Zhongdong Hua

Hybrid Therapy for Pulmonary Atresia With Intact Ventricular Septum

BACKGROUND In patients with pulmonary atresia with intact ventricular septum without right ventricular-dependent coronary circulation, catheter techniques, including the use of a stiff wire, lasers, and radiofrequency, have been most widely used for initial therapy; however, percutaneous perforation and balloon valvuloplasty have been associated with higher rates of procedural failure and serious complications. We report our experience with a hybrid approach involving the combination of surgery and interventional catheterization techniques for right ventricular decompression. METHODS Between March 2005 and April 2010, we performed a hybrid procedure in 30 newborns and infants (age, 1 day to 2 years; median age, 3 months) with favorable anatomy. The heart was exposed by performing midline sternotomy. A purse-string suture was placed in the right ventricular outflow tract 2 cm away from the pulmonary trunk. Then, a 16-gauge intravenous catheter was placed periventricular with the guidance of echocardiography to perforate the atretic pulmonary ventricle. Subsequently, a guide wire was inserted into the sheath and used to guide a balloon across the pulmonary ventricle. Sequential dilations were performed under the guidance of epicardial echocardiography until full opening of the pulmonary ventricle was obtained. In newborns, ductal ligation was performed, followed by modified Blalock-Taussig (mBT) shunt placement. Patients aged greater than 1 month were treated mostly with ductal ligation, and an mBT shunt was inserted if severe systemic oxygen desaturation occurred after ductal ligation; bidirectional Glenn shunt placement was performed if a patient showed severe hypoplasia. RESULTS The hybrid procedure was successfully performed in all patients. Patent ductus arteriosus ligation was simultaneously performed in 27 cases. Six newborns were treated with mBT shunt placement after pulmonary valvuloplasty and patent ductus arteriosus ligation, and 2 patients aged greater than 1 month were treated with mBT shunt placement alone. Another 2 patients were selected for univentricular palliation with bidirectional Glenn procedure because of a diminutive monopartite right ventricle. No pericardial effusion or cardiac tamponade was observed. One patient in whom ductal ligation could not be performed was treated with mBT shunt placement because of hypoxemia 3 days after the hybrid procedure; the other patients were discharged without any further surgical intervention. During the follow-up period of 1.5 months to 5 years, 5 patients died; 25 (83.3%) survived and all were in New York Heart Association functional class 1. Saturation of peripheral oxygen in the survivors increased from 73.1±8.5% to 94±3.5% (p<001). Two-ventricle circulation was achieved in 24 patients, whereas 1 patient had a single-ventricle pathway. CONCLUSIONS In conclusion, periventricular balloon pulmonary valvuloplasty using a hybrid approach was safe and feasible for patients with pulmonary atresia with intact ventricular septum.

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.

Outcomes of the rehabilitative procedure for patients with pulmonary atresia, ventricular septal defect and hypoplastic pulmonary arteries beyond the infant period

OBJECTIVES To determine the effect and safeness of the right ventricle to pulmonary artery connection with occlusion of major aortopulmonary collaterals and pulmonary artery angioplasty to rehabilitate the hypoplastic pulmonary arteries in patients with pulmonary atresia and ventricular septal defect beyond the infant period. METHODS From December 2009 to August 2012, 37 consecutive patients (mean age 1.9 ± 1.7 years, range 0.6-7.2 years) diagnosed with pulmonary atresia, ventricular septal defect and pulmonary artery hypoplasia (Nakata index 90.9 ± 42.6 mm(2)/m(2); McGoon ratio 1.0 ± 0.2) were included in this retrospective study. All patients underwent the procedure of right ventricle to pulmonary artery connection, during which most of them received transcatheter occlusion of major aortopulmonary collaterals and/or pulmonary angioplasty. Mean follow-up was 1.6 ± 0.8 years (range 0.6-3.3 years). Continuous variables are expressed as means ± standard deviation. RESULTS There were no early deaths, but one patient died of myocarditis 1 year after the rehabilitation. Significant pulmonary artery growth was obtained (Nakata index 215.1 ± 95.1 mm(2)/m(2), P < 0.001; McGoon ratio 1.6 ± 0.5, P < 0.001) in all of the 37 patients, and among them, 17 patients (45.9%) whose pulmonary growth was considered adequate obtained a complete repair without perforation of the ventricular septal defect. The preoperative McGoon ratio might be a good predictor for adequate pulmonary growth. There was one early death after anatomical repair. At the last visit, all survivors who underwent anatomical repair were in New York Heart Association class I-II with satisfactory haemodynamics. CONCLUSIONS Connection of the right ventricle to the pulmonary artery is safe and effective to promote the growth of the native pulmonary arteries in patients with pulmonary atresia, ventricular septal defect and hypoplastic native pulmonary arteries. Ultimately, this strategy allows complete repair in the majority of patients beyond infancy.

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.

Palliative pulmonary artery banding versus anatomic correction for congenitally corrected transposition of the great arteries with regressed morphologic left ventricle: Long-term results from a single center

OBJECTIVE We aimed to compare the long-term results between palliative pulmonary artery banding and anatomic correction for congenitally corrected transposition of the great arteries with regressed morphologic left ventricle. METHODS From 2003 to 2012, 40 consecutive patients underwent first-stage pulmonary artery banding. The second-stage operation-double switch-was performed in 15 patients (double-switch group). The other 25 patients retained pulmonary artery banding without further operation (pulmonary artery banding group). In-hospital mortality, long-term mortality, and heart function were studied as primary outcomes. RESULTS The median time of follow-up was 3.4±0.7 years (range, 6 months-9.5 years). Overall survival rate was 66.7% in the double-switch group versus 96.0% in the pulmonary artery banding group (P=.03). The ratio of New York Heart Association functional class I-II (80.0% vs 95.9%; P=.02) and the mean functional left ventricle ejection fraction (51.4%±9.6% vs 61.0%±6.4%; P=.01) were higher in the pulmonary artery banding group at follow-up. In univariate analysis, age at pulmonary artery banding was the only risk factor for late deaths (odds ratio, 7.30; P=.01) and left ventricle dysfunction (odds ratio, 4.77; P=.03) after the double switch. For patients who experienced prolonged pulmonary artery banding, mean oxygen saturation was 95%±3.1% and the trans-banding pressure gradient was 46.9±21.5 mm Hg. CONCLUSIONS In patients with congenitally corrected transposition of the great arteries with deconditioned morphologic left ventricle pulmonary artery banding may be considered an ideal procedure because it allows left ventricle training while improving tricuspid regurgitation. Compared with the double-switch procedure after pulmonary artery banding, prolonged palliative pulmonary artery banding provided a lower mortality rate and indicated better cardiac function.

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.

Uncontrolled Antegrade Pulmonary Blood Flow and Delayed Fontan Completion After the Bidirectional Glenn Procedure: Real-World Outcomes in China

BACKGROUND Given the low rate of Fontan completion, an aggressive policy for maintaining antegrade pulmonary blood flow (AnPBF) during the bidirectional Glenn procedure (BDG) was developed for the functional single ventricle. METHODS From 2008 to 2013, 294 patients who underwent the BDG were divided into two groups: group 1 (uncontrolled AnPBF, n = 270) and group 2 (controlled AnPBF, n = 24). Pulmonary artery banding was performed because of the high central venous pressure in group 2. In group 1, the patients who underwent BDG from 2008 to 2012 were further divided into group DF (delayed Fontan completion, n = 109) and group FC (Fontan completion, n = 42). RESULTS The Fontan completion rate was 16.3%, and the average interval time was 2.2 ± 1.1 years. The delay of Fontan completion did not reduce body weight gain or the survival rate. Furthermore, oxygen saturation was slightly reduced in group DF. Although no impairments of heart function were observed, the uncontrolled AnPBF in group DF resulted in an increase in ventricular end-diastolic diameter and aggravation of atrioventricular valve regurgitation over 24 months after BDG. Logistic regression analysis revealed that systemic right ventricular morphology was a risk factor for the aggravation of valve regurgitation. CONCLUSIONS The low Fontan achievement rate is a critical issue in China. Although the patients with delayed Fontan completion exhibited an acceptable survival rate and acceptable body weight gain, uncontrolled AnPBF was associated with ventricular enlargement and aggravation of valve regurgitation. Strategies for improving the Fontan completion rate in China should be explored and could benefit outcomes.

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.