Moderate Congenital Heart Defects Research Articles

Predictors of Developing Heart Failure in Adults with Congenital Heart Defects

Background: The population of adults with congenital heart defects (ACHD) is growing. The leading cause of premature death in these patients is heart failure (HF). However, there is still limited information on the predictive factors for HF in ACHD patients. Objectives: This study re-examined a group of patients with repaired or palliated congenital heart defects (CHD) that were initially studied in 2003. A follow-up period of 15 years has allowed us to identify and evaluate predictors for the development of HF in ACHD. Methods: All patients with repaired or palliated CHD who participated in the initial study (n = 364) were invited for a follow-up examination. The effects of maximum oxygen uptake (VO2max) during exercise stress testing, the cardiac biomarker N-terminal pro brain natriuretic peptide (NT-proBNP), and QRS complex on the development of HF during the follow-up period were investigated. Results: From May 2017 to April 2019, 249 of the initial 364 (68%) patients participated in the follow-up study. Of these, 21% were found to have mild CHD, 60% had moderate CHD, and 19% had complex CHD. Significant predictors for the development of HF were: NT-proBNP level >1.7 times the upper normal limit, VO2max <73% of predicted values, and QRS complex duration >120 ms. Combination of these three parameters resulted in the highest area-under-the-curve of 0.75, with a sensitivity of 75% and specificity of 63% for predicting the development of HF. Conclusions: In this cohort of ACHD patients, the combination of VO2max%, NT-proBNP, and QRS duration was predictive of HF development over a 15-year follow-up period. Enhanced surveillance of these parameters in patients with ACHD may be beneficial for the prevention of HF and early intervention.

Functional characterization of GATA6 genetic variants associated with mild congenital heart defects

Damaging GATA6 variants can cause moderate congenital heart defects. With the application of next-generation sequencing approaches, various novel GATA6 variants with unknown significance have been identified from a broad spectrum of congenital heart defects. However, functional assessment for distinct GATA6 variants from different severity of congenital heart defects, especially from mild defects, is lacking, which hinders our understanding of the genotype-phenotype correlations and underlying mechanisms.Here, we assessed the functional consequences of nine rare GATA6 variants, which had been implicated as the most significant variants associated with mild congenital heart defects using the largest case and control cohort. We examined the effects of these variants on subcellular localization, transcriptional activity, and protein interactions in 293T or AC16 cells and their ability to rescue heart malformation in gata6 zebrafish mutant.We found that two of these nine variants, Q120X and S424I, significantly decreased transcriptional activity. Additionally, Q120X altered subcellular localization. Consistent with the in vitro results, the in vivo results showed that Q120X and S424I lost their potency to rescue ventricular malformation in gata6 −/− embryos.The results indicated that Q120X and S424I are pathogenic in mild congenital heart defects. Further, the inconsistence of severely impaired Q120X function and mild CHDs phenotype suggested the complexity of the genotype-phenotype correlation between the GATA6 variant and heart phenotype, which may help to inform prenatal genetic counseling and pre-implantation genotyping for congenital heart defects.

Morbidity and mortality in adults with congenital heart defects in the third and fourth life decade

ObjectivesThe population of adults with congenital heart defects (ACHD) is continuously growing. Data on morbidity and mortality of ACHD are limited. This longitudinal observational study examined a group of ACHD with surgically corrected or palliated congenital heart defects (CHD) during a 15-year period.MethodsACHD that had participated in the initial study were invited for a follow-up examination. Mortality and hospitalization data were compared with a healthy control group.ResultsFrom 05/2017 to 04/2019 a total of 249/364 (68%) ACHD participated in the follow-up study: 21% had mild, 60% moderate and 19% severe CHD. During the observational period, 290 health incidents occurred (cardiac catheterization 37%, cardiovascular surgery 27%, electrophysiological study/ablation 20%, catheter interventional treatment 14%, non-cardiac surgery 3%). Events were more frequent in ACHD with moderate (53%) and severe (87%) compared to those with mild CHD (p < 0.001). 24 individuals died at a median age of 43 years during the observation period. 29% of them had moderate and 71% severe CHD corresponding to a mortality rate of 0%, 0.29% and 1.68% per patient-year in ACHD with mild, moderate and severe CHD. Long-term survival was significantly reduced in patients with severe CHD in comparison to individuals with mild and moderate CHD (p < 0.001).ConclusionAfter correction or palliation of CHD, there was remarkable ongoing morbidity and mortality in ACHD patients over the 15-year observation period, particularly in individuals with moderate and severe CHD when compared with the general population. Thus, life-long special care is required for all surgically corrected or palliated ACHD patients.Graphical abstract

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).

P2576Sports in children and adolescents with congenital heart defects

Abstract Background Age-appropriate physical activity is an important basis for healthy physical and mental development for children and adolescents with congenital heart defects (CHD) Purpose The main goal of this study was to learn more about the physical activity of young and adolescent CHD-patients to understand patient's needs physical activity and to improve the medical advice regarding physical activity and sports by taking the CHD-severity into account. Methods An online-questionnaire was developed. CHD patients registered in the German National Register for Congenital Heart Defects were invited by email/post to join the study. In total, 1,718 patients took part. 1,262 patients have finished the questionnaire and are included in the statistical analysis (average age: 11.6±3.1 years; 588 women). CHD severity: 411 patients with simple CHD, 423 with moderate CHD, 364 with complex CHD and 64 with unclassified CHD. The study participants were asked to answer questions regarding physical activity and (school) sports to evaluate their activity level and their individual needs regarding sports. Results We identified 831 (65.9%) patients who reported they were exercising sports for at least three days or more for 60 minutes or longer per week (simple CHD: 68.9%; moderate CHD: 64.5%; complex CHD: 63.5%; other CHD: 68.8%). Over all 47 patients (3.7%) stated that they had no regular school sports and 12 patients (1%) reported to have less than one regular sports lessen per week. Out of the 1,215 patients who reported to have regular school sports (simple CHD: 97.3%; moderate CHD: 98.1%; complex CHD: 94.5%; other CHD: 87.5%), the patients stated about how hard they usually work in a regular sports lesson as follows: “without sweating and without shortness of breath” (188 patients, 15.5%; simple CHD: 15.4%; moderate CHD: 17.1%; complex CHD: 14.8%; other CHD: 7.1%), “a little bit sweaty and a bit short of breath” (825 patients, 67.9%; simple CHD: 68.8%; moderate CHD: 65.3%; complex CHD: 68.9%; other CHD: 75%), “a lot of sweat and a lot of shortness of breath” (202 patients, 16.6%; simple CHD: 15.8%; moderate CHD: 17.6%; complex CHD: 16.3%; other CHD: 17.9%). Conclusions Overall, young and adolescent patients with CHD appear to be exercising sports regularly and are well integrated into school sports. Although there are slight differences between the individual degrees of CHD severity. These differences can be interpreted as minor fluctuations.

Abstract MP67: Pregnancy Outcomes in Women With Congenital Heart Defects in Colorado: Results From the Colorado CHD Surveillance System in Adolescents and Adults (COCHD)

Introduction: The normal hemodynamic changes of pregnancy can trigger cardiac dysfunction in women with congenital heart defects (CHD), leading to an increased risk of cardiac events. Further, utero-placental flow is impaired in women with CHD which can result in higher risk of adverse offspring outcomes. Hypothesis: Severity of maternal CHD is associated with adverse maternal cardiovascular events and offspring events by severity of CHD in 1074 pregnancies that resulted in a live birth in Colorado between 2011-13. Methods: Using data from the Colorado CHD Surveillance System in Adolescents and Adults, 926 pregnant women with CHD were identified that resulted in 1074 live births between 2011-2013. Maternal CHD type was dichotomized as severe (n=149) and moderate (total n=882). The risk of adverse maternal cardiac and offspring events were calculated out of the total number of pregnancies in each severity category. Results: Primary maternal cardiac events including cardiovascular mortality, arrhythmia, heart failure, thromboembolic events (pulmonary embolism, valve thrombosis or deep venous thrombosis), vascular events (stroke, myocardial infarction or dissection) and endocarditis occurred in 15% and 7% of women with severe and moderate CHD, respectively. Premature birth (delivery &lt;37 weeks) and/or low birth weight (&lt;2,500 grams) occurred in &gt;18% of the women with severe CHD and 17% of women with moderate CHD. The risk of offspring congenital anomalies among women with severe CHD was extremely high for circulatory/respiratory, musculoskeletal/integumentary, cyanotic congenital heart disease and Down syndrome. The risk of infant congenital anomalies was lower for women with moderate CHD, with the most common being circulatory/respiratory, urogenital, Down syndrome and cyanotic congenital heart disease. Conclusions: Population-level surveillance system of CHD in Colorado provides novel assessment of the substantial risk of maternal and neonatal cardiac events associated with pregnancy in women with CHD.

Somatic Development in Children with Congenital Heart Defects

Somatic development is impaired in children with congenital heart defects (CHDs), and head circumference seems to be a strong predictor of neurodevelopmental prognosis. The aim of this study was to generate up-to-date reference values for the somatic development (head circumference, body weight, and length/height) of children with CHDs. Our study population consisted of all patients included in the PAN study (Prävalenz angeborener Herzfehler bei Neugeborenen in Deutschland), which was conducted prospectively over a 3-year study period by the Competence Network for Congenital Heart Defects. All children with mild, moderate, and severe CHDs born in 2006-2009 in Germany were enrolled. For computing of z-scores, only children with the following characteristics were included: appropriate for gestational age, nonsyndromic disease, term or post-term delivery, and no cardiac surgery. There were 2818 patients included. New z-scores for the described somatic measures of children with mild, moderate, and severe CHDs were computed. Comparisons with the KiGGS study (Gesundheit von Kindern und Jugendlichen in Deutschland) and the Berlin Longitudinal Study revealed significantly lower measurements for all measures-most notably in children with severe CHDs and/or cardiac surgery. In our cohort, no catch-up growth was seen after cardiac surgery. Children with severe CHDs demonstrated the most abnormal pattern in growth, including head circumference before and after cardiac surgery, which is indicative of accompanying brain pathology unrelated to operative injury.

Intellectual Functioning in Children with Congenital Heart Defects Treated with Surgery or by Catheter Interventions.

Studies suggest that children with congenital heart defects (CHD) are at risk for adverse intellectual functioning. However, factors related to lower intellectual functioning in this group are largely unknown. This study describes intellectual functioning in children with CHD in relation to severity of the heart defect, the child's age, and the socioeconomic status of the family (SES). Two hundred twenty-eight children treated with surgery or by catheter technique were tested using the Wechsler intelligence scales to determine full scale IQ (FSIQ). FSIQ was then analyzed in relation to age (3-, 5-, 9-, and 15-year olds), severity of the diagnosis (mild, moderate, and severe), and SES (low, medium, and high). The median age was 70 months (5.8 years) with a range of 162 months [30 months (2.5 years) to 192 months (16.0 years)]. The total mean score on FSIQ was 100.8 (SD = 14.5). Children with severe CHD had significantly lower FSIQ than children with mild and moderate CHD, and 9- and 15-year olds had significantly lower FSIQ compared to the 3-year olds. Children from families with low SES had significantly lower FSIQ than children from medium SES and high SES families. No interaction between severity of diagnosis, age, and SES was found for FSIQ. Eighty-three percent of the children with CHD performed at or above average with respect to FSIQ. SES and severity of diagnosis had significant main effects on FSIQ. These factors should be considered when planning interventions and follow-up programs for children with CHD.

The incidence of congenital heart defects in the world regarding the severity of the defect.

BACGROUND/AIM. Congenital heart defects (CHDs) are structural or functional abnormalities of the heart present at birth even if they are detected much later. Their importance lies in the fact that, depending on the severity, they change the quality of life, and may be life threating. In addition, we should not ignore the high costs of treating people with congenital heart disease. The aim of this study was to analyze the incidence of congenital heart disease in relation to the severity in the world based on the available literature. All the available literature on the incidence of CHD cases regarding the severity of CHD published from 1955 to 2012 was analyzed. The researcher was able to read the titles and abstracts of 128 papers on the subject. Due to methodological inconsistency, 117 of the papers were rejected. Based on the criteria of reliability, availability and comparability, our analysis included 11 studies testing CHD incidence regarding the severity of the defect conducted all over the world. The Yates' χ2-test was used to compare the observed incidences. The frequency of severe congenital heart defects, ranged from 0.414 to 2.3/1,000 live births, the incidence of moderate congenital heart defects from 0.43 to 2.6/1,000 live births while in the group of minor congenital heart defects the incidence ranged from 0.99 to 10.3/1000 live births. There were no statistically significant differences in the incidence of mild, moderate and severe CHDs. The results obtained studying of the available data suggest that no statistically significant difference in the incidence of mild, moderate and severe congenital heart defects. A universal methodological approach to the incidence of CHD is essential.

Abstract 18284: Incidence of Epilepsy is Elevated in Children and Young Adults With Congenital Heart Defects Compared With the General Population: A Nationwide Cohort Study

Perioperative seizures related to surgery for congenital heart defects (CHD) are well described; however, few data exist on the long-term risk of epilepsy in patients with CHD. We aimed to estimate the incidence of epilepsy in children and young adults with CHD compared with the general population. Methods: Utilizing data from the Danish National Registry of Patients (DNRP) we identified all patients diagnosed with CHD before the age of 15 years between 1980 and 2010 who were born during the same period. The DNRP is a nationwide hospital discharge registry covering all Danish hospitals. Previously validated methodology using the DNRP was applied to measure the outcome, epilepsy, as well as presence of extra cardiac defects (ECD) and/or syndromes. We used the Danish Medical Birth Registry to identify preterm birth (gestational age&lt;37 weeks). For each CHD subject, we identified 10 controls from the general population using the Danish Civil Registration System, matched by sex and birth year. A unique personal identifier assigned at birth and used in all Danish public registries enabled virtually complete follow up for migration, death, or epilepsy until January 1, 2013. We computed cumulative incidences and hazard ratios (HR) (split at 5 years of age to obtain proportional hazards) of time from CHD diagnosis (index date for controls) to epilepsy. Results: We identified 14,665 CHD subjects with a median age at diagnosis of 2 (IQR 19) months. By 15 years of age, the cumulative incidence of epilepsy was 4% among CHD subjects. The HR of epilepsy among CHD subjects compared with the control cohort was 3.7 (95% CI: 3.2-4.3) below 5 years of age, and 2.4 (95% CI: 2.1-2.7) from 5 to 33 years of age. In the older age group, HR for patients with severe CHD was 2.8 (95% CI: 2.3-3.5), and for mild and moderate CHD was 2.2 (95% CI: 1.8-2.6). After exclusion of all subjects with ECDs and/or syndromes and preterm birth, corresponding HRs were 2.2 (95% CI: 1.6-3.0) and 1.7 (95% CI: 1.3-2.2), respectively. Conclusion: The epilepsy risk was markedly increased in CHD subjects compared with the age and gender matched controls. These findings add evidence to support the importance of developing neuro-protective measures and potentially long-term epilepsy surveillance strategies in the CHD population.

Analysis of associations between congenital heart defect complexity and health-related quality of life using a meta-analytic strategy

BackgroundAs a consequence of heterogeneous results of relatively small individual trials, the impact of congenital heart defects (CHD) and the effect of disease severity on patient reported outcome measures (PROs) of quality of life (QoL) remains uncertain. We aimed to systematically summarize QoL data in CHD patients using meta-analytic methods. Methods and resultsWe performed a systematic review of the literature focusing on QoL in CHD. The search yielded 234 publications meeting the inclusion criteria, with a median of 88 patients per study (46% females, average age 24years). In total, QoL was reported using PROs in 47,471 CHD-patients. More than 95 different PROs were used to evaluate QoL. The most commonly used tool was the SF36 form (69 publications). Analysis of available quantitative QoL data from SF36 publications (n=4217 CHD patients) showed that QoL was reduced in patients with moderate or complex cardiac disease (e.g. relative physical functioning scores 0.96 [0.93–0.99] and 0.91 [0.88–0.95] compared with controls), while no such effect was evident in those patients with simple cardiac lesions. Similar results were found for the general health domain of the SF36 domain. ConclusionsDespite the proliferation of QoL-studies in CHD no standardized approach for measuring and reporting QoL has emerged and the published results are heterogeneous. In aggregation, however, the results of this study suggest that QoL is impaired in moderate or complex CHD, while no such impact of CHD on QoL could be established — on average — in patients with simple defects.

Incidence of Congenital Heart Defects in a Public Maternity Hospital during the Period 1998-2011 in the Beginning of the National Congenital Heart Defects Program

Background: Congenital heart defects (CHD) are among the most common congenital diseases with great impact in infantmorbidity and mortality; however, their real incidence in our country is unknown. The Ministry of Health created the NationalCongenital Heart Defect Program to contribute to the reduction of infant mortality for this cause. Therefore, determiningthe incidence and the relative frequency of CHD at the Hospital Materno Infantil Ramon Sarda, a public tertiary institutionspecialized in high risk pregnancies and congenital defects would provide information to the Program.Objectives: 1) To determine the incidence and relative frequency of CHD in liveborn (LB) infants at the Hospital MaternoInfantil Ramon Sarda in the beginning of the PNCC program. 2) To evaluate the association with other congenital defects.3) To analyze the distribution of CHD by sex.Methods: The study has a descriptive, observational and retrospective design. The digital clinical records of 1,161 LB infantswith confirmed diagnosis of CHD from the Section of Pediatric Cardiology, Hospital Materno Infantil Ramon Sarda, betweenJanuary 1, 1998 and December 31, 2011, evaluated during hospitalization and follow-up at the outpatient clinic, were analyzed.Congenital heart defects were classified based on their severity according to the meta-analysis published by Hoffman andKaplan in USA assessing their incidence and relative frequency.Categorical data are presented as number of absolute cases and percentage. Results: From a total of 92,725 LB infants during the study period, CHD were present in 1,161 of cases (12.5/1000). Afterexcluding small muscular ventricular septal defects, the prevalence decreased to 7/1000 LB infants.The prevalence of moderate and severe CHD was 44% (3 to 3.5/1000).Ventricular septal defects were the most common CHD.Conclusion: Despite being a tertiary center for high risk pregnancy and congenital defect, the incidence of CHD determinedat the Hospital Materno Infantil Ramon Sarda is similar to the one published worldwid

Relationship Satisfaction Among Mothers of Children With Congenital Heart Defects: A Prospective Case-Cohort Study

To assess the level of partner relationship satisfaction among mothers of children with different severity of congenital heart defects (CHD) compared with mothers in the cohort. Mothers of children with mild, moderate, or severe CHD (n = 182) and a cohort of mothers of children without CHD (n = 46,782) from the Norwegian Mother and Child Cohort Study were assessed at 5 time points from pregnancy to 36 months postpartum. A 5-item version of the Relationship Satisfaction scale was used, and relevant covariates were explored. The trajectories of relationship satisfaction among mothers of children with varying CHD severity did not differ from the trajectories in the cohort. All women in the cohort experienced decreasing relationship satisfaction from 18 months after delivery up to 36 months after delivery. Having a child with CHD, regardless of severity, does not appear to exacerbate the decline in relationship satisfaction.

Well-being in mothers of children with congenital heart defects: a 3-year follow-up

This prospective case-cohort study compared subjective well-being (SWB) among mothers whose children had various degrees of congenital heart defects (CHD) with mothers of children without CHD (controls). Nationwide CHD registry data were linked to data collected from the Norwegian Mother and Child Cohort Study at gestational week 30, 6 months, and 36 months postpartum. A total of 175 mothers of children with mild, moderate, and severe CHD were identified in a cohort of 44,144 mothers. The SWB index was operationalized by means of three subscales: a cognitive aspect, positive affect, and negative affect. Mothers of children with severe CHD reported significantly lower SWB than the controls at 6 months postpartum (p = .003), with further decrease in SWB at 36 months postpartum (p = .001). SWB levels in the mild and moderate CHD group did not deviate significantly from controls. The findings suggest a pattern in which all stressors concerning the severely ill child lead to significant deterioration of maternal well-being. Having a child with mild or moderate CHD, which is a less severe and shorter-term stressor, did not reduce mothers' well-being.

Trajectories of Maternal Mental Health: A Prospective Study of Mothers of Infants With Congenital Heart Defects From Pregnancy to 36 Months Postpartum

To chart mothers' trajectories of mental health from pregnancy to 36 months postpartum in order to investigate the association between infants' congenital heart defects (CHD) and compromised maternal mental health. Mothers of infants with mild, moderate, or severe CHD (n = 141) and mothers (n = 36,437) enrolled in the Norwegian Mother and Child Cohort Study were assessed at regular intervals from pregnancy up to 36 months postpartum, including measurements at 6 and 18 months, using an 8-item version of the Hopkins Symptom Checklist-25. Mean score trajectories of SCL-8 for mothers of infants with severe CHD deviated significantly from cohort controls 6, 18, and 36 months postpartum, indicating heightened symptoms of depression and anxiety. Mothers of infants with severe CHD are at risk of compromised mental health from delivery to 36 months postpartum. Strain due to CHD-related interventions is identified as a possible partial mediator of the distress.

Occurrence and Predictors of Developmental Impairments in 3-Year-Old Children with Congenital Heart Defects

To examine the occurrence of developmental impairments in 3-year-old children with varying severity of congenital heart defects (CHD) and to identify predictors associated with developmental impairment in children with severe CHD. Prospective data collected at birth, 6, 18, and 36 months from the Norwegian Mother and Child Cohort Study (MoBa), conducted by the Norwegian Institute of Public Health, were linked with a nationwide medical CHD registry, and 175 three year olds with CHD in a cohort of 44,044 children were identified. Children with mild/moderate (n = 115) and severe (n = 60) CHD were compared with children without CHD (43,929) on motor, communication, and social impairments as reported by mothers in Norwegian Mother and Child Cohort Study questionnaires. Predictors of developmental impairment were analyzed for the group with severe CHD. Children with severe CHD had >3 times higher odds of communication and gross motor impairments compared with controls, and had 2 times higher odds of any developmental impairment compared with controls. Children with mild and moderate CHD had >2 times higher odds of gross motor impairment but did not otherwise differ from controls. Predictors of impairment identified were previous developmental impairments and smaller head circumference at birth. Children with severe CHD have increased odds of developmental impairments at age 3 years. Early developmental impairments are associated with later developmental impairments, suggesting lasting impairments and not merely temporary delay. Patient-specific conditions at birth should be considered and motor and communication support provided to potentially improve outcomes in children with CHD.

Mothers of infants with congenital heart defects: well-being from pregnancy through the child’s first six months

This study compared the well-being among mothers of children with congenital heart defects (CHD) with mothers of children without CHD (controls), at pregnancy and at 6 months postpartum. We linked prospective data from the Norwegian Mother and Child Cohort Study (MoBa), conducted by the Norwegian Institute of Public Health, with a nationwide medical CHD registry. In the MoBa cohort of 61,456 mothers, we identified 212 mothers of infants with mild (n=92), moderate (n=50), or severe CHD (n=70). Subjective well-being was operationalized by means of maternal life satisfaction, joy, and anger at the 30thweek of gestation and at 6months postpartum. Subjective well-being in mothers of children with CHD remained unchanged and similar to that of controls on satisfaction with life (P=0.120) and feelings of joy (P=0.065). However, at child age 6months, mothers of infants with severe CHD reported slightly elevated feelings of anger compared with controls (P=0.006). Joy and life satisfaction remained intact among mothers of children with CHD. Yet, elevated feelings of anger in mothers of children with the most severe CHD suggest that they may experience more frustration.

Longitudinal findings from a Norwegian case–cohort study on internalizing problems in children with congenital heart defects

To examine the association of the severity of congenital heart defects (CHDs) with internalizing problems in 18-month-olds and to explore the extent to which the internalizing problems are influenced by maternal distress and emotional reactivity in the child at age 6 months. We linked prospective data from the Norwegian Mother and Child Cohort Study, conducted by the Norwegian Institute of Public Health, with a nationwide CHD registry and identified 198 18-month-olds with CHDs in a cohort of 47 692 toddlers. Maternal reports on the children's emotional reactivity at age 6 months, the children's internalizing problems (anxiety, sleep problems, emotional reactivity) at age 18 months and maternal distress were assessed by questionnaires. We found an association at age 18 months between the severity of the CHD and anxiety but not sleep problems or emotional reactivity. Children with severe but not with mild or moderate CHDs were twice as likely to experience the symptoms of anxiety compared with controls. These symptoms are not merely sequelae of earlier psychological reactions or concurrent maternal distress. Should these findings be replicated, future studies ought to investigate the mechanisms leading to elevated anxiety in toddlers with CHDs. In addition, clinical interventions should address the child's anxiety as well as the interaction between the parents and the child.

Long-Term Symptoms of Depression and Anxiety in Mothers of Infants with Congenital Heart Defects

To examine the relationship between the severity of infants' congenital heart defects (CHD) and their mothers' symptoms of depression and anxiety from pregnancy to 18 months postpartum. Mothers of infants with mild, moderate, or severe CHD (n=162) and mothers (n=44,400) within the Norwegian Mother and Child Cohort Study were assessed with an eight-item short version (SCL-8) of the Hopkins Symptom Checklist-25 at the 30th week of gestation and at 6 and 18 months postpartum. Only the postpartum mental health trajectory of mothers of infants with severe CHD deviated from the mental health trajectory of the cohort at 6 and 18 months postpartum, showing significantly elevated levels of depression and anxiety symptoms. The results elucidate the relationship between infants' CHD severity and maternal symptoms of depression and anxiety, possibly identifying a specifically vulnerable patient dyad in need of postoperative interventions.

Emotional reactivity in infants with congenital heart defects: findings from a large case‐cohort study in Norway

Advances in medical treatment in recent years have led to dramatically improved survival rates of children with severe congenital heart defects (CHD). However, very little is known about the psychological consequences for these children, particularly during and after the early period of invasive treatment. In this study, we investigated the extent to which the severity of the CHD affects the child's emotional reactivity at 6 months of age. We linked prospective data from the Norwegian Mother and Child Cohort Study, conducted by the Norwegian Institute of Public Health with a nationwide medical CHD registry and identified 212 infants with CHD in a cohort of 61 299 infants. Mothers reported on their child's emotional reactivity at age 6 months by means of a standardized questionnaire. Infants with severe to moderate CHD had 60% higher odds for severe emotional reactivity (cut-off at the 85 percentile) compared with healthy infants, after controlling for important maternal and child confounders. Our study is the first to show elevated emotional reactivity in children with moderate to severe CHD, suggesting a need for special parental attention to soothe their distress. Follow-up studies will show whether this emotional reactivity is transient or an early marker of continuing emotional or behavioural problems.