Objective To investigate the efficacy of leaflet augmentation technique to repair the recurrent mitral valve (MV) regurgitation after mitral repair in children. Methods A retrospective analysis was conducted on the clinical data of children who underwent redo MV repair for recurrent regurgitation after initial MV repair, using a leaflet augmentation technique combined with a standardized repair strategy at Fuwai Hospital, Chinese Academy of Medical Sciences, from 2018 to 2022. The pathological features of the MV, key intraoperative procedures, and short- to mid-term follow-up outcomes were analyzed. Results A total of 24 patients (12 male, 12 female) were included, with a median age of 37.6 (range, 16.5–120.0) months. The mean interval from the initial surgery was (24.9±17.0) months. All children had severe mitral regurgitation preoperatively. The cardiopulmonary bypass time was (150.1±49.5) min, and the aortic cross-clamp time was (94.0±24.2) min. There were no early postoperative deaths. During a mean follow-up of (20.3±9.1) months, 3 (12.5%) patients developed moderate or severe mitral regurgitation (2 severe, 1 moderate). One (4.2%) patient died during follow-up, and one (4.2%) patient underwent a second MV reoperation. The left ventricular end-diastolic diameter was significantly reduced postoperatively compared to preoperatively [ (43.5±8.6) mm vs. (35.8±7.8)mm, P<0.001]. Conclusion The leaflet augmentation technique combined with a standardized repair strategy can achieve satisfactory short- to mid-term outcomes for the redo mitral repair after previous MV repair. It can be considered a safe and feasible technical option for cases with complex valvular lesions and severe pathological changes.

Objectives:This study aims to investigate optimal surgical management strategies for pediatric patients diagnosed with volume-overloaded mitral regurgitation. Methods:A comprehensive retrospective analysis was conducted on a cohort of 110 pediatric patients who underwent primary mitral valve repair for volume-overloaded mitral regurgitation at Fuwai Hospital between April 2020 and March 2022.The cohort,with an average age of(14.5±15.1)months and 38.2%males,was divided into standardized group for patients receiving 3-step standardized mitral valvuloplasty(n=69)and annuloplasty group for patients undergoing annuloplasty only(n=41).After propensity score matching,a total of 38 pairs of patients were included,comparing the primary endpoint(functional mitral failure and postoperative heart failure)between the two groups. Results:Over a median follow-up of 26.3(19.8,32.9)months,and with a median echocardiographic follow-up of 11.9(7.5,14.8)months,no death was recorded.Among the cases,one patient(0.8%)necessitated unplanned reoperation;and seven patients(6.4%)experienced a recurrence of moderate-severe mitral regurgitation as observed through echocardiography beyond 6 months post-surgery.Additionally,nine patients developed heart failure at one month post-discharge.Above events were similar between the two groups.Following propensity score matching,patients in the standardized group demonstrated significantly longer cardiopulmonary bypass and aortic cross-clamp times compared to the annuloplasty group(both P＜0.05),other outcomes were similar between the two groups.Subgroup analysis based on age indicated that infants(＜1 year old)in the standardized group exhibited a significantly lower incidence of major endpoint events compared to the annuloplasty group.Additionally,postoperative echocardiography in annuloplasty group indicated that Z score of left ventricular end diastolic diameter was still greater than 2 during the latest follow-up. Conclusions:Patients with volume-overloaded mitral regurgitation in the standardized group exhibited comparable perioperative recovery and postoperative outcomes as in the annuloplasty group.For pediatric patients suffering from volume-overloaded mitral regurgitation,particularly those under one year of age,the standardized surgical approach exhibited reduced rates of heart failure and major endpoint events,and this strategy is more suitable for this patient group.

Two pediatric heart failure patients were treated with pulmonary artery banding(PAB)at Fuwai Hospital,from December 2021 to January 2022.In the first case,an 8-month-old patient presented with left ventricular non-compaction cardiomyopathy(LVNC),left ventricular systolic dysfunction,ventricular septal defect,and atrial septal defect.The second case was a 4-month-old patient with LVNC,left ventricular systolic dysfunction,and coarctation of the aorta.After PAB,the left ventricular function and shape of both patients were significantly improved,without serious surgery-related complications.In these individual cases of pediatric heart failure,pulmonary artery banding exhibited a more satisfactory efficacy and safety compared to pharmacological treatment,especially for those with unsatisfactory medication results.Future clinical data are needed to promote the rational and broader application of this therapeutic option for indicated patients.

Objectives:To analyze the clinical prognosis stratification of fetal heart disease(FHD)and the mid-term follow-up results following integrated prenatal and postnatal management. Methods:Present retrospective analysis was performed on 817 fetuses diagnosed with FHD by fetal echocardiography in Fuwai Hospital,Chinese Academy of Medical Sciences(Fuwai Hospital)from the 8th February 2018 to 30th April 2022.According to the Chinese expert consensus on fetal prognosis score of congenital heart disease(CHD)and Fuwai Hospital Pediatric Center score,FHD was divided into grade Ⅰ(0 point),grade Ⅱ(1-3 points),grade Ⅲ(4-6 points)and grade Ⅳ(7-9 points).For FHD fetuses that need to be treated in the neonatal period,the whole closed-loop management and treatment were completed,including pregnancy,birth,neonatal monitoring,surgery,and follow-up.The clinical data and mid-term follow-up results of FHD fetuses were analyzed. Results:A total of 752 fetuses completed the mid-term follow-up.The mean follow-up time was(18.5±2.3)months.There were 111 cases(14.8%)of FHD grade Ⅰ,251 cases(33.4%)of FHD grade Ⅱ,275 cases(33.6%)of FHD grade Ⅲ and 115 cases(15.3%)of FHD grade Ⅳ.There were 393 cases(52.2%)of fetal preservation,1 case(0.2%)of intrauterine death,and 358 cases(47.6%)of induced abortion.Compared with the induced fetus,the pregnant women in the reserved fetus group were older,the gestational age was longer,the rate of receiving noninvasive DNA and amniocentesis was lower,the proportion of chromosomal abnormalities or genetic abnormalities was smaller,and the FHD prognostic score was lower(all P<0.05).One case(0.9%)of FHD grade Ⅰ fetus experienced intrauterine fetal death,and 110 cases(99.1%)were born and recovered.164 cases(65.3%)of grade Ⅱ fetuses were born,and all of them were cured by surgery or spontaneously.118 cases(42.9%)of grade Ⅲ fetuses were born,117 cases completed biventricular radical operation,and 1 case completed two-stage operation waiting for the second stage biventricular radical operation.One case(0.9%)of grade Ⅳ was born and completed the two-stage bidirectional Glenn procedure.Among the induced FHD grade Ⅱ fetuses,8(9.2%)had abnormal chromosomal or genetic testing results,and 8(9.2%)had other organ abnormalities.Among the induced FHD grade Ⅲ fetuses,5 cases(3.2%)had chromosomal abnormalities,5 cases(3.2%)had genetic abnormalities,and 9 cases(5.7%)had other organ abnormalities.There was only one case(0.9%)of FHD Ⅳ with hypoplastic left heart syndrome,which was retained as one of the twins and was born successfully.Norwood one-stage procedure was performed in the neonatal period,and two-stage bidirectional Greene procedure was performed at 6 months after birth.Other fetuses were induced following the choice of maternity and their family members. Conclusions:Hierarchical management based on clinical prognosis is helpful to scientifically guide the integrated treatment of FHD fetuses and improve the success rate of treatment of FHD fetuses with good prognosis.

Objectives:To summarize the experience of echocardiographic diagnosis and the short-term post-operative outcome of children with cardiac tumors. Methods:A total of 27 infants and children,who underwent echocardiography examination and were preliminarily diagnosed with cardiac tumors in Fuwai Hospital from January 2018 to June 2023,were retrospectively included in this study.Clinical symptoms,echocardiographic results,surgical and perioperative results and early outcomes were analyzed. Results:Among the 27 patients,16 were males.The age ranged from 1 month to 11 years(mean age:[3.2±2.5]years)and the body weight ranged from 5.7 to 40.0 kg(mean body weight:[17.2±11.2]kg).Cardiac tumors were pathologically confirmed in 25 cases,including 14 cases of fibroma,5 cases of rhabdomyoma,3 cases of myxoma,2 cases of lipoma,1 case of hemangioma.Preoperative echocardiographic diagnosis was consistent with postoperative pathologic diagnosis in 14 cases.Nontumor lesions were misdiagnosed as tumors by echocardiography in 2 cases.All the patients underwent surgery,complications occurred in 2 cases early post surgery and underwent reoperations and recovered well.There were no deaths during a median follow-up of 29.8(6.5,72.0)months,recurrence occurred in one child with myxoma. Conclusions:Pediatric patients with cardiac tumors have specific echocardiographic manifestations.It is necessary to sum up related experience constantly to improve the diagnostic accuracy.Short-term outcome post-surgery is satisfactory for the treatment of benign pediatric cardiac tumors.

Objective:To explore the anatomic repair strategy for congenital corrected transposition of great arteries (ccTGA).Methods:At the retrospective study, from August 2004 to May 2019, all 120 consecutive ccTGA were included and all accepted anatomic repair. There were 36 cases with with left ventricular outlet obstruction(LVOTO) and cardiac malpositon [ages(4.6±2.2) years, weight(17.7±5.9)kg] underwent the one and a half ventricle repair(hemi-Mustard and bidirectional Glenn procedures combined with the Rastelli), 49 cases[ages(3.4±2.7) years, weight(17.7±11.4)kg] underwent double switch operation(Great artery swtich with Senning operation), 24 cases [ages(5.7±4.3) years, weight(19.1±8.6)kg] with LVOTO and ventricular sept defect(VSD) accepted the Rastelli with Senning operation, and 14 cases with LVOTO and remote VSD [ages(6.9±4.8) years, weight(23.0±12.9)kg] accepted the Double root transposition(DRT) with Senning operation. Follow up data were collected by telephone interviews and echo. The median follow-up time were 49 months varied from 20 to 84 months, 46 months varied from 18 to 108 months, 35 months varied from 7 to 84 months and 98 months varied from 72 to 145 months. Statistical analysis was performed with SPSS 19.0.Results:There were 6 in-hospital deaths and 2 follow-up deaths. The survival probability were(84.0±6.0)% and(84.0±6.0)% at 5 and 10 years after operation. The probability of freedom from re-intervention were(95.0±11.8)% and(89.0±11.8)% at 5 and 10 years after operation. All 6 patients need implant pacemaker for Ⅲ A-V block. Seven patients had moderate or more than moderate tricuspid regurgitation. The left ventricular(systemic ventricle) EF were 0.61±0.09, 0.63±0.08, 0.59±0.01 and 0.65±0.07 in one and a half ventricle repair group, double switch(AS group), Rastelli with Senning(RS group) and DRT with Senning(DS group) patients. There were 1 heart failure in one and a half ventricle repair group, 1 in AS group and 1 in RS group. For 36 pure ccTGA patients, compared with direct double switch patients these patients accepting double switch after pulmonary banding(PAB) had more EF(0.54±0.09 vs. 0.65±0.08, P=0.00). There were significantly less patients need re-operation in one and a half ventricle repair group compared with RS group(0 vs. 13.6%, P=0.03). Conclusion:For ccTGA/LVOTO/cardiac malpositon, the one and a half ventricle repair was ideal strategy with significant less RV-PA conduit stenosis and re-operation. For pure ccTGA patients, second staged double switch after PAB had better long-term heart function. For ccTGA/ LVOTO/ remote VSD patients DRT with Senning was ideal strategy.

Objective    To summarize the reoperation experience for complete atrioventricular septal defect (CAVSD) with severe left atrioventricular valve regurgitation (LAVVR) by standardized mitral repair-oriented strategy. Methods    From 2016 to 2019, 11 CAVSD patients underwent reoperation for severe LAVVR by standardized mitral repair-oriented strategy at Fuwai Hospital, including 5 males and 6 females with a median age of 56 (22-152) months. The pathological characteristics of severe LAVVR, key points of repair technique and mid-term follow-up results were analyzed. Results    The interval time between the initial surgery and this surgery was 48 (8-149) months. The aortic cross-clamp time was 54.6±21.5 min and the cardiopulmonary bypass time was 107.4±38.1 min, ventilator assistance time was 16.4±16.3 h. All patients recovered smoothly with no early or late death. The patients were followed up for 29.0±12.8 months, and the echocardiograph showed trivial to little mitral regurgitation in 5 patients, little regurgitation in 5 patients and moderate regurgitation in 1 patient. The classification (NYHA) of cardiac function was class Ⅰ in all patients. Conclusion    Standardized mitral repair-oriented strategy is safe and effective in the treatment of severe LAVVR after CAVSD surgery, and the mid-term results are satisfied.

Objective    To summarize the clinical outcomes and experience of surgical treatment for patients with complete atrioventricular septal defect (CAVSD) above the optimal age for surgery. Methods    We retrospectively reviewed clinical data of 163 simple type CAVSD patients less than 7 years who underwent operations in Fuwai Hospital from 2002 to 2013. The patients were divided into a normal group (n=84, including 37 males and 16 females with an average age of 7.6±2.7 months) and an over-age group (n=79, including 30 males and 49 females with an average age of 34.6±19.6 months) according to whether the age was more than 1 year. Results    The average aortic cross clamp time (88.3±24.4 min vs. 106.1±35.4 min, P<0.001) and cardiopulmonary bypass time (123.6±31.1 min vs. 142.6±47.1 min, P=0.003) were statistically different between the two groups. During the follow-up period (the normal group 53.3±43.9 months, the over-age group 57.2±48.2 months), there was no statistical difference in all-cause mortality (10.7% vs. 8.9%, P=0.691), the incidence of moderate or severe left atrioventricular valve regurgitation (16.7% vs. 21.5%, P=0.430) and reintervention rate (3.6% vs. 0.0%, P=0.266) between the two groups. No left ventricular outflow tract obstruction and complete atrioventricular block occurred in both groups. Conclusion    For CAVSD children above the optimal age, rational surgical treatments can also achieve satisfying results.

Objective:To analyze the echocardiographic features of operation-related aorto-cardiac fistula(ACF) after surgery or transcatheter procedure, to explore the value of echocardiography on diagnosis of operation-related ACF, and summarize the key points of its diagnosis.Methods:Eight patients with operation-related ACF who were admitted to Fuwai Hospital were collected from July 2002 to December 2020. Echocardiographic features of the 8 patients with operation-related ACF were reviewed and analyzed. The diagnosis methodology was summarized.Results:Of the 8 patients with operation-related ACF, 3 had aortic right atrial fistula and 5 had aortic right ventricular fistula. The fistula was single, which can be located but not limited to the aortic sinus. The median size of the fistula was 4 mm (range: 3-5 mm). There was no aneurysmal dilation of the aortic sinus where the fistula was located. The fractured end of the fistula did not thin, and the fistula had a regular shape. Six of the 8 patients undertook cardiac catheterization and occlusion. One patient received surgical repairment. One patient was treated conservatively.Conclusions:Echocardiography can help diagnose operation-related ACF and provide valuable information for further clinical diagnosis and treatment. Sonographers should avoid missed diagnosis and pay attention to distinguishing from other causes of aortic-cardiac shunt disease.

Objective    To report the short-term outcomes of a standardized, simplified and reproducible strategy of mitral valvuloplasty (MVP), which was focused on leaflet foldoplasty and anatomic anomalies of congenital mitral regurgitation (MR). Methods    Consecutive 74 patients who underwent MVP by our standardized strategy in our institution from 2016 to 2018 were included retrospectively. There were 30 males and 44 females with a median age of 18.5 (6-146) months and weight of 15.4 (7-51) kg. Results    Anatomic anomalies of MR included: (1) subvalvular apparatus: 72 (97.3%) patients with mal-connected chordae tendineae, 31 (41.9%) with absent chordae tendineae and 14 (18.9%) with fused or dysplastic papillary muscle; (2) leaflet: 10 (13.5%) patients with cleft of anterior leaflet, 61 (82.4%) with leaflet prolapse including 56 (91.8%) with anterior leaflet prolapse; (3) annulus: 71 (95.9%) patients with annular dilatation. Leaflet foldoplasty was performed in 61 (82.4%) patients with leaflet prolapse. All patients were successfully discharged and 4 (5.4%) patients were with moderate MR. The follow-up time was 22.0 (9.1-41.8) months. During the follow-up period, 3 patients had moderate MR and 1 patient had reoperation for severe MR. All patients were in normal cardiac function with a mean left ventricular ejection fraction of 66.0%±6.1%. In addition, the mean left ventricular end-diastolic dimension was 31.8±6.0 mm, which was significant smaller than that before the operation (t=6.090, P<0.000 1). Conclusion    The standardized leaflet foldoplasty with resection of mal-connected chordae tendineae and posterior annuloplasty technique is safe and feasible with favorable short-term outcomes in MR patients.