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There are contradictory reports whether exercise capacity is reduced in patients on long-term follow-up after coarctation repair. Data from unselected patient groups are missing. In a cross-sectional, long-term follow-up study of a tertiary congenital cardiology referral center, 260 patients (30.2 ± 11.4 years old, 84 women), after surgical repair for isolated aortic coarctation (age at surgery 11.5 ± 11.2 years), underwent a symptom-limited exercise test. Peak workload was 180 ± 52 W, significantly less than the age- and height-related reference values (p <0.0005). A peak workload under 80% of expected was found in 200 patients (77%). Exercise performance of the patients was independent from age at surgery, type of surgery, or the systolic brachial-ankle blood pressure difference. The only exercise-limiting factor found was the chronic administration of diuretics to treat hypertension (p = 0.005). Exercise hypertension, defined as a systolic blood pressure >2 SD above the load-dependent reference value, was found in 73 patients (28%). It was independently related to the systolic brachial-ankle blood pressure difference (p <0.0005) and diuretics administration (p = 0.037). In conclusion, most patients after coarctation repair have a reduced exercise performance. This reduction is not related to the surgical results. Particularly, as these patients are at risk of early atherosclerosis, exercise should be promoted as primary prevention after restenosis, aortic or cerebral aneurysms, and severe exercise hypertension are ruled out. [Copyright &y& Elsevier]

Patients after coarctation repair still have an increased risk of cardiovascular or cerebrovascular events. This has been explained by the persisting hypertension and alterations in the peripheral vessels. However, involvement of the central vessels such as the retinal arteries is virtually unknown. A total of 34 patients after coarctation repair (22 men and 12 women; 23 to 58 years old, age range 0 to 32 years at surgical repair) and 34 nonhypertensive controls underwent structural and functional retinal vessel analysis. Using structural analysis, the vessel diameters were measured. Using functional analysis, the endothelium-dependent vessel dilation in response to flicker light stimulation was assessed. In the patients after coarctation repair, the retinal arteriolar diameter was significantly reduced compared to that of the controls (median 182 μm, first to third quartile 171 to 197; vs 197 μm, first to third quartile 193 to 206; p <0.001). These findings were independent of the peripheral blood pressure and age at intervention. No differences were found for venules. The functional analysis findings were not different between the patients and controls (maximum dilation 3.5%, first to third quartile 2.1% to 4.5% vs 3.6%, first to third quartile 2.2% to 4.3%; p = 0.81), indicating preserved autoregulative mechanisms. In conclusion, the retinal artery diameter is reduced in patients after coarctation repair, independent of their current blood pressure level and age at intervention. As a structural marker of chronic vessel damage associated with past, current, or future hypertension, retinal arteriolar narrowing has been linked to stroke incidence. These results indicate an involvement of cerebral microcirculation in aortic coarctation, despite timely repair, and might contribute to explain the increased rate of cerebrovascular events in such patients. [Copyright &y& Elsevier]

The aim of the study was to assess the quantity and nature of emergencies affecting adults with congenital cardiac disease (CCD) and evaluate infrastructural requirements for adequate management. There is an increasing number of adults with CCD requiring specialized complex care. This multicenter study evaluated all emergency admissions to 1 of 5 centers for adults with CCD within 1 year. Within 1 year, there were 1,033 admissions of adults with CCD, and 201 (160 patients; age 16 to 71 years) were emergencies. Underlying cardiac anomalies were univentricular heart (22%), complete transposition (14%), tetralogy of Fallot (21%), and others (43%). Seventy percent of patients had undergone previous cardiac surgery. The main reason for acute admission was cardiovascular (arrhythmia, heart failure, syncope, aortic dissection, and endocarditis). Diagnostic procedures most often assigned were echocardiography (n = 223), chest x-ray (n = 95), Holter electrocardiography (n = 85), cardiac catheterization/electrophysiologic study (n = 39), and others (n = 143). Forty-six patients underwent surgery (cardiovascular n = 41, general n = 5) or electrophysiologic treatment (n = 41). One hundred twenty-six of 201 emergencies (63%) required cooperation with another specialized department: surgery (n = 46), internal medicine (n = 42), neurology (n = 12), ophthalmology (n = 6), otorhinolaryngology (n = 5), gynecology (n = 5), psychiatry (n = 4), radiology (n = 3), dermatology (n = 2), and orthopedics (n = 2). In conclusion, physicians and consultants attending adult patients with CCD need a high degree of specialized experience concerning the cardiac anomaly to manage emergencies properly. Furthermore, a wide range of noncardiac diagnostic and therapeutic procedures must be available. Data support the demand for a multidisciplinary approach in specialized centers for adequate care of adults with CCD. [Copyright &y& Elsevier]

Sedentary lifestyle is reported to be associated with diminished exercise capacity, resulting in increased cardiovascular risk in adults with congenital heart disease (CHD). This cross-sectional study examined the association between objectively measured physical activity (PA) and exercise capacity in children and adolescents with CHD. Therefore, 107 patients (aged 13.0 ± 2.7 years, 41 girls) with various CHD performed a cardiopulmonary exercise test to quantify their peak oxygen uptake (peakV'O2). Moderate to vigorous PA (MVPA) and daily step count were assessed using Garmin vivofit jr. (Garmin, Germany) for 7 consecutive days. For association between PA and submaximal exercise capacity, Spearman correlation was performed. Patients with CHD showed almost normal values compared with the reference (79.5 ± 17.2% [31.6 to 138.1] %peakV'O2 predicted), with roughly normal ventilatory anerobic thresholds (50.6 ± 14.0% [20.3 to 97.9] % oxygen uptake at ventilatory anaerobic threshold [VATV'O2]). Step counts are below the recommendations (9,304 ± 3,792 steps/day [1,701 to 20,976]), whereas MVPA data are above the recommendations for children with ≥60 min/day (83.6 ± 34.6 min/day [10.1 to 190.9]). The Spearman rho showed significant positive correlations to VATV'O2 (r = 0.353, p <0.001) and %VATV'O2 (r = 0.307, p = 0.001), with similar results regarding MVPA (VATV'O2: r = 0.300, p = 0.002 and %VATV'O2: r = 0.270, p = 0.005). In conclusion, submaximal exercise capacity and PA correlate positively, making both assessments relevant in a clinical setting: PA in the context of cardiovascular prevention and peakV'O2 as the strongest predictor for morbidity and mortality., Competing Interests: Declaration of competing interest JH and LW received an unrestricted grant from Stiftung KinderHerz Deutschland gGmbH. The authors have no competing interests to declare., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

With rising survival rates of patients with congenital heart disease (CHD), functional health variables have become the key aspect in treatment evaluation. The effectiveness of various treatment options on the health-related quality of life (HRQoL) and the objectively measured exercise capacity as peak oxygen uptake (VO 2 peak) remains rather unclear and hence, its investigation is the primary aim of this study. Data from 1014 patients (≥14-years-old, various CHD) were retrospectively reviewed. The patients had completed at least twice the SF-36 questionnaire on HRQoL prior to a cardiopulmonary exercise test. Each patient was assigned to 1 of 4 treatment groups (i.e., surgery, catheter intervention, drug therapy, and surveillance) according to the received treatment between the baseline and the follow-up examination. After 4.0 ± 2.2 years of follow-up, patients with surgery and catheter intervention showed an increase in the physical summary score of HRQoL as compared to the other treatment groups (p <0.001). This effect remained also significant in a multivariable model accounting for anthropometric and baseline data. No significant differences in the mental summary score of HRQoL and the VO 2 peak were evident between the different treatment groups in the multivariable model. No significant correlation was found between the changes in HRQoL and VO 2 peak over time. In conclusion, despite insignificant changes in aerobic capacity, adolescents and adults with CHD report better physical HRQoL following surgery and catheter intervention compared to the other treatment options. HRQoL and exercise capacity need to be considered concurrently in the evaluation of adolescents and adults with CHD., (Copyright © 2019 Elsevier Inc. All rights reserved.)