophilic properties of the scaffolds were characterized by scanning electron microscopy (SEM) and water uptake, respectively. The cytocompatibility of electrospun scaffolds and their potential cytotoxic effects on cells were studied over 8 days by seeding rat BMSCs on the scaffolds of PLLA containing 0-15% lecithin. Then cell proliferation was tested by CCK-8 assay and cell injury was assessed by measuring the release of intracellular lactate dehydrogenase (LDH). And cell morphology was studied by Toluidine blue staining, propidium iodide staining and SEM. The hydrophilicity was dramatically increased with an increase of lecithin content in the modified PLLA/lecithin scaffolds, as determined by rate of water uptake. In addition, the results obtained from in vitro assays suggested that the electrospun PLLA/5%lecithin scaffolds could possess the optimal hydrophilicity, higher adhesion and proliferation of BMSCs than PLLA, and they also did not lead to any significant toxic effects to cells. Moreover, BMSCs on PLLA/5%lecithin scaffolds tended to maintain their phenotypic shape and integrate with the microfibers to form a 3D cellular network, indicating a favorable interaction between this electrospun lecithin-based scaffolds and cells. The study demonstrated that the electrospun PLLA/lecithin fibrous scaffolds showed much better hydrophilicity and biocompatibility by introducing lecithin into the polymer in comparison with pure PLLA, which would offer a promising strategy for constructing tissue engnineered blood vessel.

Objective: This study was aimed to elucidate the feasibility of using right ventricular (RV) strain and strain rate to evaluate right heart function of Ebstein anomaly (EA) patients before and after operation. Methods: Sixty EA patients and 30 healthy controls underwent echocardiography (UCG) for evaluation of right heart function. Preoperative UCG and 1-week and 3-month postoperative UCG were performed in EA patients. RV strain and strain rate were measured on the four-chamber section of tissue Doppler imaging (TDI). Results: The strain and strain rate representative of right ventricle systolic function were reduced prior to operation. RV strain and strain rate improved after the operation (P<.001), most significantly in the basal segment and middle segment of the free wall of the right ventricle as well as the basal segment of the interventricular septum (P<.001). Conclusions: The measurement of RV strain and strain rate on tissue Doppler imaging can be employed to assess the preoperative and postoperative RV function, proves the positive effect of tricuspid valve repair on right heart function, and offers more insight on right heart function evaluation.

目的:阐明三尖瓣环收缩期位移(TAPSE)联合Tei指数对Ebstein畸形(EA)患者手术前后右心功能评价的可行性.方法:本研究中入选的40例EA患者和20名健康对照者均完成了超声心动图(UCG)检查评估右心功能,其中EA患者分别于术前、术后1周、术后3月进行UCG.于四腔心切面测量常规超声心动指标,测量三尖瓣环收缩期位移(TTAPSE),留取组织多普勒(TDI)四腔心切面,并计算出右心室心肌做功(RVTei)指数.结果:术前反映右心室收缩功能的TAPSE、右心室面积变化分数(RVFAC)指标降低,同时反映右心室收缩和舒张功能的RVTei指数升高.EA患者矫治术后1周、3月时TAPSE值高于术前(P＜0.01),而RVTei指数低于术前(P＜0.05),研究结果表明可通过TAPSE、RVTei评估EA矫治术后右心功能.TAPSE与RVFAC有一定的相关性,RVFAC在用于EA矫治手术前后右心功能的评估中,不适于术后与术前的比较,仅适于术后不同阶段之间的比较.结论:利用TAPSE、RVTei指数,可评估EA患者术前术后右心室功能,并认识到三尖瓣修复对右心功能改善的积极作用,为右心功能的评估积累经验.

Background and Aims: Bicuspid aortic valve (BAV) related aortopathy has been a controversial issue in the past few years. Most of the researches focused on BAV with dilated ascending aorta after aortic valve replacement (AVR), but there were limited documents of patients with the normal-sized proximal aorta. We retrospectively analyzed the clinical data of patients undergoing AVR in our institution and evaluated the progression of the unreplaced ascending aorta in a relatively long term follow-up. Methods: In our institution, 165 patients were consecutively recruited from July 2004 to December 2017. Detailed perioperative information and follow-up data were comprehensively collected and quantitatively analyzed. Results: 48 patients (29.1%) had BAV, while TAV was found in 117 patients. A significant difference was observed in diameters of ascending aorta at baseline between BAV and TAV group (37.5 ±4.2mm vs 35.1 ±4.4mm; p=0.001). The overall survival rates were 89% and 95.8% at 10 years postoperatively in BAV versus TAV group (Plog rank=0.138). Only 1 patient suffered an aortic dissection and underwent proximal aortic surgery. No difference in the progression of ascending aorta (0.8±4.7mm vs 0.6±3.5mm, p=0.821) was observed. The diameter of ascending aorta at baseline was a significant predictor of progression in ascending aorta. Conclusions: BAV patients with a normal-sized ascending aorta have a considerable low incidence of late adverse aortic events after AVR. Meanwhile, the progression of the unreplaced ascending aorta in BAV patients is not different from that in TAV patients.

目的 总结我院用主动脉瓣成形术治疗儿童主动脉瓣疾病的方法和结果.方法 回顾性分析我院2005年1月至2017年12月30例行主动脉瓣成形术患儿的临床资料,其中男17例、女13例,年龄4个月～16岁,平均年龄(6.7±4.3)岁,体重5.6～62.4(23.7±14.1)kg.结果 30例患者均存活,围术期无严重并发症,术后平均跨瓣压差(21.1±17.0)mm Hg.随访9个月～l3年,共有4例患儿出现中度以上的主动脉瓣狭窄或关闭不全,其中1例行再次手术.其余患者瓣膜功能良好.结论 主动脉瓣成形术安全可靠,近中期效果良好,少数患者仍需再次手术,可作为治疗儿童主动脉瓣疾病的首选术式.

BACKGROUND Acute kidney injury (AKI) is one of the common complications in infants and children after complex congenital heart surgery. Peritoneal dialysis (PD) is usually applied for renal replacement therapy (RRT), especially in infants. We investigated the efficacy and safety of modified PD for the treatment of acute renal failure and congestive heart failure after cardiac surgery for congenital heart disease in infants. METHODS We retrospectively analyzed five consecutive patients from October 2015 to February 2017. The patients were aged from four days to five years old, and all had acute renal failure and congestive heart failure after cardiac surgery. In the five patients treated with modified PD (five males; average weight: 11.2 ± 5.5 kg), we used the Seldinger technique percutaneous abdominal puncture 16 G single lumen central venous catheter instead of the Tenckhoff peritoneal dialysis catheter as a PD catheter. Modified PD was intermittent. We recorded and monitored circulation and metabolism index. RESULTS Five cases (100%) with modified PD were restored to normal renal function. Congestive heart failure was gradually alleviated, and pulmonary and cardiovascular function were improved. Urine volume increased. Neither peritonitis nor catheter leakage occured in any of our cases. Urine volume increased due to PD, from 0.16 + 0.18 mL/kg*h before PD to 2.63 + 1.05 ml/kg*h at the end of PD (P < .05). Serum creatinine, serum urea nitrogen, and serum K+ changed from 85.0 ± 36.5 μmol/L, 17.1 ± 7.5 mmol/L, and 4.57 ± 0.30 mmol/L before PD, to 76.0 ± 36.7 μmol/L, 20.1 ± 11.0 mmol/L, and 4.42 ± 0.42 mmol/L at the end of PD, respectively (P > .05). Acidosis, hyperkalemia, hypoxemia and low cardiac output syndrome were improved. All patients were cured and discharged with normal renal function. CONCLUSION We conclude that modified single lumen central venous catheter for PD is a safe, feasible, and less invasive therapeutic strategy for AKI in infants undergoing cardiac surgery, and is worthy of being widely applied in clinical practice.

Objective: To evaluate the early and long-term outcomes cardiac surgery of patients with Ebstein anomaly. Methods: The clinic data of 237 patients with Ebstein anomaly received surgical procedures from March 2004 to December 2017 at Department of Cardiac Surgery, First Hospital of Tsinghua University was analyzed retrospectively. There were 105 male and 132 female patients with age of (19.4±16.7) years (ranging from 3 months to 64 years). The surgical procedures include anatomical repair in 188 patients, one and a half ventricle repair in 37 patients, tricuspid valve repair in 4 patients, tricuspid valve replacement in 10 patients, and Fontan procedure in 3 patients (total cavopulmonary connection in 2 patients; Glenn procedure in 1 patient). Results: The early mortality was 2.1% (n=5). One case of atrioventricular (0.4%) newly occurred. There were 228 patients available to follow-up. The range of follow-up duration was 3 to 168 months. Late survival was 99.1% (2 cases of late death) at 10 years. Three patients received reoperation (1.3%), including tricuspid valve repair of 1 patient and one and a half ventricle repair of 2 patients). Indication of tricuspid valve regurgitation improved from 3.6±0.3 to 1.5±0.4. Survival rate at 5 and 10 years was 98.6% and 98.2%, respectively. Conclusions: The principle of the techniques is to reconstruct the tricuspid valve and right ventricle anatomically. For most cases, the anatomical repair was demonstrated with low mortality, less complications and excellent durability at long-term follow-up. If the tricuspid valve is severely hypoplastic, one and a half ventricle repair and valve replacement may be alternatie.

Background: Double outlet right ventricle (DORV) is a group of complex congenital heart abnormalities. Preoperative pulmonary hypertension (PH) is considered an important risk factor for early death during the surgical treatment of DORV. The aim of this study was to report our experience on surgical treatment of DORV complicated by PH. Methods: From June 2004 to November 2016, 61 patients (36 males and 25 females) aged 2 weeks to 26 years (median: 0.67 years and interquartile range: 0.42–1.67 years) with DORV (two great arteries overriding at least 50%) complicated by PH underwent surgical treatment in our center. All patients were categorized according to surgical age and lesion type, respectively. Pulmonary artery systolic pressure (PASP), pulmonary artery diastolic pressure (PADP), and mean pulmonary artery pressure (mPAP) were measured directly before cardiopulmonary bypass (CPB) was established and after CPB was removed. An intracardiac channel procedure was performed in 37 patients, arterial switch procedure in 19 patients, Rastelli procedure in three patient, Senning procedure in one patients, and Mustard procedure in one patient. The Student's t-test and Chi-squared test were performed to evaluate clinical outcomes of the surgical timing and operation choice. Results: Fifty-five patients had uneventful recovery. PASP fell from 55.3 ± 11.2 mmHg to 34.7 ± 11.6 mmHg (t = 14.05, P < 0.001), PADP fell from 29.7 ± 12.5 mmHg to 18.6 ± 7.9 mmHg (t = 7.39, P < 0.001), and mPAP fell from 40.3 ± 10.6 mmHg to 25.7 ± 8.3 mmHg (t = 11.85, P < 0.001). Six (9.8%) patients died owing to complications including low cardiac output syndrome in two patients, respiratory failure in two, pulmonary hemorrhage in one, and sudden death in one patient. Pulmonary artery pressure (PAP) dropped significantly in infant and child patients. Mortality of both infants (13.9%) and adults (33.3%) was high. Conclusions: PAP of patients with DORV complicated by PH can be expected to fall significantly after surgery. An arterial switch procedure can achieve excellent results in patients with transposition of the great arteries type. Higher incidence of complications may occur in patients with ventricular septal defect (VSD) type before 1 year of age. For those with remote VSD type, VSD enlargement and right ventricle outflow tract reconstruction are usually required with acceptable results. The degree of aortic overriding does not influence surgical outcome.

患儿男，生后1 h，主因“发现先天性完全性房室传导阻滞”于2016年4月入住清华大学第一附属医院心脏小儿科。母亲孕25周时发现胎儿心率慢，超声检查提示胎儿房室传导阻滞，孕35周超声提示胎儿完全性房室传导阻滞（心房率135次／min，心室率44次／min），全心扩大，心胸比例0．7，左室舒张末内径20 mm，射血分数59％，心脏结构未见异常。胎儿期生长发育尚正常，胎龄36周剖宫产出生，出生体重为2．5 kg，1、5、10 min Apgar评分分别为9、9、10分。为行“起搏治疗”收入我科。患儿为第2胎第2产，其姐姐（12岁）体健，出生史及既往史无特殊，母亲（43岁）和父亲（44岁）既往体健，否认晕厥、猝死家族史，母亲自身抗体谱［抗核抗体、干燥综合征（ SS ）－A、SS－B、双链DNA （ dsDNA ）抗体、抗心磷脂抗体等］阴性。

Primary cardiac tumors are rare in children, with the prevalence of 0.0017–0.28% in autopsy series.[1] Benign tumors account for over 90% of the total. Cardiac fibromas (CFs) represent the second most common benign cardiac tumor in the pediatric population following rhabdomyomas. Patients can be asymptomatic or present with palpitations, cardiac murmur, arrhythmias, congestive heart failure, and even sudden death according to the size and location of the tumor. Surgical resection should be considered as the best option in symptomatic patients.[23] This study reported four surgical cases of large left ventricular fibroma in children. Between December 2013 and April 2016, four children (two males and two females) diagnosed with primary intramural tumors arising from the left ventricle (LV) underwent a complete surgical resection. Their ages ranged from 4 to 11 years, and weight ranged from 19.9 kg to 34.0 kg. Echocardiography and cardiac magnetic resonance imaging (MRI) confirmed the diagnosis of a large cardiac mass located at the free wall of LV in all the patients. Of them, cardiac systolic and diastolic function in a patient with nonsustained ventricular tachycardia (VT) was worsening with ejection fraction (EF) of 50%. Basal characteristics of the patients are shown in Table 1.Table 1: Clinical characteristics of the four patients in this studySurgery was performed through a median sternotomy with cardiopulmonary bypass and cold cardioplegic arrest. The pericardium was opened, and a large mass was evident arising from the free wall of LV. Dissection was started by incising the epicardium which appeared to be the thinnest portion over the mass. The mass was pale, firm, and nonencapsulated, which was carefully dissected free of the surrounding myocardium using sharp dissection [Figure 1a]. Then, the mass was completely removed [Figure 1b] with or without entering the ventricular cavity, leaving a large defect in the lateral wall of LV. The residual defect together with the cut edge of LV wall was closed with a running 5-0 Prolene suture. Then, the area was covered with a piece of autologous fresh pericardium.Figure 1: Intraoperative view revealing resection of the tumor along the plane of the normal myocardium using sharp dissection (a); the resected tumor (b).There were no hospital and late deaths and no major complications. Postoperative electrocardiogram of all the patients showed normal sinus rhythm and echocardiography showed normal EF with no residual mass. Histopathology confirmed the diagnosis of CF in four patients. All patients remained symptom-free without episodes of ventricular arrhythmias (VA) during the follow-up period. The echocardiography revealed normal left ventricular size and function of each patient without tumor recurrence. The EF in the third patient [Table 1] increased from 50% to 60% after the operation and to 64% after 18 months. Large fibromas of LV are exceptionally rare in children. CF is usually solitary, nonencapsulated, firm, nodular, and gray-white intramural tumors. They are composed of elongated fibroblasts in broad spiral bands and whirls mixed with collagen and elastin fibers. The clinical presentation of CF depends on the size and location of the tumor. Congestive heart failure and VA may be the first clinical manifestation in large fibromas.[4] Patients with large fibromas seem to be the most concerning group, with a VT risk exceeding 50%.[5] Diagnosis is usually established by echocardiography, CT, and MRI. Surgery is required as an effective management strategy in the children when CF cause ventricular inflow/outflow tract obstruction, valve dysfunction, heart failure, and arrhythmia. In the present study, three patients presented with some form of VA, ranging from frequent premature ventricular beats to nonsustained VT. The onset of VA for displacement or compression of the conduction system is often life-threatening and difficult to control, which can result in sudden death. The VA was eliminated after our patients underwent complete fibroma excision, suggesting that significant debulking of the tumor mass might be sufficient to reverse the arrhythmogenic substrate.[5] Although the goal of surgery is as complete a resection as possible, the large tumor size in relationship to the left ventricular cavity has often been considered a limiting factor for complete excision of fibromas. Of our two patients, the size and extent of the rare giant fibromas (7 cm × 5 cm) had put them in the realm of incompletely resectable tumors, but they still underwent the complete excision of the tumors without recurrence, with resolution of symptoms and arrhythmic events. Hence, we think that the key to operation is not only carefully and completely dissect the tumor from healthy surrounding muscle without damage to other normal structures, but also to reconstruct the left ventricular wall for preventing dyskinetic motion and the formation of the ventricular aneurysm, and avoid bleeding after closure of the excision. In conclusion, complete surgical resection should be recommended as the first-choice treatment for large left ventricular fibromas in children. The cardiac function at short- to medium-term follow-up is excellent, with a resolution of symptoms and arrhythmic events. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Objective:To investigate the efficacy of ultrasound-guided percutaneous atrial septal defect (ASD) occlusion and open heart surgery.Methods:29 cases of patients with ASD were divided into repair group (16 cases,surgical repair) and occlusion group (13 cases,percutaneous closure),and the curative effect was evaluated by comparison of perioperative dataof the two groups.Results:In 13 cases of occlusion group,11 cases were successful and surgical repair was performed in 2 cases;repair group of 16 cases (+ 2 casespercutaneous closure) were successful.Time of operation,time of ventilation and the hospitalization time of the occlusion group were significantly lower than that in the repair group,P ＜0.001.Conclusion:Ultrasound guided ASD is less invasive and has no scar,which is better than intracardiacrepair under direct vision.

Primary cardiac tumors in infancy and childhood are rare and usually benign. We report two children with giant left ventricular fibromas and discuss their surgical management.

目的 探讨主动脉瓣关闭不全合并巨大左心室患者外科治疗的适应证、危险因素及远期结果.方法 回顾性分析了2004年3月至2012年12月共收治主动脉瓣关闭不全合并巨大左心室患者42例(左心室舒张期末内径≥70 mm和左心室收缩期末内径≥50 mm)的临床资料.其中男38例、女4例,年龄16 ～ 73 (45.86±14.99)岁.所有患者均行主动脉瓣置换术,术前、术后1周、术后3个月行超声心动图检查,并对患者随访12～132个月,分析影响术后心功能恢复及导致死亡的危险因素.结果 术后早期死亡1例,远期死亡5例.41例患者术后1周经胸超声心动图检查,左心室舒张期末内径(62.00±13.21) mm、左心室收缩期末内径(50.71±14.02) mm、左心室舒张期末内径指数(35.23±8.58) mm/m2、左心室收缩期末内径指数(28.92±9.08) mm/m2,与术前相比均明显减小(P＜0.01).左心室射血分数(46.41％±12.4％)明显低于术前(P＜0.01).术前心功能分级、射血分数、左心室舒张期末内径、左心室舒张期末内径指数是预测术后早期左心室功能恢复的预测因子.左心功能不全及室性心律失常是导致术后死亡的危险因素.术后1年、5年、10年生存率分别为92.9％、90.2％和83.8％.结论 大多数主动脉瓣关闭不全合并巨大左心室患者仍有手术指征,术后疗效满意.但术前左心功能不全、室性心律失常是影响手术疗效和远期存活的危险因素.

A dying neonate with congenital complete atrioventricular block underwent an emergency temporary pacing via the umbilical vein 1 h after birth. Implantation of a permanent epicardial pacemaker system was performed at the age of 10 days. During the follow-up period of 3 months, the child had been growing well with the VVIR pacemaker.

IntroductIon Complete transposition of the great arteries (TGAs) is a common cyanotic congenital heart defect, with an incidence rate of 0.02%, accounting for 5–7% of congenital heart diseases.[1] After birth, infants with TGA largely rely on ventricular septal defect (VSD), atrial septal defect (ASD), and patent ductus arteriosus (PDA) to sustain life. Without systemic‐pulmonary shunt and surgical correction, these infants will probably die on the day after birth.

Background: Double outlet right ventricle (DORV) is a conotruncal anomaly that is a defining element of many types of complex congenital heart disease. Because of a big variety of pathology, there are still some controversies with respect to the definition, classification, and surgical treatment. We report our experience with surgical treatment for DORV (as defined by the “90% rule”) with pulmonary outflow tract obstruction (POTO). Methods: From July 2005 to July 2015, 90 patients underwent surgical treatment of DORV with POTO at the First Hospital of Tsinghua University. There were 55 males and 35 females whose age varies from 3 months to 36 years (mean age 7.1 ± 9.0 years old), and body weights ranged from 5 to 63 kg (mean weight 20.4 ± 16.6 kg). Besides DORV, ventricular septal defect, and POTO, this group of patients includes some with additional associated cardiac abnormalities. Results: Fourteen patients (15.6%) died. The main cause of death was low cardiac output syndrome. Conclusions: The DORV is usually associated with a variety of cardiac abnormalities and POTO is a common defining feature. Acceptable surgical results can be achieved by individualized surgical treatment of most patients. Some patients may require reoperation, and a close follow-up is needed.