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AbstractObjectives:Atrioventricular valve regurgitation in patients with univentricular heart is a well-known risk factor for adverse outcomes and atrioventricular valve repair remains a particular surgical challenge.Methods:We reviewed all surgical atrioventricular valve procedures in patients with univentricular heart and two separate atrioventricular valves who underwent surgical palliation. Endpoints of the study were reoperation-free survival and cumulative incidence of reoperation.Results:Between 1994 and 2021, 202 patients with univentricular heart and two separate atrioventricular valve morphology underwent surgical palliation, with 15.8% (32/202) requiring atrioventricular valve surgery. Primary diagnoses were double inlet left ventricle (n = 14, 43.8%), double outlet right ventricle (n = 7, 21.9%), and congenitally corrected transposition of the great arteries (n = 7, 21.9%). Median weight at valve surgery was 10.6 kg (interquartile range, 7.9–18.9). Isolated left or right atrioventricular valve surgery was required in nine (28.1%) and 22 patients (68.8%), respectively. Concomitant left and right atrioventricular valve surgery was performed in one patient (3.1%). Closure of the left valve was conducted in four patients (12.5%) and closure of the right valve in three (9.4%). Operative and late mortality were 3.1% and 9.7%, respectively. Reoperation-free survival and cumulative incidence of reoperation at 10 years after surgery were 62.3% (standard error of the mean: 6.9) and 30.9% (standard error of the mean: 9.6), respectively.Conclusions:In patients with univentricular heart and two separate atrioventricular valves, surgical intervention on these valves is required in a minority of patients and is associated with low mortality but high incidence of reoperation.

Hintergrund: Die Zahl der Erwachsenen mit angeborenen Herzfehlern (EMAH) steigt stetig. Die vorliegende Querschnittsstudie wurde konzipiert, um die EMAH-Versorgung aus Sicht der Patienten und der Hausärzte (Fachärzte für Allgemeinmedizin und für Innere Medizin, praktische Ärzte) zu untersuchen. Methode: Fragebogenbasierte Querschnittsstudie zur Analyse der realen Versorgungssituation von EMAH in Deutschland aus Sicht der Patienten sowie der Hausärzte. Ergebnisse: Der Fragebogen wurde von 4493 EMAH (53,7 % weiblich; 41,3 ± 16,9 Jahre) und 1055 Hausärzten ausgefüllt. Die Mehrheit der EMAH (79,8 %) besuchten ihren Hausarzt wegen nichtkardialer Gesundheitsprobleme, aber auch wegen kardialer Probleme. Fast alle EMAH hatten erheblichen medizinischen Beratungsbedarf (Leistungsfähigkeit, Berufsfindung etc.). 2014 Patienten (44,8 %) kannten keine zertifizierten EMAH-Spezialisten oder -Zentren. 2816 Befragte (62,7 %) waren nicht über EMAH-Patientenorganisationen informiert. 87,5 % der Hausärzte hatten EMAH aller Schweregrade wegen vitientypischer Rest- und Folgeerscheinungen betreut. Viele kannten keine zertifizierten EMAH-Spezialisten. Nur 28,5 % zogen einen EMAH-Spezialisten konsiliarisch hinzu. Nur 23,5 % kannten EMAH-Patientenorganisationen. Schlussfolgerung: Hausärzte sind eine tragende Säule der EMAH-Versorgung in Deutschland. Die vorliegende Studie zeigt, dass EMAH und ihre Hausärzte, trotz des großen Bedarfs an spezialisierter Betreuung, weitgehend uninformiert sind über die bundesweit vorhandenen EMAH-Versorgungsstrukturen. Um die Morbidität und Mortalität der Betroffenen niedrig zu halten, müssen Lösungen entwickelt werden, um in Kooperation mit den auf EMAH spezialisierten Kinderkardiologen, Kardiologen und Zentren die Hausärzte stärker in die vorhandenen EMAH-Versorgungsstrukturen einzubinden bzw. zu integrieren.

Background:This study aims to evaluate clinical outcomes and hemodynamic variables late after the Björk procedure, regarding the pulmonary flow pattern. Methods:Patients who survived more than 15 years after the Björk procedure were included and then divided into two groups according to their pulmonary flow pattern by pulsed-wave Doppler assessment of echocardiography: patients with pulsatile systolic pulmonary flow (Group P) and those without (Group N). Results:A total of 43 patients were identified, of whom 13 patients were divided into Group P and 30 in Group N. Median age at the Björk procedure was 5.7 (2.1-7.3) years, and median follow-up was 32 (28-36) years. Survival after 15 years was higher in Group P, compared with Group N (100% vs 76% at 30 years, P= .045). Cardiac catheterization data demonstrated higher cardiac index in Group P patients compared with Group N patients (3.5 vs 2.8 L/m2, P= .014). Cardiac magnetic resonance imaging study revealed that Group P patients had higher right ventricular end-diastolic volume index (96 vs 57 mL/m2, P= .005), higher end-systolic volume index (49 vs 30 mL/m2, P= .013) and higher right ventricular stroke volume index (48 vs 25 mL/m2, P< .001), compared with Group N patients. Exercise capacity tests demonstrated that Group P patients showed a higher percent predicted peak oxygen consumption, compared with Group N patients (73 vs 58%, P< .001). Conclusions:Late after the Björk procedure, patients with a pulsatile systolic pulmonary flow had a larger right ventricle and better exercise capacity compared with those without pulsatile systolic pulmonary flow.

Background We aimed to evaluate incidence, outcomes, and predictors of protein-losing enteropathy (PLE) and plastic bronchitis (PB) in a cohort of total cavopulmonary connection (TCPC).Methods We included 620 consecutive patients undergoing TCPC between 1994 and 2021. Prevalence and predictors for onset of PLE/PB were evaluated. Death and heart transplantation after onset of PLE/PB were examined.Results A total of 41 patients presented with PLE/PB (31 with PLE, 15 with PB, and 5 developed both PLE and PB). Their median age at TCPC was 2.2 (interquartile ranges [IQRs], 1.7-3.7) years, and time period to onset for PLE was 2.6 (IQR: 1.0-6.6) years and for PB was 1.1 (IQR: 0.3-4.1) years after TCPC. Independent factors for developing PLE/PB were dominant right ventricle (RV, hazard ratio [HR], 2.243; 95% confidence interval [CI], 1.129-4.458, P= .021) and prolonged pleural effusion after TCPC (HR, 2.101; 95% CI, 1.090-4.049, P= .027). In PLE/PB population, freedom from death or transplantation after PLE/PB diagnosis at 5 and 10 years were 88.7% and 76.4%, respectively. Eleven surgical interventions were performed in 10 patients, comprising atrioventricular valve repairs (n = 4), Fontan pathway revisions (n = 2), pacemaker implantation (n = 2), secondary fenestration (n = 1), diaphragm plication (n = 1), and ventricular assist device implantation (n = 1). In nine patients, a recovery from PLE with the resolution of PLE symptoms and normal protein levels was achieved. Eight patients died and the remaining continued to have challenging protein loss.Conclusions Protein-losing enteropathy and PB remain severe complications in the cohort of TCPC. Patients with dominant RV, and prolonged pleural effusions, were at risk for PLE/PB.

AbstractBackground:Impairments and developmental delay are often reported in infants and young children with CHD. However, currently, there is no data regarding cognitive abilities assessed by standardised intelligence tests in adults with CHD. This study assesses the cognitive function in Fontan patients compared with acyanotic CHD patients whether restrictions in cognitive function are present in adulthood and its association with health-related quality of life.Methods:Forty-four adult CHD (female n = 21 (47.7%); mean age 34.7 ± 11.9 years), 22 with Fontan circulation and 22 with acyanotic CHD, underwent the Wechsler Intelligence Scale for adults as patients during routine follow-up in 2018. The Medical Outcomes Study Questionnaire Short-Form 36 Health Survey (SF-36) assessed health-related quality of life.Results:Fontan patients showed significantly better results in the FSIQ (p = 0.020) and perceptual reasoning (p = 0.017) in comparison with patients with acyanotic CHD. All adult CHD patients showed normal IQ in subscales and full-scale IQ (FSIQ). In health-related quality of life, no association with cognitive function was found and no significant difference between both CHD groups, but trends to reduced values in acyanotic adult CHD.Conclusions:Interestingly, our study results in adult Fontan patients showed that it is possible to live an adult life with normal cognitive function and good health-related quality of life with a univentricular heart. Thus, this study could be a guidepost for more in-depth studies on cognitive function in Fontan survivors. In addition, the focus should be on health-related quality of life of adult CHD with simple CHD in particular, since a reduced health-related quality of life is not only medically based.

Heart-focused anxiety (HFA) and peak oxygen uptake (V˙o2peak) were assessed in adults with congenital heart disease (CHD). This study shows that HFA is a common sequela in adults with CHD, and high HFA is associated with reduced V˙o2peak. Psychological screening and counseling for HFA therefore seem indicated in the aftercare of adults with CHD.

AbstractBackground:Lung function and cardiac function are naturally correlated by sharing the thoracic cage and handling the whole cardiac output sequentially. However, lung function studies are rare in patients with CHD, although results worthy of investigation could be expected. This review summarises existing studies with the lung function parameters (spirometry and body plethysmography) in CHD patients during the last decade.Methods:A systematic review was performed in the relevant database (PubMed, Cochrane, and Scopus) in studies including paediatric and adult patients with CHD where lung parameters (spirometry, body plethysmography) were investigated from January 2010 to December 2020. Two independent reviewers evaluated the studies according to the Study Quality Assessment Tool for Observational Cohort and Cross-Sectional Studies of the National Heart, Lung, and Blood InstituteResults:Eight studies investigated patients with Fontan palliation including 704 patients (306 female). Four studies included patients after repaired tetralogy of Fallot examining 219 patients (103 female), with one study using double. Further six studies included 3208 (1324 female) children and adults with various CHDs. Overall, four studies were categorised as “good”, ten as “fair”, and four as “poor”. While the measurements were consistently standardised, references to calculate %predicted differed substantially across all studies. All evaluated studies showed reduced forced vital capacity in the majority of CHD patients.Conclusions:Many CHD patients have a reduced forced vital capacity independent of their underlying defect. Spirometry should not only follow a standardised measure according to ATS (update 2019) but also stick to the 2012 GLI reference values

ObjectiveResilience is a complex, yet rather unexplored topic in patients with congenital heart disease (CHD). The goal of this study was to assess and compare resilience in children with CHD with healthy controls during the COVID-19 pandemic.Design and patientsFrom June 2020 to June 2021, 124 children with various CHDs (14.6±2.1 years, 49 girls) and 124 matched healthy controls (14.8±2.0 years, 49 girls) completed the Resilience Scale-11 short version.ResultsResilience was significantly reduced in children with CHD compared with healthy controls (CHD: 59.0±10.0 vs healthy controls: 64.4±6.5, p<0.001). That reduction was prominent in all CHD subgroups except those with left heart obstruction (aortic stenosis and coarctation of the aorta) and patients with transposition of the great arteries. Complex CHD had the lowest resilience of 57.6±8.4 (p<0.001) after adjusting for age and sex according to group differences. There was no difference between native CHD and CHD with open-heart surgery (native: 59.5±12.2 vs surgery: 58.8±9.3, p=0.758).ConclusionsResilience was reduced in children and adolescents with CHD compared with healthy peers during the COVID-19 pandemic. Children with complex severity appeared to be particularly affected. These findings emphasise continued efforts to provide a holistic and multidisciplinary approach in medical aftercare of these patients and their families.

Severe hypoxemia in the early postoperative period after bidirectional cavopulmonary shunt (BCPS) is a critical complication. We aimed to evaluate patients who underwent additional systemic to pulmonary shunt and septation of central pulmonary artery (partial takedown) after BCPS.

AbstractIntroduction:Congenital heart defects (CHDs) are the most common congenital malformations. Patients with CHD have a higher morbidity and mortality rate and are at greater risk for infectious diseases. The risk might even be higher if complex CHD occurs and if CHD is associated with additional co-morbidities. Therefore, immunisations in these children are essential.Materials and Methods:Individuals were recruited at the outpatient centre of the Department of Congenital Heart Defects and Pediatric Cardiology at the German Heart Center Munich in the time between February 2016 and February 2017. Included were children between 23 months and 17 years and a diagnosis of CHD. The vaccination certificate aimed to assess the immunization status.Results:In total, 657 children with CHD were included and analysed. Regarding primary immunisation, only 34 % (n = 221) of the children reached the complete vaccination status within the allowed catch-up time. Among these primary immunisation rates, vaccinations against Hepatitis B, Meningococci, Varicella and Pneumococci were found to have the lowest coverage with all being below 80%. The vaccination rate was partly influenced by the previously performed number of surgeries but not by the diagnosis of specific genetic diseases. At the age of school entry, the immunisation rate in children with CHD was also lower than in the comparable healthy population.Conclusion:The vaccination coverage rate in children with CHD is lower than in comparable healthy children, although this is a vulnerable patient group. Further education of parents and treating physicians of children with CHD regarding vaccination is still needed.

Staged palliation for hypoplastic left heart syndrome (HLHS) is associated with substantial mortality [1].

ObjectivesGrip strength is known to be reduced in adults with congenital heart disease (CHD). This study compared grip strength in paediatric patients with CHD with healthy controls and determined a possible association between grip strength and health-related physical fitness (HRPF).MethodsGrip strength and HRPF were assessed in 569 children (12.4 years, 95% CI 12.16 to 12.72; 238 girls) with various CHD and compared with 2551 healthy controls (11.4 years, 95% CI 11.3 to 11.5; 1424 girls). Grip strength was determined as the maximum value of three repetitions with each hand. HRPF was tested by five motor tasks (FITNESSGRAM) and converted into an SD score (z-score).ResultsAfter adjusting for age, sex and weight, children with CHD showed significantly lower grip strength compared with healthy controls (CHD: 20.8 kg, 95% CI 20.4 to 21.2; controls: 24.5 kg, 95% CI 24.3 to 24.8). CHD subgroup analysis also revealed significantly lower grip strength than the controls, with the lowest values in patients with total cavopulmonary connection (19.1, 95% CI 18.0 to 20.2). Children with complex CHD showed the lowest values with 19.8 kg (95% CI 19.2 to 20.4), those with moderate 20.7 kg (95% CI 19.9 to 21.4) and those with simple 22.5 kg (95% CI 21.6 to 23.3), respectively. HRPF was also lower (z-score: −0.46, 95% CI −0.49 to –0.35) compared with healthy controls and poorly associated with grip strength (r=0.21).ConclusionsGrip strength is already reduced in children with CHD and poorly associated with HRPF. This suggests that grip strength and HRPF are different domains and have to be assessed separately.

ObjectivePatients with tetralogy of Fallot (ToF) have limited pulmonary blood flow before corrective surgery and ongoing dysfunction of the pulmonary valve and right ventricle throughout life leading to lower exercise capacity and lung volumes in many patients. Inhalation training can increase lung volumes, improve pulmonary blood flow and consequently exercise capacity. This study tests whether home-based daily breathing training improves exercise capacity and lung volumes.MethodsFrom February 2017 to November 2018, 60 patients (14.7±4.8 years, 39% female) underwent spirometry (forced vital capacity (FVC); forced expiratory volume in 1 s (FEV1)), cardiopulmonary exercise testing (peak oxygen uptake (peak [Formula]O2)) and breathing excursion measurement. They were randomised into immediate breathing exercise or control group (CG) and re-examined after 6 months. The CG started their training afterwards and were re-examined after further 6 months. Patients trained with an inspiratory volume-oriented breathing device and were encouraged to exercise daily. The primary endpoint of this study was the change in peak [Formula]O2. Results are expressed as mean±SEM (multiple imputations).ResultsIn the first 6 months (intention to treat analysis), the training group showed a more favourable change in peak [Formula]O2(Δ0.5±0.6 vs −2.3±0.9 mL/min/kg, p=0.011), FVC (Δ0.18±0.03 vs 0.08±0.03 L, p=0.036) and FEV1(Δ0.14±0.03 vs −0.00±0.04 L, p=0.007). Including the delayed training data from the CG (n=54), this change in peak [Formula]O2correlated with self-reported weekly training days (r=0.282, p=0.039).ConclusionsDaily inspiratory volume-oriented breathing training increases dynamic lung volumes and slows down deconditioning in peak [Formula]O2in young patients with repaired ToF.

AbstractBackground/hypothesis:Disadvantages of intravenous therapeutic unfractionated heparin, the first-line anti-coagulant agent in children with complex congenital heart disease, include unpredictable pharmacokinetics requiring frequent phlebotomies and the need for continuous intravenous access.Objective:To compare efficacy and safety of low-molecular-weight heparin administered by a subcutaneous indwelling catheter with intravenous unfractionated heparin.Materials and methods:Clinical data from 31 inpatients prospectively enrolled to receive subcutaneous low-molecular-weight heparin were compared with those from a historical group of 44 inpatients receiving intravenous unfractionated heparin. Investigation of parents’ satisfaction by telephone survey.Results:The percentage of anti-factor Xa levels outside therapeutic range was lower in the subcutaneous low-molecular-weight heparin group compared with the percentage of activated partial thromboplastin times outside therapeutic range in the intravenous unfractionated heparin group (40% versus 90%, p < 0.001). Neither group had a major complication. Transient local reactions occurred in 19% of patients of the subcutaneous low-molecular-weight heparin group. The number of needle punctures and that of placement of indwelling catheters were significantly lower in the subcutaneous low-molecular-weight heparin compared with the intravenous unfractionated heparin group (p < 0.001). In total, 84.2% of parents in the subcutaneous low-molecular-weight heparin group reported a positive experience when asked about comparison with prior intravenous unfractionated heparin treatment.Conclusion:Subcutaneous low-molecular-weight heparin offers a safe anti-coagulation regimen for children with complex congenital heart disease providing more efficient therapeutic anti-coagulation and a reduction in needle punctures, thus causing less pain and anxiety in this children.

Supplemental Digital Content is Available in the Text.Heart rate variability measurement and a cardiopulmonary exercise test were performed in 222 patients with congenital heart disease (CHD) and 57 healthy controls. Patients with CHD showed reduced parasympathetic activity and exercise capacity compared with the controls. Impaired physical activity suggests limited function of the autonomic nervous system in patients with CHD.

Home-based exercise training is a promising alternative to conventional supervised training for patients with congenital heart disease (CHD). Even though the beneficial effect of exercise interventions is well established in patients with CHD, knowledge concerning variety and utility of existing programmes is still lacking. Therefore, the aim of this review is to give an overview about existing home-based exercise interventions in patients with CHD. A systematic search was performed in PubMed, Cochrane, Scopus and PEDro (2008–2018) for relevant clinical trials that provided any kind of home-based exercise with patients with CHD. All articles were identified and assessed by two independent reviewers. Seven articles with 346 paediatric CHD (18 months to 16 years) and five articles with 200 adults with CHD (21–41 years) were included. Most studies performed a supervised home-based exercise intervention with children and adolescents exercising at least three times per week with duration of 45 min for 12 weeks. Reported outcome measurements were health-related quality of life and physical activity, but mostly exercise capacity measured as peak oxygen uptake that improved in four studies (1.2%, 7%, 7.7%, 15%; p<0.05), walking distance in two (3.5%, 19.5%, p<0.05,) or walking time (2 min, p=0.003) in one. The dropout rates were high (15%), and compliance to the training programme was not reported in the majority of the studies (58%). Home-based exercise interventions are safe, feasible and a useful alternative to supervised cardiac rehabilitation for all age groups of patients with CHD. Nevertheless, training compliance represents a major challenge.

The clinical significance of left ventricular rehabilitation for borderline left ventricular hypoplasia is controversial. This study aimed to review the surgical results of patients with borderline left ventricular hypoplasia and to evaluate the impact of the left ventricular rehabilitation on outcomes.

Age‐related cardiovascular diseases are a relevant risk in the aging population of adults with congenital heart diseases (ACHD). Risk factors such as the metabolic syndrome (MetS) impact the risk of increased carotid intima‐media thickness (cIMT) and thereby long‐term cardiovascular diseases. The aim of the study was to assess MetS in ACHD and outline a possible association to cIMT. In total, 512 ACHD (43.0 ± 9.6 years, 48.9% female) were screened for MetS by the standards of the International Diabetes Federation, and their cIMT by ultrasound from January 2017 to June 2019. MetS was prevalent in 72 (14.1%) of the ACHD population (34 female, 15.5%). Regarding severity class, patients with simple forms of CHD had a MetS prevalence of 11.8%, moderate 16.7%, and severe 13.8%. ACHD with MetS had significantly increased cIMT compared to ACHD without MetS (ACHD with MetS: 0.587 ± 0.079 mm, ACHD without MetS: 0.560 ± 0.087 mm, mean difference: 0.028 mm, P = .013). Such a difference in vascular structure corresponds to roughly five years of normal vascular aging of the vessels. ACHD with MetS have a thicker cIMT compared to ACHD without MetS. Screening for MetS and targeting risk factors in ACHD might help to prevent structural alterations of the vessels at an early stage.

The Fontan circulation is highly dependent on ventilation, improving pulmonary blood flow and cardiac output. A reduced ventilatory function is reported in these patients. The extent of this impairment and its relation to exercise capacity and quality of life is unknown and objective of this study. This multicenter retrospective/cross‐sectional study included 232 patients (140 females, age 25.6 ± 10.8 years) after Fontan palliation (19.8% atrioventricular connection; 20.3% atriopulmonary connection; 59.9% total cavopulmonary connection). Resting spirometry, cardiopulmonary exercise tests, and quality‐of‐life assessment (SF‐36 questionnaire) were performed between 2003 and 2015. Overall, mean forced expiratory volume in one second (FEV1) was 74.7 ± 17.8%predicted (%pred). In 59.5% of the patients, FEV1was <80%pred., and all of these patients had FEV1/forced vital capacity (FVC) > 80%, suggestive of a restrictive ventilatory pattern. Reduced FEV1was associated with a reduced peakVO2of 67.0 ± 17.6%pred. (r= 0.43, P< .0001), even if analyzed together with possible confounding factors (sex, BMI, age, years after palliation, number of interventions, scoliosis, diaphragmatic paralysis). Synergistically to exercise capacity, FEV1was associated to quality of life in terms of physical component summary (r= 0.30, P= .002), physical functioning (r= 0.25, P= .008), bodily pain (r= 0.22, P= .02), and general health (r= 0.16, P= .024). Lower FEV1was associated with diaphragmatic paralysis (P= .001), scoliosis (P= .001), higher number of interventions (P= .002), and lower BMI (P= .01). No correlation was found to ventricular morphology, type of surgeries, or other perioperative/long‐term complications. This study shows that the common restrictive ventilatory pattern in Fontan patients is associated with lower exercise capacity and quality of life. Risk factors are diaphragmatic paralysis, scoliosis, a high total number of interventions and low BMI.

Background The respiratory benefits of muscle strength are well-known in heart-healthy populations, but recommendations and research often focus instead on aerobic fitness (peak oxygen uptake) or total activity. Independent benefits of strength thus may be underestimated, especially in congenital heart disease where perceived dangers of certain types of exercise may outweigh perceived benefits. To assess whether it is plausible that pulmonary benefits of strength in heart-healthy populations also apply in congenital heart disease, we simultaneously correlated these patients’ lung function with fitness, strength, and cardiac diagnosis.Methods Lung function (forced expiratory volume in one second percentage predicted (FEV1%pred)) was modeled as function of handgrip strength, congenital heart disease diagnosis, peak oxygen uptake and the interactions of handgrip with sex and diagnosis in 538 Germans (58% male, ages 6–82 years) in linear models corrected for age, sex, height and weight. Congenital heart disease diagnoses were: complex cyanotic; Fallot/Truncus arteriosus communis (common arterial trunk) (TAC); shunts; transposition of the great arteries (TGA); left heart; and other/none.Results Each kg of handgrip was associated with 0.74% higher FEV1%pred (p< 0.001) and handgrip explained almost 10% of variance in FEV1%pred. While some groups had higher FEV1%pred than others (pfor global null <0.0001), all experienced similar associations with strength (pfor interaction with handgrip >0.10 for both sex and diagnosis.) Correction for peak oxygen uptake eliminated the association with congenital heart disease, but not handgrip.Conclusion Strength was associated with better lung function in all ages even after correction for peak oxygen uptake, regardless of sex and congenital heart disease. This suggests that strength may be at least as important for lung function as aerobic fitness. Heart-safe strength training may improve pulmonary function in congenital heart disease.

ObjectiveFunctional outcome measures are of growing importance in the aftercare of patients with congenital heart disease. This study addresses the functional status with regard to exercise capacity, health-related physical fitness (HRPF) and arterial stiffness in a recent cohort of children, adolescents and young adults with tetralogy of Fallot (ToF) after repair.DesignSingle-centre, uncontrolled and prospective cohort study.SettingOutpatient department of the German Heart Centre Munich; July 2014–January 2018.PatientsOne hundred and six patients with ToF after repair (13.5±3.7 years, 40 females) were included. Data were compared with a recent cohort of healthy controls (HCs) (n=1700, 12.8±2.6 years, 833 females).Main outcome measuresPatients underwent a symptom-limited cardiopulmonary exercise test, performed an HRPF test (FitnessGram) and had an assessment of their arterial stiffness (Mobil-O-Graph).ResultsCompared with HC, patients with ToF showed lower predicted [Formula]O2peak (ToF: 80.4% ± 16.8% vs HC: 102.6% ± 18.1%, p<0.001), impaired ventilatory efficiency (ToF: 29.6 ± 3.6 vs HC: 27.4 ± 2.9, p<0.001), chronotropic incompetence (ToF: 167 ± 17 bpm vs HC: 190 ± 17 bpm, p<0.001) and reduced HRPF (ToF z-score: −0.65 ± 0.87 vs HC z-score: 0.03 ± 0.65, p<0.001). Surrogates of arterial stiffness, central and peripheral systolic blood pressure, did not differ between the two groups.ConclusionsContemporary children, adolescents and young adults with ToF still have functional limitations. How impaired HRPF and limited exercise capacity interact and how they can be modified needs to be evaluated in further intervention studies.

ObjectivesImproved treatments for patients with congenital heart disease (CHD) have led to a growing interest in long-term functional outcomes such as health-related quality of life (HRQoL). Studies on HRQoL in children with CHD have contradicting results. Therefore, we compared HRQoL of children with CHD with that of current healthy peers and stratify CHD cases by severity and diagnostic subgroups.MethodsWe included 514 patients (191 girls) aged 7–17 (12.9±3.1) years who were recruited at our institution between July 2014 and May 2017. The self-reported and age-adapted KINDL questionnaire was used to assess HRQoL. Patient data were compared with that of a recent control group of 734 healthy children (346 girls, 13.4±2.1 years).ResultsPatients with CHD scored at least as high as healthy peers in HRQoL (CHD: 78.6±9.8; healthy: 75.6±10.1; P<0.001). After correction for sex and age, patients with CHD presented a 2.3-point higher HRQoL (P<0.001). The sex-specific and age-specific analyses showed that there were no differences between boys with and without CHD in childhood (P=0.255), but in adolescence, boys with CHD had on average 3.9-point higher scores (P=0.001), whereas girls with CHD had statistically higher HRQoL perception than healthy girls in childhood (4.2 points; P=0.003) and adolescence (4.2 points; P=0.005). There were no differences between the severity classes or diagnostic subgroups in the total HRQoL score or in the six subdomains.ConclusionThe high HRQoL in young patients with CHD suggests that they can cope well with their disease burden. This holds true for all severity classes and diagnostic subgroups.

ObjectiveDespite decreasing mortality, functional impairments in children with transposition of the great arteries (TGA) are still a concern. This study analyses health-related physical fitness (HRPF), arterial stiffness and health-related quality of life (HRQoL) in children with anatomically repaired TGA regarding congenital ventricular septal defect (VSD) and coronary patterns.Patients and methods68 children with anatomically repaired TGA with or without VSD (12.9±3.7 years, 19.1% female) were investigated between August 2014 and October 2017. HRPF was assessed by five tests of the FITNESSGRAM, arterial stiffness was measured by oscillometric measurement using Mobil-O-Graph and HRQoL was analysed with a self-report questionnaire (KINDL-R). All test results were compared with a healthy reference cohort (n=2116, 49.1% female) adjusted for sex and age.ResultsChildren with anatomically repaired TGA had significantly worse HRPF (z-score: −0.58±0.81, p<0.001), increased pulse wave velocity (TGA: 4.9±0.3 m/s vs healthy: 4.8±0.3 m/s, p=0.028) and central systolic blood pressure (TGA: 105.9±5.8 mm Hg vs healthy: 103.3±5.7 mm Hg, p=0.001). No difference was found for HRQoL between the two groups (total HRQoL score: TGA: 76.5±10.2 vs healthy: 75.2±10.1, p=0.315). Neither the coronary pattern nor a congenital VSD resulted in significant differences in all functional outcomes.ConclusionChildren with anatomically repaired TGA exhibit impaired HRPF and increased arterial stiffness whereas their HRQoL is normal. The underlying coronary pattern seems to have no influence on the functional outcome, nor does an accompanied congenital VSD.

With increased long-term survival, children with congenital heart disease (CHD) are at increased risk of early-onset adult cardiovascular disease. Carotid intima–media thickness (cIMT) is a surrogate marker of atherosclerosis. The aim of this present study was to detect high-risk diagnostic subgroups by measuring cIMT and determine its correlates in children with CHD and subgroups of CHD compared with healthy controls.

AbstractObjectivesThis study aimed to develop a method for retrieval of the new meshed nitinol atrial septal defect occluders – Ceraflex and Occlutech.BackgroundThe newly developed atrial septal defect occluders have potential benefits considering implantation, but concerns regarding their removal in case of embolisation have been raised.MethodsOver 21 years, 1449 patients underwent interventional atrial septal defect occlusion in our institution. We reviewed the cases of embolisation of the device, developed a strategy for device removal, and tested it on the benchside and in animal tests.ResultsIn 11 patients (0.8%), the intended atrial septal defect occlusion was complicated by an embolisation of the device. In contrast to the Amplatzer septal occluders, retrieval of Occlutech devices larger than 16 mm with snare techniques was impossible. In benchside tests, this was confirmed and a new method for removal of large meshed devices was developed. This involved the commercially available Maslanka® biopsy forceps. The feasibility of this technique in vivo was tested in a pig model. During animal tests, using the Maslanka biopsy forceps it was possible to interventionally retrieve embolised Ceraflex and Occlutech devices of different sizes – 10, 16, 30, and 40 mm – into a 12-F sheath.ConclusionIt was impossible to retrieve Occlutech and Ceraflex devices larger than 16 mm into a large sheath in vivo and during benchside tests. However, this was feasible on the bench and in vivo using the Maslanka biopsy forceps even with the largest available devices.

Objective Central systolic blood pressure (SBP) is a measure of arterial stiffness and strongly associated with atherosclerosis and end-organ damage. It is a stronger predictor of cardiovascular events and all-cause mortality than peripheral SBP. In particular, for children with congenital heart disease, a higher central SBP might impose a greater threat of cardiac damage. The aim of the study was to analyse and compare central SBP in children with congenital heart disease and in healthy counterparts.Patients and methods Central SBP was measured using an oscillometric method in 417 children (38.9% girls, 13.0 ± 3.2 years) with various congenital heart diseases between July 2014 and February 2017. The test results were compared with a recent healthy reference cohort of 1466 children (49.5% girls, 12.9 ± 2.5 years).Results After correction for several covariates in a general linear model, central SBP of children with congenital heart disease was significantly increased (congenital heart disease: 102.1 ± 10.2 vs. healthy reference cohort: 100.4 ± 8.6, p< .001). The analysis of congenital heart disease subgroups revealed higher central SBP in children with left heart obstructions (mean difference: 3.6 mmHg, p< .001), transpositions of the great arteries after arterial switch (mean difference: 2.2 mmHg, p= .017) and univentricular hearts after total cavopulmonary connection (mean difference: 2.1 mmHg, p= .015) compared with the reference.Conclusion Children with congenital heart disease have significantly higher central SBP compared with healthy peers, predisposing them to premature heart failure. Screening and long-term observations of central SBP in children with congenital heart disease seems warranted in order to evaluate the need for treatment.

AbstractWe report a case of successful, life-saving implantation of a covered Cheatham Platinum stent, an all-in-one NuDEL catheter system, in an adult with aortic rupture after bare-metal stenting for re-coarctation of the aorta.

AbstractObjectivesWe aimed to investigate whether early postoperative extubation following the Fontan operation is universally feasible and can be used as a management tool in unstable patients.MethodsAll patients undergoing the Fontan operation in our centre between 2004 and 2013 (n=253) were analysed. Until 2008, patients were extubated according to standard criteria and comprised group 1. Group 2 included all patients presenting after 2009, when early extubation was always aimed regardless of the haemodynamic status. Patients who exceeded the 75th percentiles for volume requirements and inotrope scores for the respective group were defined as unstable. Comparisons of outcomes between groups and subgroups and analysis of the changes in haemodynamic and treatment parameters with extubation in unstable patients after 2009 were performed.ResultsCompared with group 1, patients from group 2 were ventilated for shorter duration (p<0.001), had similar re-intubation rates (p=0.50), and needed less volume (p=0.01). In group 2, the unstable patients were not ventilated for longer durations (p=0.19), but had higher re-intubation rates (p=0.03) than the stable patients. Compared with the unstable patients from group 1, the unstable patients from group 2 were ventilated for shorter duration (p<0.001), had similar re-intubation rates (p=0.66), and needed less volume (p=0.006). There was a significant acute and sustained increase in mean arterial pressure with extubation and a parallel reduction in volume requirements and inotrope scores in the unstable patients from group 2.ConclusionsTimely extubation is universally applicable following the Fontan operation. Early postoperative extubation can be valuable for improving Fontan haemodynamics.

ObjectiveImproved survival has yielded to growing importance of functional outcome measures in patients with congenital heart disease (CHD). This study applied the International Physical Activity Questionnaire (IPAQ) to assess self-reported physical activity (PA) in patients with CHD and their association with exercise capacity and health-related quality of life (HrQoL).Patients and methodsPrior to cardiopulmonary exercise testing (CPET), 786 consecutive patients (335 female, 31.1±11.6 years) with CHD filled in the short form of the IPAQ and the HrQoL questionnaire 36-Item Short Form.ResultsIn total, 393 (50.0%) patients reported health-enhancing physical activity (HEPA), 237 (30.2%) minimal activity and 156 (19.8%) inactivity. In comparison with the HEPA group, the inactive group had significant lower peak oxygen uptake (74.2%±20.7% vs 86.8%±22.3%; p<0.001) as well as lower physical (91.0%±16.9% vs 97.4%±13.6%; p<0.001) and mental (97.1%±22.2% vs 104.1%±15.6%; p<0.001) HrQoL. Independent of severity class, surgery, age, beta-blocker, pacemaker and oxygen saturation, the HEPA group in comparison with the inactive group showed significantly less probability for impairments in mental (OR: 0.21, 95% CI: 0.10 to 0.45; p<0.001) and physical (OR: 0.46, 95% CI: 0.23 to 0.92; p=0.027) HrQoL and exercise capacity (OR: 0.36, 95% CI: 0.22 to 0.59; p<0.001).ConclusionsCategorisation of patientswith CHD with the IPAQ quickly provides clinical information as HEPA patients have a less probability for impaired HrQoL and diminished exercise capacity. Nevertheless, the IPAQ cannot substitute an accelerometer-based assessment for PA, nor a CPET for exercise capacity.

AbstractWe report on a 12-year-old girl with Ebstein’s anomaly after a unidirectional Glenn procedure with surgical ligation of the proximal right pulmonary artery, who suffered from significant central cyanosis caused by multiple arterio-venous fistulas in the right lung. The continuity between the right pulmonary artery and the pulmonary trunk was restored with the use of radiofrequency perforation and consecutive covered stent implantation.