BACKGROUND: Congenital heart disease (CHD) is a leading cause of birth defect-related mortality. However, more recent CHD mortality data for China are lacking. Additionally, limited studies have evaluated sex, rural-urban, and region-specific disparities of CHD mortality in China. METHODS: We designed a population-based study using data from the Dataset of National Mortality Surveillance in China between 2008 and 2021. We calculated age-adjusted CHD mortality using the sixth census data of China in 2010 as the standard population. We assessed the temporal trends in CHD mortality by age, sex, area, and region from 2008 to 2021 using the joinpoint regression model. RESULTS: From 2008 to 2021, 33,534 deaths were attributed to CHD. The period witnessed a two-fold decrease in the age-adjusted CHD mortality from 1.61 to 0.76 per 100,000 persons (average annual percent change [AAPC] = -5.90%). Females tended to have lower age-adjusted CHD mortality than males, but with a similar decline rate from 2008 to 2021 (females: AAPC = -6.15%; males: AAPC = -5.84%). Similar AAPC values were observed among people living in urban (AAPC = -6.64%) and rural (AAPC = -6.12%) areas. Eastern regions experienced a more pronounced decrease in the age-adjusted CHD mortality (AAPC = -7.86%) than central (AAPC = -5.83%) and western regions (AAPC = -3.71%) between 2008 and 2021. Approximately half of the deaths (46.19%) due to CHD occurred during infancy. The CHD mortality rates in 2021 were lower than those in 2008 for people aged 0-39 years, with the largest decrease observed among children aged 1-4 years (AAPC = -8.26%), followed by infants (AAPC = -7.01%). CONCLUSIONS CHD mortality in China has dramatically decreased from 2008 to 2021. The slower decrease in CHD mortality in the central and western regions than in the eastern regions suggested that public health policymakers should pay more attention to health resources and health education for central and western regions.
Humans ; Heart Defects, Congenital/mortality* ; Male ; Female ; China/epidemiology* ; Infant ; Child, Preschool ; Adult ; Child ; Adolescent ; Infant, Newborn ; Middle Aged ; Young Adult ; Aged ; Rural Population

OBJECTIVES: To provide reference basis for reducing the mortality for children under 5 years old and promote the healthy development, the mortality for children under 5 years old and the main causes for death in Liuyang City from 2013 to 2020 are analyzed. METHODS: The data of 725 cases of death for children under 5 years old in Liuyang City from 2013 to 2020 were collected.The causes and difference of death among the children were analyzed retrospectively by descriptive statistic methods. RESULTS: There were a total of 144 516 live births in Liuyang City from 2013 to 2020. The mortality for children under 5 years old was 5.01‰, for infants was 3.39‰, and for newborns was 1.63‰. The male child mortality was 5.28‰, and the female child mortality rate was 4.72‰, with significant difference (P>0.05). The mortality for children under 5 years old was seasonal fluctuation, without significant difference among seasons (P>0.05). For the past 5 years, the top 3 causes for death among children under 5 years old were preterm birth and low birth weight, congenital heart disease, and pneumonia. Before death, 341 cases (47.04%) were treated in provincial hospitals, 198 cases (27.31%) in county-level hospitals, 56 cases (7.72%) in village-level hospitals, and 130 cases (17.93%) were not treated. CONCLUSIONS The mortality for children under 5 years old in Liuyang City is gradually reduced in the past 5 years. The main causes for death are premature birth and low birth weight, congenital heart disease and pneumonia. We should develop healthy education, improve the rate of prenatal diagnosis, promote the construction of obstetrics and paediatrics, and fundamentally reduce the mortality for children under 5 years old.
Cause of Death ; Child ; Child Mortality ; Child, Preschool ; Female ; Heart Defects, Congenital ; Humans ; Infant ; Infant Mortality ; Infant, Newborn ; Male ; Pneumonia/epidemiology* ; Pregnancy ; Premature Birth ; Retrospective Studies

OBJECTIVE: To investigate the morbidity of congenital heart defects(CHDs) in children with anorectal malformation, and to summarize appropriate treatment. METHODS: The clinical data and echocardiographic findings of 155 children with congenital anorectal malformations from the Third Affiliated Hospital of Zhengzhou University during January 2016 and October 2019 were reviewed. According to the surgical findings of anorectal malformations, the patients were categorized as the high/intermediate group and the low group; the CHDs were classified as minor CHDs and major CHDs. Multiple logistic regression was used to analyze the correlation of wingspread classification, and extracardiac malformations with the severity of CHDs. RESULTS: Out of 155 children with anorectal malformations, 47 (30.3%) had different types of cardiac structural malformations, including 18 cases of minor CHDs (11.6%) and 29 cases of major CHDs (18.7%). Sixty children (38.7%) had extracardiac malformations, of which 38 cases (24.5%) had a single extracardiac malformation, 15 cases (9.7%) had multiple extracardiac malformations, 6 had trisomy 21 syndrome, and 1 had VATER syndrome. Multivariate logistic regression analysis showed that wingspread classification of anorectal malformation and extracardiac disorders were independent risk factors for major CHDs. The probability of major CHDs in children with high/intermediate anorectal malformation was 4.709 times higher than that with low anorectal malformation ( CONCLUSIONS The morbidity of major CHDs is higher in severe cases with high/intermediate anorectal malformation and acute cases without fistula or with obstructed fistula and cases with multiple congenital disorders. Echocardiography can define the type and severity of CHDs, which are useful to develop the optimal diagnosis and treatment plan for children with anorectal malformation.
Abnormalities, Multiple ; Anorectal Malformations/therapy* ; Child ; Heart Defects, Congenital/mortality* ; Humans ; Retrospective Studies

Percutaneous pulmonary valve implantation (PPVI) is recognized as a feasible and low risk alternative to surgery to treat dysfunctional right ventricular outflow tract (RVOT) in usually pluri-operated patients. Evolving technology allowed to develop different kind of prosthesis and to go from an initial treatment exclusively of stenotic conduit to an actual approach extended also to wide native RVOT. The Melody transcatheter pulmonary valve (TPV) and the Edwards Sapien valve are nowadays the most commonly implanted prostheses. However, other devices have been developed to treat large RVOT (i.e., the Venus p-valve, the Medtronic Harmony TPV, the Alterra Adaptive Prestent, and the Pulsta valve). Indications for PPVI are the same as for surgical interventions on pulmonary valve, with limits related to the maximum diameter of the available percutaneous prosthesis. Therefore, an accurate preoperative evaluation is of paramount importance to select patients who could benefit from this procedure. The overall periprocedural mortality incidence is around 1.4%, while freedom from RVOT reintervention ranges from 100% at 4 months to 70% at 70 months, according to the different published studies.
Freedom ; Heart Defects, Congenital ; Humans ; Incidence ; Mortality ; Prostheses and Implants ; Pulmonary Valve ; Venus

BACKGROUND AND OBJECTIVES: Adults with congenital heart disease (CHD) are an increasing group of patients thanks to the survival of over 85% of children with CHD. 20% of these patients shall warrant a surgical procedure during their life span. However, currently there is no one risk score that assess correctly the mortality of these procedures. Thus, we analyse the risk scores used at our institution. METHODS: From May 1991 till June 2017, 608 procedures in adults with CHD were performed. The 3 risk scores (risk adjustment for congenital heart surgery [RACHS-1], Aristotle, and Euroscore I) of each procedure were analysed. We used area under the receiver operating characteristic curve (c-index) to measure model discrimination, and Hosmer-Lemeshow (H-L) statistic along with calibration plots to measure calibration. RESULTS: There was no statistical difference between the area under the curve for the 3 scores (χ²=0.58 with 2 df, p=0.750). There was no evidence of lack of fit for RACHS-1 (H-L, χ²=2.61; p=0.271) and Aristotle score (H-L, χ²=5.69; p=0.459). However, there was evidence in lack of calibration in the Euroscore I scoring system (H-L, χ²=33.69; p<0.001). The calibration slope for RACHS-1 was 0.912, for Aristotle (stratified in risk groups) was −0.14 and for Euroscore 1 (stratified in risk groups) was 0.46. CONCLUSIONS: RACHS-1 seems to be best risk scoring system for calculating mortality applied to surgery in adults with CHD.
Adult ; Calibration ; Child ; Discrimination (Psychology) ; Heart Defects, Congenital ; Humans ; Mortality ; ROC Curve ; Thoracic Surgery

BACKGROUND: Heart transplantation (HTx) can be a life-saving procedure for patients in whom single ventricle palliation or one-and-a-half (1½) ventricle repair has failed. However, the presence of a previous bidirectional cavopulmonary shunt (BCS) necessitates extensive pulmonary artery angioplasty, which may lead to worse outcomes. We sought to assess the post-HTx outcomes in patients with a previous BCS, and to assess the technical feasibility of leaving the BCS in place during HTx. METHODS: From 1992 to 2017, 11 HTx were performed in patients failing from Fontan (n=7), BCS (n=3), or 1½ ventricle (n=1) physiology at Asan Medical Center. The median age at HTx was 12.0 years (range, 3–24 years). Three patients (27.3%) underwent HTx without taking down the previous BCS. RESULTS: No early mortality was observed. One patient died of acute rejection 3.5 years after HTx. The overall survival rate was 91% at 2 years. In the 3 patients without BCS take-down, the median anastomosis time was 65 minutes (range, 54–68 minutes), which was shorter than in the patients with BCS take-down (93 minutes; range, 62–128 minutes), while the postoperative central venous pressure (CVP) was comparable to the preoperative CVP. CONCLUSION: Transplantation can be successfully performed in patients with end-stage congenital heart disease after single ventricle palliation or 1½ ventricle repair. Leaving the BCS in place during HTx may simplify the operative procedure without causing significant adverse outcomes.
Angioplasty ; Central Venous Pressure ; Chungcheongnam-do ; Fontan Procedure ; Heart Defects, Congenital ; Heart Transplantation* ; Heart* ; Humans ; Mortality ; Physiology ; Pulmonary Artery* ; Surgical Procedures, Operative ; Survival Rate ; Vena Cava, Superior*

OBJECTIVETo analyze the perioperative clinical data of children with congenital heart disease complicated by acute kidney injury (AKI) after cardiopulmonary bypass (CPB) surgery, and to explore potential factors influencing the prognosis.

METHODSA retrospective analysis was performed among 118 children with congenital heart disease who developed AKI within 48 hours after CPB surgery.

RESULTSIn the 118 patients, 18 died after 48 hours of surgery. Compared with the survivors, the dead children had significantly higher incidence of cyanotic disease and Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1) scores before surgery; during surgery, the dead children had significantly longer CPB time and aortic cross-clamping time, a significantly higher proportion of patients receiving crystalloid solution for myocardial protection, and a significantly higher mean blood glucose level. Within 48 hours after surgery, the dead children had significantly higher positive inotropic drug scores, significantly higher creatinine values, a significantly higher incidence of stage 3 AKI, a significantly higher proportion of patients receiving renal replacement the, and significantly higher usage of blood products (P<0.05). The mortality rate of the patients increased with increased intraoperative blood glucose levels (P<0.05). Patients with intraoperative blood glucose levels >8.3 mmol/L had a significantly lower postoperative cumulative survival rate and a significantly shorter mean survival time than those with blood glucose levels ≤ 8.3 mmol/L (P<0.05).

CONCLUSIONSIntraoperative blood glucose levels are associated with the prognosis in children with congenital heart disease complicated by AKI after CPB surgery. Maintaining good intraoperative blood glucose control can improve the prognosis of the children.

Acute Kidney Injury ; etiology ; mortality ; therapy ; Cardiopulmonary Bypass ; Child, Preschool ; Female ; Heart Defects, Congenital ; mortality ; surgery ; Humans ; Infant ; Male ; Postoperative Complications ; etiology ; Prognosis ; Retrospective Studies

Fontan circulation is generally characterized by high central venous pressure, low cardiac output, and slightly low arterial oxygen saturation, and it is quite different from normal biventricular physiology. Therefore, when a patient with congenital heart disease is selected as a candidate for this type of circulation, the ultimate goals of therapy consist of 2 components. One is a smooth adjustment to the new circulation, and the other is long-term circulatory stabilization after adjustment. When either of these goals is not achieved, the patient is categorized as having “failed” Fontan circulation, and the prognosis is dismal. For the first goal of smooth adjustment, a lot of effort has been made to establish criteria for patient selection and intensive management immediately after the Fontan operation. For the second goal of long-term circulatory stabilization, there is limited evidence of successful strategies for long-term hemodynamic stabilization. Furthermore, there have been no data on optimal hemodynamics in Fontan circulation that could be used as a reference for patient management. Although small clinical trials and case reports are available, the results cannot be generalized to the majority of Fontan survivors. We recently reported the clinical and hemodynamic characteristics of early and late failing Fontan survivors and their association with all-cause mortality. This knowledge could provide insight into the complex Fontan pathophysiology and might help establish a management strategy for long-term hemodynamic stabilization.
Cardiac Output ; Cardiac Output, Low ; Central Venous Pressure ; Fontan Procedure ; Heart Defects, Congenital ; Hemodynamics* ; Humans ; Mortality ; Oxygen ; Patient Selection ; Physiology ; Prognosis ; Survivors ; Vascular Resistance

BACKGROUND: Patent ductus arteriosus (PDA) ligation is usually performed by congenital cardiac surgeons. However, due to the uneven distribution of congenital cardiac surgeons in South Korea, many institutions depend solely on adult cardiac surgeons for congenital cardiac diseases. We report the outcomes of PDA ligations performed by adult cardiac surgeons at our institution. METHODS: The electronic medical records of 852 neonates at Chung-Ang University Hospital, Seoul, South Korea from November 2010 to May 2014 were reviewed to identify patients with PDA. RESULTS: Of the 111 neonates with a diagnosis of PDA, 26 (23%) underwent PDA ligation. PDAs were ligated within 28 days of birth (mean, 14.5±7.8 days), and the mean gestational age of these patients was 30.3±4.6 weeks (range, 26 to 40 weeks) with a mean birth weight of 1,292.5±703.5 g (range, 480 to 3,020 g). No residual shunts through the PDA were found on postoperative echocardiography. There was 1 case of 30-day mortality (3.8%) due to pneumonia, and 6 cases of in-hospital mortality (23.1%) after 30 days, which is comparable to results from other centers with congenital cardiac surgery programs. CONCLUSION: Although our outcomes may not be generalizable to all hospital settings without a congenital cardiac surgery program, in select centers, PDA ligations can be performed safely by adult cardiac surgeons if no congenital cardiac surgery program is available.
Adult* ; Birth Weight ; Cardiac Surgical Procedures ; Diagnosis ; Ductus Arteriosus, Patent* ; Echocardiography ; Electronic Health Records ; Gestational Age ; Heart Defects, Congenital ; Heart Diseases ; Hospital Mortality ; Humans ; Infant, Newborn ; Korea ; Ligation* ; Mortality ; Parturition ; Pneumonia ; Seoul ; Surgeons* ; Thoracic Surgery

BACKGROUND AND OBJECTIVES: While the off-label use of implantable medical devices for treating congenital heart disease is not uncommon, the present conditions and outcomes of their off-label use have rarely been described. Therefore, this study evaluated the prevalence and results of using implantable devices off-label to treat congenital heart disease at a single institute. SUBJECTS AND METHODS: This was a retrospective study based on the medical records of catheter-based interventions for congenital heart disease performed from July 1, 1995 to June 1, 2015. The inclusion criterion was the off-label use of an implantable device. Patient demographic data, procedural success, and follow-up status regarding late complications were investigated, and the results of the off-label use of each device were compared. RESULTS: Off-label use occurred in 144 of 1730 interventions with device implantation, accounting for 8.3% of the interventions. The median patient age and mean body weight were 51.0 months and 16.3 kg, respectively. Immediate and late failures were found in 9 cases, and 3 cases of mortality were not directly related to the devices used. The overall success rate was 93.8%. There were no long-term complications of the off-label use of occlusion devices. No procedural failures resulted from stent implantation, but one case of stent malposition and two cases of stent fracture were identified after procedure completion. CONCLUSION: In general, the off-label use of implantable devices for treating congenital heart disease is safe and effective.
Body Weight ; Follow-Up Studies ; Heart Defects, Congenital* ; Humans ; Medical Records ; Mortality ; Off-Label Use* ; Prevalence ; Retrospective Studies ; Stents