陈求名
中国医学科学院阜外医院 小儿外科
Background Although right ventricular ( RV ) volume was significantly decreased in symptomatic patients with repaired tetralogy of Fallot ( rTOF ) after pulmonary valve replacement ( PVR ), RV size was still enlarged along with RV dysfunction. Methods and Results A prospective case-control study was conducted in a tertiary hospital; 81 asymptomatic repaired tetralogy of Fallot patients with moderate or severe pulmonary regurgitation were enrolled. The enrolled cohort was divided into 2 groups: PVR group (n=41) and medication group (n=40). Cardiac magnetic resonance, transthoracic echocardiography, and electrocardiography were scheduled after recruitment and 6 months after PVR or recruitment. Adverse events were recorded during follow-up. Three deaths, 1 heart transplantation, 3 PVR s, and 2 symptomatic heart failures in medication group and 1 redo PVR in the PVR group were observed during follow-up. Compared with the medication group, the PVR group had significantly lower adverse events rate ( P=0.023; odds ratio, 0.086; 95% CI, 0.010-0.716), and RV function was significantly improved ( P<0.05). Binary logistic regression analysis identified preoperative RV end-systolic volume index (10-mL/m2 increment, P=0.009; odds ratio, 0.64; 95% CI, 0.457-0.893) was an independent predictor of normalization of RV size after PVR . A preoperative RV end-systolic volume index cut-off value of 120 mL/m2 (area under curve, 0.819; sensitivity, 90.3%; specificity, 70%) was analyzed by receiver operating characteristic curves for normalized RV size after PVR . Conclusions PVR in asymptomatic repaired tetralogy of Fallot patients is appropriate and effective in reducing right ventricular size and preserving right ventricular function. The recommended criterion of RV end-systolic volume index for PVR is 120 mL/m2.
Journal of the American Heart Association 2019
Complex cardiac anomalies are sometimes channeled toward Fontan palliation for various reasons. Nevertheless, anatomical repair after bidirectional cavopulmonary shunt may be another option with theoretical benefits. In this study, we report our experience with anatomical repair conversion in challenging patients who had been palliated with bidirectional cavopulmonary shunt. Retrospective review was conducted in patients who underwent anatomical repair conversion from prior bidirectional cavopulmonary shunt palliation between January 2008 and March 2016. Patients who underwent a planned staged 1½-ventricular repair were excluded. Twenty-three patients underwent anatomical repair conversion at a median age of 6.5 years (range 2.7-20.0 years). The interval time between palliation and conversion was 4.6 ± 2.4 years (range 0.9-12.4). Indications for conversion were high-risk Fontan candidates (n = 11) and preference for biventricular anatomy (n = 12). In eight of the patients, bidirectional cavopulmonary shunts were taken down and superior vena cava was reconnected to the right atrium with Gore-Tex tube or bovine jugular venous tube. Mean cardiopulmonary bypass and aortic cross-clamp times were 225.6 ± 107.0 and 138.3 ± 76.6 min, respectively. After a mean follow-up of 2.7 ± 2.2 years, there was no mortality and reoperation. No patients presented sinoatrial node dysfunction and superior venous cave stenosis. All the patients were in the New York Heart Association functional class I or II. Patients with previous bidirectional cavopulmonary shunt should be re-evaluated before completion of Fontan and, if cardiac anatomy allows, anatomical repair conversion may be considered, especially in patients with high-risk Fontan completion. Initial bidirectional cavopulmonary shunt palliation should not be considered as a one-way path to Fontan. Although technically challenging, early- and mid-term clinical results of anatomical repair conversion were satisfactory.
Pediatric cardiology 2018
OBJECTIVES:The hemi-Mustard and bidirectional Glenn (BDG) procedures combined with the Rastelli procedure have been applied to selected cases of congenitally corrected transposition of the great arteries (ccTGA) for potential benefit over the classic atrial switch procedure. The aim of this study was to analyse our experience with the hemi-Mustard, BDG and Rastelli procedures as an anatomical correction for patients with ccTGA/left ventricular outflow tract obstruction (LVOTO) with positional heart anomalies.METHODS:In this retrospective study, 31 consecutive patients with corrected transposition underwent the hemi-Mustard/BDG procedures with the Rastelli operation between 2011 and 2015. The median age was 5.4 (range: 0.75-12) years. Positional anomalies were present in all patients. Eleven patients underwent BDG initially; they then had the second-stage hemi-Mustard and Rastelli procedures; 'one-stage repair' (hemi-Mustard/BDG and the Rastelli procedures) was performed in 20 cases.RESULTS:There were no in-hospital deaths, and 3 patients received a pacemaker. One patient had an atrial baffle obstruction that was observed in the early postoperative period; 7 patients had prolonged pleural effusions that developed more frequently in the one-stage repair group (7/20 vs 0/11, P = 0.033). During the mean follow-up of 3.3 years, 1 late death was noted, and no conduit replacements were required. Twenty-five (83.3%) patients are in New York Heart Association classes I and II at the latest follow-up.CONCLUSIONS:Hemi-Mustard, BDG and the Rastelli procedures are technically feasible for correction of ccTGA/left ventricular outflow tract obstruction (LVOTO) and cardiac malposition. Nevertheless, postoperative pleural effusion is the most prevalent complication in the one-stage repair. Two-stage repair may reduce the risk of pleural effusion-related complications.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 2017
BACKGROUND:One important determinant of longevity in congenital heart disease is right ventricular (RV) function, and this is especially true in cyanotic congenital heart disease. However, there is a paucity of data concerning right ventricular remodeling (RVR) in the setting of chronic hypoxia. Dimethyloxalylglycine (DMOG) is a competitive inhibitor of hypoxia-inducible factor (HIF)-hydroxylated prolyl hydroxylase and has been shown to play an important role against ischemia-reperfusion myocardial injury.METHODS:We tested the hypothesis that DMOG prevents the development RVR after chronic hypoxia exposure. Rats were injected with saline or DMOG and exposed to room air or continued hypoxia for 4 weeks. In addition, we explored the response of myocardial erythropoietin and its receptor to hypoxic exposure.RESULTS:Treatment with DMOG attenuated myocardial fibrosis, apoptosis, and oxidative stress, which lead to enhanced RV contractile function. As an endpoint of HIF-dependent cardioprotection, a novel pathway in which nuclear factor kappa B links HIF-1 transcription was defined.CONCLUSIONS:This study supports a role for HIF-1 stabilizers in the treatment of RVR and brings into question the commonly held concept that RVR follows a linear relationship with increased RV afterload.
Journal of cardiovascular pharmacology 2016
BACKGROUND:Report results of neoaortic regurgitation (NAR) after arterial switch for patients with d-transposition of the great arteries (d-TGA) and corrected transposition of the great arteries.METHODS:From 2003 to 2013, 583 patients who underwent arterial switch operation for d-TGA and 31 patients who underwent double switch (DS) for congenitally corrected transposition of the great arteries (cc-TGA) were included in this retrospective study. Since 2011, concomitant neoaortic sinotubular junction reconstruction was performed if aorta and pulmonary artery discrepancy was present in patients with d-TGA.RESULTS:The long-term survival rate was 92.5% (544/583) in patients with d-TGA and 74.2% (23/31) in patients with cc-TGA. More NAR developed in patients with cc-TGA than with d-TGA. Moreover, significant NAR (7.1% [38/539] versus 26.1% [(6/23], p = 0.010) and the aortic valve replacement (0.6% [3/539] versus 8.7% [2/23], p = 0.003) were less in the d-TGA group. Previous pulmonary artery banding and aortic and pulmonary artery diameter discrepancy were identified as risk factors for significant NAR in patients with d-TGA. However, no specific risk factors were identified in patients with cc-TGA. With the application of neoaortic sinotubular junction reconstruction, no significant NAR was recorded in patients with aortic and pulmonary artery discrepancy.CONCLUSIONS:After an arterial switch operation, we report a favorable incidence of NAR and rare neoaortic valve replacement. Significant NAR was associated with aorta-pulmonary discrepancy and previous pulmonary artery banding. Patients with cc-TGA may require close monitoring. Patients with aortic and pulmonary artery diameter discrepancy may benefit from sinotubular junction reconstruction.
The Annals of thoracic surgery 2016
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.
PloS one 2016
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.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 2016
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.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 2015
OBJECTIVE: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.METHODS:Using microRNAs microarray, we profiled the microRNAs in the lung specimen from 12 congenital heart disease patients, (6 with pulmonary arterial hypertension and the others without). We validated the microRNAs expression using RT-PCR experiments. Then, we predicted the target genes of the promising microRNAs by bioinformatical analysis and verified its regulating role by luciferase assay and western blot experiments.RESULTS:All the 12 patients were uneventfully recovered from cardiac surgery. Comparing to the non-pulmonary arterial hypertension lung tissue, 62 microRNAs were significantly up-regulated and 12 were significantly de-regulated in the pulmonary arterial hypertension lung tissue. Among them 27 microRNAs reached P values ≤ 0.05, we validated the up-regulation of microRNA-27b by RT-PCR experiments and found the expression level of microRNA-27b was correlated with preoperative mean pulmonary arterial pressure. In vitro, overexpression of microRNA-27b decreased the protein expression of NOTCH1 and significantly reduced luciferase activity.CONCLUSIONS:The current study revealed for the first time that microRNAs may be important regulators in pulmonary arterial hypertension secondary to congenital heart disease, and demonstrated the correlation between microRNA-27b and pulmonary arterial hypertension with the implication of NOTCH1.
Pediatric pulmonology 2015
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.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 2015
