Association of Congenital Heart Defects (CHD) with Factors Related to Maternal Health and Pregnancy in Newborns in Puerto Rico

Background:Given the pervasive issues of obesity and diabetes both in Puerto Rico and the broader United States,there is a compelling need to investigate the intricate interplay among body mass index(BMI),pregesta-tional,and gestational maternal diabetes,and their potential impact on the occurrence of congenital heart defects(CHD)during neonatal development.Methods:Using the comprehensive System of Vigilance and Surveillance of Congenital Defects in Puerto Rico,we conducted a focused analysis on neonates diagnosed with CHD between 2016 and 2020.Our assessment encompassed a range of variables,including maternal age,gestational age,BMI,pregestational diabetes,gestational diabetes,hypertension,history of abortion,and presence of preeclampsia.Results:A cohort of 673 patients was included in our study.The average maternal age was 26 years,within a range of 22 to 32 years.The mean gestational age measured 39 weeks,with a median span of 38 to 39 weeks.Of the 673 patients,274(41%)mothers gave birth to neonates diagnosed with CHD.Within this group,22 cases were linked to pre-gestational diabetes,while 202 were not;20 instances were associated with gestational diabetes,compared to 200 without;and 148 cases exhibited an overweight or obese BMI,whereas 126 displayed a normal BMI.Conclusion:We identified a statistically significant correlation between pre-gestational diabetes mellitus and the occurrence of CHD.However,our analysis did not show a statistically significant association between maternal BMI and the likelihood of CHD.These results may aid in developing effective strategies to prevent and manage CHD in neonates.

Maternal Vascular Dysfunction in Congenital Heart Defects

Background:Research on fetal congenital heart defect(CHD)mostly focuses on etiology and mechanisms.However,studies on maternal complications or pathophysiology are limited.Our objective was to determine whether vascular dysfunction exists in pregnant women carrying a fetus with congenital heart defects.Methods:We conducted a case-control study.27 cases of pregnant women carrying a fetus with major CHD admitted to our hospital for delivery between April 2021 and August 2022 were selected.Every case was matched with about 2 pregnant complication-free controls without fetal abnormalities.The proangiogenic and anti-angiogenic factors and pregnancy outcomes were compared.Results:The proangiogenic factors include vascular endothelial growth factor(VEGF)and placental growth factor(PlGF).The anti-angiogenic factors involve soluble fms-like tyrosine kinase 1(sFlt-1)and soluble endoglin(sEng).No differences were found in maternal plasma concentrations of PlGF,VEGF,and sFlt-1 between case-control groups when analyzed at 36 weeks≤gestational age(GA)<39 weeks and 39 weeks≤GA≤41 weeks.The concentrations of sEng in maternal plasma in the fetal CHD group were significantly higher than those in the control group:0.60(0.77)vs.0.32(0.26)ng/ml at 36 weeks≤GA<39 weeks,p=0.001 and 0.75(0.55)vs.0.28(0.27)ng/ml at 39 weeks≤GA≤41 weeks,p<0.001.Conclusion:Vascular dysfunction exists in pregnant women with fetal congenital heart defects,manifesting significantly elevated sEng concentration at delivery.

Real-Time Remote-Mentored Echocardiography in Management of Newborns with Critical Congenital Heart Defects

Background:The management of suspected critical congenital heart defects(CCHD)relies on timely echocardiographic diagnosis.The availability of experienced echocardiographers is limited or even non-existent in many hospitals with obstetric units.This study evaluates remote-mentored echocardiography performed by physicians without experience in imaging of congenital heart defects(CHD).Methods:The setup included a pediatric cardiologist in a separate room,guiding a physician without experience in echocardiographic imaging of CHD in the examination of a symptomatic newborn.This remote-mentoring pair was blinded to the diagnosis of the newborn and presented with a simplified patient history.The echocardiographic images were streamed to the laptop of the mentor,along with a webcam feed showing the probe position.The task was to identify CCHD in need of immediate transfer to a pediatric cardiac surgical center.The result was compared to the previously completed echocardiographic report and the clinical decision of the patient-responsible pediatric cardiologist.Results:During 17 months,15 newborns were recruited.All six newborns with CCHD were correctly labeled by the remotementoring pair.One newborn with Tetralogy of Fallot was erroneously labeled as needing immediate transfer.Eight newborns without CCHD were correctly labeled.Conclusions:Remote-mentored echocardiography performed by examiners without experience in imaging CHD identified all newborns with CCHD in need of immediate transfer for specialist care.The setup shows promising results for improving the management of CCHD in hospitals without continuous pediatric cardiology service.

Circulating microRNAs as potential biomarkers for diagnosis of congenital heart defects

BACKGROUND: Micro RNAs are small non-coding RNAs of approximately 22 nucleotides in length, and play important regulatory roles in normal heart development and the pathogenesis of heart diseases. Recently, a few prospective studies have implicated the diagnostic role of micro RNAs in congenital heart defects(CHD).DATA RESOURCES: This review retrieved the research articles in Pub Med focusing on the altered microR NAs in cardiac tissue or serum of patients with CHD versus healthy normal controls, as well as the studies exploring circulating microR NAs as potential biomarkers for(fetal) CHD.RESULTS: Most of the studies of interest were conducted in recent years, implicating that the topic in this review is a newly emerging field and is drawing much attention. Moreover, a number of differentially expressed microR NAs between CHD specimens and normal controls have been reported.CONCLUSION: Circulating micro RNAs may serve as potential biomarkers for diagnosis of CHD in the future, with more efforts paving the road to the aim.

Current Advances in Transcatheter Intervention for Children Born with Congenital Heart Defects: A Review of Literature

This review aims to sum up the improvements witnessed in the field of interventional cardiology during recent times. The last decade has witnessed significant technical advances in the field of radiological imaging and also in interventional cardiology which has helped to offer more non-invasive solutions for the management of congenital heart defects. This has resulted from the use of advanced 3-dimensional fusion imaging instead of conventional 2-dimensional angiography, applying interactive real-time enhancement and using computed tomography and Magnetic Resonance Imaging for interventional procedures. Similarly the introduction of next generation devices, have not only improved the final outcome of the procedure but also has helped in reducing the challenges that were faced before and with the initial generation of devices. These advances have helped not only in reducing the radiation exposure, the use of contrast medium dose but also have resulted in improved early survival. The field of neonatal cardiology has advanced at an unprecedented pace. The transcatheter closure of patent ductus arteriosus has evolved over time and now it has been made possible at much lower body weight. Similarly, early use of stents for aortic coarctation has been found effective in some patients, especially when surgical intervention had been denied. The application of the hybrid approach for the management of complex congenital heart defects has also been effectively applied. More recently transcatheter placement of the pulmonary valve has been introduced for severely stenotic and/or regurgitant pulmonary valve in adolescents and adults. It is anticipated that in near future, this procedure would be available for relatively younger patients. In conclusion: last 2 decades have improvised pediatric interventional cardiology to incorporate less invasive solutions for CHD. The current advances in radio-diagnostic imaging, gadgetry and technical expertise have improved significantly and led to manage many of such defects by trans-catheter approach. This has led also, to replace the early surgical intervention with a more subtle hybrid approach, thus reducing not only the major surgical trauma but also been found to be cost-effective due to a shorter hospital stay. But a learning curve for performers is required for optimum results and also such procedures should be performed in a fully developed facility with an optimum surgical backup.

Morphological Characteristics of the Artery of the Atrioventricular Node by Congenital Heart Defects

The objective of the work is to study the topographic and anatomical characteristics of the atrioventricular node’s artery in complex congenital heart defects.The material for the work was the following:44 specimens including hearts of fetuses and children of the first three years having congenital defects and 50 specimens of the hearts of a similar age with normal development as the control.A complex of generally accepted morphological methods was used to identify the conductive system of the heart,its blood supply.According to the data obtained given congenital heart defects,the artery of the atrioventricular node arises from an artery,that determines the type of blood supply to the heart.With the full form of the common atrioventricular canal,the presence of the“U”-shaped bend of the coronary artery at the point of departure of the atrioventricular artery from it is inconstant,which is apparently due to the localization of the atrioventricular node in the embryonic position.The intensity of the lateral branches from the artery of the atrioventricular node to the heart partitions is determined by the hemodynamic features with congenital heart defects.

Family lifestyle factors related to children's congenital heart defects in China:a case-control study

Objective To explore the multiple risk factors for family lifestyle of children with congenital heart defects(CHDs)in Shaanxi Province,China.Methods A 1∶1 case-control study was carried out to investigate 60 pairs of children and their parents.The univariate and multivariable logistic regression models were used to analyze the influence of risk factors related to parents’ lifestyle on CHDs.Results Several possible risk factors were found for CHDs,including fever(OR=4.465,P=0.017),pesticides contact(OR=2.234,P=0.083),passive smoking during pregnancy(OR=20.529,P=0.007)and father’s smoking(OR=3.342,P=0.005);fever(OR=2.428,P=0.012)and passive smoking during pregnancy(OR=1.201,P=0.037)were also correlated with ventricular sepal defect(VSD).Conclusion Fever,pesticides contact and passive smoking are associated with CHDs during pregnancy.We should focus our attention on health care during pregnancy to avoid the above-mentioned risk factors and call on parents to hold on to a good healthy lifestyle.

Association between Maternal Drug Use and Cytochrome P450 Genetic Polymorphisms and the Risk of Congenital Heart Defects in Offspring

Objective This study aimed to assess the associations between maternal drug use,cytochrome P450(CYP450)genetic polymorphisms,and their interactions with the risk of congenital heart defects(CHDs)in offspring.Methods A case-control study involving 569 mothers of CHD cases and 652 controls was conducted from November 2017 to January 2020.Results After adjusting for potential confounding factors,the results show that mothers who used ovulatory drugs(adjusted odds ratio[a OR]=2.12;95% confidence interval[CI]:1.08-4.16),antidepressants(a OR=2.56;95%CI:1.36-4.82),antiabortifacients(a OR=1.55;95%CI:1.00-2.40),or traditional Chinese drugs(a OR=1.97;95%CI:1.26-3.09)during pregnancy were at a significantly higher risk of CHDs in offspring.Maternal CYP450 genetic polymorphisms at rs1065852(A/T vs.A/A:OR=1.53,95%CI:1.10-2.14;T/T vs.A/A:OR=1.57,95%CI:1.07-2.31)and rs16947(G/G vs.C/C:OR=3.41,95%CI:1.82-6.39)were also significantly associated with the risk of CHDs in offspring.Additionally,significant interactions were observed between the CYP450 genetic variants and drug use on the development of CHDs.Conclusions In those of Chinese descent,ovulatory drugs,antidepressants,antiabortifacients,and traditional Chinese medicines may be associated with the risk of CHDs in offspring.Maternal CYP450 genes may regulate the effects of maternal drug exposure on fetal heart development.

Prenatal congenital heart defects in southern China:detection rate and termination of preqnancy,2006-2016

Objective Congenital heart defect (CHD) is one of the most common birth anomalies with high morbidity and mortality. Previous studies of CHD mostly focus on the postnatal prevalence, mortality and successful rate of operation, etc. This study aimed to explore the detection rate of prenatal CHD and CHD factors that attribute to termination of pregnancy (TOP).

Interaction between ozone and paternal smoking on fetal congenital heart defects among pregnant women at high risk a multicenter maternal-fetal medicine study

Background Evidence remains limited on the association between maternal ozone(O,)exposure and congenital heart defects(CHDs)in offspring,and few studies have investigated the interaction and modification of paternal smoking on this association.Methods Using a sample including pregnant women at high risk of fetal CHD(with metabolic disease,first-trimester viral infection,family history of CHD,etc.)from a maternal-fetal medicine study covering 1313 referral hospitals in China during 2013-2021,we examined the associations between maternal O_(3)exposure during 3-8 weeks of gestational age and fetal CHD in offspring and investigated the interaction and modification of paternal smoking on this association.CHD was diagnosed by fetal echocardiograms,maximum daily 8-hour average O,exposure data at a 10 km×10 km spatial resolution came from the Tracking Air Pollution in China dataset,and paternal smoking was collected using questionnaires.Logistic regression models were used to estimate adjusted odds ratios(ORs)and 95%confidence intervals(CIs).Results Among 27,834 pregnant women at high risk of fetal CHD,17.4%of fetuses were diagnosed with CHD.Each 10μg/m^(3)increase in maternal O_(3)exposure was associated with a 17%increased risk of CHD in offspring(OR=1.17,95%CI=1.14-1.20).Compared with paternal nonsmoking and maternal low O_(3)exposure,the ORs(95%CI)of CHD for smoking and low O_(3)exposure,nonsmoking and high O_(3)exposure,and smoking and high O_(3)exposure were 1.25(1.08-1.45),1.81(1.56-2.08),and 2.23(1.84-2.71),respectively.Paternal smoking cessation seemingly mitigated the increased risk of CHD.Conclusions Maternal O_(3)exposure and paternal smoking were interactively associated with an increased risk of fetal CHD in offspring,which calls for effective measures to decrease maternal exposure to O_(3)pollution and secondhand smoke for CHDprevention.

Implementation of a High-Risk Outpatient Clinic for Children with Complex Congenital Heart Disease in a Reference Service in Brazil

Background:Children with congenital heart disease(CHD),even after surgical approaches,and especially those who undergo staged procedures in thefirst months of life,remain vulnerable to readmissions and complications,requiring very close monitoring and differentiated intervention strategies.Methods:Descriptive and exploratory study,of the experience report type,which presents the process of building the high-risk outpatient clinic for complex congenital heart diseases(AAR)at the Instituto do Coração(InCor).Results:Report of the path taken to structure the AAR,demonstrating the organization,interface with the multidisciplinary team,admission and discharge criteria,training,and patient profile.In thesefive years of care,275 patients were treated,59.65%with biventricular interstage physiology,followed by univentricular interstage physiology(34.55%),residual defects after surgical procedures(3.63%),tumors with risk of mechanical obstruction(1.45%)and patients with an intrauterine approach(0.72%).The significant number of critical patients who were successfully discharged from hospital(44.72%),the low mortality rate due to sudden deaths at home and the high adherence to follow-up cor-roborate the impact of this specialized assistance.Conclusions:Despite the limitations of the study,this experi-ence report showed that with few resources,there is the possibility of organizing an AAR with differentiated care,with the objective of early detection and treatment of residual injuries,identification of early interventions,edu-cation of parents for follow-up of their children,resulting in individualized treatment,promoting a better quality of life for this population.

Right Axillary Thoracotomy Should Be the Standard of Care for Repair of Non-Complex Congenital Heart Defects in Infants and Children

Minimally invasive approaches for cardiac surgery in children have been lagging in comparison to the adult world.A wide range of the most common congenital heart defects in infants and children can be repaired suc-cessfully through a variety of non-sternotomy incisions.This has been shown to be associated with superior cos-metic results,shorter hospital stays,and rapid return to full activity compared to sternotomy.These approaches have been around for decades,but they have not been widely adopted for a variety of reasons.Right axillary thor-acotomy is one of these approaches that we believe should be the new standard for the repair of a wide variety of heart defects in children and will be the focus of our current review.

Prenatal Diagnosis of an Apically Located Congenital Left Ventricular Aneurysm: A Rare Case

Congenital ventricular aneurysm is a very rare cardiac anomaly.A diagnosis can be made during the prenatal period using fetal echocardiography.This study presents a very rare apically located left ventricular aneurysm case,and the relevant literature was reviewed and discussed.In this case,a 35-year-old,gravida 2,parity 1 preg-nant woman at 24 weeks of gestation,displayed a wide aneurysmal image in the left ventricular apical wall on fetal echocardiography.There was a 1.79 mm muscular ventricular septal defect at the apical region of the interven-tricular septum.In the course of the color Doppler ultrasonography examination,an aberrantfibrous band within the left ventricle and consequent turbulentflow during systole were observed.The baby,born via cesarean section at 37 weeks of gestation,is now in its postnatal seventh month.However,during echocardiographic follow-ups,changes have been observed,including mild to moderate mitral insufficiency and a decrease in systolic function.Despite thesefindings,the clinical condition remains asymptomatic.It is of great importance to use a multidis-ciplinary approach in managing these rare cases that could lead to potential adverse outcomes during the antena-tal or postnatal periods.

Congenital heart defects through 30 years

Aim: To assess basic trends in epidemiology of congenital heart defects (CHDs). Method: Population based prospective observational study. Material: CHDs in infants born alive in a Norwegian county 1982-2011. Results: In 828/71 217 infants (12 per 1000) a CHD was diagnosed. The prevalence increased from 8 to 12 per 1000 after introduction of early echocardiography in newborns with suspected CHD from 1986 (p = 0.0001). Ventricular septal defect (VSD) was the dominating CHD (474;57%). In 222 (27%) the CHDs were missed and diagnosed after discharge from hospital after birth. Twelve critical CHDs (causing death or requiring invasive procedures before 28 days of life) were missed. Prenatal diagnosis of critical CHDs increased from 4/67 (6%) born 1997-2006 to 4/11 (36%) born 2007-2011 (p = 0.01). In 177 (21%) a syndrome or extracardial defect occurred. The occurrence of CHDs associated with chromosomal disorders (60/73 (82%) trisomies) more than doubled from the cohort born in the first 10-year period 1982-1991 (0.6 per 1000) to the last 2002-2011 (1.4 per 1000) (p < 0.0001) in parallel with increasing births in women ≥ 35 years old in the population. 237 (29%) underwent therapeutic procedures (203 (86%) surgery, of whom 16 after initial catheter intervention, and 34 (14%) catheter intervention alone). 39/237 (16%) died, 101 (43%) were repaired and 97 (41%) had some minor residual defect. The death rate declined significantly from 65/532 (12%) born 1982-2001 to 11/296 (4%) born 2002-2011 (p = 0.0001). 37/76 (49%) deaths occurred within 28 days after birth. Conclusions: The rate of detection of CHDs increased substantially after introduction of echocardiography in newborns with suspected CHD, especially VSDs. Some critical CHDs were overlooked. The prenatal detection rate of such defects increased. The prevalence of CHDs with associated chromosomal disorders increased in parallel with increasing maternal age in the population. Most deaths occurred during the neonatal period. Increasing survival increases the load on long-term follow-up programmes.

Genetic Analysis of Variants of the MYH6 Gene Promoter in Congenital Atrial Septal Defects

Background:Atrial septal defect(ASD)is one of the common congenital heart diseases.The MYH6 gene has a critical role in cardiac development but the role of MYH6 promoter variants in patients with ASD has not been explored.Methods:In 613 subjects including 320 ASD patients,we investigated the MYH6 gene promoter variants and verified the effect on gene expression by using cellular functional experiments and bioinformatics analysis.Results:Eleven variants were identified in the MYH6 gene promoter,of which four variants were found only in ASD patients,and two variants(g.3434G>C and g.4524C>T)were identified for the first time.Cellular functional experiments indicated that all four variants reduced the transcriptional activity of the MYH6 gene promoter(p<0.05).Subsequent analysis through the JASPAR(A database of transcription factor binding profiles)suggests that these variants may alter transcription factor binding sites,which may in turn lead to changes in myocardin subunit expression and ASD formation.Conclusions:Our study for the first time focuses on variants in the promoter region of the MYH6 gene in Chinese patients with ASD and the discovered variants have functional significance.The study provides new insights in the role of the MYH6 gene promoter region to better understand the genetic basis of ASD formation and facilitates clinical diagnosis.

The associations between resilience and socio-demographic factors in parents who care for their children with congenital heart disease

Objective To examine the resilience of parents of children with congenital heart disease and to investigate socio-demographic factors that may influence parents’resilience.Methods This is a web-based survey study using a cross-sectional design.A purposive sampling method was utilized to recruit 515 parents who care for children with congenital heart disease.Resilience was assessed using the Dispositional Resilience Scale-Ⅱ.Based on expert-interviews,a questionnaire was designed to collect socio-demographic data.Descriptive statistics,factor analysis,and linear regressions were used to analyze data.Results A total of 413 parents completed the survey study.The mean resilience score was 3.75(SD=0.61;range=1.89–4.89)with higher scores indicating higher resilience.The linear regression models demonstrated that parents who had lower education levels and lower gross household income had lower resilience(P<0.05).Conclusions Parents reported resilience that reflected their ability to cope with stressful events and mitigate stressors associated with having and caring for children with congenital heart disease.Lower education levels and lower gross household income are associated with lower resilience.To increase parents’resilience,nursing practice and nurse-led interventions should target screening and providing support for parents at-risk for lower resilience.As lower education level and financial hardship are factors that are difficult to modify through personal efforts,charitable foundations,federal and state governments should consider programs that would provide financial and health literacy support for parents at-risk for lower resilience.

Genetics of congenital heart defects in DiGeorge syndrome

Background Di George syndrome（DGS） is the most common microdeletion syndrome in humans and a disorder caused by a defect in chromosome 22. Almost 80% of DGS patients manifest congenital heart defects（CHD）, which are highly variable and severe. However, the genetics of CHD in DGS remain elusive. This review concludes that the TBX1 gene plays a critical role in cardiovascular defects, involving many additional genes, such as Six1, Eya1, Fgf8, Fox, and Shh. Concerning the variable manifestations of CHD in DGS,additional modifiers have been shown of involvement, such as Wnt, MOZ, micro RNAs, VEGF, and CRK.Knowledge of the genetics underlying CHD in DGS has the potential to early detection and treatment of this disease.

Factors Affecting the Genetic Diagnostic Rate in Congenital Heart Disease

Background: Over 400 genes contribute to the development of congenital heart disease (CHD). Additionally,multisystemic manifestations accompanying syndromic CHD pose a higher risk of genetic diseases. This studyinvestigated the diagnostic yield of whole-exome sequencing (WES) in patients with sporadic syndromic CHDand the phenotypic factors affecting the genetic diagnostic rate. Methods: Sixty-four patients with sporadic syndromicCHD aged <18 years underwent WES between May 2018 and December 2020 in a single tertiary center,and the association between genetic testing data and extracardiac phenotypes was analyzed. Results: Extracardiacphenotypes were measured as 3.66 ± 3.05 (standard deviation, interquartile range: 2–5) items per patient. WESdetected diagnostic variants in 19 (29.7%) patients: seven (36.8%), seven (36.8%), and five (26.3%) with pathogenicvariants, likely pathogenic variants, and variants of unknown significance, respectively. Post-diagnosis surveillanceidentified the extracardiac phenotype in 54.5% (6/11) of patients. De novo variants accounted for 76.2%(15/19) of variants and autosomal dominant inheritance for 94.7% (18/19). Most diseases were ultra-rare. No significantdifferences were noted in cardiac and extracardiac phenotypes, single or combined (all P > 0.05), betweenthe groups with and without a diagnostic variant. However, patients with ≥3 extracardiac phenotypes had a significantlyhigher likelihood of having a diagnostic variant than those with ≤2 (38.3% vs. 5.9%, odds ratio = 9.93,95% confidence interval = 1.21–81.44, P = 0.013). Conclusions: The number of extracardiac phenotypes is importantin predicting the possibility of genetic diagnosis. Physicians will be able to select patients with a high probabilityof genetic diagnosis and provide appropriate genetic counseling based on the results of this study.

Lesion-based Patterns of Morbidity and Mortality in Hospitalized Adolescents with Congenital Heart Disease

Objective:The objective of this analysis is to describe the characteristics and morbidity during hospitalizations among adolescents with congenital heart disease(AdoCHD)from the Pediatric Health Information System(PHIS)database.Methods:The PHIS database was queried for all AdoCHD admissions aged 12–18 years(1/1/2004–12/31/2013).Major forms of CHD were identified by their International Classification of Diseases,ninth revision codes,further verified based on their secondary diagnosis and/or procedure codes.Patient characteristics,diagnoses,procedures and vital status were assessed.Results:In total,there were 4,267 adolescents admitted to 42 Children’s Hospitals,58.3%were males,24.6%single ventricle(SV)patients,64.1%bi-ventricle(BV),and 11.3%could not be classified.They accounted for 8,512 hospitalizations(41,240 total hospital days),of which 31.6%were intensive care unit(ICU)stays.ICU stay was similar for the SVand BV patients with similar duration of mechanical ventilation between the two groups.Overall,the most common CHD among in-patients was tetralogy of Fallot(TOF,36.4%).Larger proportion of the BVAdoCHD admissions were for elective surgical and electrophysiological procedures.There were 109(2.5%)heart transplantations(1.3%SV vs.0.6%BV)and 120 in-hospital deaths(2.8%)(1.1%SV vs.1.3%BV).Hypoplastic left heart syndrome was the most common diagnosis in transplanted patients(46%)and those who died(28%);TOF(29%)was frequent in 91(2.1%)patients who had cardiac arrests.Conclusions:Different hospitalization patterns exist for BV and SV AdoCHD.Recognizing this risk may encourage directing resources toward optimizing long-term care of CHD patients.

Characteristics of In-Hospital Patients with Congenital Heart Disease Requiring Rapid Response System Activations: A Japanese Database Study

Objectives:This study aimed to study the characteristics of in-hospital deterioration in patients with congenital heart disease who required rapid response system activation and identify risk factors associated with 1-month mortality.Methods:We retrospectively analysed data from a Japanese rapid response system registry with 35 participating hospitals.We included consecutive patients with congenital heart disease who required rapid response system activation between January 2014 and March 2018.Logistic regression analyses were performed to examine the associations between 1-month mortality and other patient-specific variables.Results:Among 9,607 patients for whom the rapid response system was activated,only 82(0.9%)had congenital heart disease.Only few patients with congenital heart disease were being treated at the cardiology and cardiovascular surgery departments(12.3%and 9.9%,respectively).Moreover,the incidences of rapid-response events after intensive care unit discharge or surgery were low(6.8% and 12.2%,respectively).The most common reason for rapid response system activation was respiratory dysfunction(desaturation:35.4%,tachypnoea:25.6%,and new dyspnoea:19.5%).Rapid response system interventions and intensive care unit transfers were required for 65.9% and 20.7%of patients,respectively.The mortality rate was 1.2% at the end of the rapid response system intervention and 11.0% after 1 month.Moreover,decreased respiratory rate and decreased heart rate at rapid response system activation were associated with increased 1-month mortality.The adjusted odds ratio was 1.10(95% confidence interval 1.02–1.19)and 1.02(95% confidence interval,1.00–1.04) for respiratory rate and heart rate,respectively.Conclusions:Rapid response systems were rarely activated after cardiac surgery and intensive care unit discharge,which were situations with a high risk of sudden deterioration in patients with congenital heart disease.Therefore,encouraging the use of the rapid response system in these departments will enable intervention by a third,specialised team for in-hospital emergencies and help provide comprehensive medical care to patients.Furthermore,1-month mortality was associated with vital signs at rapid response system activation.These findings may guide treatment selection for patients with congenital heart disease showing deterioration.