The Application and Benefits of C-CHEWS in Infants with Left-to-Right Shunt Congenital Heart Disease during the Transition Period after Surgery.

Objective: This study aimed to explore the predictive value of various indicators in the application of C-CHEWS (Cardiac-Children's Hospital Early Warning Score) during the transition period of infants with left-to-right shunt congenital heart disease after surgery. Methods: A retrospective study was conducted on 229 infants who underwent surgery for left-to-right shunt congenital heart disease at a tertiary pediatric hospital in Anhui Province from January 2019 to March 2022. The infants' status was evaluated using C-CHEWS scores within 1 hour of transfer from the ICU to the transitional ward. A cutoff score 6 was used, with scores ≤6 defining the control group and scores ≥7 defining the observation group. The predictive value of various indicators during this period was analyzed. Results: The 229 infant patients were divided into the control group (n = 154) and the observation group (n = 75). All infants received sufficient oxygen inhalation, and 210 infants underwent VIS (Vasoactive-inotropic Score) evaluation, with 137 in the control group and 73 in the observation group, showing a statistically significant difference between the two groups. All infants were discharged without recurrence of ICU admission within 48 hours. In the C-CHEWS evaluation, medical staff attention and parental concern were assigned 1 point, while the consciousness level received 0 points. The respiratory system scores ranged from 2 to 3 points without a statistically significant difference, whereas the cardiovascular system scores ranged from 0 to 3 points and showed a statistically significant difference. Among the 75 observation group patients, 43 were boys, accounting for 57.33%. Conclusions: During the transition period after surgery for congenital heart disease in infants, monitoring the cardiovascular system, along with the effective application of VIS, through C-CHEWS scoring, can help detect warning signs. Focusing on managing cardiovascular function is crucial to reduce the risk of disease deterioration, promoting comfort, and aiding in the infants' recovery.

Newborn Pulse Oximetry for Infants Born Out-of-Hospital.

BACKGROUND AND OBJECTIVES: Conventional timing of newborn pulse oximetry screening is not ideal for infants born out-of-hospital. We implemented a newborn pulse oximetry screen to align with typical midwifery care and measure its efficacy at detecting critical congenital heart disease. METHODS: Cohort study of expectant mothers and infants mainly from the Amish and Mennonite (Plain) communities with limited prenatal ultrasound use. Newborns were screened at 1 to 4 hours of life ("early screen") and 24 to 48 hours of life ("late screen"). Newborns were followed up to 6 weeks after delivery to report outcomes. Early screen, late screen, and combined results were analyzed on the basis of strict algorithm interpretation ("algorithm") and the midwife's interpretation in the field ("field") because these did not correspond in all cases. RESULTS: Pulse oximetry screening in 3019 newborns (85% Plain; 50% male; 43% with a prenatal ultrasound) detected critical congenital heart disease in 3 infants. Sensitivity of combined early and late screen was 66.7% (95% confidence interval [CI] 9.4% to 99.2%) for algorithm interpretation and 100% (95% CI 29.2% to 100%) for field interpretation. Positive predictive value was similar for the field interpretation (8.8%; 95% CI 1.9% to 23.7%) and algorithm interpretation (5.4%; 95% CI 0.7% to 18.2%). False-positive rates were ≤1.2% for both algorithm and field interpretations. Other pathologies (noncritical congenital heart disease, pulmonary issues, or infection) were reported in 12 of the false-positive cases. CONCLUSIONS: Newborn pulse oximetry can be adapted to the out-of-hospital setting without compromising sensitivity or prohibitively increasing false-positive rates.

Alternative to Body Surface Area as a Solution to Correct Systematic Bias in Pediatric Echocardiography z Scores.

BACKGROUND: Z scores are the method of choice to report dimensions in pediatric echocardiography. Z scores based on body surface area (BSA) have been shown to cause systematic biases in overweight and obese children. Using aortic valve (AoV) diameters as a paradigm, the aims of this study were to assess the magnitude of z score underestimation in children with increased body mass index z score (BMI-z) and to determine if a predicting model with height and weight as independent predictors would minimise this bias. METHODS: In this multicentre, retrospective, cross-sectional study, 15,006 normal echocardiograms in healthy children 1-18 years old were analyzed. Residual associations with body size were assessed for previously published z score. BSA-based and alternate prediction models based on height and weight were developed and validated in separate training and validation samples. RESULTS: Existing BSA-based z scores incompletely adjusted for weight, BSA, and BMI-z and led to an underestimation of > 0.8 z score units in subjects with higher BMI-z compared with lean subjects. BSA-based models led to overestimation of predicted AoV diameters with increasing weight or BMI-z. Models using height and weight as independent predictors improved adjustment with body size, including in children with higher BMI-z. CONCLUSIONS: BSA-based models result in underestimation of z scores in patients with high BMI-z. Prediction models using height and weight as independent predictors minimise residual associations with body size and generate well fitted predicted values that could apply to all children, including those with low or high BMI-z.

Electrophysiological Characteristics of Intra-Atrial Reentrant Tachycardia in Adult Congenital Heart Disease: Implications for Catheter Ablation.

Background Ultra-high-density mapping enables detailed mechanistic analysis of atrial reentrant tachycardia but has yet to be used to assess circuit conduction velocity (CV) patterns in adults with congenital heart disease. Methods and Results Circuit pathways and central isthmus CVs were calculated from consecutive ultra-high-density isochronal maps at 2 tertiary centers over a 3-year period. Circuits using anatomic versus surgical obstacles were considered separately and pathway length <50th percentile identified small circuits. CV analysis was used to derive a novel index for prediction of postablation conduction block. A total of 136 supraventricular tachycardias were studied (60% intra-atrial reentrant, 14% multiple loop). Circuits with anatomic versus surgical obstacles featured longer pathway length (119 mm; interquartile range [IQR], 80-150 versus 78 mm; IQR, 63-95; P<0.001), faster central isthmus CV (0.1 m/s; IQR, 0.06-0.25 versus 0.07 m/s; IQR, 0.05-0.10; P=0.016), faster non-isthmus CV (0.52 m/s; IQR, 0.33-0.71 versus 0.38 m/s; IQR, 0.27-0.46; P=0.009), and fewer slow isochrones (4; IQR, 2.3-6.8 versus 6; IQR 5-7; P=0.008). Both central isthmus (R2=0.45; P<0.001) and non-isthmus CV (R2=0.71; P<0.001) correlated with pathway length, whereas central isthmus CV <0.15 m/s was ubiquitous for small circuits. Non-isthmus CV in tachycardia correlated with CV during block validation (R2=0.94; P<0.001) and a validation map to tachycardia conduction time ratio >85% predicted isthmus block in all cases. Over >1 year of follow-up, arrhythmia-free survival was better for homogeneous CV patterns (90% versus 57%; P=0.04). Conclusions Ultra-high-density mapping-guided CV analysis distinguishes atrial reentrant patterns in adults with congenital heart disease with surgical obstacles producing slower and smaller circuits. Very slow central isthmus CV may be essential for atrial tachycardia maintenance in small circuits, and non-isthmus conduction time in tachycardia appears to be useful for rapid assessment of postablation conduction block.

Element profiles in blood and teeth samples of children with congenital heart diseases in comparison with healthy ones.

BACKGROUND: Some elements were claimed to play a role in the pathogenesis of congenital heart defects (CHD) and influence the general well-being and health of these children. OBJECTIVES: We aimed to assess the levels of some elements simultaneously in the blood and teeth samples of children with cyanotic and acyanotic CHD compared with healthy children. METHODS: A total of 39 children with CHD (11 with cyanotic and 28 with acyanotic CHD) and 42 age- and sex-adjusted controls were enrolled. Levels of 13 elements, including magnesium, phosphorus, calcium, chromium, manganese, iron, copper, zinc, strontium, cadmium, lead, mercury, and molybdenum, were assessed using inductively coupled plasma mass spectrometry. RESULTS: Children with cyanotic and acyanotic CHD had significantly lower teeth calcium and calcium/phosphorus ratio as compared to the controls after adjusting for confounders. The mean blood iron level was found to be significantly higher in the cyanotic CHD group compared to the other groups. In addition, children with acyanotic CHD had significantly higher teeth copper levels, higher blood molybdenum and lower blood magnesium levels compared to the healthy control group. Blood cadmium and mercury levels were found to be significantly elevated in both the cyanotic and acyanotic CHD groups compared to the healthy control group. There were no differences in toxic metal levels of teeth in cases with CHD. CONCLUSION: Monitoring adequate and balanced gestational micronutrient intake might support not only maternal health but also fetal cardiac development and infant well-being. Supplementation of magnesium should be evaluated in patients having CHD.

The Effect of Continuous Field Block through Intercostal Muscles after Atrial Septal Defect Closure via a Mini-Right Thoracotomy in Pediatric Patients.

BACKGROUND: Postoperative pain management in thoracotomy patients often is difficult. Furthermore, pediatric patients present more challenges because of their inability to effectively communicate their pain intensity. The purpose of this study was to evaluate the use of continuous field block through intercostal muscles as postoperative pain management in pediatric thoracotomy. METHODS: Between 2014 and 2018, 11 patients underwent an ASD closure using a cardiopulmonary bypass via a mini-right thoracotomy through the fourth intercostal space. At the time of chest closure, a single-shot field block via the fourth intercostal muscles was performed with levobupivacaine (0.6 mg/kg). The first five patients were only given the single-shot field block (Single group). The remaining six patients were given levobupivacaine continuously (0.1 mg/kg/hr) through an indwelling catheter until the chest tube removal (Continuous group). The groups' vital signs, total amounts of acetaminophen used, postoperative courses were compared. RESULTS: Although the heart rate did not differ between the groups, the respiratory rate was significantly higher in the Single group versus the Continuous group at 16 and 32 hr post-surgery (35.6 ± 9.7/min vs. 18.5 ± 4.7/min; p=0.007, 43.0 ± 10.4 vs. 25.3 ± 3.1; p=0.042, respectively). The accumulated dosage of acetaminophen given by postoperative day 2 was significantly higher in the Single group versus the Continuous group (55.3 ± 22.1 mg/kg vs. 7.8 ± 17.4 mg/kg; p=0.012). CONCLUSIONS: Continuous field block via intercostal muscles after ASD closure via a mini-right thoracotomy in children was effective to stabilize the vital signs and reduce the analgesic medication use.

Frequency of thyroid dysfunction in pediatric patients with congenital heart disease exposed to iodinated contrast media - a long-term observational study.

Background The thyroid gland of patients with congenital heart disease may be exposed to large doses of iodine from various sources. We assessed the thyroid response after iodine exposure during conventional angiography in cardiac catheterization and angiographic computer tomography in childhood. Methods Retrospective mid- to long-term follow-up of 104 individuals (24% neonates, 51% infants, 25% children) with a median age and body weight of 104 days [0-8 years] and 5.3 kg [1.6-20]. Serum levels of thyroid-stimulating hormone, free triiodthyronine and free thyroxine were evaluated at baseline and after excess iodine. We also assessed risk factors that may affect thyroid dysfunction. Results Baseline thyroidal levels were within normal range in all patients. The mean cumulative iodinate contrast load was 6.6 ± 1.6 mL/kg. In fact, 75% had experienced more than one event involving iodine exposure, whose median frequency was three times per patient [1-12]. During the median three years follow-up period [0.5-10], the incidence of thyroid dysfunction was 15.4% (n=16). Those patients developed acquired hypothyroidism (transient n=14, long-lasting n=2 [both died]) with 10 of them requiring temporary replacement therapy for transient thyroid dysfunction, while four patients recovered spontaneously. 88 individuals (84.6%) remained euthyroid. Repeated cardiac interventions, use of drugs that interfere with the thyroid and treatment in the intensive care unit at the index date were strong predictors for acquired thyroid dysfunction. Conclusions The incidence of acquired hypothyroidism after iodine excess was 15.4%. However, most patients developed only transient hypothyroidism. Systemic iodine exposure seems to be clinically and metabolically well tolerated during long-term follow-up.

Neonatal surgical outcomes after prenatal diagnosis of complex congenital heart disease: experiences of a perinatal integrated diagnosis and treatment program.

BACKGROUND: This study aimed to evaluate neonatal surgical outcomes of patients diagnosed with complex congenital heart disease (CHD) during pregnancy and treated by the newly initiated "perinatal integrated diagnosis and treatment program (PIDTP)". METHODS: We reviewed clinical data of 207 neonates (surgical age ≤ 28 days) who underwent cardiac surgeries in a single center from January 2017 to December 2018, including 31 patients with referrals from the "PIDTP" (integration group) and 176 patients with routine referral treatment (non-integrated group). RESULTS: In the integration group, median admission age was 0 days and median age at surgery was 4 days. In the non-integrated group, median admission age was 8 days (P = 0.001) and median age at surgery was 13 days (P = 0.001). The emergency surgery rate in patients with duct-dependent defects was 36% in the integration group and 59% (P = 0.042) in the non-integrated group, respectively. The in-hospital mortality was 16% in the integration group and 14% (P = 0.78) in the non-integrated group. The 2-year cumulative survival rate after surgery was 83.9% ± 6.6% in the integration group and 80.3% ± 3.1% (P = 0.744) in the non-integrated group. According to multivariable regression analysis, independent risk factors for early mortality of overall neonatal cardiac surgery were low body weight, high serum lactate level, postoperative extracorporeal membrane oxygenation (ECMO) support and prolonged cardiopulmonary bypass (CPB) time. CONCLUSIONS: PIDTP shortens the postnatal transit interval, reduces the emergency operation rate of neonatal critical CHD, and provides better preoperative status for surgery. Patients treated by the PIDTP tend to have more complicated anatomical deformity and a greater requirement for the operation and postoperative management, but early outcome and follow-up prognosis are satisfactory.

Longitudinal Improvements in Radiation Exposure in Cardiac Catheterization for Congenital Heart Disease: A Prospective Multicenter C3PO-QI Study.

BACKGROUND: The C3PO-QI (Congenital Cardiac Catheterization Project on Outcomes - Quality Improvement), a multicenter registry launched in 2015, instituted quality improvement (QI) initiatives to reduce patient radiation exposure. Through regular collaboration, this initiative would allow for harmony among active participants, maximizing efforts and efficiency at achieving radiation best practices. This study sought to report these efforts with a detailed methodology for which institutions can target initiatives, reducing radiation exposure, and increasing patient safety. METHODS: Data were collected prospectively by 8 C3PO-QI institutions between January 1, 2015 and December 31, 2017. Radiation exposure was measured in dose area product per body weight (dose area product/kg; µGy*m2/kg) and reported by expected radiation exposure categories (REC) and institution for 40 published unique procedure types. Targeted interventions addressing selected strategic domains for radiation reduction were implemented in the pediatric catheterization labs of the C3PO-QI institutions. RESULTS: The study consisted of 15 257 unique cases. Median exposure (dose area product/kg) was decreased by 30% for all procedures. Dose area product/kg was reduced in all 3 REC, with the greatest improvement observed in REC I (REC I, -37%; REC II, -23%; REC III, -27%). Although the baseline radiation exposures and exact percent decrease varied across all C3PO-QI sites, each institution demonstrated improvements in radiation dose over time. These improvements occurred with the implementation of institution-specific QI interventions accelerated by participation in the C3PO-QI multicenter collaborative. CONCLUSIONS: Substantial radiation dose reductions can be achieved using targeted QI methodology and interventions. Participation in a multicenter QI collaborative may accelerate improvement across all centers due to enhanced engagement and shared learning between sites.

Pulse oximetry screening in a midwifery-led maternity setting with high antenatal detection of congenital heart disease.

AIM: To assess local and individual factors that should be considered in the design of a pulse oximetry screening strategy in New Zealand's midwifery-led maternity setting. METHODS: An intervention study was conducted over 2 years. Three hospitals and four primary maternity units participated in the study. Post-ductal saturation levels were measured on well infants with a gestation of ≥35 weeks. Infant activity and age (hours) at the time of the test were recorded. RESULTS: Screening was performed on 16 644 of 27 172 (61%) eligible infants. The age at which the screening algorithm was initiated varied significantly among centres. The probability of achieving a pass result (saturations ≥95%) in the context of no underlying pathology ranged from .94 for an unsettled infant screened <4 hours of age to .99 (P < .001) when the test was performed after 24 hours on a settled infant. Forty-eight (0.3%) infants failed to reach saturation targets: 37 had significant pathology of which three had cardiac disease. CONCLUSION: Screening practices were influenced by the setting in which it was undertaken. Infant activity and age at the time of testing can influence saturation levels. Screening is associated with the identification of significant non-cardiac pathology.

Iron intake and iron status during pregnancy and risk of congenital heart defects: A case-control study.

BACKGROUND: The relationships between iron nutritional status and congenital heart defects (CHDs) among humans are still unclear. This study aimed to explore the associations of maternal iron intake during pregnancy and maternal and neonatal iron status with CHDs. METHODS: This hospital-based case-control study analyzed 474 cases and 948 controls in Shaanxi China. Eligible women waiting for delivery in the hospital were interviewed to report their diets and characteristics during pregnancy. We conveniently collected maternal blood before delivery and neonatal cord blood to get a subgroup of 50 cases and 100 controls. Mixed logistic regression models were used to estimate ORs (95%CIs) for CHDs associated with iron intake. Mixed linear regression models were used to assess the relationships between CHDs and iron status. RESULTS: Mothers whose fetuses have CHDs were less likely to have higher intakes of total iron and heme iron during pregnancy, and the tests for linear trend were significant (all P < 0.05). Mothers whose fetuses have CHDs were less likely to take iron supplements during pregnancy (OR = 0.28, 95%CI: 0.21, 0.36) and during the first trimester (OR = 0.32, 95%CI: 0.12, 0.84). Maternal SF and Hb concentrations before delivery were lower and maternal sTfR/SF before delivery was higher among the cases than the controls. CONCLUSIONS: Mothers whose fetuses have CHDs are less likely to have higher intakes of total iron and heme iron and take iron supplements during pregnancy compared to their counterparts. Maternal iron status before delivery is low among mothers whose fetuses have CHDs.

Ultrahigh-density mapping supplemented with global chamber activation identifies noncavotricuspid-dependent intra-atrial re-entry conduction isthmuses in adult congenital heart disease.

OBJECTIVE: To evaluate the role of ultrahigh-density mapping for conduction isthmus (CI) characterization in adult congenital heart disease (ACHD). BACKGROUND: Catheter ablation remains suboptimal for ACHD with atypical intra-atrial reentrant tachycardias (IART) that can be challenging to define using existing mapping technology. METHODS: An ultrahigh-density mapping system was selectively employed over a 1-year period for procedures involving noncavotricuspid isthmus-dependent-IART. A global activation histogram (GAH) was assessed for the ability to predict ablation targets. Procedural characteristics were compared to a group of matched controls. RESULTS: Twenty patients (mean age 43 ± 15, 70% male) underwent 20 procedures targeting 34 tachycardias during the study period. Diagnoses included single ventricle (8), tetralogy of Fallot (2), left heart obstruction (3), Ebstein's anomaly (2) atrial septal defect (2), Mustard operation (2), and Rasteilli operation (1). Prior catheter ablation/Maze operation had been performed in 12 (60%). The median time per map was 21 minutes (interquartile range [IQR], 16-32), representing 14 834 points per map (IQR 9499-43 191; P < .001 vs controls). Review of GAH maps showed lower trough values were associated with more favorable IART CI characteristics (P ≤ =.001 for all). Acute success was achieved in 19/20 (95%) procedures, with tachycardia termination during the first lesion in eight cases (P = .02 vs controls). There was one recurrence during 0.6 years follow-up. CONCLUSIONS: Ultrahigh-density mapping supplemented with the GAH tool was effective for CI identification in a cohort of complex ACHD patients. Catheter ablation was more efficient compared to controls, suggesting precise CI characterization using this technology.

Long term follow-up after ventricular tachycardia ablation in patients with congenital heart disease.

BACKGROUND: Ventricular tachycardia (VT) is frequently encountered in patients with repaired and unrepaired congenital heart disease (CHD), causing significant morbidity and sudden cardiac death. Data regarding underlying VT mechanisms and optimal ablation strategies in these patients remain limited. OBJECTIVE: To describe the electrophysiologic mechanisms, ablation strategies, and long-term outcomes in patients with CHD undergoing VT ablation. METHODS: Forty-eight patients (mean age 41.3 ± 13.3 years, 77.1% male) with CHD underwent a total of 57 VT ablation procedures at two centers from 2000 to 2017. Electrophysiologic and follow-up data were analyzed. RESULTS: Of the 77 different VTs induced at initial or repeat ablation, the underlying mechanism in 62 (81.0%) was due to scar-related re-entry; the remaining included four His-Purkinje system-related macrore-entry VTs and focal VTs mainly originating from the outflow tract region (8 of 11, 72.7%). VT-free survival after a single procedure was 72.9% (35 of 48) at a median follow-up of 53 months. VT-free survival after multiple procedures was 85.4% (41 of 48) at a median follow-up of 52 months. There were no major complications. Three patients died during the follow-up period from nonarrhythmic causes, including heart failure and cardiac surgery complication. CONCLUSION: While scar-related re-entry is the most common VT mechanism in patients with CHD, importantly, nonscar-related VT may also be present. In experienced tertiary care centers, ablation of both scar-related and nonscar-related VT in patients with CHD is safe, feasible, and effective over long-term follow-up.

Operator-Directed Procedural Sedation in the Congenital Cardiac Catheterization Laboratory.

OBJECTIVES: The purpose of this study was to evaluate the association between the method of procedural sedation and outcomes for congenital cardiac catheterization procedures. BACKGROUND: The safety of operator-directed sedation (ODS) in the pediatric/congenital cardiac catheterization laboratory has been questioned. To our knowledge, the relative safety of ODS versus general anesthesia (GA) in these cases has not to date been critically evaluated. METHODS: A single-center retrospective cohort study was performed to compare the relative safety, cost, and times of catheterization procedures performed with ODS and those performed with GA from a cardiac anesthesiologist. The risk of adverse outcomes was compared using propensity-score-adjusted models. Using the same propensity score, procedure times and relative charges were also compared. RESULTS: Over the study period, 4,424 procedures in 2,547 patients were studied. Of these, 27% of cases were performed with ODS. ODS procedures were 70% diagnostic procedures, 17% device closure of patent ductus arteriosus, 5% balloon pulmonary valvuloplasty, and 3% pulmonary artery angioplasty. The risk of adverse event in adjusted models for ODS cases was significantly lower than in GA cases (odds ratio: 0.66; 95% confidence interval: 0.45 to 0.95; p = 0.03). Total room time and case time were also significantly shorter (p < 0.001). Professional (charge ratio: 0.88; p < 0.001) and hospital (charge ratio: 0.84; p < 0.001) charges for ODS cases were also lower than those for GA cases. CONCLUSIONS: This study demonstrates that clinical judgment can identify subjects in whom ODS is not associated with increased risk of adverse events. The use of ODS was associated with reduced case times and charges. In combination, these findings suggest that the selective use of ODS can allow for greater efficiency and higher value care without sacrificing safety.

Improving health perception through a transition care model for adolescents with congenital heart disease.

AIMS: The aim of this study was to assess the impact of a transition clinic model on adolescent congenital heart disease (CHD) patients' health perception outcomes. The transition clinic model consists of multidisciplinary standardized interventions to educate and support CHD patients and represents a key element in the adequate delivery of care to these individuals during their transition from childhood to adulthood. Currently, empirical data regarding the impact of transition clinic models on the improvement of health perceptions in CHD adolescent patients are lacking. METHODS: A quasi-experimental design was employed. Quality of life, satisfaction, health perceptions and knowledge were assessed at the time of enrolment (T0) and a year after enrolment (T1), respectively. During the follow-up period, the patients enrolled (aged 11-18 years) were involved in the CHD-specific transition clinic model (CHD-TC). RESULTS: A sample of 224 CHD adolescents was enrolled (60.7% boys; mean age: 14.84 ±â€Š1.78 years). According to Warnes' classification, 22% of patients had simple heart defect, 56% showed moderate complexity and 22% demonstrated severe complexity. The overall results suggested a good impact of the CHD-TC on adolescents' outcomes, detailing in T1 the occurrence of a reduction of pain (P < 0.001) and anxiety (P < 0.001) and an improvement of knowledge (P < 0.001), life satisfaction (P < 0.001), perception of health status (P < 0.001) and quality of life (P < 0.001). CONCLUSION: The CHD-TC seems to provide high-quality care to the patient by way of a multidisciplinary team. The results of the present study are encouraging and confirm the need to create multidisciplinary standardized interventions in order to educate and support the delivery of care for CHD adolescents and their families.

Advanced care planning in adult congenital heart disease: Transitioning from repair to palliation and end-of-life care.

As a result of advances in pediatric care, the majority of patients born with congenital heart disease (CHD) survive into adulthood [1]. Effective transfer and transition programs assure that patients with CHD remain in follow-up and receive continuous holistic care. Unfortunately, adult patients with CHD carry residual lesions and sequelae putting them at risk for premature death related to re-interventions or complications; most commonly heart failure and arrhythmia [2]. The scientific adult CHD (ACHD) community has been working hard to identify variables related to worse outcomes, modifying those where possible in order to improve survival. Indeed, survival in adults with CHD has increased, but consequently, on top of CHD-related complications, patients are increasingly exposed to the standard cardiovascular risk factors. Therefore, a program for lifelong coaching on health behavior and life style management becomes indispensable. More emerging is that a substantial number of patients, in particular those with complex heart defects, will eventually end up in a stage with hardly any medical or interventional options left. Our healthcare provision has to be prepared to organize care for this specific group of patients who will die prematurely and require the timely development and establishment of advanced care planning. Advanced care planning should preferentially be set-up in expert CHD centers. The long-lasting relationship in ACHD care with healthcare providers offers an excellent basis with regards to prognosis, advanced care planning and end-of-life issues.