Critical Congenital Heart Disease Research Articles

Neonatal Pulse Oxymetry Screening for Detection of Congenital Heart Disease in Asymptomatic Newborns: A Cross-sectional Study from a Tertiary Care Hospital

Introduction: Screening for Congenital Heart Diseases (CHD) depends on the antenatal ultrasonography and clinical examination of the newborn, however both these methods have low detection rates and often life-threatening congenital heart diseases are missed. Pulse Oximetry (PO) is an easy, accurate, rapid, non invasive method of detecting hypoxaemia. The purpose of using PO to identify Critical Congenital Heart Disease (CCHD) is that clinically non detectable minimal hypoxemia can be detected by pulseoximetry. Aim: To study the accuracy of pulse oxymetry as a screening tool for early detection of critical congenital heart diseases in asymptomatic newborns. Materials and Methods: This cross-sectional study was conducted in the Department of Paediatrics and the Postnatal Ward of Obstetrics and Gynaecology at Sri Venkateswara Medical College, Tirupathi, Andhra Pradesh, India, from January 2017 to December 2020. All the term asymptomatic newborns of age more than 24 hours were screened using PO. Screening was positive if -a: PO was< 90% in right hand or foot at any stage of screening, b; PO was 90%-< 95% on both; there was> 3% absolute difference in oxygen saturation between the right hand and foot on three consecutive measures (each separated by one hour). All the screen positive babies were subjected to 2D echocardiography. All statistical analyses were performed using OpenEpi website epicalculator, and Chi-square’s test was used to calculate the p-value. Results: The mean gestational age (weeks) was 38±4 days. Out of 14400, PO screening was positive in 45 babies, and subsequent echocardiography detected CHD in 30 babies. The sensitivity was 66.67%, positive predictive value was 66.67%, negative predictive value was 99.90%, with a diagnostic accuracy of 99.79%. Results of 2D electrocardiography, 30 were true positive cases, whereas, false positives and false negatives were 15 each. Remaining 14340 newborns were true negatives. Conclusion: Pulse oximetry is a safe, accessible, feasible test that can be used for early detection of CCHD’s that are often undetected on antenatal ultrasonography.

What is Known About Critical Congenital Heart Disease Diagnosis and Management Experiences From the Perspectives of Family and Healthcare Providers? A Systematic Integrative Literature Review

The experience of diagnosis, decision-making and management of Critical Congenital Heart Disease (CCHD) is complex and taxing for families and clinicians. We systematically reviewed evidence relating to the family and healthcare provider experience of CCHD diagnosis and its management.

Prenatal Diagnosis of Congenital Heart Diseases and Associations with Serum Biomarkers of Aneuploidy: A Multicenter Prospective Cohort Study.

PurposeWe assessed prenatal detection rates of congenital heart disease (CHD) and associations between maternal serum biomarkers and non-chromosomal CHD in singleton pregnancies.Materials and MethodsThis study was conducted as a secondary analysis of data obtained during a multicenter prospective cohort study that investigated the cost-effectiveness of prenatal testing for fetal aneuploidy. We analyzed the prenatal detection rate and accuracy for CHD screening via ultrasound during the second trimester, as well as associations between serum biomarkers and CHDs, in singleton newborns without chromosomal abnormalities.ResultsAmong 6715 women, 142 (2.1%) newborns were born with CHDs, of which 67 (1.0%) newborns had major CHDs. The prenatal detection rate for all CHDs and major CHDs were 34.5% and 58.2%, respectively. After excluding isolated ventricular septal defects, the detection rate for critical CHDs was 85.9%. Women with low pregnancy-associated plasma protein A (PAPP-A) (<0.4 multiples of the median, MOM) face increased risks of non-chromosomal CHDs [adjusted odds ratio (aOR) 2.76; 95% confidence interval (CI) 1.36–5.13] and major CHDs (aOR 7.30; 95% CI 3.18–15.59), compared to those without CHDs. A higher inhibin A level (≥2.5 MOM; aOR 4.84; 95% CI 1.42–12.46) was associated with non-chromosomal major CHDs.ConclusionUltrasonography performed during the second trimester by obstetricians detected over 85% of critical CHDs. Low maternal serum PAPP-A or high inhibin-A was associated with non-chromosomal CHDs. These results may contribute to an improvement in prenatal diagnosis of CHDs.

A nationwide survey of pulse oximetry screening for critical congenital heart disease in Japan

A nationwide survey of pulse oximetry screening for critical congenital heart disease in Japan

The Role of Pulse Oximetry as a Screening Tool for Early Detection of Critical Congenital Heart Disease in Newborn

The Role of Pulse Oximetry as a Screening Tool for Early Detection of Critical Congenital Heart Disease in Newborn

Impact of Prenatal Diagnosis on the Management and Prognosis of Infants with Congenital Heart Disease- A Retrospective Study

Introduction: Prenatal diagnosis is important in outcome of various congenital anomalies in recent times especially in congenital heart diseases. This study would help in recognising the importance of prenatal diagnosis and in understanding the management of newborns with congenital heart diseases. Aim: To evaluate the impact of prenatal diagnosis on the management and prognosis of infants with congenital heart disease. Materials and Methods: This retrospective study was conducted in Department of Paediatrics at Dr. Vasantrao Pawar Medical College, Nashik, Maharshtra, India, from January 2018 to January 2021. The study was performed on newborns who were diagnosed with Congenital Heart Diseases (CHD) during hospitalisation, in an inborn and outborn unit of Neonatal Intensive Care Unit (NICU). The data recorded for requirement of inotropes, ventilation, arterial blood gas analysis and outcome in the form of survival. The babies were classified into Prenatal ECHO (PNE) group and No Prenatal ECHO Available (NPEA) group, based on availability of prenatal Echocardiogram (ECHO) diagnostic report. CHDs were categorised into critical/major and minor. For the comparison of quantitative variables, Unpaired t-test/Mann- Whitney test was used. For the comparison of qualitative variable, Fisher’s-exact test/Chi-square test was used, as necessary. Results: There were 5000 admissions in the inborn and outborn neonatal unit during the study period, of which 159 cases had a diagnosis of CHDs. The PNE group consisted of 61 neonates who had been diagnosed with CHD and the NPEA group consisted of 67 neonates who were not antenatally diagnosed to have CHD. Ionotropic support needed for PNE group (4.5%) was comparatively lesser than NPEA group (14.3%). Need for ventilation was reduced in PNE group (4.5%) as compared to NPEA group (14.3%). Mean for serum lactate and serum bicarbonate was 3.49±2.58 and 16.24±4.31 in PNE group, whereas, it was 5.08±2.79 and 15.12±4.13 in NPEA group on admission in NICU. In management of critical CHDs, 2/31 (6.5%) babies died in PNE group compared to 11/37 (29.7%) in NPEA group (p-value=0.09). Conclusion: Antenatal diagnosis helps in meticulous management of neonates with congenital heart diseases in terms of fewer requirement of inotropes, need of ventilation and improved management. There was no significant difference in outcome in the form of survival.

In infants with congenital heart disease autonomic dysfunction is associated with pre-operative brain injury.

Background:Brain injury is a serious and common complication of critical congenital heart disease (CHD). Impaired autonomic development (assessed by heart rate variability; HRV) is associated with brain injury in other high-risk neonatal populations.Objective:To determine whether impaired early neonatal HRV is associated with pre-operative brain injury in CHD.Methods:In infants with critical CHD, we evaluated HRV during the first 24 hours of cardiac ICU (CICU) admission using time-domain (RMS1, RMS2, and alpha 1) and frequency-domain metrics (LF, nLF, HF, nHF). Pre-operative brain MRI was scored for injury using an established system. Spearman’s correlation coefficient was used to determine the association between HRV and pre-operative brain injury.Results:We enrolled 34 infants with median birth gestational age (GA) of 38.8 weeks (IQR 38.1-39.1). Median postnatal age (PNA) at pre-operative brain MRI was 2 days (IQR 1-3 days). Thirteen infants had MRI evidence of brain injury. RMS 1 and RMS 2 were inversely correlated with pre-operative brain injury.Conclusions:Time-domain metrics of autonomic function measured within the first 24 hours of admission to the CICU are associated with pre-operative brain injury, and may perform better than frequency domain metrics under non-stationary conditions such as critical illness.

Preoperative in-hospital mortality in neonates with critical CHD.

Data regarding preoperative mortality in neonates with critical CHD are sparse and would aid patient care and family counselling. The objective of this study was to utilise a multicentre administrative dataset to report the rate of and identify risk factors for preoperative in-hospital mortality in neonates with critical CHD across US centres. The Pediatric Health Information System database was utilised to search for newborns ≤30 days old, born 1 January 2009 to 30 June 2018, with an ICD-9/10 code for d-transposition of the great arteries, truncus arteriosus, interrupted aortic arch, or hypoplastic left heart syndrome. Preoperative in-hospital mortality was defined as patients who died prior to discharge without an ICD code for cardiac surgery or interventional catheterisation. Overall preoperative mortality rate was at least 5.4% (690/12,739) and varied across diagnoses (d-TGA 2.9%, TA 8.3%, IAA 5.5%, and HLHS 7.3%) and centres (0-20.5%). In multivariable analysis, risk factors associated with preoperative mortality included preterm delivery (<37 weeks) (OR 2.3, 95% CI: 1.8-2.9; p < 0.01), low birth weight (<2.5 kg) (OR 3.8, 95% CI: 3.0-4.7; p < 0.01), and genetic abnormality (OR 1.6, 95% CI: 1.2-2.2; p < 0.01). Centre average surgical volume was not a significant risk factor. Approximately 1 in 20 neonates with critical CHD suffered preoperative in-hospital mortality, and rates varied across diagnoses and centres. Better understanding of the factors that drive the variation (e.g. patient factors, preoperative care models, surgical timing) could help identify patient care improvement opportunities and inform conversations with families.

Association Between Children With Life-Threatening Conditions and Their Parents’ and Siblings’ Mental and Physical Health

Despite concerns regarding the potential deleterious physical and mental health outcomes among family members of a child with a life-threatening condition (LTC), few studies have examined empirical measures of health outcomes among these family members. To examine whether mothers, fathers, sisters, and brothers of children with 1 of 4 types of pediatric LTCs have higher rates of health care encounters, diagnoses, and prescriptions compared with families of children without these conditions. This retrospective cohort study included US families with commercial insurance coverage from a single carrier. Children who had 1 of 4 LTCs (substantial prematurity, critical congenital heart disease, cancer, or a condition resulting in severe neurologic impairment) were identified by a diagnosis in their insurance claim data between July 1, 2015, and June 30, 2016. Each case child and their family was matched with up to 4 control children and their families based on the age of the case and control children. Data were analyzed between August 2020 and March 2021. Having a child or sibling with substantial prematurity, critical congenital heart disease, cancer, or a condition resulting in severe and progressive neurologic impairment. Rates of occurrence of health care encounters, physical and mental health diagnoses, and physical and mental health medication prescriptions, identified from insurance claims data, were compared between case and control families using a multivariable negative binomial regression model. The statistical analysis adjusted for observed differences between case and control families and accounted for clustering at the family level. The study included 25 528 children (6909 case children [27.1%] and 18 619 control children [72.9%]; median age, 6.0 years [IQR, 1-13 years]; 13 294 [52.1%] male), 43 357 parents (11 586 case parents [26.7%] and 31 771 control parents [73.3%]; mean [SD] age, 40.4 [8.1] years; 22 318 [51.5%] female), and 25 706 siblings (7664 case siblings [29.8%] and 18 042 control siblings [70.2%]; mean [SD] age, 12.1 [6.5] years; 13 114 [51.0%] male). Overall, case mothers had higher rates of the composite outcome of health care encounters, diagnoses, and prescriptions compared with control mothers (incident rate ratio [IRR], 1.61; 95% CI, 1.54-1.68), as did case fathers compared with control fathers (IRR, 1.55; 95% CI, 1.46-1.64). Sisters of children with LTCs had higher rates of the composite outcome compared with sisters of children without LTCs (IRR, 1.68; 95% CI, 1.55-1.82), as did brothers of children with LTCs compared with brothers of children without LTCs (IRR, 1.70; 95% CI, 1.56-1.85). In this cohort study, mothers, fathers, sisters, and brothers who had a child or sibling with 1 of 4 types of LTCs had higher rates of health care encounters, diagnoses, and medication prescriptions compared with families who did not have a child with that condition. The findings suggest that family members of children with LTCs may experience poorer mental and physical health outcomes. Interventions for parents and siblings of children with LTCs that aim to safeguard their mental and physical well-being appear to be warranted.

Central and Peripheral Cannulation for Cardiopulmonary Bypass in Fetal Sheep: A Comparative Study.

Objective: In-utero correction is an option for treatment of critical congenital heart diseases (CHDs). Fetal cardiac surgery for CHDs is dependent on the reliable use of fetal cardiopulmonary bypass (CPB), but this technology remains experimental. In this study, we established fetal CPB models with central and peripheral cannulation to explore the differences between the two cannulation strategies.Methods: Ten fetal sheep with 90–110 gestational days were randomized into central cannulation (n = 5) and peripheral cannulation (n = 5) groups. All fetal CPB models were successfully established. At each time point (0, 30, and 60 min after initiation of CPB), echocardiography was performed. Blood samples were also collected for blood gas analysis and tests of myocardial enzymes and liver and kidney function.Results: In the central cannulation group, right ventricular Tei index significantly increased (p = 0.016) over time. Compared with the peripheral cannulation group, the left ventricular Tei index of the central cannulation group was significantly higher (1.96 ± 0.31 vs. 0.45 ± 0.19, respectively; p = 0.028) and the stroke volume was lower (0.46 ± 0.55 vs. 2.13 ± 0.05, respectively; p = 0.008) at 60 min after CPB. Levels of liver and kidney injury markers and of acid-base balance, including alanine aminotransferase (ALT), aspartate aminotransferase/ALT ratio, blood urea nitrogen (BUN), BUN/creatinine ratio, base excess and bicarbonates, were significantly higher for peripheral than for central cannulation. Other important physiologic parameters, including heart rate, blood pressure, myocardial enzymes, umbilical artery beat index and resistance index, left ventricular Tei index, and left and right ventricular stroke volume, were comparable between the two groups.Conclusions: Both central and peripheral cannulations can be used to establish fetal CPB models. Central cannulation causes more adverse impacts for cardiac function, whereas peripheral cannulation is more susceptible to complications related to inadequate organ perfusion.

Effectiveness of newborn pulse oximetry screening for the identification of critical congenital heart disease in a tertiary care hospital in Sri Lanka

The Journal is the primary organ of Continuing Paediatric Medical Education in Sri Lanka. The journal also has a website. Free full text access is available for all readers.The Sri Lanka Journal of Child Health is now indexed in SciVerse Scopus (Source Record ID 19900193609), Index Medicus for South-East Asia Region (IMSEAR), CABI (Centre for Agriculture and Bioscience International Global Health Database), DOAJ and is available in Google, as well as Google Scholar.The policies of the journal are modelled on the Committee on Publication Ethics (COPE) Guidelines on Principles of Transparency and Best Practice in Scholarly Publishing. Sri Lanka Journal of Child Health is recognised by the International Committee of Medical Journal Editors (ICMJE) as a publication following the ICMJE Recommendations.

Folic Acid and the Risk of Congenital Heart Defects

Source: Wang D, Jin L, Zhang J, et al. Maternal periconceptional folic acid supplementation and risk for fetal congenital heart defects [published online ahead of print September 8, 2021]. J Pediatr. doi: 10.1016/j.jpeds.2021.09.004.Investigators from Peking University and Tongzhou Maternal and Child Health Hospital, both in Beijing, China, conducted a retrospective study to assess the effectiveness of periconceptional folic acid (FA) supplementation in preventing congenital heart defects (CHD). For the study, they reviewed data contained in the Prenatal Health Care System (PHCS) and Birth Defects Surveillance System (BDSS) databases on singleton deliveries (including live births, stillbirths, and terminated pregnancies) between 2013 and 2018 in the Tongzhou District, Beijing, China. PHCS is a registry that includes data on women during pregnancy and after delivery, including maternal age and educational level, delivery date, and periconceptional FA supplementation. Information about FA supplementation was based on maternal self-report and included type of FA supplementation and when it started (prior to or after conception). In China, women are provided FA in a daily dose of 0.4 mg by the central government. However, some women take micronutrient supplements containing FA (MMFA). The BDSS includes information on birth defects, beginning at 13 weeks of gestation, including CHD.Primary study outcomes were any CHD, ventricular septal defect (VSD), and critical CHD (defined using standardized criteria). Poisson regression was used to compare the risks of these outcomes in women with FA/MMFA supplementation and in those without supplementation. Risk of CHD also was compared in women taking FA vs MMFA, and with FA/MMFA supplementation beginning prior to or after conception. Regression models also included maternal age, education, and year of delivery.Data were analyzed on 63,969 women and their offspring. Women using FA/MMFA supplementation were significantly older and more educated than those who didn’t. There were 308 cases of CHD in the offspring of study women, including 152 live births and 156 stillbirths or terminated pregnancies; the most common CHD was VSD (125 cases). The use of periconceptional FA/MMFA was associated with a reduced risk of any CHD (adjusted risk ratio [ARR], 0.60; 95% CI, 0.44, 0.83), VSD (ARR, 0.47; 95% CI, 0.30, 0.74) and critical CHD (ARR, 0.41; 95% CI, 0.26, 0.67). There were no statistical differences between FA and MMFA supplementation for risk of any CHD (ARR, 0.84; 95% CI, 0.66, 1.09), VSD (ARR, 0.94; 95% CI, 0.63, 1.41) or critical CHD (ARR, 0.64; 95% CI, 0.44, 1.00). Initiation of FA/MMFA supplementation before conception, compared to post-conception, was associated with a reduced risk of any CHD (ARR, 0.68; 95% CI, 0.48, 0.95) and critical CHD (ARR, 0.26; 95% CI, 0.10, 0.71).The authors conclude that maternal periconceptional FA supplementation was associated with a decreased risk of CHD in off-spring, particularly when initiated prior to conception.Dr Doolittle has disclosed no financial relationship relevant to this commentary. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.FA, one of the B vitamins, is a micronutrient found in fresh fruit, vegetables, nuts, and eggs.1 FA deficiency has been associated with megaloblastic anemia and neural tube defects in infants, as well as heart disease and cancer in adults.1 Humans are unable to synthesize FA, and thus must rely on ingestion in the form of food or conversion of precursors in the gut microflora.1 FA is an important co-factor in DNA and RNA synthesis, and the formation of the heart neural crest cells are highly sensitive to FA.2 Since 1992, FA and other micronutrient deficiencies have been associated with CHD.3 However, the results of supplementation trials to prevent CHD have been equivocal.4 The results of the current investigation provide convincing evidence to support FA/MMFA supplementation to prevent CHD.The study design was robust, with a governmental registry of more than 63,000 women who were followed prospectively over 5 years. Of note, the investigators evaluated self-report of FA/MMFA supplementation. Although self-report may be imperfect, the study represents a real-world evaluation. Women who took FA/MMFA supplementation were older and better educated. As maternal age is a risk for CHD, and education usually confers better health outcomes, the investigators controlled for these variables.The results of the current study raises several provocative questions that merit future investigation. Pre-conception supplementation was associated with reduced CHD compared to peri-conception supplementation. This suggests that some independent maternal factors may influence CHD formation prior to conception. Also, despite the reduction of CHD due to supplementation, the continued prevalence of CHD suggests other factors such as genetic defects, exogenous toxin, or infection are at play.Maternal periconceptual supplementation with FA may be associated with a reduced risk of CHD, especially when initiated before conception. (See AAP Grand Rounds. 2020;44[6]:68.)5Perhaps the greater protective efficacy of preconceptual FA supplementation is related to the time it takes to achieve effective folate levels during the critical period of heart development.2

A Machine Learning Driven Pipeline for Automated Photoplethysmogram Signal Artifact Detection.

Recent advances in Critical Congenital Heart Disease (CCHD) research using Photoplethysmography (PPG) signals have yielded an Internet of Things (IoT) based enhanced screening method that performs CCHD detection comparable to SpO2 screening. The use of PPG signals, however, poses a challenge due to its measurements being prone to artifacts. To comprehensively study the most effective way to remove the artifact segments from PPG waveforms, we performed feature engineering and investigated both Machine Learning (ML) and rule based algorithms to identify the optimal method of artifact detection. Our proposed artifact detection system utilizes a 3-stage ML model that incorporates both Gradient Boosting (GB) and Random Forest (RF). The proposed system achieved 84.01% of Intersection over Union (IoU), which is competitive to state-of-the-art artifact detection methods tested on higher resolution PPG.

A Failed CCHD Screen.

A Failed CCHD Screen.

Pulse oximetry for diagnosis of Critical Congenital Heart Disease

Pulse oximetry for diagnosis of Critical Congenital Heart Disease

Major heart defects: the diagnostic evaluations of first-year-olds

BackgroundSevere or critical congenital heart defects (CHDs) constitute one third of the heart defect cases detected only after birth. These prenatally unrecognised defects usually manifest as cyanotic or acyanotic lesions and are diagnosed postnatally at various times. The aim of the study was to identify their clinical symptoms and determine individual risk periods for CHD manifestation.MethodsData were assessed retrospectively based on a cohort of patients born between 2009 and 2018 in a population of 175,153 live births. Occurrence of the first symptoms of CHD was classified into: early neonatal (0–7 days), late neonatal (8–28 days), early infancy (1–6 months), or late infancy (6–12 months). The first symptom for which the child was referred to a paediatric cardiologist was defined as a symptom of CHD.ResultsThere were 598 major CHDs diagnosed in the studied region, 91% of which were isolated anomalies. A concomitant genetic disorder was diagnosed in 6% of the cases, while 3% presented extracardiac pathology with a normal karyotype. In total, 47% (282/598) of all CHDs were not identified prenatally. Of these, 74% (210/282) were diagnosed as early neonates, 16% (44/282) as late neonates, and 10% (28/282) as infants. The most common symptoms leading to the diagnosis of CHD were heart murmur (51%, 145/282) and cyanosis (26%, 73/282). Diagnosis after discharge from the hospital occurred in 12% (72/598) of all major CHDs. Ventricular septal defect and coarctation of the aorta constituted the majority of delayed diagnoses.ConclusionsIn conclusion, murmur and cyanosis are the most common manifestations of prenatally undetected CHDs. Although most children with major CHDs are diagnosed as neonates, some patients are still discharged from the maternity hospital with an unidentified defect.

Two approaches for newborns with critical congenital heart disease: a comparative study.

Prenatal diagnosis and planned peripartum care is an unexplored concept in China. This study aimed to evaluate the effects of the "prenatal diagnosis and postnatal treatment integrated model" for newborns with critical congenital heart disease. The medical records of neonates (≤ 28days) admitted to Fuwai Hospital were reviewed retrospectively from January 2019 to December 2020. The patients were divided into "prenatal diagnosis and postnatal treatment integrated group" (n = 47) and "non-integrated group" (n = 69). The age of admission to the hospital and the age at surgery were earlier in the integrated group than in the non-integrated group (5.2 ± 7.2days vs. 11.8 ± 8.0days, P < 0.001; 11.9 ± 7.0days vs. 16.5 ± 7.7days, P = 0.001, respectively). The weight at surgery also was lower in the integrated group than in the non-integrated group (3.3 ± 0.4kg vs. 3.6 ± 0.6kg, P = 0.010). Longer postoperative recovery time was needed in the integrated group, with a median mechanical ventilation time of 97h (interquartile range 51-259h) vs. 69h (29-168h) (P = 0.030) and with intensive care unit time of 13.0days (8.0-21.0days) vs. 9.0days (4.5-16.0days) (P = 0.048). No significant difference was observed in the all-cause mortality (2.1 vs. 8.7%, P = 0.238), but it was significantly lower in the integrated group for transposition of the great arteries (0 vs. 18.8%, log rank P = 0.032). The prenatal diagnosis and postnatal treatment integrated model could significantly shorten the diagnosis and hospitalization interval of newborns, and surgical intervention could be performed with a lower risk of death, especially for transposition of the great arteries.

Treatment and prognosis of 826 infants with critical congenital heart disease: a single center retrospective study

Objective: To analyze the current status of clinical treatment and factors influencing postoperative mortality in infants with critical congenital heart disease (CCHD) in China, optimize the perioperative management of CCHD, and provide a new scientific basis for clinical decision-making for the optimal management of these patients. Methods: This is a retrospective single-center study. Infants diagnosed with CCHD in Guangdong Provincial People's Hospital from January 2017 to December 2019 (aged 0-1 years at admission) were enrolled. General clinical information, inpatient treatment information, prognosis and complications were collected and analyzed. Multivariate logistic regression analysis was used to explore the independent risk factors of postoperative death in infants with CCHD. Results: A total of 826 infants with CCHD were included, including 556 males (67.3%) and the age at first admission was 51.0 (5.0,178.3) days. 264 (32.0%) cases were tetralogy of Fallot and 137 (16.6%) cases were total anomalous pulmonary venous return. 195 cases (23.6%) were diagnosed prenatally. 196 cases (23.7%) were treated with prostaglandin. The preoperative invasive ventilation time was 0 (0, 0) hour, and the postoperative invasive ventilation time was 95.0 (26.0, 151.8) hours. A total of 668 cases (80.9%) underwent surgical treatment. The age was 100.5 (20.0, 218.0) days during operation and the operation time was 190.0 (155.0, 240.0) hours. Sixty-two cases (7.5%) received medical treatment, and 96 cases (11.6%) gave up treatment. A total of 675 cases (81.7%) were discharged with improvement, 96 cases (11.6%) were discharged after giving up treatment, 55 cases (6.7%) died and 109 cases (13.2%) were readmitted within one year. Complications occurred in 565 (68.6%) cases, including pneumonia in 334 cases (40.4%) and cardiac arrhythmias in 182 cases (22.0%). Multifactorial analysis showed that delayed chest closure (OR=49.775, 95%CI 3.291-752.922, P=0.005), prolonged post-operative invasive ventilator ventilation (OR=1.003, 95%CI 1.000-1.005, P=0.038) and cardiac hypoplasia syndrome (OR=272.658, 95%CI 37.861-1 963.589, P<0.001) were the independent risk factors for mortality in CCHD infants post-operation. Conclusions: Tetralogy of Fallot and total anomalous pulmonary venous return account for the majority of infants with CCHD. The proportion of infants diagnosed prenatally was less than 1/4. The majority CCHD infants received surgical treatment. The main complications are pneumonia and arrhythmia. Delayed chest closure, prolonged postoperative invasive ventilator ventilation and low cardiac output syndrome are the independent risk factors for postoperative death in infants with CCHD.

C39. Ductus Arteriosus Stenting: A Promising and Life Saving Procedure for Ductal-Dependent Pulmonary Blood Flow

Abstract Background Duct-dependant congenital heart disease in newborn presents as a life-threatening emergency and the survival depends on patency of the ductus arteriosus. Stent implantation into ductus arteriosus has been proposed as non-surgical management for critical congenital heart disease with duct-dependent. Case Summary We present full-term newborn babies who had critical congenital heart disease with ductal-dependent pulmonary blood blow and successfully managed with ductal stenting. Patient A, a 13-days-old male had Tetralogy of Fallot-Pulmonary Atresia and restrictive Ventricular Septal Defect (VSD). Patient B, a 3-days-old male had Pulmonary Atresia-Intact Ventricular Septum (PA-IVS) with Patent Foramen Ovale (PFO). Patient C, a 5-days-old female had Tricuspid Atresia with restrictive VSD and multiple congenital anomaly. Patient D, a 2-days-old female had PA-IVS with PFO stretch. Prostaglandin-E1 infusion was started at first and stop 6 hours prior to the procedure. Ductus arteriosus stenting using drug-eluting stent and femoral artery approach was successful in all 4 patients. All patient had improvement of oxygen saturation and good post-procedure outcome. Discussion Ductal stenting in patient with duct-dependent pulmonary circulation appears to be an alternative to surgery. It provides a bridging palliation in cyanotic congenital heart disease until the time of definitive surgery. Stenting of ductus arteriosus is feasible and safe with good result, thus early recognition and timely management are important to save the life.

C39. Ductus Arteriosus Stenting: A Promising and Life Saving Procedure for Ductal-Dependent Pulmonary Blood Flow

Abstract Background Duct-dependant congenital heart disease in newborn presents as a life-threatening emergency and the survival depends on patency of the ductus arteriosus. Stent implantation into ductus arteriosus has been proposed as non-surgical management for critical congenital heart disease with duct-dependent. Case Summary We present full-term newborn babies who had critical congenital heart disease with ductal-dependent pulmonary blood blow and successfully managed with ductal stenting. Patient A, a 13-days-old male had Tetralogy of Fallot-Pulmonary Atresia and restrictive Ventricular Septal Defect (VSD). Patient B, a 3-days-old male had Pulmonary Atresia-Intact Ventricular Septum (PA-IVS) with Patent Foramen Ovale (PFO). Patient C, a 5-days-old female had Tricuspid Atresia with restrictive VSD and multiple congenital anomaly. Patient D, a 2-days-old female had PA-IVS with PFO stretch. Prostaglandin-E1 infusion was started at first and stop 6 hours prior to the procedure. Ductus arteriosus stenting using drug-eluting stent and femoral artery approach was successful in all 4 patients. All patient had improvement of oxygen saturation and good post-procedure outcome. Discussion Ductal stenting in patient with duct-dependent pulmonary circulation appears to be an alternative to surgery. It provides a bridging palliation in cyanotic congenital heart disease until the time of definitive surgery. Stenting of ductus arteriosus is feasible and safe with good result, thus early recognition and timely management are important to save the life.