Double outlet right ventricle (DORV) is a complex cardiac malformation with many anatomic variations and various approaches for surgical repair. DORV is mainly defined as the congenital heart disease with ventriculoarterial connection in which both pulmonary artery and aorta arising primarily (>50%) from the right ventricle, associated with continuity or discontinuity between the aorta and mitral valve. DORV can be subclassified by various ways. Now subclassification is usually performed according to the relationship between the ventricular septal defect (VSD) and the great arteries. Various approaches for surgical repair of DORV ranging from single ventricle palliation to biventricular repair are reported from many centers. However, the high-grade guideline of surgical management of DORV is still absent. Hence, we developed the Chinese expert consensus on DORV as the evidence for surgical strategies.
ObjectiveTo explore the appropriate method of biventricular repair and analyze the risk factors for reoperation, by summarizing the 15-year treatment experience of biventricular repair for double outlet right ventricle with non-committed ventricular septal defect (DORVncVSD).MethodsClinical data of 162 consecutive patients with DORVncVSD who had biventricular repair from 2005 to 2019 in our center were retrospectively analyzed. The children were divided into two groups according to the path of intracardiac tunnel: 110 patients with ventricular septal defect rerouted to the aorta were recruited into a group A (75 males and 35 females aged 3.6±3.2 years); 52 patients with ventricular septal defect rerouted to the pulmonary artery were into a group B (30 males and 22 females aged 2.8±2.7 years). In order to establish a smooth intracardiac tunnel, enlargement of VSD, the resection of conus muscle and the transfer of tricuspid tendon or papillary muscle, etc were performed at the same time.ResultsIn the patients with biventricular repair, there were 9 (5.6%) early deaths and 6 (3.7%) early intracardiac baffle obstructions. During the follow-up of 7.5±7.0 years, and 8 (4.9%) late deaths occurred. The 1-year, 5-year, 10-year and 15-year survival rates of the group A were 92.7%, 91.1%, 91.1%, 85.4%, respectively and those of the group B were 92.2%, 85.2%, 85.2%, 85.2%, respectively. The difference between the two groups was not statistically significant (P=0.560). The follow-up results showed that 10 (6.2%) patients had late-onset intracardiac tunnel obstruction, and 8 patients underwent reoperation. There were more late-onset intracardiac tunnel obstruction patients and overall intracardiac tunnel obstruction patients in the group A than those in the group B (9 patients vs. 1 patient, P=0.017; 15 patients vs. 1 patient, P=0.001). No significant difference of early mortality and late mortality was noted for the group A (P=0.386) and the group B (P=0.223). Also it was noted that performing tricuspid valve operation at the same time in the group A had a significant impact to reduce the occurrence rate of intracardiac obstruction (1/46 vs. 15/64, P=0.004), without any tricuspid regurgitation or stenosis. The reoperation rate of patients with Rastelli after right ventricular outflow tract lesions was significantly higher than that of REV surgery and double root replacement surgery (5/14 vs. 0/38, P<0.001).ConclusionThe effect of biventricular repair for DORVncVSD is satisfactory. And concomitant tricuspid procedures can help reduce the occurrence of intracardiac obstructions. Reconstruction of right ventricular outflow tract with biological valved conduit is a risk factor for reoperation.
ObjectiveTo evaluate the mid- and long-term outcomes of different surgical techniques for subaortic stenosis.MethodsThe clinical data of 75 patients with subaortic stenosis who underwent surgery in our hospital from January 2008 to January 2018 were retrospectively analyzed, including 48 males and 27 females, with a median age of 72 (48, 132) months and mean weight of 21.35±15.82 kg. There were 40 (53.3%) patients combined with aortic regurgitation; 38 (50.7%) patients were the first time and 37 patients were the second time to receive the operation. According to the surgical techniques, 75 patients were divided into two groups: a group A (40 patients with simple subaortic membrane resection) and a group B (35 patients with subaortic membrane and muscle resection or modified Konno procedure).ResultsTwo (2.67%) patients died in hospital. There was one late death in the group B. The average preoperative and postoperative pressure gradient of all patients was 69.96±42.02 mm Hg and 7.44±12.45 mm Hg, respectively. All patients were followed up for 51 (12, 120) months. Pressure gradient at follow-up in the group A and the group B was 8.83±14.52 mm Hg and 5.86±9.53 mm Hg, respectively with no statistical difference (P=0.294). Four patients in the group A and 2 patients in the group B needed reintervention. However, there was no statistical difference in the long-term reintervention rate between the two groups (P=0.480).ConclusionFor the different degree of lesions in the left ventricular outflow tract, our management strategy is feasible. Although there is no statistical difference between two the groups in the long-term reintervention rate after simple valvular membrane resection, prolonged follow-up is necessary to examine the long-term outcomes of different surgical techniques.
ObjectiveTo explore the operative strategy after palliative shunt for correcting congenitally corrected transposition of great artery (cTGA) patients with left ventricular outflow tract obstruction (LVOTO) and cardiac malpostion.MethodsWe retrospectively analyzed the clinical data of 54 patients with onsecutive cTGA with LVOTO and cardiac malpositon from June 2011 to May 2019. The patients were devided into two groups. There were 24 patients (16 males and 8 females at mean age of 5.4±2.2 years) who underwent one and a half ventricle repair as a one and half ventricle group. And there were 30 patients (19 males and 11 females at age of 8.6±6.2 years) who underwent one ventricle repair operation as a one ventricle group. Follow-up data were collected by telephone interviews.ResultsThere was no statistical difference in systemic atrioventricular valve regurgitation and systemic ventricular ejection fraction between the two groups (P>0.05). Compared with one and a half ventricle group, the cardiopulmonary bypass time (CPB) time, mechanical ventilation time and intensive care unit stay were significant shorter than those in the one ventricle group (P<0.05), but prolonged pleural effusions developed more frequently in the one ventricle repair group (P<0.05). There was no in-hospital death but 1 follow-up death in each group. The follow-up time was 49 (17-38) months in the one and half ventricle group at follow-up rate of 93.9%, and 47 (12-85) months at follow-up rate at 90.9% in the one ventricle group. One and a half ventricle group had better systemic ventricular ejection fraction (EF) than that in the one ventricle repair group. And the rate of heart function (NYHA) class Ⅲ and class Ⅳ in one and a half ventricle group was lower than that in the ventricle group. No significant difference of survival and freedom from re-intervention probability between the two groups was found.ConclusionFor patients of correction of cTGA with LVOTO and cardiac malposition after palliative shunt, the one-and-a-half ventricular repair procedure is ideal operative strategy.
ObjectiveTo analyze the results of surgical treatment of severe aortic stenosis in infants.MethodsFrom August 2012 to December 2019, 28 infants undergoing aortic valvuloplasty in our hospital were selected, including 22 males and 6 females, aged 62.00 (47.00, 82.50) d. The baseline characteristics of the patients, postoperative complications and follow-up results were analyzed.ResultsTwenty (71.43%) patients had bicuspid aortic valves. Five (17.86%) patients had heart failure and two (7.14%) patients used prostaglandin before surgeries. Postoperative mechanical ventilation time was 25.00 (17.00, 62.75) h, ICU stay was 3.50 (2.00, 8.50) d and postoperative hospital stay was 10.00 (7.00, 16.50) d. Four (14.29%) patients got delayed recovery (ICU stay>14 d). One (3.57%) perioperative death was observed. The follow-up time was 55.00 (43.25, 82.25) months. No death was found during follow-up. Four (14.81%) patients underwent a second operation, including three (11.11%) patients with severe aortic stenosis, and one (3.70%) patient with severe regurgitation.ConclusionInfants with severe aortic stenosis are seriously ill and have a long postoperative recovery time, requiring early surgery. The postoperative follow-up results are satisfactory.
ObjectiveTo evaluate the long-term effects of fenestration on patients at different risk levels, who performed external conduit total cavo-pulmonary connection operation.MethodsThis was a retrospective analysis which enrolled 383 patients undergoing external conduit total cavo-pulmonary connection in Fuwai Hospital from 2008 to 2015. Based on the preoperative data and whether fenestration in the operation, the whole cohorts were divided into four subgroups: a high risk group with fenestration(mean age: 10.53±7.06 years, 55 males), a high risk group with non-fenestration(mean age: 9.30±7.83 years, 43 males), a low risk group with fenestration(mean age: 8.91±7.13 years, 65 males) and a low risk group with non-fenestration(mean age: 8.23±5.34 years, 67 males). Then we collected and analyzed the perioperative data and long-term prognosis of this cohorts in different risk levels.ResultIn the high-risk group, the duration of chest drainage in fenestration group was significantly shorter than that of the non-fenestration group (12.39±12.03 d vs. 23.30±15.36 d, P=0.001). The incidence of delayed chest drainage in the fenestration group was lower than that in the non-fenestration group (25.0% vs. 47.1%, P=0.002). In addition, the length of hospital stay was shorter than that of the non-fenestration group (18.91±12.79 d vs. 29.68±37.77 d, P=0.004), with significant statistical difference. In the low risk group, there were 3 (2.7%) and 2 (1.6%) deaths at the follow-up in the non-fenestration and fenestration groups respectively (P=0.761). And 1 patient (1.3%), 1 patient (1.4%) died in the fenestration and non-fenestration group (P=0.593) in high risk group. However, there was no statistically significant difference among the fenestration and non-fenestration groups in terms of long-term intestinal protein loss syndrome and arrhythmia in different risk level groups.ConclusionFenestration can reduce the incidence of early complications and hospital stay, effectively, especially for the high-risk patient. Fenestration is recommended for high-risk patients with external conduit total cavo-pulmonary connection operation.
ObjectiveTo summarize the surgical experience of patients with anomalous left coronary artery from the pulmonary artery (ALCAPA) and intramural left coronary artery and analyze the early and mid-term clinical outcomes. Methods The infants with ALCAPA and intramural left coronary artery who underwent surgical treatment in Fuwai Hospital from January 2014 to September 2020 were retrospectively enrolled, and the clinical data of the patients were analyzed. Results A total of 10 patients were included. There were 8 males and 2 females, with a median age of 7.5 (3-46) months at surgery. The surgical techniques included coronary unroofing in 7 patients, coronary unroofing with coronary reimplantation in 2 patients, and coronary unroofing with ligation of left coronary artery ostium in 1 patient. Seven infants received additional procedures, including 5 mitral valve repair. Delayed chest closure was required in 2 infants, and no operative death or major complications occurred in the whole group. Postoperative chest radiograph showed that the mean cardiothoracic ratio was lower than that before surgery (0.62±0.05 vs. 0.67±0.06, P=0.006). Postoperative echocardiography indicated that the mean left ventricular ejection fraction was increased than that before surgery, but there was no statistical difference (38.7%±15.9% vs. 30.0%±16.1%, P=0.066). The follow-up was available for all 10 survivors, with an average follow-up time of 13-92 (46.6±25.0) months. During the follow-up period, the patients had no obvious symptoms, death, coronary complications or other major complications. The chest radiograph at last follow-up showed that the mean cardiothoracic ratio was further decreased (0.60±0.07 vs. 0.62±0.05, P=0.024). The echocardiography at last follow-up showed that the mean left ventricular ejection fraction was further improved (60.1%±9.3% vs. 38.7%±15.9%, P=0.002). Conclusion ALCAPA with intramural left coronary artery is a rare malformation. It can be treated with different surgical techniques with satisfactory early and mid-term outcomes.
ObjectiveTo analyze the mid-long-term outcomes of surgical balloon valvuloplasty (SBV) for right ventricular decompression in the treatment of pulmonary atresia with intact ventricular septum (PA/IVS).MethodsClinical data of consecutive 91 patients who were diagnosed with PA/IVS and underwent SBV in our institution from January 2005 to December 2017 were retrospectively analyzed, including 52 (57.1%) males and 39 (42.9%) females. The median age was 3 months (1 d, 24 months) and the median weight was 4.1 (2.5, 12.0) kg.ResultsThe SBV was performed in all patients, and 62 of whom received other simultaneous surgeries, including ligation of patent ductus arteriosus (PDA, 33 patients), ligation of PDA with modified Blalock-Taussig shunt (23 patients), ligation of PDA with bidirectional Glenn shunt (6 patients). There was no early postoperative death. The median follow-up time was 8.8 (2.5, 13.4) years, 4 patients were lost. There were 7 (8.0%) deaths and 1 (1.1%) patient with a re-SBV for pulmonary stenosis. The one and a half ventricular repair was performed in 5 (5.7%) patients and Fontan procedure in 2 (2.3%) patients. In addition, the mean Z-value of tricuspid valve annulus was −1.7±1.5, which was significant bigger than that before the operation (t=5.587, P<0.001).ConclusionSBV via right ventricular outflow tract for right ventricular decompression in the treatment of PA/IVS is safe and reliable. The majority of patients can receive biventricular repair instead of single ventricular palliation by SBV with individually customized shunt.
ObjectiveTo 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).MethodsConsecutive 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.ResultsAnatomic 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).ConclusionThe 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.
ObjectiveTo summarize our experience of critical congenital heart diseases treatment system for the newborn and to report its surgical results.MethodsWe reviewed the clinical data of 97 neonates with congenital heart diseases who admitted to pediatric cardiac center from January 2019 to August 2020 in our hospital. The patients were divided into a prenatal and postnatal diagnosis and treatment integration group (integrated group, n=41), and a postnatal diagnosis and rapid admission by green channel group (non-integrated group, n=56).ResultsThe age of admission in the integrated group was younger than that in the non-integrated group (3.0 d vs. 11.0 d, P<0.001), and the weight was lighter (3.3±0.4 kg vs. 3.6±0.6 kg, P=0.006), operation age was younger (13.0 d vs. 17.5 d, P=0.004), proportion of palliative surgery was smaller (2.4% vs. 8.9%, P=0.396), time for ventilator assistance was longer (153.0 h vs. 65.0 h, P=0.020), hospital mortality was lower (0.0% vs. 7.1%, P=0.135). There was no significant difference in the follow-up (11.0 months vs. 12 months, P=1.000), out-of-hospital mortality (2.4% vs. 1.8%, P=1.000) and total mortality (2.4% vs. 8.9%, P=0.396) between the two groups.ConclusionPrenatal and postnatal diagnosis and treatment integration can significantly shorten the diagnosis and the hospitalization interval of newborn, that surgical intervention could be performed timely. It can reduce the risk of death before surgery but need longer time for recovery after surgery. Patients with postnatal diagnosis and admitted hospital through green channel also can get perfect results if surgical intervention is performed timely.