COVID-19 infection in patients with history of pediatric heart transplant in Germany, Austria, and Switzerland.

COVID-19 is a heterogenous infection-asymptomatic to fatal. While the course of pediatric COVID-19 infections is usually mild or even asymptomatic, individuals after adult heart transplantation are at high risk of a severe infection. We conducted a retrospective, multicenter survey of 16 pediatric heart transplant centers in Germany, Austria and Switzerland to evaluate the risk of a severe COVID-19 infection after pediatric heart transplantation between 02/2020 and 06/2021. Twenty-six subjects (11 male) with a median age of 9.77 years at time of transplantation and a median of 4.65 years after transplantation suffered from COVID-19 infection. The median age at time of COVID-10 infection was 17.20 years. Fourteen subjects had an asymptomatic COVID-19 infection. The most frequent symptoms were myalgia/fatigue (n = 6), cough (n = 5), rhinitis (n = 5), and loss of taste (n = 5). Only one subject showed dyspnea. Eleven individuals needed therapy in an outpatient setting, four subjects were hospitalized. One person needed oxygen supply, none of the subjects needed non-invasive or invasive mechanical ventilation. No specific signs for graft dysfunction were found by non-invasive testing. In pediatric heart transplant subjects, COVID-19 infection was mostly asymptomatic or mild. There were no SARS-CoV-2 associated myocardial dysfunction in heart transplant individuals.

"Take it or leave it": Analysis of pediatric heart offers for transplantation in Switzerland.

BACKGROUND: There is a shortage of donor hearts in Switzerland, especially for pediatric recipients. However, the rate and reason for refusals of pediatric donor hearts offered in Switzerland has not been systematically analyzed. METHODS: The national transplant database, Swiss Organ Allocation System, was searched for all hearts from Swiss and foreign donors younger than 16 years from 2015 to 2020. The numbers of accepted and refused hearts and early outcome were assessed, and the reasons for refusal were retrospectively analyzed. RESULTS: A total of 136 organs were offered to the three Swiss pediatric heart centers and foreign donor procurement organizations. Of these, 26/136 (19%) organs were accepted and transplanted: 18 hearts were transplanted in Switzerland, and 13 of these were foreign. Reasons for refusal were (1) no compatible recipient due to blood group or weight mismatch, 89.4%; (2) medical, meaning organ too marginal for transplantation, 7.4%; (3) logistic, 1.4%; and (4) other, 1.8%. Five organs were refused in Switzerland by one center but later accepted and successfully transplanted by another center. Hearts from outside Switzerland were transplanted significantly less than Swiss hearts (n = 16/120 vs. 10/16, p < .001). CONCLUSION: The most common reason for refusing a pediatric donor heart is lack of compatibility with the recipient. Few hearts are refused for medical reasons. A more generous acceptance seems to be justified in selected patients. Switzerland receives a high number of foreign offers, but their rate of acceptance is lower than that of Swiss donations.

Association of Lymphatic Abnormalities with Early Complications after Fontan Operation.

BACKGROUND: Increased central venous pressure is inherent in Fontan circulation but not strongly related to Fontan complication. Abnormalities of the lymphatic circulation may play a crucial role in early Fontan complications. METHODS: This was a retrospective, single-center study of patients undergoing Fontan operation from 2008 to 2015. The primary outcome was significant early Fontan complication defined as secondary in-hospital treatment due to peripheral edema, ascites, pleural effusions, protein-losing enteropathy, or plastic bronchitis. All patients received T2-weighted magnetic resonance images to assess abdominal and thoracic lymphatic perfusion pattern 6 months after Fontan completion with respect to localization, distribution, and extension of lymphatic perfusion pattern (type 1-4) and with application of an area score (0-12 points). RESULTS: Nine out of 42 patients developed early Fontan complication. Patients with complication had longer chest tube drainage (mean 28 [interquartile range [IQR]: 13-60] vs. 13 [IQR: 2-22] days, p = 0.01) and more often obstructions in the Fontan circuit 6 months after surgery (56 vs. 15%, p = 0.02). Twelve patients showed little or no abnormalities of lymphatic perfusion (lymphatic perfusion pattern type 1). Most frequently magnetic resonance imaging showed lymphatic congestion in the supraclavicular region (24/42 patients). Paramesenteric lymphatic congestion was observed in eight patients. Patients with early Fontan complications presented with higher lymphatic area score (6 [min-max: 2-10] vs. 2 [min-max: 0-8]), p = 0.001) and greater distribution and extension of thoracic lymphatic congestion (type 3-4: n = 5/9 vs. n = 1/33, p = 0.001). CONCLUSION: Early Fontan complication is related to hemodynamic factors such as circuit obstruction and to the occurrence and extent of lymphatic congestion.

A new approach of three-dimensional guidance in paediatric cath lab: segmented and tessellated heart models for cardiovascular interventions in CHD.

BACKGROUND: Optimal imaging is essential for catheter-based interventions in CHD. The three-dimensional models in volume-rendering technique currently in use are not standardised. This paper investigates the feasibility and impact of novel three-dimensional guidance with segmented and tessellated three-dimensional heart models in catheterisation of CHD. In addition, a nearly radiation-free two- to three-dimensional registration and a biplane overlay were used.Methods and resultsWe analysed 60 consecutive cases in which segmented tessellated three-dimensional heart models were merged with live fluoroscopy images and aligned using the tracheal bifurcation as a fiducial mark. The models were generated from previous MRI or CT by dedicated medical software. We chose the stereo-lithography format, as this promises advantage over volume-rendering-technique models regarding visualisation. Prospects, potential benefits, and accuracy of the two- to three-dimensional registration were rated separately by two paediatric interventionalists on a five-point Likert scale. Fluoroscopy time, radiation dose, and contrast dye consumption were evaluated. Over a 10-month study period, two- to three-dimensional image fusion was applied to 60 out of 354 cases. Of the 60 catheterisations, 73.3% were performed in the context of interventions. The accuracy of two- to three-dimensional registration was sufficient in all cases. Three-dimensional guidance was rated superior to conventional biplane imaging in all 60 cases. We registered significantly smaller amounts of used contrast dye (p<0.01), lower levels of radiation dose (p<0.02), and less fluoroscopy time (p<0.01) during interventions concerning the aortic arch compared with a control group. CONCLUSIONS: Two- to three-dimensional image fusion can be applied successfully in most catheter-based interventions of CHD. Meshes in stereo-lithography format are accurate and base for standardised and reproducible three-dimensional models.

Accuracy and Specific Value of Cardiovascular 3D-Models in Pediatric CT-Angiography.

Computed tomography (CT)-angiography is routinely performed prior to catheter-based and surgical treatment in congenital heart disease. To date, little is known about the accuracy and advantage of different 3D-reconstructions in CT-data. Exact anatomical information is crucial. We analyzed 35 consecutive CT-angiographies of infants with congenital heart disease. All datasets are reconstructed three-dimensionally using volume rendering technique (VRT) and threshold-based segmentation (stereolithographic model, STL). Additionally, the two-dimensional maximum intensity projection (MIP) reconstructs two-dimensional data. In each dataset and resulting image, measurements of vascular diameters for four different vessels were estimated and compared to the reference standard, measured via multiplanar reformation (MPR). The resulting measurements obtained via the STL-images, MIP-images, and the VRT-images were compared with the reference standard. There was a significant difference (p < 0.05) between measurements. The mean difference was 0.0 for STL-images, -0.1 for MIP-images, and -0.3 for VRT-images. The range of the differences was -0.7 to 1.0 mm for STL-images, -0.6 to 0.5 mm for MIP-images and -1.1 to 0.7 mm for VRT-images. There was an excellent correlation between the STL-, MIP-, VRT-measurements, and the reference standard. Inter-reader reliability was excellent (p < 0.01). STL-models of cardiovascular structures are more accurate than the traditional VRT-models. Additionally, they can be standardized and are reproducible.

Third-generation dual-source 70-kVp chest CT angiography with advanced iterative reconstruction in young children: image quality and radiation dose reduction.

BACKGROUND: Many technical updates have been made in multi-detector CT. OBJECTIVE: To evaluate image quality and radiation dose of high-pitch second- and third-generation dual-source chest CT angiography and to assess the effects of different levels of advanced modeled iterative reconstruction (ADMIRE) in newborns and children. MATERIALS AND METHODS: Chest CT angiography (70 kVp) was performed in 42 children (age 158 ± 267 days, range 1-1,194 days). We evaluated subjective and objective image quality, and radiation dose with filtered back projection (FBP) and different strength levels of ADMIRE. For comparison were 42 matched controls examined with a second-generation 128-slice dual-source CT-scanner (80 kVp). RESULTS: ADMIRE demonstrated improved objective and subjective image quality (P < .01). Mean signal/noise, contrast/noise and subjective image quality were 11.9, 10.0 and 1.9, respectively, for the 80 kVp mode and 11.2, 10.0 and 1.9 for the 70 kVp mode. With ADMIRE, the corresponding values for the 70 kVp mode were 13.7, 12.1 and 1.4 at strength level 2 and 17.6, 15.6 and 1.2 at strength level 4. Mean CTDIvol, DLP and effective dose were significantly lower with the 70-kVp mode (0.31 mGy, 5.33 mGy*cm, 0.36 mSv) compared to the 80-kVp mode (0.46 mGy, 9.17 mGy*cm, 0.62 mSv; P < .01). CONCLUSION: The third-generation dual-source CT at 70 kVp provided good objective and subjective image quality at lower radiation exposure. ADMIRE improved objective and subjective image quality.

Three-Dimensional Rotational Angiography in the Pediatric Cath Lab: Optimizing Aortic Interventions.

The aim of this study was to evaluate usability and accuracy of three-dimensional rotational angiography (3DRA) during interventions of the aorta in congenital heart disease (CHD). 3DRA is an accurate, encompassing and fast imaging technique in the cath lab. However, there is only few published data about its use during interventions in CHD. Between January 2010 and January 2014, 3DRA was performed in 77 patients with aortic issues: in 65 % cases, an intervention was performed, of which 72 % had aortic isthmus stenosis. Data were obtained retrospectively. The accuracy of 3DRA was evaluated on the basis of comparative measurements (n = 60) of the aortic diameter between 3DRA and conventional biplane angiography. Measurements presented a high accuracy with an average deviation of 3.89 % [±3 %] and a significant correlation of r = 0.99 after Pearson (p < 0.0001). Clinical benefit was assessed using a five-point Likert scale and could be shown in 98 %. Comparison with a control group showed a reduced fluoroscopy time from 10.2 to 8.30 min (median, p < 0.01) and decreased radiation dose of 0.18 compared to 0.56 Gy cm(2)/kg (median, p < 0.02). The use of 3DRA in patients with aortic anomalies has advantages in comparison with conventional angiography. It improves diagnostic accuracy, and 3D guidance enables a faster and simplified intervention with enhanced patients' safety and the potential to reduce radiation dose.

Aortic Arch Reconstruction in Neonates with Biventricular Morphology: Increased Risk for Development of Recoarctation by Use of Autologous Pericardium.

BACKGROUND: The aim of this study was to analyze risk factors promoting development of recoarctation (Re-CoA) in neonates who survived aortic arch repair from an anterior approach. METHODS: Fifty consecutive neonates with biventricular morphology and ductal-dependent lower body perfusion who were discharged home following aortic arch repair with cardiopulmonary bypass between 2000 and 2012 were retrospectively reviewed. Arch anatomy was either interruption (n = 10) or hypoplasia with coarctation (n = 40). Aortic arch reconstruction was performed by using patch material (bovine pericardium, n = 30, homograft, n = 10, or glutaraldehyde-treated autologous pericardium, n = 7), and three patients underwent direct end-to-side anastomosis. Antegrade cerebral and continuous myocardial perfusion was performed in 39 and 21 patients, respectively. Kaplan-Meier freedom from Re-CoA was calculated. Morphologic and perioperative data indicating increased risk of Re-CoA by univariate analysis were included in multivariate Cox regression analysis. RESULTS: Mean follow-up was 5.3 ± 4.1 years. Re-CoA occurred in 13 patients and was treated successfully by balloon dilatation (n = 6) or surgery (n = 7). Freedom from Re-CoA after 1 and 5 years was 83 ± 5 and 79 ± 6%, respectively. Two patients died early after surgical repair of Re-CoA. The use of autologous pericardium for aortic arch augmentation was the only independent risk factor for development of Re-CoA (hazard ratio: 4.3 [95% confidence interval: 1.2-16.1]; p = 0.028). CONCLUSION: Re-CoA following neonatal aortic arch surgery can be treated by balloon dilatation or surgery, if adequate. In this study, the risk for development of Re-CoA was independently increased by the use of autologous pericardium during initial arch repair.

Pediatric cardiac MRI: automated left-ventricular volumes and function analysis and effects of manual adjustments.

BACKGROUND: Cardiac MRI is an accurate and reproducible technique for the assessment of left ventricular volumes and function. The accuracy of automated segmentation and the effects of manual adjustments have not been determined in children. OBJECTIVE: To evaluate automated segmentation and the effects of manual adjustments for left ventricular parameter quantification in pediatric cardiac MR images. MATERIALS AND METHODS: Left ventricular parameters were evaluated in 45 children with suspected myocarditis (age 13.4 ± 3.5 years, range 4-17 years) who underwent cardiac MRI. Dedicated software was used to automatically segment and adjust the parameters. Results of end-diastolic volume, end-systolic volume, stroke volume, myocardial mass, and ejection fraction were documented before and after apex/base adjustment and after apex/base/myocardial contour adjustment. RESULTS: The software successfully detected the left ventricle in 42 of 45 (93.3%) children; failures occurred in the smallest and youngest children. Of those 42 children, automatically segmented end-diastolic volume (EDV) was 151 ± 47 ml, and after apex/base adjustment it was 146 ± 45 ml, after apex/base/myocardial contour adjustment 146 ± 45 ml. The corresponding results for end-systolic volume (ESV) were 66 ± 32 ml, 63 ± 29 ml and 64 ± 28 ml; for stroke volume (SV) they were 85 ± 25 ml, 83 ± 23 ml and 83 ± 23 ml; for ejection fracture (EF) they were 57 ± 10%, 58 ± 9% and 58 ± 9%, and for myocardial mass (MM) they were 104 ± 31 g, 95 ± 31 g and 94 ± 30 g. Statistically significant differences were found when comparing the EDV/ESV/MM results, the EF results after apex/base adjustment and after apex/base/myocardial contour adjustment and the SV results (except for comparing the SVs after apex/base adjustment and after apex/base/myocardial contour adjustment). CONCLUSION: Automated segmentation for the evaluation of left ventricular parameters in pediatric MR images proved to be feasible. Automated segmentation + apex/base adjustment provided clinically acceptable parameters for the majority of cases.

Impact of postoperative necrotizing enterocolitis after neonatal cardiac surgery on neurodevelopmental outcome at 1 year of age.

Objectives: We analyzed the impact of postoperative necrotizing enterocolitis (NEC) after cardiac surgery in neonatal age on neurodevelopmental (ND) outcome at 1 year of age. Methods: Using data from the Swiss Neurodevelopmental Outcome Registry for Children with Congenital Heart Disease (ORCHID), we analyzed perioperative variables including postoperative NEC (Bell's stage ≥2) and 1-year ND outcome (Bayley III). Results: The included patients (n = 101) had congenital heart disease (CHD), categorized as follows: 77 underwent biventricular repair for CHD with two functional chambers, 22 underwent staged palliation until the Fontan procedure for CHD with single ventricle physiology (n = 22), or 4 underwent single ventricle palliation or biventricular repair for borderline CHD (n = 4). Neonatal cardiopulmonary bypass (CBP) surgery was performed at a median age (IQR) of 8 (6) days. NEC occurred in 16 patients. Intensive care unit (ICU) length of stay (LOS) and the total duration of the hospitalization were longer in children with NEC than those in others (14 with vs. 8 days without NEC, p < 0.05; 49 with vs. 32 days without NEC, p < 0.05). The Bayley III scores of the analyzed patients determined at an age of 11.5 ± 1.5 months showed cognitive (CCS) (102.2 ± 15.0) and language scores (LCS) (93.8 ± 13.1) in the normal range and motor composite scores (MCS) (88.7 ± 15.9) in the low-normal range. After adjusting for socioeconomic status and CHD type, patients with NEC had lower CCS scores [ß = -11.2 (SE 5.6), p = 0.049]. Using a cumulative risk score including NEC, we found a higher risk score to be associated with both lower CCS [ß = -2.8 (SE 1.3), p = 0.030] and lower MCS [ß = -3.20 (SE 1.3), p = 0.016]. Conclusions: Postoperative NEC is associated with longer ICU and hospital LOS and contributes together with other complications to impaired ND outcome at 1 year of age. In the future, national and international patient registries may provide the opportunity to analyze large cohorts and better identify the impact of modifiable perioperative risk factors on ND outcome. Clinical Trial Registration: ClinicalTrials.gov identifier: NCT05996211.

Multimodality 3D-roadmap for cardiovascular interventions in congenital heart disease--a single-center, retrospective analysis of 78 cases.

BACKGROUND: Modern fluoroscopic angiography systems permit rendering of three-dimensional volumetric data sets using rotational angiography (3D-RA). Along with magnetic resonance imaging (MRI) or multi-detector-row computed tomography (MDCT) data sets, they can be fused with live fluoroscopy images for roadmapping during therapeutic procedures, but the value of multimodality fused data sets has not been clarified. METHODS: In a pediatric cardiac catheterization laboratory, we analyzed 78 interventional cardiovascular procedures in which 3D models of 3D-RA, MRI, or MDCT were used for 3D-guidance. Accuracy of 2D-3D registration as well as overall procedural benefit was independently rated by two pediatric interventionalists. Fluoroscopy time, radiation dose, and contrast dye consumption were evaluated and, grouping a subgroup analysis, the parameters were compared between patients who underwent stenting of aortic coarctation with and without 3D-roadmapping. RESULTS: 3D-guiding was used in 78 cases, 75 of these cases were with accurate 2D-3D registration. 3D-roadmapping was rated superior to conventional biplane imaging in 74 cases. 3D-guidance was used in 64 cases to define the ideal C-arm angulation and in 60 cases for accurate device positioning. Median dose-area product in the total investigation was 706.3 µGym(2) (104.8-7249.7 µGym(2) ), 3.3 ml/kg (0.9-13.7 ml/kg) of contrast dye was used, and total fluoroscopy time was 14.5 min (2.9-68.1 min). Fluoroscopy time for 3D-guided stenting of aortic coarctation is significantly lower (8.35 versus 10.2 min; P = 0.04). CONCLUSION: 3D-image fusion with live fluoroscopy can be applied successfully in catheter-based interventions of congenital heart disease. 3D-guidance facilitates catheter manipulations and interventions, allows preselection of ideal projection angles, reduces fluoroscopic time and the number of control angiographies.

Assessment of cavopulmonary connections by advanced imaging: value of flat-detector computed tomography.

OBJECTIVES: To investigate the impact of flat-detector computed tomography on the clinical assessment of patients with cavopulmonary connections, and to evaluate the obtained diagnostic accuracy and supplementary information, as well as the value of overlaid three-dimensional reconstructions on fluoroscopic images during catheter-based interventions. METHODS: We analysed 31 consecutive patients retrospectively in whom flat-detector computed tomography was used to visualise the cavopulmonary connection. We investigated patients with cavopulmonary connections either early post-operatively (first group), before converting to a total cavopulmonary connection (second group), and patients with failing total cavopulmonary connection (third group). Flat-detector computed tomography based on a single rotational angiography was used to create a three-dimensional vascular model. The clinical value of flat-detector computed tomography was evaluated using standard categories of diagnostic utility. Used contrast volume and radiation exposure were quantified. RESULTS: Within 18 months, flat-detector computed tomography was performed in 31 cases with cavopulmonary connections. The median age was 1.9 years (range 0.3-43 years). In the first group, we found anomalies in 4 out of 8 cases, which led to therapeutic or prophylactic procedures; in the second and third groups, we performed interventions in 14 out of 23 cases. The overall clinical value was always rated superior to conventional biplane angiography. The median dose area product was 91.8 microgray square metres (range 33.0-679.3 microgray square metres). The required contrast medium was 2.08 millilitres per kilogram (range 0.66-4.7 millilitres per kilogram). CONCLUSION: Flat-detector computed tomography improves the diagnostic accuracy in cavopulmonary connections and provides additional diagnostic information, which may lead to therapeutic or prophylactic procedures. Overlaid three-dimensional images on fluoroscopy facilitate and provide security for interventions.

Swiss Evaluation Registry for Pediatric Infective Endocarditis (SERPIE) - Risk factors for complications in children and adolescents with infective endocarditis.

BACKGROUND: Infective endocarditis (IE) in pediatric patients is a severe cardiac disease and its actual epidemiology and clinical outcome in Switzerland is scarcely studied. METHODS: Retrospective nationwide multicenter data analysis of pediatric IE in children (<18 years) between 2011 and 2020. RESULTS: 69 patients were treated for definite (40/69;58%) or possible IE (29/69;42%). 61% (42/69) were male. Diagnosis was made at median 6.4 years (IQR 0.8-12.6) of age with 19 patients (28%) during the first year of life. 84% (58/69) had congenital heart defects. IE was located on pulmonary (25/69;35%), mitral (10/69;14%), tricuspid (8/69;12%) and aortic valve (6/69;9%), and rarely on ventricular septal defect (VSD;4/69;6%) and atrial septal defect (ASD;1/69;1%). In 22% (16/69) localization was unknown. 70% (48/69) had postoperative IE, with prosthetic material involved in 60% (29/48; right ventricular to pulmonary artery conduit (24), VSD (4), ASD (1)). Causative organisms were mostly Staphylococci spp. (25;36%) including Staphylococcus aureus (19;28%), and Streptococci spp. (13;19%). 51% (35/69) suffered from severe complications including congestive heart failure (16;23%), sepsis (17;25%) and embolism (19;28%). Staphylococcus aureus was found as a predictor of severe complications in univariate and multivariate analysis (p = 0.02 and p = 0.033). In 46% (32/69) cardiac surgery was performed. 7% (5/69) died. CONCLUSIONS: IE in childhood remains a severe cardiac disease with relevant mortality. The high morbidity and high rate of complications is associated with Staphylococcus aureus infections. Congenital heart defects act as a risk factor for IE, in particular the high number of cases associated with prosthetic pulmonary valve needs further evaluation and therapeutic alternatives.

ORCHID (Outcome Registry for CHIldren with severe congenital heart Disease) a Swiss, nationwide, prospective, population-based, neurodevelopmental paediatric patient registry: framework, regulations and implementation.

INTRODUCTION: Congenital heart disease (CHD) is the most frequent birth defect. As survival has significantly improved, attention has turned to neurodevelopmental outcomes of children undergoing heart surgery in early infancy. Since multiple risk factors contribute to neurodevelopmental alterations, a nationwide registry collecting data on medical characteristics, interventions, clinical course and neurodevelopment until school-age is needed to improve the quality of management, identify risk- and protective factors affecting neurodevelopment, and facilitate multicentre trials. METHODS AND ANALYSIS: The Swiss Outcome Registry for CHIldren with severe congenital heart Disease (ORCHID) is a nationwide, prospective, population-based patient registry developed (1) to collect baseline characteristics and clinical data of CHD patients operated with bypass-surgery or hybrid procedures in the first 6 weeks of life in Switzerland, (2) to monitor long-term neurodevelopment, and (3) to relate clinical characteristics and neurodevelopment to identify risk and protective factors in these children. This registry started data collection relating to pregnancy, birth, preoperative course, catheter-based and surgical treatment, postoperative course and reinterventions in 2019. The primary outcome includes standardised neurodevelopmental assessments at 9 to 12 months, 18 to 24 months and 5.5 to 6 years. We expect to include 80 to 100 children per year. Correlation and regression analyses will be used to investigate risk- and protective factors influencing neurodevelopment. ETHICS AND DISSEMINATION OF RESULTS: Swiss ORCHID received support by the Accentus Charitable Foundation, the Anna Mueller Grocholoski Stiftung, the Swiss Society of Paediatric Cardiology, the Verein Kinderherzforschung, and the Corelina - Stiftung für das Kinderherz, and was approved by the cantonal ethics committees. Findings will be presented at national and international scientific meetings, and published in peer-reviewed journals. Results will also be shared with patient organizations, primary health care providers, and public health stakeholders to ensure a widespread dissemination of the results.

The value of flat-detector computed tomography during catheterisation of congenital heart disease.

OBJECTIVES: To analyse the diagnostic utility of flat-detector computed tomography imaging (FD-CT) in patients with congenital heart disease, including the value of image fusion to overlay three-dimensional (3D) reconstructions on fluoroscopic images during catheter-based interventions. METHODS: We retrospectively analysed 62 consecutive paediatric patients in whom FD-CT was used during catheterisation of congenital heart disease. Expert operators rated the clinical value of FD-CT over conventional fluoroscopic imaging. Added radiation exposure and contrast medium volume were evaluated. RESULTS: During a 12-month period, FD-CT was performed in 62 out of 303 cardiac catheterisations. Median patient age was 3.5 years. In 32/62 cases, FD-CT was used for diagnostic purposes, in 30/62 cases it was used in the context of interventions. Diagnostic utility was never rated as "misleading". It was classified as "not useful" in six cases (9.7%), "useful" in 18 cases (29.0%), "very useful" in 37 cases (59.7%) and "essential" in one case (1.6%). The median added dose-area product was 111.0 µGym(2), the required additional quantity of contrast medium was 1.6 ml/kg. CONCLUSION: FD-CT provides useful diagnostic information in most of the patients investigated for congenital heart disease. The added radiation exposure and contrast medium volume are reasonable.

The 'Cauliflower Heart': a case report of congenital bi-atrial aneurysms causing non-controllable arrhythmia.

BACKGROUND: Congenital aneurysms of the atrium are very rare malformations. Known complications are therapy-resistant arrhythmias. Different treatments such as medical therapy, electrophysiological ablation, and surgery have been proposed. However, there are no guidelines on treatment. CASE SUMMARY: We describe the case of a neonate with bi-atrial aneurysms causing atrial arrhythmia. Arrhythmia was first observed in the 28th week of gestation. Maternal digoxin treatment did not show any effect. After birth, bi-atrial aneurysms were diagnosed and determined as the probable cause of the atrial tachycardia and later of atrial flutter. Antiarrhythmic drug treatment was initiated. However, only frequency control could be achieved. At the age of 7 months, the patient underwent surgical resection. Since surgery, sinus rhythm is present. CONCLUSION: Atrial aneurysms are rare malformations, known complications are atrial arrhythmia. If medical treatment fails, surgery correction appears to be indicated going along with low operative risk and a high probability of successful termination of arrhythmia.

Right Axillary Thoracotomy in Congenital Cardiac Surgery: Analysis of Percutaneous Cannulation.

BACKGROUND: Vertical right axillary minithoracotomy (VRAMT) represents a minimally invasive and cosmetically attractive alternative for selected congenital heart defects. We report our institutional experience with VRAMT, especially regarding the performance of percutaneous femoral venous access to establish extracorporeal circulation in this pediatric population. METHODS: A retrospective single-center analysis was made of children to 16 years of age who underwent corrective cardiac surgery using VRAMT over a period of 5 years. VRAMT involved a 4 cm to 5 cm vertical incision parallel to the anterior axillary fold and aortic/bicaval cannulation. Since 2016, the technique has been modified and the inferior vena cava was cannulated using femoral percutaneous venous access. The primary endpoints were all-cause mortality, with additional secondary endpoints of major adverse cardiac and cerebrovascular events and conversion to median sternotomy. RESULTS: A total of 110 patients with biventricular congenital malformations were included. Age was 2.3 years (range, 0.2 to 16), and body weight was 11 kg (range, 3 to 47). Extracorporeal circulation time was 66 minutes (range, 24 to 167), cross-clamp time was 41 minutes (range, 9 to 95). Fast-track-management with on-table extubation was achieved in 34.5% (n = 38). For patients with percutaneous femoral venous cannulation (n = 38, 34.5%), thrombosis at the cannulation site was recorded in 5 cases (13.5%). There was no early or late mortality during the follow-up of 14.4 months (range, 0.8 to 47.19). No wound infection or thoracic deformities were observed. CONCLUSIONS: VRAMT can be considered as an alternative, minimally invasive, and cosmetically attractive access for the repair of frequent congenital heart defects in newborns and young children. Percutaneous femoral venous cannulation provides sufficient extracorporeal circulation flow and can be used even in infants with early postoperative heparin prophylaxis.

The impact of obesity on left ventricular hypertrophy and diastolic dysfunction in children and adolescents.

Childhood obesity continues to escalate worldwide and may affect left ventricular (LV) geometry and function. The aim of this study was to investigate the impact of obesity on prevalence of left ventricular hypertrophy (LVH) and diastolic dysfunction in children. In this analysis of prospectively collected cross-sectional data of children between 5 and 16 years of age from randomly selected schools in Peru, parameters of LV geometry and function were compared according to presence or absence of obesity (body mass index z-score > 2). LVH was based on left ventricular mass index (LVMI) adjusted for age and sex and defined by a z-score of > 2. LV diastolic function was assessed using mitral inflow early-to-late diastolic flow (E/A) ratio, peak early diastolic tissue velocities of the lateral mitral annulus (E'), early diastolic transmitral flow velocity to tissue Doppler mitral annular early diastolic velocity (E/E') ratio, and left atrial volume index (LAVI). Among 1023 children, 681 children (mean age 12.2 ± 3.1 years, 341 male (50.1%)) were available for the present analysis, of which 150 (22.0%) were obese. LVH was found in 21 (14.0%) obese and in 19 (3.6%) non-obese children (padjusted < 0.001). LVMI was greater in obese than that in non-obese children (36.1 ± 8.6 versus 28.7 ± 6.9 g/m2.7, p < 0.001). The mean mitral E/E' ratio and LAVI were significantly higher in obese than those in non-obese individuals (E/E': 5.2 ± 1.1 versus 4.9 ± 0.8, padjusted = 0.043; LAVI 11.0 ± 3.2 versus 9.6 ± 2.9, padjusted = 0.001), whereas E' and E/A ratio were comparable. Childhood obesity was associated with left ventricular hypertrophy and determinants of diastolic dysfunction.ClinicalTrials.gov Identifier: NCT02353663.

Surgical Management of Congenital Bilateral Multiple Atrial Aneurysms.

Congenital atrial aneurysms are a rare malformation, often associated with supraventricular arrhythmias. Here, we present the case of a child with biatrial aneurysms and a type 2 atrioseptal defect. Directly after birth the girl became symptomatic with incessant ectopic atrial tachyarrhythmia. On echocardiography, multiple biatrial aneurysms and septations were observed. The diagnosis was confirmed with computed tomography. After 7 months of antiarrhythmic therapy, the child underwent surgical intervention by aneurysm resection, atrioseptal defect closure, and ablation. Since then the patient has been in stable sinus rhythm.

Three-Dimensional Rotational Angiography during Catheterization of Congenital Heart Disease - A ten Years' experience at a single center.

This paper aims to assess the usability and advantages of three-dimensional rotational angiography (3DRA) in patients with congenital heart disease (CHD) and its application in the cath lab. Up to now, its use in CHD is not widespread or standardized. We analyzed all patients with CHD who underwent a 3DRA at our facility between January 2010 and May 2019. The 3DRAs were evaluated for radiation exposure, contrast dye consumption, diagnostic utility and image quality. We performed 872 3DRAs. 3DRA was used in 67.1% of the cases for interventional procedures and in 32.9% for diagnostic purposes. Two different acquisition programs were applied. The median dose-area product (DAP) for all 872 rotations was 54.1 µGym2 (21.7-147.5 µGym2) and 1.6 ml/kg (0.9-2.07 ml/kg) of contrast dye was used. Diagnostic utility of the generated 3D-model was rated superior to the native 3D angiography in 94% (819/872). 3DRA is an excellent and save diagnostic and interventional tool. However, 3DRA has not become a standard imaging procedure in pediatric cardiology up to now. Effort and advantage seems to be unbalanced, but new less invasive techniques may upgrade this method in future.