It is often assumed, that adult patients with CHD (ACHD) have impairments regarding their cognitive function (CF) and health-related quality of life. In particular, it seems reasonable to assume that cyanosis may have a potential impact on CF as well as surgical or drug treatment into adulthood. This study assesses neuromental health aspects such as CF and health-related quality of life in ACHD patients.

Seventy-eight ACHD patients (female n = 39 (50%); 34.1 ± 12.9 years; cyanotic CHD n = 49 (62.8%) with a cyanosis duration of 159.8 ± 196.2 month) who underwent open heart surgery as first intervention were asked to participate during routinely follow-up in 2018. Wechsler Intelligence Scale IV was used for CF and the Short Form 36 Health Survey to assess health-related quality of life.

Intelligence quotient measures showed significant differences comparing never cyanotic and with a cyanotic phase in verbal comprehension (p = 0.013). There was no association of CF with cyanosis duration, number of surgery or catheter, CHD severity, and time of first surgery. The group of early surgery showed significantly better results in physical function (p = 0.040) of health-related quality of life, and in comparison with their assigned reference, both groups showed significantly reduced results in all domains except in bodily pain and mental health. Full-Scale intelligence quotient correlates with physical function of health-related quality of life.

The results show normal CF in ACHD. Health-related quality of life was weak in comparison with the reference. There is a need to improve the well-being of our ACHD with structured programmes, including physical activity programmes. This growing ACHD population should be focused in order of their needs, medical ones on one hand and on the other hand psychosocial matters.

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.

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 Institute

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.

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

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.

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.

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.

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.

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.

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.

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.

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.

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.

To compare efficacy and safety of low-molecular-weight heparin administered by a subcutaneous indwelling catheter with intravenous unfractionated heparin.

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.

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.

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.

Catheterization in small children should be performed with the lowest diameter introducer sheaths to prevent permanent vessel damage. The objective of this study is to evaluate the clinical safety and efficacy of the Glidesheath Slender in small children.

We present a group of 52 patients (male: n = 36) with median age 118.5 days (min. 3; max. 1302), median weight: 5.3 kg (min. 1.4; max. 14.0), median height: 60.5 cm (min. 39; max. 102), and median body surface area 0.28 m2 (min. 0.12; max. 0.63) in whom percutaneous catheter interventions (n = 55) were performed via a Glidesheath Slender. In 49 children, the intervention was performed from femoral access (artery n = 35; vein n = 14) in 2 from the axillary arterial access and in 1 from the jugular venous access. In all patients, the vessel access was obtained under ultrasound guidance. After the catheterization, the pulse on the peripheral arteries (posterior tibial artery or radial artery) was palpable in all patients, and no signs for vessel dysfunction were present.

The Glidesheath Slender effectively reduces the outer sheath diameter for various types of interventions in small children by one French, reducing the risk of vessel complications (stenosis, occlusion). Interventions via Glidesheath Slender in small patients are safe and feasible and extend the transcatheter possibilities in small children with congenital heart diseases.

This study aimed to develop a method for retrieval of the new meshed nitinol atrial septal defect occluders – Ceraflex and Occlutech.

The newly developed atrial septal defect occluders have potential benefits considering implantation, but concerns regarding their removal in case of embolisation have been raised.

Over 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.

In 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.

It 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.

Acute kidney injury is a frequent complication after cardiac surgery with cardiopulmonary bypass in infants. Neutrophil gelatinase-associated lipocalin has been suggested to be a promising early biomarker of impending acute kidney injury. On the other hand, neutrophil gelatinase-associated lipocalin has been shown to be elevated in systemic inflammatory diseases without renal impairment. In this secondary analysis of data from our previous study on acute kidney injury after infant cardiac surgery, our hypothesis was that neutrophil gelatinase-associated lipocalin may be associated with surgery-related inflammation.

We prospectively enrolled 59 neonates and infants undergoing cardiopulmonary bypass surgery for CHD and measured neutrophil gelatinase-associated lipocalin in plasma and urine and interleukin-6 in the plasma. Values were correlated with postoperative acute kidney injury according to the paediatric Renal-Injury-Failure-Loss-Endstage classification.

Overall, 48% (28/59) of patients developed acute kidney injury. Of these, 50% (14/28) were classified as injury and 11% (3/28) received renal replacement therapy. Both plasma and urinary neutrophil gelatinase-associated lipocalin values were not correlated with acute kidney injury occurrence. Plasma neutrophil gelatinase-associated lipocalin showed a strong correlation with interleukin-6. Urinary neutrophil gelatinase-associated lipocalin values correlated with cardiopulmonary bypass time.

Our results suggest that plasma and urinary neutrophil gelatinase-associated lipocalin values are not reliable indicators of impending acute kidney injury in neonates and infants after cardiac surgery with cardiopulmonary bypass. Inflammation may have a major impact on plasma neutrophil gelatinase-associated lipocalin values in infant cardiac surgery. Urinary neutrophil gelatinase-associated lipocalin may add little prognostic value over cardiopulmonary bypass time.

We 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.

All 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.

Compared 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.

Timely extubation is universally applicable following the Fontan operation. Early postoperative extubation can be valuable for improving Fontan haemodynamics.

In this study we sought to determine, first, whether maximal exercise capacity reflects ventricular function, and second, whether the age of the patient, and the age of completion of the Fontan circulation, influence ventricular function and exercise performance.

Cardiac magnetic resonance imaging and cardiopulmonary exercise testing were performed in 29 patients at a median time of 6.9 years after completion of the Fontan circulation. We divided the patients into 2 groups, the first 19 having their operation below the age of 18 years, and the second group, of 10 patients, having completion of the Fontan circulation when they were older than 18 years. Parameters for ventricular function and exercise were compared for both groups with controls.

Compared to controls, the younger patients had normal end-diastolic ventricular volumes, but significantly impaired ventricular function, lower maximal work load and consumption of oxygen. The older patients had greater end-diastolic ventricular volumes, and significantly poorer ventricular function than both the younger patients and the controls. Maximal work load and consumption of oxygen were significantly lower in the older patients than in the younger ones and the controls.

Patients with the Fontan circulation have an impaired systolic ventricular function, which correlates with maximal exercise capacity and uptake of oxygen. Those having completion of the Fontan circulation when younger than 18 years had significantly better ventricular function and exercise performance than those who had completion of the Fontan circulation at an older age. An early creation of the Fontan circulation may preserve cardiac function and exercise capacity.

To assess results of closure of atrial septal defects within the oval fossa by devices delivered by catheterisation in symptomatic infants and children under 2 years of age.

The Amplatzer septal occluder was used. Results, and complications of transcatheter device treatment in patients aged below 2 years were compared to previous results from our institution.

We attempted closure in 12 consecutive patients below the age of 2 years who presented with an atrial septal defect between May 1997 and 1999. Symptoms were failure to thrive in 6, frequent chest infections in 5, and the need for treatment of heart failure in the other. All were thought to have a defect suitable for interventional closure. The atrial defects were seen in isolation in 10 children, but 2 had associated pulmonary stenosis which had been treated by balloon dilation prior to placement of the Amplatzer occluder.

The Amplatzer septal occluder was implanted at a mean age of 1.4 ± 0.4, with a range from 0.8 to 1.8 years. Ratios of pulmonaryto-systemic flow had been 2.1 ± 0.5, with a range from 1.6 and 3.2, and the defect was measured at 12 ± 4 mms. Fluoroscopy time was 12.8 ± 10.2 minutes, with a range from 5 to 43 minutes, and the time of the overall procedure was 162 ± 70 minutes, with a range from 85 to 360 minutes. It proved necessary to remove the device in 2 patients (16%) because of a residual shunt and movement after release. One of these developed transient neurological complications. Both subsequently underwent surgical treatment.

Symptomatic patients less than 2 years of age can undergo successful closure of an atrial septal defect using the Amplatzer device, but the rates of success are less, and procedure time longer, than in older children or adults.