BACKGROUND: Pulmonary artery hypertension associated with congenital heart disease (PAH-CHD) occurs predominantly among patients with uncorrected CHD. Treatment of severe pediatric PAH-CHD remains a major intractability. This study evaluated the predictors and prognoses of children with PAH-CHD who underwent surgical correction. METHODS: The data for 59 children with severe PAH-CHD who underwent surgical correction, with or without postoperative medication, between May 2011 and June 2015 at the Guangdong Provincial People's Hospital were analyzed retrospectively. A regression analysis, receiver-operating characteristic (ROC) curves, and Kaplan-Meier curves were used for survival analysis. RESULTS: Fifty-nine children with severe PAH-CHD underwent heart catheterization and correction, with or without specific anti-PAH drugs postoperatively, were included in this study. The pulmonary pressure, heart function, and ending events were observed and median observation period was 49 ± 20 months. Twenty-eight patients (50%) received at least one additional anti-PAH drug after correction. The survival rate after 2 years was 91.5% (54/59); two patients were in a critical condition, and three were lost to follow-up. Twelve patients (29%) still received over one additional PAH-specific therapy at follow-up, whereas 42 (75%) had successfully stopped drug treatment. Two patients (3.5%) died and one underwent a second thoracotomy to remove the ventricular septal defect patch. Acute vasoreactivity test (AVT) criteria had limited efficacy in predicting pediatric PAH-CHD, whereas pulmonary vascular resistance (PVR) ≤ 6.65 Wood units (WU)/m or PVR/systemic vascular resistance (SVR) ≤ 0.39 during AVT indicated a good prognosis after surgical correction with an AUC of 98.3% (95% confidence interval [CI]: 96.0-100%), 98.4% (95% CI: 96.0-100%) sensitivity of 100%, 100% and specificity of 82.1%, 92.9%, respectively. CONCLUSIONS Although the criteria for positive AVT currently used are unsuitable for pediatric patients with PAH-CHD, PVR and PVR/SVR during AVT are excellent predictors of outcome in pediatric PAH-CHD. Surgery aided by anti-PAH drugs is an effective strategy and should be recommended for severe pediatric PAH-CHD with PVR ≤ 6.65 WU/m and PVR/SVR ≤ 0.39 after iloprost aerosol inhalation.
Adolescent ; Child ; Child, Preschool ; Echocardiography ; Female ; Heart Defects, Congenital ; pathology ; surgery ; Humans ; Hypertension, Pulmonary ; pathology ; surgery ; Kaplan-Meier Estimate ; Male ; Prognosis ; ROC Curve ; Retrospective Studies ; Risk Factors

To investigate the incidence of postoperative abnormal liver function test (aLFT) for the children with heart surgery, and to analyze the clinical characteristics and risk factors.
 Methods: A total of 143 children younger than 18 years old who underwent heart surgery in 2017 were enrolled in this study. The liver function were examined one day preoperation and consecutive 5 days after operation. The clinical data of perioperative period were recorded and the risk factors for aLFT were analyzed.
 Results: There were 43/143 (30%) cases had aLFT, including 5/143 (3.5%) acute liver injury (ALI). In the 6 liver function tests, total bilirubin, and glutamic-oxalacetic aminotransferase and glutamic-pyruvic aminotransferase increased to the peak at the first day and the second day after operation, respectively, and albumin declined to the lowest level at the fourth day after operation. aLFT happened most common at the first day after operation(22/43, 51.2%). The patients in the aLFT(+) group had smaller body size, more proportion of the risk adjustment for congenital heart sugery-1 (RACHS-1) score ≥ 3 and cyanosis, longer cardiopulmonary bypass (CPB) time, higher postoperative cardiac troponin (cTnI) value, higher inotropic score (IS), more transfusion, and longer mechanical ventilation time than those in the aLFT(-) group. The aLFT(+) group had longer ICU and hospital time, higher morbidity and mortality than those in the aLFT(-) group (P<0.05). Logistic regression showed that RACHS-1≥3, cyanosis, CPB time, cTnI, IS, transfusion, and mechanical ventilation time were the risk factors for aLFT. Multiple factor analysis showed the mechanical ventilation time was an independent risk factor for aLFT (P<0.05).
 Conclusion: aLFT is common in children after congenital heart operation, which could deteriorate to poor outcome. The mechanical ventilation time is an independent risk factor for aLFT.
Adolescent ; Cardiac Surgical Procedures ; Child ; Child, Preschool ; Heart Defects, Congenital ; surgery ; Humans ; Liver ; injuries ; pathology ; Logistic Models ; Postoperative Complications ; Respiration, Artificial ; Retrospective Studies ; Risk Factors ; Treatment Outcome

Tetralogy of Fallot (TOF) is one of the most common forms of cyanotic congenital heart disease usually managed by serial surgical repairs. The repaired prosthetic valve or conduit is susceptible to life-threatening infection. FDG-PET is an effective alternative to evaluate the source of infection when other examinations are inconclusive. We report an unusual case of an infected pulmonary artery conduit after TOF repair although the echocardiogram was negative for vegetation, which was later confirmed by surgery and pathology. The case highlights the role of FDG-PETas a problem-solving tool for potential endocarditis and cardiac device infection cases after complex cardiac surgery.
Endocarditis ; Heart Defects, Congenital ; Pathology ; Pulmonary Artery ; Tetralogy of Fallot ; Thoracic Surgery

Fontan surgery is a widely used palliative procedure that significantly improves the survival period of patients with complex congenital heart disease (CHD). However, it does not decrease postoperative complication rate. Previous studies suggested that elevated mean pulmonary artery pressure (mPAP) and vascular resistance lead to decreased exercise tolerance and myocardial dysfunction. Therapy with endothelial receptor antagonists (Bosentan) has been demonstrated to improve the patients' prognosis. A double-blind, randomized controlled trial was performed to explore the efficacy of Bosentan in treating patients who underwent the Fontan procedure. Eligible participants were randomly divided into Bosentan group and control group. Liver function was tested at a local hospital and the results were reported to the phone inspector every month. If the results suggested abnormal liver function, treatment would be adjusted or terminated. All the participants finished the follow-up study, with no patients lost to follow-up. Unblinding after 2-year follow-up, no mortality was observed in either group. However, secondary end-points were found to be significantly different in the comparable groups. The cardiac function and 6-min walking distance in the Bosentan group were significantly superior to those in the control group (P=0.018 and P=0.027). Bosentan could improve New York Heart Association (NYHA) functional status and improve the results of the 6-min walking test (6MWT) in Fontan patients post-surgery, and no other benefits were observed. Furthermore, a primary meta-analysis study systematically reviewed all the similar clinical trails worldwide and concluded an overall NYHA class improvement in Fontan patients who received Bosentan treatments.
Adolescent ; Child ; Double-Blind Method ; Female ; Follow-Up Studies ; Fontan Procedure ; Heart Defects, Congenital ; drug therapy ; pathology ; surgery ; Humans ; Liver ; drug effects ; pathology ; Male ; Palliative Care ; Prognosis ; Sulfonamides ; administration & dosage ; Treatment Outcome

OBJECTIVETo discuss the experience of surgical treatment of total anomalous pulmonary venous connection (TAPVC) in infants.

METHODSThe clinic data of 84 cases with TAPVC under 6 months of age underwent surgical treatment at Department of Cardiac Surgery, Guangzhou Women and Children's Medical Center from January 2012 to October 2015 were analyzed retrospectively. There were 58 male and 26 female patients. The patients were aged 1 days to 6 months with a mean of (2.4±2.2) months at surgery, including 22 newborns. Body weight was 1.8 to 6.8 kg with a mean of (4.3±1.2) kg. There were 24 cases of intracardiac type, 46 cases of supracardiac type, 10 cases of infracardiac type and 4 cases of mixed type. There were 26 cases received emergent operation. There were 14 cases used Sutureless technique in operations and 46 cases used conventional methods in the no-intracardiac type cases, and 2 cases enlarged the anastomsis with autologous pericardium. According to the condition, corrective surgeries of other anomalies were performed in the meantime, including 3 Warden operations (right side), 3 bilateral bidirectional Gleen operation, 2 correction of unroofed coronary sinus syndrome, 1 coarctation of aorta correction with deep hypothermic circulation arrest, and 1 repair of ventricular septal defect.

RESULTSThe ratio of newborn was higher in Sutureless technique group than in conventional methods group (7/14 vs. 32.6%, χ(2)=4.927, P=0.043), and mean age was less ((1.8±0.4) months vs. (2.4±2.2) months, F=4.257, P=0.042), but there were no difference in body weight, cardiopulmonary bypass time and aorta clamped time between the two groups. Followed up for 1 to 46 months, 10 cases (11.9%) died overall and the mortality of intracardiac (3/10) and mixed (2/4) type were much higher than in intracardiac (4.2%) and supracardiac (13.0%) type. The mortality were no difference between newborn and infants, or whether emergent operation, or Sutureless technique and conventional methods. The maximal pulmonary venous flow velocity was abnormal speed-up >1.8 m/s at 1 week and 1 to 3 months post-operation mostly.

CONCLUSIONSThe mortality of TAPVC was differed by different types. Intrinsic pulmonary vein stenosis maybe the main cause of mortality. The high quality of anastomsis could reduce the operative mortality.

Aortic Coarctation ; Body Weight ; Cardiac Surgical Procedures ; methods ; Circulatory Arrest, Deep Hypothermia Induced ; Female ; Heart Defects, Congenital ; mortality ; surgery ; Heart Septal Defects, Ventricular ; Humans ; Infant ; Infant, Newborn ; Male ; Postoperative Period ; Pulmonary Veins ; pathology ; surgery ; Retrospective Studies

The management of pulmonary atresia with intact ventricular septum (PA/IVS) remains controversial. The goal of separating systematic and pulmonary circulation can be achieved by biventricular or uni-ventricular (Fontan or one and a half ventricle repair) strategies. Although outcomes have been improved, these surgical procedures are still associated with high mortality and morbidity. An optimal strategy for definitive repair has yet to be defined. We searched databases for genetically randomized controlled trials (RCTs) comparing biventricular with uni-ventricular repair for patient with PA/IVS. Data extraction and quality assessment were performed following the guidelines of the Cochrane Collaboration. Primary outcome measures were overall survival, and secondary criteria included exercise function, arrhythmia-free survival and treatment-related mortality. A total number of 669 primary citations were screened for relevant studies. Detailed analysis revealed that no RCTs were found to adequately address the research question and no systematic meta-analysis would have been carried out. Nevertheless, several retrospective analyses and case series addressed the question of finding right balance between biventricular and uni-ventricular repair for patient with PA/IVS. In this review, we will discuss the currently available data.
Arrhythmias, Cardiac ; physiopathology ; prevention & control ; Cardiac Valve Annuloplasty ; methods ; mortality ; Fontan Procedure ; methods ; mortality ; Heart Defects, Congenital ; mortality ; pathology ; surgery ; Heart Ventricles ; abnormalities ; pathology ; surgery ; Humans ; Pulmonary Atresia ; mortality ; pathology ; surgery ; Retrospective Studies ; Survival Analysis ; Treatment Outcome

OBJECTIVEBranch pulmonary artery stenosis is one of the common congenital heart disease. Stent implantation to relieve branch pulmonary artery stenosis (BPAS) is an alternative to failed surgical or balloon angioplasty. The aim of this study was to explore the indication, methods and complications of using balloon expandable stent placement to treat branch pulmonary artery stenosis, and evaluate the results of stent implantation in the treatment of branch pulmonary artery stenosis.

METHODFrom August 2005 to December 2012, 19 patients underwent an attempt at stent implantation. The median age of those patients was 9.1 years (range 4.0-15.0 years). The median weight was 31.7 kg (range 17.0-60.5 kg); 14/19 patients underwent post surgical repair of tetralogy of Fallot, one patient received post surgical repair of pulmonary atresia with ventricular septal defect, one patient underwent post surgical repair of pulmonary atresia with intact septum, one with native left BPAS, and one was after surgical repair of aortopulmonary window and the other truncus arteriosus. CP stent and NuMED Balloon-in-Balloon catheter were selected according to digital subtracted angiography measurements. After checking for correct position by angiography, the inner balloon and outer balloon was inflated successively to expand the stent to desired diameter. Statistical analysis was performed with the unpaired Student t test.

RESULTA total of 26 stents were implanted successfully in 19 patients. The systolic gradient across the stenosis fell from a median of (36.0 ± 18.3) to (3.8 ± 3.4) mmHg (P < 0.01, 1 mmHg = 0.133 kPa) and the diameter of the narrowest segment improved from (6.0 ± 1.9) to (11.6 ± 3.1) mm (P < 0.01). The right ventricle to aortic pressure ratio fell from 0.68 to 0.49 (P < 0.01). Complications included the following: two stents were malpositioned in the right ventricular outflow tract and one balloon ruptured when dilated the hole of the stent. No other complications occurred. All patients were followed up for 6 months to 6.0 (2.5 ± 1.8) years. One patient underwent stent re-dilation in order to accommodate somatic growth two years later.

CONCLUSIONBalloon expandable stents are safe and effective in relieving BPAS. Stent implantation should be considered the treatment of choice for most patients with BPAS. Stents placed into growing children will require further dilation to keep up with normal somatic growth. Intermediate and long-term follow up studies have shown excellent results after further dilation over time.

Adolescent ; Angioplasty, Balloon ; methods ; Blood Vessel Prosthesis Implantation ; Child ; Child, Preschool ; Constriction, Pathologic ; surgery ; Female ; Heart Defects, Congenital ; complications ; surgery ; Humans ; Male ; Postoperative Complications ; epidemiology ; surgery ; Pulmonary Artery ; pathology ; surgery ; Pulmonary Valve Stenosis ; etiology ; surgery ; Retrospective Studies ; Stents ; Treatment Outcome ; Vascular Surgical Procedures ; methods

Regardless of the preoperative morphology and the type of operation, left ventricular outflow tract obstruction (LVOTO) after biventricular repair of double outlet right ventricle (DORV) may develop. This report presents our 10-yr experience with surgical management of LVOTO after biventricular repair of DORV. Between 1996 and 2006, 15 patients underwent reoperation for subaortic stenosis after biventricular repair of DORV. The mean age at biventricular repair was 23.3+/-18.3 months (1.1-64.2). Biventricular repairs included tunnel constructions from the left ventricle to the aorta in 14 cases and an arterial switch operation in one. The mean left ventricle-to-aorta peak pressure gradient was 54.0+/-37.7 mmHg (15-140) after a mean follow-up of 9.5+/-6.3 yr. We performed extended septoplasty in nine patients and fibromuscular resection in six. There were no early or late mortality. There was one heart block and one aortic valve injury after an extended septoplasty, and two and one after a fibromuscular resection. No patient required reoperation for recurrent subaortic stenosis. The mean pressure gradient was 11.2+/-11.4 mmHg (0-34) after a mean follow-up of 5.6+/-2.7 yr. Extended septoplasty is a safe and effective method for the treatment of subaortic stenosis, especially in cases with a long-tunnel shaped LVOTO.
Blood Pressure/physiology ; Child, Preschool ; Double Outlet Right Ventricle/pathology/*surgery ; Female ; Heart Defects, Congenital/pathology/surgery ; Humans ; Infant ; Male ; Postoperative Complications/*surgery ; Reoperation ; Retrospective Studies ; Treatment Outcome ; *Ventricular Outflow Obstruction/etiology/surgery

BACKGROUNDThe present study was aimed to compare the effects of bilateral and unilateral bidirectional superior cavopulmonary shunt (b-BDG and u-BDG) on pulmonary artery growth and clinical outcomes.

METHODSThe 51 subjects enrolled in this study were divided into two groups: those receiving b-BDG (n = 21) and those receiving u-BDG (n = 30). Clinical records were reviewed retrospectively at a mean of 43.3 months after BDG procedures. Chi square and t-tests were performed to analyze the data.

RESULTSLeft and right pulmonary artery diameters increased 27% - 37% in both groups. The pulmonary artery index increased 37.2% after b-BDG and 27.0% after u-BDG. b-BDG patients experienced a significant decrease in mean hemoglobin concentration and hematocrit value, and a correlated change in postoperative diameter of left pulmonary artery (LPA) and pulmonary artery index (y = 0.2719, x = -1.8278; R = 0.564, P = 0.008). The change ratio of hemoglobin and postoperative LPA were also correlated in b-BDG patients (y = -0.0522x + 0.3539; R = -0.479, P = 0.028). Only one b-BDG patient versus twelve u-BDG patients needed total cavopulmonary connections 31.8 months after BDG surgery (P = 0.0074). Moreover, only one (4.8%) b-BDG patient but eight u-BDG patients (26.7%) developed pulmonary arteriovenous malformations.

CONCLUSIONSb-BDG increases bilateral pulmonary blood flow and promotes growth of bilateral pulmonary arteries, with preferable physiological outcomes to u-BDG. Results may imply that subsequent Fontan repair may not always be needed.

Cardiopulmonary Bypass ; methods ; Child ; Child, Preschool ; Female ; Fontan Procedure ; methods ; Heart Defects, Congenital ; physiopathology ; surgery ; Humans ; Infant ; Lung ; pathology ; surgery ; Male ; Pulmonary Circulation ; Retrospective Studies ; Treatment Outcome

Adolescent ; Aortic Valve ; abnormalities ; Aortic Valve Insufficiency ; complications ; pathology ; surgery ; Aortic Valve Stenosis ; complications ; pathology ; surgery ; Child ; Endocarditis ; complications ; pathology ; surgery ; Female ; Heart Defects, Congenital ; complications ; pathology ; surgery ; Heart Valve Prosthesis Implantation ; Humans ; Male ; Rheumatic Heart Disease ; complications ; pathology ; surgery