Qiuming Chen

Arterial Switch for Transposed Great Vessels With Intact Ventricular Septum Beyond One Month of Age

BACKGROUND Late referral of patients with transposition of the great arteries (TGA) and intact ventricular septum (IVS) is common in China. This study investigates the impact of later age on the arterial switch operation (ASO) performed for TGA-IVS beyond 1 month of age. METHODS From 2000 to 2011, a total 109 patients with TGA-IVS were referred over 1 month of age. In group A, 78 patients with satisfactory left ventricular (LV) geometry underwent a one-stage ASO. In group B, 31 patients with LV regression underwent a two-stage ASO with prior LV retraining. RESULTS The median age at ASO was older in group B (6 months, versus group A 1.9 months; p = 0.01). Group A had more frequent patent ductus arteriosus (70.5%, versus group B 38.7%; p = 0.02). The in-hospital mortality was similar in both groups (group A 2.6%, group B 9.7%; p = 0.14). Late mortality was higher in group B (16%, versus group A 2.7%; p = 0.03), as well as aortic regurgitation rate (group A 9.8% versus group B 33.3%; p = 0.01). The median duration of retraining in group B was 18 days. There were no deaths at retraining, although 2 patients required revision of the pulmonary artery banding. The only significant risk factor for late mortality in group B was age at retraining, as continuous variable (p = 0.04). Age beyond 3 months at LV retraining was associated with late impaired LV ejection fraction (p = 0.01). CONCLUSIONS The overall outcomes of ASO for TGA-IVS performed beyond 1 month of age are satisfactory. Two-stage ASO has higher late mortality and more neoaortic regurgitation. Later age at retraining is associated with higher late mortality. Age beyond 3 months at retraining is associated with impaired LV function.

Multistage pulmonary artery rehabilitation in patients with pulmonary atresia, ventricular septal defect and hypoplastic pulmonary artery

OBJECTIVES The aim of this study was to determine the effect of multistage pulmonary artery (PA) rehabilitation consisting of right ventricle to pulmonary artery (RV-PA) connection, major aortopulmonary collateral artery (MAPCA) closure and PA angioplasty in patients with pulmonary atresia, ventricular septal defect (VSD) and hypoplastic pulmonary arteries. In addition, the effects of the PA reintervention were reported and risk factors were analysed. METHODS This study was a retrospective review of 69 consecutive patients with pulmonary atresia, VSD and hypoplastic pulmonary arteries (mean Nakata index 100.9 ± 57.6 mm(2)/m(2)) who underwent multistage rehabilitation of hypoplastic PA from December 2009 to December 2014. RESULTS RV-PA connection was performed at a median age of 1.0 years with 2 hospital deaths in the hybrid operation theatre. Thirty-two patients underwent concomitant pulmonary angioplasty with 28 collateral occlusions. After a mean duration of 15.4 ± 12.7 months, 16 patients had interventional catheterization consisting of 14 balloon dilatations, 12 stent implantations and 16 collateral occlusions. At a mean age of 2.7 ± 1.9 years, complete repair was performed in 31 patients within 1.2 ± 0.6 years of the palliation with 1 hospital death. Twenty-two patients had concomitant PA angioplasty. The estimated complete repair rate was 60.1 ± 7.1% ∼3 years after the palliation by the Kaplan-Meier method. During a mean follow-up of 2.8 ± 1.3 years, 49.3% (33/67) of the patients had PA reintervention. Pulmonary stenosis requiring angioplasty at palliation is associated with PA reintervention (P = 0.003). The actuarial survival rate for the overall population was 93.8 ± 3.0% at 5 years after the placement of an RV-PA connection. CONCLUSIONS The multistage strategy consisting of a RV-PA connection, MAPCA closure and PA angioplasty is effective in rehabilitating the hypoplastic PA in patients with pulmonary atresia, VSD and hypoplastic pulmonary arteries. However, PA reinterventions may be required in specific patients.

Surgical outcomes of 380 patients with double outlet right ventricle who underwent biventricular repair.

OBJECTIVES The study objective was to report the outcomes of biventricular repair in patients with double outlet right ventricle. METHODS Patients with double outlet right ventricle who underwent biventricular repair at Fuwai Hospital from January 2005 to December 2012 were included. Patients were excluded if double outlet right ventricle was combined with atrioventricular septal defect, heterotaxy syndrome, atrioventricular discordance, or univentricular physiology. RESULTS A total of 380 consecutive patients with a mean age of 1.9 ± 2.1 years (range, 1 month to 6 years) were included. Varied types of biventricular repair were customized individually. Follow-up was 90.4% complete, and the mean follow-up time was 3.4 ± 3.9 years. There were 17 (4.5%) early deaths and 7 (2.1%) late deaths. Preoperative pulmonary hypertension was the only risk factor for early mortality. Postoperative significant left ventricular outflow tract obstruction was present in 9 survivors. Patients with noncommitted ventricular septal defect had a longer crossclamp time, longer cardiopulmonary bypass time, and higher incidence of postdischarge left ventricular outflow tract obstruction. There were 4 reoperations, all of which were caused by subaortic left ventricular outflow tract obstruction. All of the pressure gradients were decreased to less than 20 mm Hg after the modified Konno procedure with an uneventful postoperative course. CONCLUSIONS Optimal results of varied types of biventricular repair for double outlet right ventricle have been acquired. Although noncommitted ventricular septal defect is technically difficult, the outcomes of patients are favorable. Late-onset left ventricular outflow tract obstruction is the main reason for reoperation but can be successfully relieved by the modified Konno procedure.

Human lung microRNA profiling in pulmonary arterial hypertension secondary to congenital heart defect

Although several microRNAs were reported to play essential roles in pulmonary artery hypertension due to hypoxia or monocrotaline, their potential role in pulmonary arterial hypertension secondary to congenital heart disease is largely unknown. This study aimed to indentify microRNAs implicated in pulmonary arterial hypertension secondary to congenital heart disease in children.

Outcomes of Surgical Repair for Persistent Truncus Arteriosus from Neonates to Adults: A Single Center's Experience

Objective This study aimed to report our experiences with surgical repair in patients of all ages with persistent truncus arteriosus. Methods From July 2004 to July 2014, 50 consecutive patients with persistent truncus arteriosus who underwent anatomical repair were included in the retrospective review. Median follow-up time was 3.4 years (range, 3 months to 10 years). Results Fifty patients underwent anatomical repair at a median age of 19.6 months (range, 20 days to 19.1 years). Thirty patients (60%) were older than one year. The preoperative pulmonary vascular resistance and mean pulmonary artery pressure were 4.1±2.1 (range, 0.1 to 8.9) units.m2 and 64.3±17.9 (range, 38 to 101) mmHg, respectively. Significant truncal valve regurgitation was presented in 14 (28%) patients. Hospital death occurred in 3 patients, two due to pulmonary hypertensive crisis and the other due to pneumonia. Three late deaths occurred at 3, 4 and 11 months after surgery. The actuarial survival rates were 87.7% and 87.7% at 1 year and 5 years, respectively. Multivariate analysis identified significant preoperative truncal valve regurgitation was a risk factor for overall mortality (odds ratio, 7.584; 95%CI: 1.335–43.092; p = 0.022). Two patients required reoperation of truncal valve replacement. One patient underwent reintervention for conduit replacement. Freedom from reoperation at 5 years was 92.9%. At latest examination, there was one patient with moderate-to-severe truncal valve regurgitation and four with moderate. Three patients had residual pulmonary artery hypertension. All survivors were in New York Heart Association class I-II. Conclusions Complete repair of persistent truncus arteriosus can be achieved with a relatively low mortality and acceptable early- and mid-term results, even in cases with late presentation. Significant preoperative truncal valve regurgitation remains a risk factor for overall mortality. The long-term outcomes warrant further follow-up.

Biventricular repair for double outlet right ventricle with non-committed ventricular septal defect

OBJECTIVES Outcomes of biventricular repair for patients with double outlet right ventricle and non-committed ventricular septal defect (DORVncVSD) are not well defined. We aim to report our experience with biventricular repair of this anomaly in proposing an original surgical management that simplifies the anatomical correction. METHODS From January 2005 to December 2013, 75 consecutive patients with DORVncVSD who had undergone biventricular repair in our institution were retrospectively included. The patients were divided into 2 groups: 40 patients in Group A had the ventricular septal defect rerouted to the aorta, and 35 patients in Group B had the ventricular septal defect rerouted to the pulmonary artery. Concomitant tricuspid procedures, conal resection and ventricular septal defect enlargement were used to favour intracardiac tunnel geometry. RESULTS Five types of biventricular repair and 16 concomitant procedures were performed. Mean age at biventricular repair was 2.2 ± 2.0 years. There were 6 (8.0%) early deaths and 4 (6.1%) early intracardiac baffle obstructions. During the 4.1 ± 4.0 years follow-up, 3 (4.3%) late deaths occurred with an 87.1% estimated overall survival at 5 years (early deaths included). Six late-onset intracardiac tunnel obstructions were noted and three of them required reoperation. Comparing the 2 groups, Group A patients have more late-onset (6 in Group A vs 0 in Group B, P = 0.026) and overall tunnel obstructions (10 in Group A vs 0 in Group B, P = 0.001). Concomitant tricuspid procedures significantly reduced intracardiac obstruction (0 in 16 vs 10 in 24, P = 0.003) without development of any tricuspid regurgitation and stenosis. CONCLUSIONS Using appropriate intracardiac tunnel establishment strategy and techniques, biventricular repair of DORVncVSD is feasible with encouraging outcomes. Concomitant tricuspid procedures can reduce intracardiac tunnel geometry without increase of mortality and morbidity.

Outcomes of coronary transfer for anomalous origin of the left coronary artery from the pulmonary artery

OBJECTIVES To determine outcomes of patients presenting with an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) who underwent coronary transfer, and to investigate the role of the left ventricular ejection fraction (LVEF) and preoperative myocardial viability as the predictors for incomplete LV functional recovery. METHODS From 2002 to 2012, 40 consecutive patients whose diagnosis was ALCAPA and who underwent coronary transfer were included. Seventeen patients (42.5%, 17/40) presented with LV dysfunction (Group I) while the other 23 (57.5%, 23/40) did not (Group II). All patients in Group I underwent myocardial perfusion/metabolism imaging and the global viability index was calculated. The mean follow-up time was 54 ± 37 months. RESULTS The median age at repair was 27.6 months (range, 3-66 months) and the mean preoperative LVEF was 49.0 ± 15.6%. Two extracorporeal membrane oxygenation support systems were required after correction. There were no in-hospital deaths and 1 late death. The mean LVEF was significantly improved to 61.0 ± 12.2 and 64.0 ± 11.2% at discharge and follow-up, respectively. Both the LVEF at discharge (52.7 ± 17.1% in Group I versus 66.1 ± 8.0% in Group II, P = 0.02) and the LVEF at the latest follow-up (55.2 ± 10.1% in Group I versus 67.6 ± 8.0% in Group II, P = 0.03) were lower in Group I than Group II. Moderate mitral regurgitations (MRs) were noted in 5 patients (12.8%, 5/39) at follow-up without difference between groups. Restrained functional recovery was observed in 6 patients (35.2%, 6/17) in Group I and the global viability index (P = 0.02) was identified as a predictor for incomplete LV functional recovery. CONCLUSIONS Coronary transfer is a technique of choice with favourable outcomes for this anomaly. In the majority of patients, both ventricular function and MR tend to be normalized over time. Myocardial viability may be an alternative predictor for restrained LV functional recovery.

The role of LV in the autograft complication after ROSS operation

We have read the interesting article from Luciani and colleagues1 documenting the outcomes into the second decade after the Ross procedure in infants and children from the Italian Paediatric Ross Registry. The conclusion concisely stated that the Ross procedure was a low-risk palliative procedure for aortic valve abnormalities at the expense of valve-related reoperation. Contrary to prior evidence, autograft reoperation was more common than homograft in their cohort, raising the concern about the autograft failure in paediatric population. …

ASSA13-17-5 The Effects of National Transfer Network System For Congenital Heart Disease Children in Early Intervention and Comprehensive Treatment

Objective To analysis the effects of early prognosis, safety and medical costs in congenital heart diseases (CHD) children transferred through the newly-built National Transfer Network System for Congenital Heart Disease Children (NTNS) to get emergency transferring. Methods NTNS was founded by the National Center for Cardiovascular Disease (NCCD), combined with the major medical centre, which is based on the on-line network and covers most areas of China. NTNS has formed a CHD transferring and collaborative network. This system is based on the most experienced medical resources in Beijing and regional hospitals. In the meanwhile, the CHD database was established to exchange the medical files, records and photos among the collaborative health care facilities, making medical suggestions and decisions of transferring for further urgent surgical procedures decided by on-line medical councils for the severely sick CHD children even in the most undeveloped provinces. After the major operations, the CHD children who need the continuous recovery are discussed by the multi-disciplinary team on-line and the children will be transferred the proper health care centres for further treatment. All of these form a comprehensive CHD therapy system combined with early diagnosis, fast cooperative medical councils, emergent transferring, early intervention and postoperative rehabilitation among the medical centres. Results NTNS has transferred 121 severely sick CHD children and run 143 distanced medical councils. These children’s median age was 10 months (5.6, 21.0) with youngest age at 4 days, the mean hospital stays of them was 12.9 ± 4.8 days, which is much shorter than the children with same diagnosis in the same period of time transferred traditional ways. However, the post-operative PICU stays (p = 0.58) and hospital cost (p = 0.17) were no statistical differences. The out-patient treatment cycles and out-patient cost of the children in NTNS-transferred group were 2.7 days and 78234.43RMB, which were less than those in the children in the traditional group. Conclusions The National Transfer Network System for Congenital Heart Disease Children has been founded as a safe and effective working platform, which provided the prenatal diagnosis, emergent transferring, early surgical intervention and post-operative rehabilitation. This system shortened the hospital stays, treatment cycles, medical cost with sharing the capital’s experienced medical resources. NTNS will continue to provide the experiences in health policies and emergent transferring, which will bring more good results to the CHD children.