Executive functioning of patients with congenital heart disease: 45 years after surgery.

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.

Daily life and psychosocial functioning of adults with congenital heart disease: a 40-53 years after surgery follow-up study.

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 (< 15 years) between 1968 and 1980 for one of the following diagnoses: atrial septal defect, ventricular septal defect, pulmonary stenosis, tetralogy of Fallot or transposition of the great arteries. Psychosocial functioning was measured every 10 years, using standardized and validated questionnaires. Results were compared with the general Dutch population and over time. 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.

Fate of the Arterial Origin of Major Aortopulmonary Collateral Arteries After Unifocalization.

BACKGROUND: During unifocalization procedures for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries, collateral arteries are either ligated or detached. Not much is known of the fate of the remaining arterial origins in the long term. Available computed tomography (CT) or magnetic resonance (MR) imaging of the intrathoracic arteries was examined to investigate possible abnormalities at the former position of the collateral arteries as well as ascending aortic diameters. METHODS: From 1989 to 2018, we performed 66 unifocalization procedures in 39 patients. One hundred and twenty-nine collateral arteries were ligated or detached. In 52% (15) of the surviving patients (with a total of 55 ligated or detached collaterals), sufficient imaging of the thoracic aorta from CT (11) and/or MR (9) was available for evaluation. RESULTS: The median interval between unifocalization procedure and imaging was 15 years (interquartile range [IQR]: 9-19 years). In 93% (14) of the scanned patients, 18 blunt ends were detected at the location of a former collateral artery. No aneurysm formation of the descending aorta was observed. The median diameter of the ascending aorta was 35 mm (IQR: 31-40 mm). During follow-up, no aortic dissection or rupture occurred. CONCLUSIONS: Aortic imaging late after unifocalization showed abnormalities in 93% of the scanned patients. Abnormalities consisted mostly of blunt ends of the former collateral artery. We recommend to include routine imaging of the aorta during late follow-up to detect eventual future abnormalities and monitor aortic diameters. Ascending aortic diameters showed slight dilatation with no clinical implications so far.

Transcatheter device closure of atrial septal defects in patients aged 40 years and older.

OBJECTIVE: To evaluate the safety and effect of transcatheter device closure in ostium secundum atrial septal defects (ASD II) in patients aged 40 years and older. METHODS: Retrospective single-centre study concerning 47 consecutive ASD transcatheter occlusion procedures performed between January 1999 and December 2008. Electrocardiography, echocardiography and clinical assessments of the patients were conducted pre- and post-intervention and at follow-up. RESULTS: Of the 130 patients who were referred for interventional ASD closure, 47 were 40 years and older and all of them actually had the device inserted. There were no major complications during the intervention. Mean follow-up time was 15±15 months. During follow-up, three patients needed surgical reintervention because of device embolisation (n=2) or dislocation (n=1). Of the patients with severe right ventricular (RV) dilatation, more than half (58%) had no or mild dilatation at last follow-up. Reduction of RV dilatation was not related to age. Pulmonary hypertension was present in 63% before the procedure and was reduced to 38% at follow-up. NYHA class improved in all age groups, also in patients over 60 years of age. In two of the three patients who died during follow-up, no cause of death could be established, but both had responded well to treatment regarding the echocardiographic and clinical findings. CONCLUSION: Transcatheter device closure of ASD is a successful and effective treatment, also for patients aged 40 years and older. Patients showed regression of right ventricular enlargement and an improvement in functional class. (Neth Heart J 2010;18:537-42.).

Endovascular stenting for aortic (re)coarctation in adults.

Objectives. Stenting for native and recurrent coarctation (COA) in adults has become an important therapeutic strategy. In this prospective observational study we evaluated the intermediate-term outcome of stent implantation for either native or recurrent (re)COA in adults.Methods. All adults who underwent stent implantation in our institution between January 2003 and December 2008 were included. Diagnosis of (re)COA was based upon a combination of clinical signs, noninvasive imaging or invasive gradient measurements. NuMED stents were implanted under general anaesthesia.Results. Twenty-four patients (50% male) underwent stent implantation for native (n=6) or reCOA (n=18) at a median age of 36 (18 to 60) years. There was significant improvement in pre- versus post-stent invasive systolic gradient (19 vs. 0 mmHg, p<0.001) and COA diameter (10 vs. 16 mm, p<0.001). Acute complications (12.5%) included death due to aortic rupture despite immediate successful coverage with a covered stent (n=1) and groin haematoma (n=2). During a median follow-up period of 33 (8 to 77) months (n=22), late complications occurred in three patients (13.6%): stent migration to the ascending aorta (n=1), pseudoaneurysm at the site of the initial stent (n=1), and occluded external iliac artery (n=1). Stent implantation did not reduce the need for antihypertensive medication or blood pressure at last follow-up. Conclusion. COA stenting results in a significant gradient decrease and increase in vessel diameter. However, serious complications do occur and hypertension remains in the majority of patients. (Neth Heart J 2010;18:430-6.).