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Background Nowadays, more than 90% of patients with congenital heart disease (CHD) reach adulthood. However, long-term impact on neurodevelopment and executive functioning in adults with CHD are not completely understood. Purpose To investigate the self- and informant-reported executive functioning in adults with CHD operated in childhood. Material and methods Longitudinal study of a cohort of patients (n = 194, median age: 49.9 [46.1–53.8]) who were operated in childhood (< 15 years old) between 1968 and 1980 (median follow-up time: 45 [40–53] years) for one of the following diagnoses: atrial septal defect (ASD), ventricular septal defect (VSD), pulmonary stenosis (PS), tetralogy of Fallot (ToF) or transposition of the great arteries (TGA). Behavior Rating Inventory of Executive Function-Adult version (BRIEF-A) questionnaire was used to assess self- and informant-reported executive functioning. Results 40–53 years after surgery, the CHD group did show significantly better executive functioning compared to the norm data. No significant difference was found between mild CHD (ASD, VSD and PS) and moderate/severe CHD (ToF and TGA). Higher education, NYHA class 1 and better exercise capacity were associated with better self-reported executive functioning, whereas females or patients taking psychiatric or cardiac medications reported worse executive functioning. Conclusions Our findings suggest favorable outcomes (comparable to normative data) regarding executive functioning in adults with CHD, both self- and informant-reported. However, further study is warranted to explore more in detail the different cognitive domains of executive functioning in these patients. Graphical abstract

Introduction Nowadays, more than 90% of patients with congenital heart disease (CHD) reach adulthood. However, knowledge about their psychosocial functioning is limited. Methods Longitudinal cohort study of patients (n = 204, mean age: 50 years, 46.1% female) who were operated during childhood ( Results After a median follow-up of 45 [40–53] years adults with CHD had a significantly lower educational level, occupation level and employment rate, but better health-related quality of life and emotional functioning compared with normative data. Patients with moderate/severe defects reported significantly more self-perceived physical restrictions and lack of physical strength due to their CHD. Compared to 2011, in 2021 patients considered their CHD as more severe and they felt more often disadvantaged. Conclusions Overall, despite a lower education, occupation level and employment rate, our sample of patients with CHD had a positive perception of their life and their psychosocial functioning was even better than the norm. Although the quality of life was very good, their view on their disease was more pessimistic than 10 years ago, especially for patients with moderate/severe CHD. Graphic abstract

Background For children with congenital heart disease (CHD), subtle neuropsychological deficits have been reported. However, very little is known about executive functioning in their adulthood. Purpose To investigate the self- and informant-reported executive functioning in adults with CHD operated in childhood ( Material and methods A cohort study of 194 patients (age 50 [46–54] years), operated in childhood between 1968 and 1980 for one of the following diagnosis: atrial septal defect (ASD), ventricular septal defect (VSD), pulmonary stenosis (PS), tetralogy of Fallot (ToF) or transposition of the great arteries (TGA), were evaluated 40–53 years after surgery. The “Behavior Rating Inventory of Executive Functions – Adult version” (BRIEF-A) questionnaire was used to assess self- and informant-reported executive functioning and compared to the general population. Results The CHD group did not show worse executive functioning compared to normative data. In addition, no significant difference was found between simple CHD (ASD, VSD and PS) and moderate/complex CHD (ToF and TGA). Higher education and NYHA class 1 were associated with better self-reported executive functioning, while male patients and patients taking psychiatric or cardiac medications reported worse executive functioning. Conclusions Overall, our findings suggest reassuring outcomes regarding executive functioning in adults with CHD. However, these findings should be confirmed with neuropsychological assessment studies. Funding Acknowledgement Type of funding sources: Foundation. Main funding source(s): Thorax Foundation

This article provides expert opinion on the use of cardiovascular magnetic resonance (CMR) in young patients with congenital heart disease (CHD) and in specific clinical situations. As peculiar challenges apply to imaging children, paediatric aspects are repeatedly discussed. The first section of the paper addresses settings and techniques, including the basic sequences used in paediatric CMR, safety, and sedation. In the second section, the indication, application, and clinical relevance of CMR in the most frequent CHD are discussed in detail. In the current era of multimodality imaging, the strengths of CMR are compared with other imaging modalities. At the end of each chapter, a brief summary with expert consensus key points is provided. The recommendations provided are strongly clinically oriented. The paper addresses not only imagers performing CMR, but also clinical cardiologists who want to know which information can be obtained by CMR and how to integrate it in clinical decision-making.

Echocardiographic left and right ventricular sequences are usually interpreted visually, but current quantitation techniques have been found to be tedious, time-consuming and associated with significant inter- and intra-observer variabilities. In an attempt to eliminate these problems, we have developed the Echocardiographic Analysis System (EAS) which uses robust automated border detection techniques both for single frames as well as for sequences. Comparison of LV cross-sectional area with the semi-automated border detection (AUTO) and those assessed from manual tracings (MAN) yielded a systematic difference (MAN-AUTO) of -6.6% (p < 0.001), and a random difference (standard deviation of paired signed differences) of 11.8%. Current developments are directed towards real-time automated border detection with an Accelerator board, integration of Acoustic Quantification (AQ) data as edge information into EAS, and intravascular echocardiographic applications.

To unravel the mechanisms underlying the previously demonstrated associations between low birthweight and cardiovascular disease in adulthood, we examined whether maternal smoking during pregnancy leads to fetal arterial resistance adaptations, and subsequently to fetal growth retardation and changes in postnatal blood pressure and cardiac development.Prospective cohort study from early fetal life onwards.Academic hospital.Analyses were based on 1120 children aged 2 years.Maternal smoking during pregnancy [non-smoking, first trimester smoking, continued smoking (5 and ≥ 5 cigarettes/day)] was assessed by questionnaire.Third trimester placental and fetal arterial resistance indices and fetal growth were assessed by ultrasound and Doppler measurements. Postnatal blood pressure and cardiac structures (aortic root diameter, left atrial diameter, left ventricular mass) were measured at 2 years of age.First trimester smoking was not associated with third trimester placental and fetal blood flow adaptations. Continued smoking of ≥ 5 cigarettes/day was associated with an increased resistance in uterine, umbilical and middle cerebral arteries, and with a decreased flow and diameter of the ascending aorta. Among mothers who continued to smoke, the third trimester estimated fetal weights and birthweights were most affected in children with the highest umbilical artery resistance. Fetal arterial resistance indices were also associated with aortic root diameter and left atrial diameter.Fetal arterial resistance adaptations may be involved in the pathways leading from maternal smoking during pregnancy to low birthweight and cardiovascular developmental changes in childhood in the offspring.

Evaluation of the first results in the Netherlands of percutaneous and transvenous closure of an ASD II in children with an Amplatzer Septal Occluder (ASO).Prospective.Data were collected from children with an ASD II prior to, during and up to 24 months after the insertion of an ASO during heart catheterisation in Leiden University Hospital, the Netherlands.Between 1 January 1998 and 29 February 2000, 28 patients (12 girls, 16 boys; mean age: 74 months (range: 15-198 months)) underwent heart catheterisation to close an ASD II with an ASO. In 26 patients an ASO could be placed without significant complications. The size of the device varied from 9-34 mm (median 16 mm). In one patient ASD closure was not attempted because of multiple ASDs. In another patient the procedure was stopped after air embolism into the coronary arteries had occurred during preparation of ASO implantation. In 23/26 patients with an implanted ASO, no residual shunt was present after 24 hours. One child, in whom the defect was found to be closed after 24 hours and after three weeks, returned abroad and was lost to follow-up. After one year all defects (n = 22) were completely closed.Percutaneous transvenous closure of an ASD II with an ASO was possible, was not associated with any significant complications and had a high success rate, even in relatively young children with large defects.

In the last four decades the survival of patients with corrected or palliated congenital heart disease has increased dramatically. However, post-operative abnormalities frequently occur and therefore a noninvasive imaging tool is mandatory for the timely detection of morphological as well as functional abnormalities. Magnetic resonance imaging (MRI) is ideally suited for the noninvasive diagnosis and post-operative follow-up of congenital heart disease. Spin-echo MRI is able to visualize structures that may be difficult to assess with other noninvasive image modalities and is sensitive in the detection of post-interventional stenoses or aneurysms. Because the function of the ventricles may deteriorate over time after correction or palliation of a congenital cardiac malformation, the use of gradient-echo MRI is essential in the follow-up after correction or palliation, as no other conventional technique allows such detailed evaluation of ventricular function, without geometrical assumptions. Phase-contrast MRI is well suited to assess valvular function, allowing accurate measurement of regurgitation or stenosis. Shunt quantification is another application of phase-contrast MRI. J. Magn. Reson. Imaging 1999;10:656-666.

In this article the value of magnetic resonance (MR) imaging for the evaluation of double outlet right ventricle (DORV) is reviewed from the literature and illustrated with several cases. MR imaging can be used for the determination of cardiac anatomy at initial diagnosis and may provide functional information during the follow-up of patients after surgical correction.

Few data exists on the differences in long-term outcome between Mustard and Senning operations. We reviewed available data of all hospital survivors of these operations and assessed risk factors for late death and sinus node dysfunction. Of those patients undergoing the Mustard operation, 60 were hospital survivors (46 simple transposition, 14 complex); of those patients undergoing the Senning operation, 62 were hospital survivors (43 simple, 19 complex). Median duration of follow-up was 16 years (maximum 25 years) for Mustard operation, 11 years (maximum 20 years) for Senning operation. No reoperations were done, except for pacemaker implantation. No differences were found between the two groups with regard to baffle-associated problems, right ventricular failure, sudden death (6% in both groups), and functional status at final follow-up (New York Heart Association class I or II, except for four patients). For patients undergoing the Mustard operation, survival at 16-year follow-up was 91% with simple transposition and 60% with complex transposition (p = 0.027); for both groups of patients undergoing the Senning operation, survival at 16-year follow-up was 78%. Survival in the absence of rhythm disturbance at 16-year follow-up was 18% for Mustard operation and 53% for Senning operation (p0.001). In multivariate analysis, significant independent risk factors for late death turned out to be complex transposition (versus simple) and active arrhythmias. The only significant risk factor for the occurrence of sinus node dysfunction was the Mustard operation. We conclude that apart from the difference in the loss of sinus rhythm, no differences were found in the long-term clinical results of the two types of operations.

Infants and children with achondroplasia are at increased risk of sudden death because of apneic attacks caused by compression of the medulla oblongata or spinal cord by a constricted foramen magnum or narrow upper cervical spinal canal. This history of an infant with achondroplasia is discussed. As a result of apneic attacks she developed severe brain damage. Cervicomedullary compression was revealed at CT-scan and NMRI of the basicranium and upper cervical canal, and confirmed at decompressive surgery. Early symptoms can be clues to the existence of cervicomedullary compression. These clues are indication for further investigations. Decompressive surgery has good results when performed at an early stage. Knowledge of the signs and symptoms of cervicomedullary compression and of factors which increase the risk of complications are important in the management of achondroplastic patients.