Congenital Heart Disease Database Research Articles

Risk-prediction nomogram for congenital heart disease in offspring of Chinese pregnant women

BackgroundThe identification and assessment of environmental risks are crucial for the primary prevention of congenital heart disease (CHD). We were aimed to establish a nomogram model for CHD in the offspring of pregnant women and validate it using a large CHD database in Northwest China.MethodsA survey was conducted among 29,204 women with infants born between 2010 and 2013 in Shaanxi province, Northwest China. Participants were randomly assigned to the training set and to the validation set at a ratio of 7:3. The importance of predictive variables was assessed using random forest. A multivariate logistic regression model was used to construct the nomogram for the prediction of CHD.ResultsMultivariate analyses revealed that the gravidity, preterm birth history, family history of birth defects, infection, taking medicine, tobacco exposure, pesticide exposure and singleton/twin pregnancy were significant predictive risk factors for CHD in the offspring of pregnant women. The area under the receiver operating characteristic curve for the prediction model was 0.716 (95% CI: 0.671, 0.760) in the training set and 0.714 (95% CI: 0.630, 0.798) in the validation set, indicating moderate discrimination. The prediction model exhibited good calibration (Hosmer-Lemeshow χ2 = 1.529, P = 0.910).ConclusionsWe developed and validated a predictive nomogram for CHD in offspring of Chinese pregnant women, facilitating the early prenatal assessment of the risk of CHD and aiding in health education.

HART: Deep learning-informed lifespan heart failure risk trajectories

BackgroundHeart failure (HF) results in persistent risk and long-term comorbidities. This is particularly true for patients with lifelong HF sequelae of cardiovascular disease such as patients with congenital heart disease (CHD). PurposeWe developed hART (heart failure Attentive Risk Trajectory), a deep-learning model to predict HF trajectories in CHD patients. MethodshART is designed to capture the contextual relationships between medical events within a patient’s history. It is trained to predict future HF risk by using the masked self-attention mechanism that forces it to focus only on the most relevant segments of the past medical events. ResultsTo demonstrate the utility of hART, we used a large cohort containing healthcare administrative data from the Quebec CHD database (137,493 patients, 35-year follow-up). hART achieves an area under the precision-recall of 28% for HF risk prediction, which is 33% improvement over existing methods. Patients with severe CHD lesion showed a consistently elevated predicted HF risks throughout their lifespan, and patients with genetic syndromes exhibited elevated HF risks until the age of 50. The impact of the birth condition decreases on long-term HF risk. The timing of interventions such as arrhythmia surgery had varying impacts on the lifespan HF risk among the individuals. Arrhythmic surgery performed at a younger age had minimal long-term effects on HF risk, while surgeries during adulthood had a significant lasting impact. ConclusionTogether, we show that hART can detect meaningful lifelong HF risk in CHD patients by capturing both long and short-range dependencies in their past medical events.

CanCHD Study of Hematopoietic Cancers in Children With and Without Genetic Syndromes.

Individuals with genetic syndromes can manifest both congenital heart disease (CHD) and cancer attributable to possible common underlying pathways. To date, reliable risk estimates of hematopoietic cancer (HC) among children with CHD based on large population-based data remain scant. This study sought to quantify the risk of HC by the presence of genetic syndrome among children with CHD. Data sources were the Canadian CHD database, a nationwide database on CHD (1999-2017), and the CCR (Canadian Cancer Registry). Standardized incidence ratios were calculated for comparing HC incidences in children with CHD withthe general pediatric population. A modified Kaplan-Meier curve was used to estimate the cumulative incidence of HC with death as a competing risk. A total of 143 794 children (aged 0-17 years) with CHD were followed up from birth to age 18 years for 1 314 603 person-years. Of them, 8.6% had genetic syndromes, and 898 HC cases were observed. Children with known syndromes had a substantially higher risk of incident HC than the general pediatric population (standardized incidence ratio, 13.4 [95% CI, 11.7-15.1]). The cumulative incidence of HC was 2.44% (95% CI, 2.11-2.76) among children with a syndrome and 0.79% (95% CI, 0.72-0.87) among children without a syndrome. Acute myeloid leukemia had a higher cumulative incidence during early childhood than acute lymphoblastic leukemia. This is the first large population-based analysis documenting that known genetic syndromes in children with CHD are a significant predictor of HC. The finding could be essential in informing risk-stratified policy recommendations for cancer surveillance in children with CHD.

Mid-term results following pulmonary artery patch augmentation in congenital heart disease.

Treatment of pulmonary artery (PA) stenosis in congenital heart disease is associated with adverse outcomes. The aim of this retrospective cohort study was to compare outcomes after surgical patch augmentation of PA stenosis in patients with biventricular congenital heart disease using different patch materials. We identified all patients from our institutional congenital heart disease database who underwent patch augmentation for PA stenosis on the main pulmonary artery (MPA) or PA branches between 2012 and 2018. Patch materials used were glutaraldehyde fixated autologous pericardium (AP), expanded polytetrafluoroethylene (ePTFE), equine pericardium (EP), and bovine pericardium (BP). The primary study endpoint was the composite of catheter-based re-intervention or re-operation to relieve recurrent stenosis at the site of prior implanted patch material. A total of 156 patients (median age, 5 months, range, 0-85 months; median weight, 6.2 kg, range, 2.8-15.0 kg) underwent patch augmentation using 163 patches (ePTFE =99, 61%; EP =34, 21%; AP =25, 15%; BP =5, 3%). Overall, 131 (84%) patients underwent patch augmentation at the MPA, and 25 (16%) patients underwent patch augmentation at one or both PA branches. Over a mean follow-up period of 4±2 years, 30 patients (19%) reached the study endpoint. Freedom from primary endpoint was 92%±3% for the MPA and 25%±9% for PA branches at 5 years, respectively (P<0.001). Comparison of patch materials revealed similar re-intervention rates between ePTFE, AP, and EP. In contrast, outcomes were significantly decreased following the usage of BP when compared to other materials (ePTFE vs. BP, P=0.01; EP vs. BP, P=0.005). In the multivariable analysis, lower weight at index operation, patch augmentation of PA branches, and usage of BP were independently associated with re-intervention. Patch augmentation of the MPA was associated with acceptable outcomes, while patch augmentation of PA branch stenosis remained independently associated with re-intervention. None of the used patch materials demonstrated superiority; however, BP had a higher rate of re-interventions.

Severe Fontan-Associated Liver Disease and Its Association With Mortality.

Background Data are rare about the incidence of severe Fontan-associated liver disease (FALD) and its association with mortality. We sought to: (1) estimate the probability of developing severe FALD in patients who undergo the Fontan procedure (Fontan patients), compared with severe liver complications in patients with a ventricular septal defect; (2) assess the severe FALD-mortality association; and (3) identify risk factors for developing severe FALD. Methods and Results Using the Quebec Congenital Heart Disease database, a total of 512 Fontan patients and 10 232 patients with a ventricular septal defect were identified. Kaplan-Meier curves demonstrated significantly higher cumulative risk of severe FALD in Fontan patients (11.95% and 52.24% at 10 and 35 years, respectively), than the risk of severe liver complications in patients with a ventricular septal defect (0.50% and 2.75%, respectively). At 5 years, the cumulative risk of death was 12.60% in patients with severe FALD versus 3.70% in Fontan patients without FALD (log-rank P=0.0171). Cox proportional hazard models identified significant associations between the development of severe FALD and congestive heart failure and supraventricular tachycardia, with hazard ratios (HRs) of 2.36 (95% CI, 1.38-4.02) and 2.45 (95% CI, 1.37-4.39), respectively. More recent Fontan completion was related to reduced risks of severe FALD, with an HR of 0.95 (95% CI, 0.93-0.97) for each more recent year. Conclusions This large-scale population-based study documents that severe FALD in Fontan patients was associated with a >3-fold increase in mortality. The risk of FALD is time-dependent and can reach >50% by 35 years after the Fontan operation. Conditions promoting poor Fontan hemodynamics were associated with severe FALD development.

Abstract 12585: Flexible Modeling of the Association Between Cumulative Exposure to Low-Dose Ionizing Radiation From Cardiac Procedures and Risk of Hematopoietic Cancer in Children With Congenital Heart Disease

Background: High-dose ionizing radiation is a well-established risk factor for childhood malignancies, including hematopoietic cancers (HC). However, data on the effect of low-dose ionizing radiation (LDIR) from medical imaging is conflicting and scant, especially in the pediatric population with congenital heart diseases (CHD). This study evaluated the association between cardiac LDIR exposure and hematopoietic cancers among children with CHD. Methods: A nationwide population-based cohort study was conducted using the Canadian Congenital Heart Disease (CanCHD) database. The study population included children born between 1999 and 2017 with at least one CHD diagnosis in their medical records. The cumulative dose of ionizing radiation corresponding to cardiac diagnostic and therapeutic procedures was quantified considering a 6-month exposure lag. The recency-weighted cumulative exposure (WCE) model, a flexible extension of Cox’s proportional hazards model, was used to assess the association. Results: We identified 139,975 children with CHD born between 1999 and 2017 and followed them for 1,388,681 person-years since birth. In this population, 718 hematopoietic cancer cases were observed. Children with HC were exposed to low-dose ionizing radiation earlier in life (median age at first exposure: 6 vs. 10 months; p=0.03) and had more procedures than those without cancer (mean number of procedures: 0.4 vs. 0.2; p&lt;0.001). The cases received higher cumulative LDIR doses than their counterparts (mean dose: 2.3 vs. 1.1 mSv; p&lt;0.001). We observed that cumulative LDIR doses within five years were associated with increased risk of hematopoietic cancer with the maximum association magnitude around 2 years. Conclusion: This is the first large population-based study documenting increased risk of HC associated with increased dose and recency of the LDIR exposure among children with CHD. Along with these findings, future studies focusing on detecting a threshold effect will help physicians decide the exposure point at which increased surveillance on LDIR exposure should be initiated.

End-of-life and palliative care provision to adults with congenital heart disease: mortality follow-back study using administrative data

Abstract Background Although many adults with congenital heart disease (CHD) still die prematurely, end-of-life care for these patients receives limited attention. There are indications that current care provision at the end of life is burdensome, expensive, and not in line with patients' needs and preferences. We sought to analyse end-of-life care in adult CHD patients to determine whether health services need to be optimized. Purpose This study aimed to describe patterns of healthcare consumption of adults with CHD who died in the last year of life. Methods This retrospective mortality follow-back study used data of the BELgian COngenital heart disease Database combining Administrative and Clinical data (BELCODAC), including individually linked healthcare claims, death certificates and clinical data from adults with CHD in Flanders (Belgium). For this study, adults with CHD who died between 2007 and 2016 from any cause except sudden death, accident or violence, were selected for inclusion. Accidental, violent, and sudden deaths were identified based on causes of death and healthcare use in the last 3 months of life. Healthcare consumption was based on nomenclature codes derived from healthcare claims data. Results A total of 327 eligible patients (median age: 58 y; 54% women; 43% mild CHD; 45% moderate CHD; 11% complex CHD; 49% cardiovascular cause of death) were identified. During the last year of life, healthcare use increased substantially (Fig. 1). During the last month of life, 54% of patients were hospitalised, 55% visited the emergency department, and 15% were admitted to an intensive care unit at least once (Fig. 2). A total of 8% and 5% of patients underwent heart surgery or catherization in the last month of life, respectively. Furthermore, 70% of patients had at least one encounter with a general practitioner and 11% with a CHD specialist in the last month of life. Specialist palliative care was provided to 13% of patients in the last month of life. When looking at the subgroup of patients with CHD that died due to a cardiovascular cause, proportions of patients that were hospitalised or had visits at the emergency department or intensive care unit in the last month of life were similar (Fig. 2). However, these patients underwent more heart surgeries (11%) and catherizations (8%), had more encounters with CHD specialists (15%), and received remarkably less specialized palliative care (4%) in the last month of life. Conclusion Resource utilization increased substantially during the last year of life, resulting in high acute healthcare consumption in the last month of life. It is remarkable that only a minority of patients received palliative care, especially when looking at patients who died due to a cardiovascular cause. Our findings motivate the need to assess if and how end-of-life is planned for adults with CHD. Future studies using qualitative analyses and survey methodology are needed to optimize the management of end-of-life care. Funding Acknowledgement Type of funding sources: Public grant(s) – EU funding. Main funding source(s): Research Foundation Flanders, European Society of Cardiology, Koning Boudewijnstichting, National Foundation on Research in Pediatric Cardiology, Swedish Research Council for Health, Working Life and Welfare-FORTE

Healthcare use at the end of life of patients with congenital heart disease: does heart failure matter?

Abstract Funding Acknowledgements Type of funding sources: Foundation. Main funding source(s): Research Foundation Flanders (to PM, EG, and LVB) European Society of Cardiology (Nursing Training Grant to LVB) Background Heart failure (HF) is a common cause of morbidity and mortality in patients with congenital heart disease (CHD). Although limited in scope, previous studies suggest that patients with heart failure follow a specific end-of-life trajectory with episodes of serious complications, which may impact the patterns of care as death approaches. Aims The study aims to identify differences in characteristics and patterns of care in the last year of life in deceased CHD patients with and without HF. Methods This retrospective study used data of deceased adult patients included in the BELgian COngenital heart disease Database combining Administrative and Clinical data (BELCODAC). To describe patterns of care in the last year of life, we captured information about hospitalisations, emergency department visits, and visits to the general practitioner using nomenclature codes. Heart failure was identified as having HF as cause of death and/or at least one prescription of a loop diuretic in the last year of life. Sensitivity analyses with a stricter definition for HF (HF as cause of death or ≥ 1 prescription of a loop diuretic combined with a prescription of digoxin, dopamine, dobutamine, other non-glycoside stimulants, metoprolol, bisoprolol, carvedilol, aldosterone antagonists, ACE inhibitors or ARBs) were performed as well. Results During the period 2007–2016, 390 adults with CHD died, of which 170 patients with HF (44%). Patients with HF were older, died more often due to a cardiovascular cause of death, and had more complex heart lesions, compared to patients without HF (Table 1). While the number of emergency department visits and hospitalisations in the last year was similar, patients with HF had almost twice as much monthly visits at the general practitioner in their last year of life (Table 1). As shown in Figure 1, the mean number of hospitalisations and emergency department visits increased in a similar fashion throughout the last year of life, but the pattern of general practitioner visits was substantially different for patients with and without HF. The sensitivity analyses, in which a stricter definition for HF was used, yield very similar results. In these analyses, the difference in mean monthly hospitalisations was also significant between the two groups. Conclusions This study shows clinically important differences in characteristics and patterns of care of deceased patients with CHD with and without heart failure. Patients with HFhave different needs and should receive a tailored approach at the end of life. Future research is needed to understand these differences and investigate these patients' end-of-life care needs in more detail. Funding acknowledgments: This work was supported by Research Foundation Flanders; European Society of Cardiology; the King Baudouin Foundation; the National Foundation on Research in Pediatric Cardiology; and the Swedish Research Council for Health, Working Life and Welfare-FORTE.

Impact of delayed sternal closure on wound infections following neonatal and infant cardiac surgery.

Delayed sternal closure is a routine procedure to reduce hemodynamic and respiratory instability in pediatric patients following cardiac surgery, particularly in neonates and infants. In this setting, the possible links between sternal wound infection and delayed sternal closure are still a matter of debate. As a part of our routine, there was a low threshold for delayed sternal closure, so we reviewed our experience with sternal wound infections with a focus on potentially related perioperative risk factors, particularly delayed sternal closure. We retrospectively identified 358 operated neonates (37%) and infants (mean age 3.6 months) in our local congenital heart disease database between January 2013 and June 2017. Potential risk factors for sternal wound infections, such as age, gender, complexity (based on Aristotle- and STS-EACTS mortality category), reoperation, use of cardiopulmonary bypass, extracorporeal membrane oxygenation, mortality and delayed sternal closure (163/358, 46%), were subjected to uni- and multivariate analysis. A total of 26/358 patients (7.3%) developed a superficial sternal wound infection. There were no deep sternal wound infections, no mediastinitis or sepsis. Applying univariate analysis, the prevalence of sternal wound infections was related to younger age, more complex surgery and delayed sternal closure. However, in multivariate analysis, sternal wound infection was only associated with delayed sternal closure (p = 0.013, odds ratio 8.6). Logistic regression revealed the prevalence of delayed sternal closure to be related to younger age, complexity, and the use of extracorporeal membrane oxygenation. In patients younger than one year, sternal wound infections are clearly related to delayed sternal closure. However, in our cohort, all sternal wound infections were superficial and acceptable, considering the improved postoperative hemodynamic stability.

Prevalence of Congenital Heart Disease in Chinese Children With Different Birth Weights and Its Relationship to the Neonatal Birth Weight.

ObjectiveThis study aimed to examine the prevalence and the related risk factors of congenital heart disease (CHD) in children with different birth weights in China and the relationship between the subtypes of CHD and birth weight (BW).MethodsThis study conducted a cross-sectional survey on the data collected in the children’s congenital heart disease database (CHDD) established in China. This database contained data from one Grade A, Level III Children’s Public Hospital in Zhengzhou, Henan. The study included all the children and their parents in the database from 2014 to 2020 as the study subjects, and the missing data were processed by means of imputation. Diagnoses of CHD were coded using the International Classification of Diseases version 10 (ICD-10), and subtypes were classified by the codes Q20 to Q26. We reported the prevalence of CHD based on birth weight and gestational age and analyzed the related risk factors for children with CHD in different birth weight groups and factors for children of the same birth weight groups between the CHD groups and the non-CHD groups. The generalized linear model was used to assess the association between the subtypes of CHD and BW by establishing three adjusting models, and the data were stratified for further analysis by urban-rural and infant gender.ResultsA total of 42,814 children were identified as having CHD among 5,071,799 live children; the overall prevalence of CHD was 8.44 per 1,000 live births during 2014–2020; and the three subtypes with the highest prevalence of CHD were atrial septal defect (ASD) (2.75‰), ventricular septal defect (VSD) (2.57‰), and patent foramen ovale (PFO) (1.12‰). The prevalence of CHD was 18.87‰ in the group with BW <1,500 g, 12.84‰ in the group with BW 1,500–2,500 g, 8.24‰ in the group with BW 2,500–4,000 g, and 4.80‰ in the group with BW ≥4,000 g. The prevalence of CHD was 16.62‰ in the small for gestational age (SGA) group, 6.99‰ in the appropriate for gestational age (AGA) group, and 6.40‰ in the larger for gestational age (LGA) group. Parental factors such as drinking, smoking, viral infections, peri-pregnancy exposure to radioactive substances, low family monthly expenditure, and low Apgar scores at 1 and 5 min were related to the increased risk of CHD in the offspring. Parental supplementation of folic acid and exercise during the peri-pregnancy period could reduce the risk of CHD in the offspring. The results of Model 3 adjusting for confounding variables showed that infants with ASD had a birth weight 461 g lower (95% CI: −1,085, –128), infants with VSD had a birth weight 426 g lower (95% CI: –932, –120), infants with tetralogy of Fallot (TOF) had a birth weight 532 g lower (95% CI: –987, –168), and without classification, infants with CHD had a birth weight 973 g lower (95% CI: –1,502, –204).ConclusionIn very low birth weight (VLBW) and low birth weight (LBW) infants, CHDs are more prevalent than in the general live-born population. Moreover, some peri-pregnancy factors of parents are closely related to the occurrence of CHD in offspring; different types of heart defects can lead to LBW. Therefore, if the fetus is found to have a heart defect during the prenatal examination, the mother should pay more attention to maintaining weight and ensuring that the fetus is within the normal weight range, thereby increasing the postpartum survival rate, reducing complications, and promoting children’s health.

Operational and Ethical Considerations for a National Adult Congenital Heart Disease Database.

As more adults survive with congenital heart disease, the need to better understand the long‐term complications, and comorbid disease will become increasingly important. Improved care and survival into the early and late adult years for all patients equitably requires accurate, timely, and comprehensive data to support research and quality‐based initiatives. National data collection in adult congenital heart disease will require a sound foundation emphasizing core ethical principles that acknowledge patient and clinician perspectives and promote national collaboration. In this document we examine these foundational principles and offer suggestions for developing an ethically responsible and inclusive framework for national ACHD data collection.

Hospital discharge codes and substantial underreporting of congenital heart disease

BackgroundHospital discharge codes are relied upon for research, accounting/invoicing and health systems planning. Congenital heart disease (CHD), however, is uniquely difficult for non-cardiologists to code due to the rarity, variety and complexity of lesions. It is therefore important that the accuracy of hospital discharge codes is regularly checked to ensure that the prevalence and burden of CHD is being correctly estimated and recorded. Methods and resultsWe identified all inpatient admissions of adults with CHD to Royal Prince Alfred Hospital in Sydney, Australia from January 2018 to March 2021 (257 admissions, 106 unique patients). The associated discharge coding summaries were extracted and compared to the codes in the separately collected and audited Adult CHD database. Only a quarter of discharge coding summaries contained any diagnosis of CHD, and just one-tenth accurately recorded all appropriate CHD diagnoses. Patients with simple lesions were most likely to have a coded diagnosis of CHD, while those with moderate and complex lesions were much less likely. Moreover, patients admitted under a cardiovascular specialty were twice as likely to have a coded diagnosis of CHD, compared with those admitted under non-cardiovascular specialties (p ​= ​0.006). Overall, less than half of patients had any hospital-coded diagnosis of CHD in any admission over the three-year study period. ConclusionsHospital discharge coding dramatically underreports CHD, especially for patients with moderate and severe CHD lesions and for admissions under non-cardiovascular specialties. This suggests that discharge coding-based estimates of the burden of CHD on hospitals and health systems may be substantially underestimated.

Late complication rates after aortic coarctation repair in patients with or without a bicuspid aortic valve

ObjectivePatients with previously repaired aortic coarctation (CoA) are at risk of developing late surgical complications. Many patients with CoA also have a bicuspid aortic valve (BAV). We sought to determine...

Provision of palliative care to adults with congenital heart disease at the end of life

Abstract Funding Acknowledgements Type of funding sources: Public grant(s) – National budget only. Main funding source(s): This work was supported by Research Foundation Flanders [grant numbers G097516N to PM, 12E9816N to EG and 1154719N to LVB]; the King Baudouin Foundation (Fund Joseph Oscar Waldmann-Berteau &amp; Fund Walckiers Van Dessel); the National Foundation on Research in Pediatric Cardiology; and the Swedish Research Council for Health, Working Life and Welfare -FORTE (grant number STYA-2018/0004). OnBehalf BELCODAC consortium Background Although recent position papers have discussed and advocated for the integration of palliative care in the treatment course of adults with congenital heart defects (CHD), empirical studies reporting to what extent palliative care is currently provided, are still lacking. Purpose (1) To explore the current provision of palliative care to adults with CHD in the last 6 months of their life; and (2) to describe the profile of patients who received palliative care. Methods In this retrospective study, data of deceased adult patients included in the BELgian COngenital heart disease Database combining Administrative and Clinical data (BELCODAC) were analysed. Palliative care provision (i.e., admission to palliative care ward, or palliative care at home) was identified using nomenclature codes. The level of anatomical complexity was based on the Bethesda classification. Descriptive analyses were performed. Results During the period 2006-2016, 480 adults with CHD died (mean age: 54.4y; 45% simple CHD, 43% moderate CHD, 12% complex CHD). We identified that 75 patients (16%) had at least one nomenclature code linked to palliative care in the last 6 months of their life. More specifically, 16 patients were admitted to an inpatient palliative care service and 67 patients received palliative care at home. Of the patients who received palliative care at home, 40 patients were cared for by a multidisciplinary team specialized in palliative care provision and 59 patients received care from nurses and/or general practitioners while being recognized as a palliative patient. A total of 8 patients received palliative care both at the inpatient palliative care service and at home. Of the 75 patients receiving palliative care, 44 (59%) had a neoplasm as the primary cause of death and a cardiac cause of death was reported for 10 patients (13%) (see Figure 1). The mean age of patients receiving palliative care was 57.9 years. Most patients receiving palliative care had a simple CHD (n = 40; 53%), 29 patients (39%) had a moderate lesion, and 6 patients (8%) had a complex lesion. That means that, respectively, 19%, 14%, and 11% of all deceased patients with a simple, moderate, and complex heart lesion received palliative care. Conclusions This is the first exploratory study on palliative care in adults with CHD. About one in six patients who died received palliative care. Of those who received palliative care, the cause of death was in most cases of a non-cardiac nature. Further research is needed to investigate the care trajectories and care needs of adults with CHD in the last months of life. Figure 1. Causes of death of adults with CHD who received palliative care in the last 6 months of life (n = 75).

Neurocognitive disorders amongst patients with congenital heart disease undergoing procedures in childhood

BackgroundAmongst children with congenital heart disease (CHD), earlier age of repair improves cardiovascular outcomes. The effects of early intervention on neurodevelopment remains unclear. We assessed the association between early life repair, cardiopulmonary bypass (CPB) and the incidence of neurocognitive disorders (NCD) amongst CHD patients. MethodsWe created two retrospective cohorts from the Quebec CHD Database; with data from 1988 to 2010. Complexity of reparative procedures for CHD lesions were used as the proxy of CPB exposure with more complex procedure means longer exposure to CPB. Study Population 1 included pediatric patients with a single reparative procedure and compared patients with complex (long CBP) versus isolated shunt (short CBP) lesions. To assess the effects of CPB alone in Study Population 2 we compared patients with isolated atrial septal defects (ASD) who had surgical (short CBP) versus percutaneous (no CBP) repairs. The primary endpoint for both cohorts was development of an NCD. ResultsIn Study population 1, 1174 patients underwent complex surgical repair and 1620 had a shunt closure. The incidence of NCDs was 2.45/100 person-years in the complex surgery group, and 2.08/100 person-years in the shunt closure group. The following were associated with increased risk of developing a NCD: surgical complexity (Hazard Ratio [HR] = 1.20, 95% Confidence Interval [CI]: 1.01–1.42), younger age at intervention (AAI) (HR = 1.20, 95% CI: 1.16–1.25), male sex (HR = 1.91, 95% CI: 1.61–2.27), and later calendar year at intervention (HR = 1.06, 95% CI: 1.04–1.07). Study population 2 had 527 isolated ASD patients; 202 underwent surgical repair and 325 had percutaneous closure. The incidence of NCDs was not statistically different between groups. Male sex (HR = 1.77, 95% CI: 1.08–2.89) and younger AAI (HR = 1.15, 95% CI: 1.06–1.25) were associated with increased NCD risk. ConclusionIncreased surgical complexity, male sex and younger AAI were associated with increased risk of NCDs in pediatric CHD patients. Surveillance protocols should be considered to identify NCDs in CHD patients after cardiac intervention.

Influenza Vaccination in Patients With Congenital Heart Disease in the Pre-COVID-19 Era: Coverage Rate, Patient Characteristics, and Outcomes

BackgroundInfluenza vaccination is the most commonly recommended immune prevention strategy. However, data on influenza vaccination in patients with congenital heart disease (CHD) are scarce. In this study, our goals were to: (1) measure vaccination coverage rates (VCRs) for influenza in a large cohort of children, adolescents, and adults with CHD; (2) identify patient characteristics as predictors for vaccination; and (3) investigate the effect of influenza vaccination on hospitalization. MethodsA nationwide cohort study in Belgium included 16,778 patients, representing 134,782 vaccination years, from the Belgian Congenital Heart Disease Database Combining Administrative and Clinical Data (BELCODAC). Data over 9 vaccination years (2006-2015) were used, and patients were stratified into 5 age cohorts: 6 months to 4 years; 5-17 years; 18-49 years; 50-64 years; and 65 years and older. ResultsIn the respective age cohorts, the VCR was estimated to be 6.6%, 8.0%, 23.9%, 46.6%, and 72.8%. There was a steep increase in VCRs as of the age of 40 years. Multivariable logistic regression showed that higher anatomical complexity of CHD, older age, presence of genetic syndromes, and previous cardiac interventions were associated with significantly higher VCRs. Among adults, men had lower and pregnant women had higher VCRs. The association between influenza vaccination and all-cause hospitalization was not significant in this study. ConclusionsThe influenza VCR in people with CHD is low, especially in children and adolescents. Older patients, particularly those with complex CHD, are well covered. Our findings should inform vaccination promotion strategies in populations with CHD.

Different levels of care for follow-up of adults with congenital heart disease: a cost analysis scrutinizing the impact on medical costs, hospitalizations, and emergency department visits.

To scrutinize the economic impact of different care levels, such as shared care, in the follow-up of adult congenital heart disease (ACHD) patients. The BELgian COngenital heart disease Database combining Administrative and Clinical data (BELCODAC) was analyzed. Patients (N = 6579) were categorized into five care levels based on their cardiac follow-up pattern between 2006 and 2010. Medical costs, hospitalizations, and emergency department visits were measured between 2011 and 2015. In patients with moderate lesions, highly specialized cardiac care (HSC; exclusive follow-up by ACHD specialists) and shared care with predominantly specialized cardiac care (SC+) were associated with significantly lower medical costs and resource use compared to shared care with predominantly general cardiac care (SC-) and general cardiac care (GCC). In the patient population with mild lesions, HSC was associated with better economic outcomes than SC- and GCC, but SC+ was not. HSC was associated with fewer hospitalizations (- 33%) and less pharmaceutical costs (- 46.3%) compared to SC+. Patients with mild and moderate lesions in the no cardiac care (NCC) group had better economic outcomes than those in the GCC and SC- groups, but post-hoc analysis revealed that they had a different patient profile than patients under cardiac care. More specialized care levels are associated with better economic outcomes in patients with mild or moderate lesions in cardiac follow-up. Shared care with strong involvement of ACHD specialists might be a management option to consider. Characteristics of patients without cardiac follow-up but good medium-term economic prospects should be further scrutinized.

Investigation of echocardiographic characteristics and predictors for persistent defects of patent foramen ovale or patent ductus arteriosus in Chinese newborns

BackgroundPersistent patent foramen ovale (PFO) and patent ductus arteriosus (PDA) increase the adult risk of cryptogenic embolic stroke and chronic pulmonary hypertension. To understand the characteristics of PFO and PDA in newborns, we investigated the spontaneous closure rate and derived the determinants for residual defects. MethodsWe utilized the database of congenital heart disease (CHD) in Xiamen ChangGung Memorial Hospital from 2015 to 2017 and allocated 2523 eligible newborns into four groups according to PDA, PFO, both or neither at birth. A total of 574, 1229, 202 and 518 newborns were assigned into the group of PFO and PDA, PFO alone, PDA alone and non-PFO/non-PDA, respectively. Regular echocardiographic follow-ups at baseline, 6, 12 and 24 months after birth were performed for evaluating the spontaneous closure rate in the subjects. Regression analysis was carried out to study the risk factors of residual congenital defects. ResultsNewborns with PFO alone had the youngest birth age and lowest birth weight among the four groups. About one in four PDA-alone newborns had concomitant small ASD, i.e., <5 mm in diameter. Echocardiographic study showed that 71.3% and 30.8% of CHD newborns had PFO and PDA, respectively, compared to less than 10% of them having ASD or VSD. However, more than 95% of newborns with PFO or PDA closed spontaneously at 6 months, in contrast to about 30% of newborns with ASD or VSD had persistent existence of the intracardiac defects. Complex CHD significantly linked to persistent PFO or PDA at 6 and 12 months, with an adjusted hazard ratio of 9.03 (95% CI 1.97–41.46) and 12.11 (95% CI 2.11–69.72), respectively. ConclusionsChinese newborns with PFO or PDA expressed differences in characteristics and concomitant congenital defects. Additionally, persistent PFO or PDA is strongly associated with complex CHD and requires long-term regular monitoring for future associated complications.

Pulmonary Vein Stenosis: A Rare Disease with a Global Reach.

Pulmonary vein stenosis (PVS) is a rare, but high mortality and resource intensive disease caused by mechanical obstruction or intraluminal myofibroproliferation, which can be post-surgical or idiopathic. There are increasing options for management including medications, cardiac catheterization procedures, and surgery. We queried the International Quality Improvement Collaborative for Congenital Heart Disease (IQIC) database for cases of PVS and described the cohort including additional congenital lesions and surgeries as well as infectious and mortality outcomes. IQIC is a quality improvement project in low-middle-income countries with the goal of reducing mortality after congenital heart surgery. Three cases were described in detail with relevant images. We identified 57 cases of PVS surgery, with similar mortality to higher income countries. PVS should be recognized as a global disease. More research and collaboration are needed to understand the disease, treatments, and outcomes, and to devise treatment approaches for low resource environments.

Epidemiology of adult congenital heart disease among the general population in Kuwait.

BackgroundAdult congenital heart disease (ACHD) is a highly underrepresented entity in medical literature, especially in the middle‐eastern region.HypothesisThis study is the first to assess the prevalence of adult congenital heart disease among the population of Kuwait.MethodsAfter a retrospective register review of patients in Kuwait being followed up in the chest diseases hospital was conducted, patients who fit the inclusion criteria were enrolled in the study. Using the American College of Cardiology Task Force 1 of the 32nd Bethesda conference classification of the severity of ACHD, the patients were classified into those with simple, moderate, and complex congenital heart diseases. The age and gender of the patients, as well as the type repair performed, and the residual cardiac findings were recorded to assess the association between the complexity and residuals. Associations were assessed using STATA 15.ResultsA total of 611 patients were evaluated over a period of 18 months. The youngest participant was 20 years of age, and the oldest participant was 88 years old. Male participants with moderate congenital heart disease class were more common in our study population. Patients with complex congenital heart disease have more residual cardiac lesion than the moderate or simple groups. Almost (70%) of patients with complex cardiac anomalies have undergone either partial or complete repair. The most prevalent cardiac defect was atrial septal defect (21.5%). Tetralogy of Fallot was the most prevalent defect in the moderate group, representing (13%) of the group. The most prevalent anomaly in the complex group was double outlet right ventricle (DORV) representing (15.38%).ConclusionAdult Congenital heart disease is a growing entity of heart disease due to advanced repair techniques. This population requires registries to document cases and assign specialists for the management and care of this special group of patients.Highlights First database of adult congenital heart disease in Kuwait.The most prevalent heart defect was ASD in Kuwait.TOF was the most prevalent defect in the moderate group; and DORV was the most prevalent in the complex group.Patients with moderate ACHD tended to have a more complete repair than those in the complex group.