Long-term outcomes following mitral valve replacement in children at heart center Leipzig: a 20-year analysis.

BACKGROUND: Although mitral valve repair is the preferred surgical strategy in children with mitral valve disease, there are cases of irreparable severe dysplastic valves that require mitral valve replacement. The aim of this study is to analyze long-term outcomes following mitral valve replacement in children in a tertiary referral center. METHODS: A total of 41 consecutive patients underwent mitral valve replacement between February 2001 and February 2021. The study data was prospectively collected and retrospectively analyzed. Primary outcomes were in-hospital mortality, long-term survival, and long-term freedom from reoperation. RESULTS: Median age at operation was 23 months (IQR 5-93), median weight was 11.3 kg (IQR 4.8-19.4 kg). One (2.4%) patient died within the first 30 postoperative days. In-hospital mortality was 4.9%. Four (9.8%) patients required re-exploration for bleeding, and 2 (4.9%) patients needed extracorporeal life support. Median follow-up was 11 years (IQR 11 months - 16 years). Long-term freedom from re-operation after 1, 5, 10 and 15 years was 97.1%, 93.7%, 61.8% and 42.5%, respectively. Long-term survival after 1, 5, 10 and 15 years was 89.9%, 87%, 87% and 80.8%, respectively. CONCLUSION: If MV repair is not feasible, MV replacement offers a good surgical alternative for pediatric patients with MV disease. It provides good early- and long-term outcomes.

"Quality of life" analysis in the long-term follow-up after "Fontan" palliation for CHDs-a single-centre experience.

BACKGROUND: Complex CHDs are life threatening, and surgical treatment is needed for survival. Fontan palliation led to a significant increase in survival rates during the last decades. Consequently, quality of life became more essential. While a reduced quality of life compared to healthy children has been reported, detailed knowledge about individual quality of life and particular areas is lacking. Furthermore, the effect of different risk factors on quality of life is only rarely evaluated. METHOD AND RESULTS: Database of the department for pediatric cardiology, Heart Center Leipzig, was screened for children after total cavopulmonary connection palliation. n = 39 patients were included in the study, the outcome after total cavopulmonary connection was analysed in detail and quality of life data were collected and analysed using the standardised questionnaire "Pediatric quality of life inventory", version 4.0. We compared the total health score of our patients to the mean score of healthy children in the literature. The mean follow-up time was 6.4 ± 3.2 years, the overall survival was 100% after maximal follow-up time of 11.1 years. We could not find any age or gender dependence, nor an influence of age at total cavopulmonary connection on the later quality of life. Yet, patients with three-staged surgery exhibited a worse quality of life than patients with two-staged palliation. Late complications might influence quality of life, but patient number is too small, to find universal results. CONCLUSION: The total cavopulmonary connection palliation affects physical and psychological quality of life as well as cardiac health independently from age and gender. More patients and longer observation should be examined to confirm the results.

Long-Term Results Following Combined Repair of Atrioventricular Septal Defect with Tetralogy of Fallot.

Atrioventricular septal defect (AVSD) in association with tetralogy of Fallot (TOF) is a rare and complex congenital cardiac malformation. We report our institutional experience and outcomes following surgical correction over a 20-year period. Patients who underwent combined surgical AVSD and TOF correction between October 2001 and February 2020 were included for analysis. All patients underwent primary repair. The study data were prospectively collected and retrospectively analyzed. Primary outcomes were in-hospital mortality and long-term freedom from reoperation. During the study period, a total of 10 consecutive patients underwent combined surgical AVSD and TOF correction. Median age at operation was 307 days (IQR 228-457) and median weight was 7.7 kg (IQR 6.7-9.5). Down Syndrome was present in six of the patients. In-hospital mortality was 0%. One patient required re-exploration due to bleeding. Median follow-up was 11 years (IQR 11 months -16 years). There was one case of reoperation due to significant residual ventricular septal defect after 2 months. None of the patients died during follow-up. Combined primary AVSD and TOF repair can be performed with low early mortality and morbidity, as well as a high long-term freedom from reoperation.

Long-term results following atrioventricular septal defect repair.

BACKGROUND: Atrioventricular septal defects (AVSD) represent 4-7% of congenital cardiac malformations. Definitive early repair is favored over prior pulmonary artery banding and delayed definitive repair in many centers. The aim of this study was to analyze long-term outcomes following AVSD repair over a 21-year period. METHODS: A total of 202 consecutive patients underwent surgical AVSD correction between June 1999 and December 2020. Surgery was performed using the double-patch technique. The study data were prospectively collected and retrospectively analyzed. Primary outcomes were In-hospital mortality and overall long-term freedom from reoperation. RESULTS: Median age at operation was 120 days (IQR 94-150), median weight was 5.0 kg (4.2-5.3). None of the patients died within the first 30 postoperative days. In-hospital mortality was 0.5% (1/202 patients). Median follow-up was 57 months (11-121). Overall freedom from reoperation at 5, 10 and 15 years was 91.8%, 86.9% and 86.9%, respectively. CONCLUSION: AVSD repair with the double-patch technique is a safe and effective procedure with good early postoperative outcomes and low long-term reoperation rates.

The association of bicuspid aortic valve on long-term outcome following one-stage repair of aortic arch obstruction associated with ventricular septal defect.

OBJECTIVE: The aim of this study was to evaluate the association of bicuspid aortic valve on contemporary outcomes, including reoperation rates, after one-stage correction for interrupted aortic arch with ventricular septal defect or for aortic coarctation with hypoplastic aortic arch and ventricular septal defect. METHODS: Seventy-four consecutive patients (35 boys, 47% and 39 girls, 53%) with interrupted aortic arch (n = 41, 55%) or aortic coarctation with hypoplastic aortic arch (n = 33, 45%) with ventricular septal defect underwent early one-stage correction. Twenty (27%) patients had bicuspid aortic valve, and the remaining 54 (73%) had a tricuspid aortic valve. The median aortic valve annulus diameter was 6.0 mm (IQR: 2.0). Patients' median age was 7 ± 29 days (range, 2-150); median weight was 3.3 ± 0.7 kg (range, 1.5-6.0), with 21 (28%) patients <3.0 kg. Selective brain perfusion through the innominate artery and selective coronary perfusion through the aortic root during aortic arch reconstruction were used in all patients. Statistical analysis was performed using SPSS version 20.0 software (SPSS Inc., Chicago, IL, USA). RESULTS: The early mortality was 1.3%. One premature neonate died in the hospital with extracorporeal membrane oxygenation after aortic coarctation plus ventricular septal defect repair. There was no further mortality. Median follow-up was 5.7 years (IQR: 10.48). Reinterventions occurred in 36 (49%) patients: balloon angioplasty in 18 (24%) patients, reoperations in 4 (5%) patients, and both in 14 (19%) patients. A total of 86 follow-up procedures were required in these 36 (49%) patients: aortic valve valvulopasty (n = 6, 8%), stent implantation (n = 8, 11%), balloon dilatation (n = 39, 53%), and reoperation (n = 33, 45%). The median time to reinterventions was 9.094 years (SE 0.890). A potential risk factor for reintervention after interrupted aortic arch and aortic coarctation with ventricular septal defect repair was bicuspid aortic valve (p = 0.019, Chi2 (1) = 5.457). In addition, a multivariate Cox analysis with backward selection and significance level <0.015 was applied to all variables that showed significant effects in univariable analyzes. This regression confirmed that bicuspid aortic valve (HR = 0.381, p = .016), and interrupted aortic arch (HR = 0.412, p = 0.043) were predictors of late reintervention. All patients had no obvious neurologic impairment in routine examinations at last follow-up. CONCLUSION: Bicuspid aortic valve was a significant risk factor for valve-related reintervention after one-stage repair for aortic arch obstruction with ventricular septal defect due to later development of stenosis associated with higher late morbidity and mortality. Particularly neonates with bicuspid aortic valve will possibly require reintervention in the future. Regular lifelong cardiac follow-up is recommended.

Pulmonary valve prostheses: patient's lifetime procedure load and durability. Evaluation of the German National Register for Congenital Heart Defects.

OBJECTIVES: We evaluated 4384 procedures performed between 1957 and 2018, collected in the National Register for Congenital Heart Defects, conducted on 997 patients with 1823 pulmonary valve replacements (PVRs), including 226 implanted via catheter [transcatheter valve (TCV)]. Main study targets are as follows: TCV benefit, valve type durability, decade-wise treatment changes and procedure frequencies over the lifetime of a PVR patient. METHODS: We studied TCV impact on surgical valve replacement (via Kaplan-Meier); pulmonary valve type-specific performance (Kaplan-Meier and Cox regressions with age group as stratification or ordinary variable); procedure interval changes over the decades (Kaplan-Meier); procedure load, i.e. frequency of any procedure/surgical PVR/interventional or surgical PVR by patient age (multistate analyses). RESULTS: TCV performance was equivalent to surgical PVRs and extended durability significantly. Homografts were most durable; Contegras lasted comparably less in older; and Hancock devices lasted less in younger patients. Matrix P-valves showed poorer performance. Age group stratification improves the precision of valve-specific explantation hazard estimations. The current median interval between procedures is 2.6 years; it became significantly shorter in most age groups below 40 years. At 30 years, 80% of patients had undergone ≥3 procedures, 20% ≥3 surgical PVRs and 42% ≥3 surgical or interventional PVRs. CONCLUSIONS: TCVs doubled freedom from explantation of conventional valves. Homografts' age group-specific explantation hazard ratio was lowest; Matrix P's hazard ratio was highest. Age-stratified Cox regressions improve the precision of prosthesis durability evaluations. The median time between procedures for PVR patients shortened significantly to 2.6 years. At 30 years, 42% had ≥3 PVRs.

First paediatric cohort for the evaluation of inflammation in endomyocardial biopsies derived from congenital heart surgery.

BACKGROUND: Endomyocardial biopsies (EMB) are the gold standard for the diagnosis of myocarditis in children and adults. The existing WHO/ISFC criteria for lymphocytic cell infiltrates by are based on the myocardium of adults. The aim of this study was to present a paediatric control cohort for the evaluation of inflammation in EMB of children. METHODS: In this study endomyocardial tissue from 62 children under 4 years of age was investigated, being collected during a planned open heart surgery with routine resection from ventricular site. Patients had no history of infection or myocardial inflammation. The heart tissue was formalin fixed and embedded in paraffin. Four µm thick tissue sections were stained with haematoxylin and eosin, Masson's trichrome, and Giemsa. Immunohistochemical stainings included quantitative evaluation of CD3+ T cells, CD20+ B cells, CD68+ macrophages and MHCII expression. RESULTS: The myocardium was obtained in 96.8% (n = 60) of the cases from the right and in 3.2% (n = 2) from the left ventricle. The median age (interquartile range) at biopsy was 0.5 years (0.3-0.9), 66.1% male. Within this cohort, a median of 2.5/mm2 (1.0-4.0) CD3+ T cells, 0.5/mm2 (0.0-0.6) CD20+ B cells and 4.0/mm2 (2.5-6.0) CD68+ macrophages were detected. The MHC II grade was 0 in 71.0% (n = 44) and 1 in 29.0% (n = 18). CONCLUSION: This is the first paediatric control cohort being relevant for the correct interpretation of inflammatory heart diseases in EMB. The lymphocytic cell numbers in children needing congenital heart surgery without myocardial inflammation are below the existing values in adults.

ChyloBEST: Chylothorax in Infants and Nutrition with Low-Fat Breast Milk.

Chylothorax occurs in 2.8-5% of infants after cardiac surgery and can increase morbidity and mortality. First-line conservative treatment consists of a chest tube drainage and a fat-free and medium-chain triglyceride (MCT)-enriched diet. This typically leads to a discontinuity of breast milk feeding due to high content of long-chain triglycerides within the breast milk. Modified breast milk with low fat content (LFBM) could provide numerous benefits like immunological properties of breast milk even for patients with chylothorax. This study was conducted at Herzzentrum Leipzig comparing clinical and growth outcomes between infants with chylothorax after surgery for congenital heart disease treated with LFBM (n = 13) versus MCT-Formula (n = 10). LFBM was prepared by centrifugation of native breast milk added with MCT-oil and fortifier. There were no differences in volume and duration of chest tube drainage between LFBM and MCT-formula treatment groups. Furthermore, no statistically significant differences with regard to weight and length gains could be observed between both feeding groups. LFBM is an efficient and unharmful treatment for chylothorax following cardiac surgery in young children.

Differences of early immunological responses in on-pump versus off-pump cardiac surgery.

INTRODUCTION: Cardiopulmonary bypass surgery is accompanied by an inflammatory response and pulmonary dysfunction that renders patients vulnerable to postoperative complications. The majority of studies investigating the inflammatory response in cardiopulmonary bypass focus on cytokine measurements. This study investigated the early response of peripheral blood cell types and early changes in lung tissue in on-pump versus off-pump cardiopulmonary bypass surgery. METHODS: Landrace pigs were assigned to the following groups (n = 6 per group): 1. off-pump cardiopulmonary bypass, 2. conventional cardiopulmonary bypass, 3. heparin-coated cardiopulmonary bypass, 4. surface-reduced cardiopulmonary bypass, and 5. surface-reduced cardiopulmonary bypass plus lung perfusion. Surgery was performed under mild hyperthermia (32°C), with 90-minute ischemia and 180-minute reperfusion. Histological and flow cytometric analyses were performed. RESULTS: Lung water content increased during reperfusion in heparin-coated (84.63 ± 2.99%) compared to conventional cardiopulmonary bypass (76.33 ± 4.56%, p = 0.04). Alveolar septal thickness increased during ischemia at heparin-coated (p < 0.01) and surface-reduced cardiopulmonary bypass plus lung perfusion (p = 0.05). Tumor necrosis factor expression increased significantly (p < 0.01) in peribronchial, perivascular, and peripheral lung areas in all on-pump groups, but not in off-pump cardiopulmonary bypass. The usage of heparin-coated cardiopulmonary bypass led to increased percentages of CD3+CD4+ (p = 0.03) and CD3+CD8+ (p = 0.01) T cells compared to an uncoated device. Natural killer and mature B lymphocytes decreased at conventional and surface-reduced cardiopulmonary bypass plus lung perfusion. Activated granulocytes and macrophages increased at conventional cardiopulmonary bypass and heparin-coated cardiopulmonary bypass. CONCLUSION: Off-pump cardiopulmonary bypass induces less immunological response and lung injury than on-pump surgery. The reduction of cardiopulmonary bypass surface reduces the inflammatory immune response induced by cardiopulmonary bypass. Lung perfusion of surface-reduced cardiopulmonary bypass diminished the extravasation caused by surface reduction of the cardiopulmonary bypass.

Complex cases of acquired pulmonary vein stenosis after radiofrequency ablation: is surgical repair an option?

AIMS: Results of catheter based interventional treatment for pulmonary vein stenosis (PVS) following radiofrequency ablation (RFA) for atrial fibrillation remain suboptimal. Surgical repair may represent an alternative therapy, though long-term results have not been thoroughly investigated. METHODS AND RESULTS: We retrospectively assessed all patients in our centre undergoing surgical repair for radiofrequency-induced PVS. Data regarding surgical technique, clinical outcome, and rate of pulmonary vein (PV) restenosis were collected and analysed. Between 2004 and 2016, the rate for PVS resulting from RFA for atrial fibrillation in our institution was 0.79% (76/9633). During this period, five male patients with multiple PVS (3 ± 1) underwent surgical repair of a total of 13 symptomatic PVS. Surgery was performed in a standard setting under cardiopulmonary bypass. Stenotic veins were incised longitudinally followed by a patch augmentation plasty using either bovine pericard (n = 7) or polytetrafluoroethylene (PTFE) patches (n = 5). Localization of incision was on the anterior side of the PV only (n = 8) or on both the anterior and posterior sides (n = 4). In one PVS lesion, mechanical dilatation was sufficient. Long-term follow-up after 60 ± 69 months revealed an average restenosis rate of 38%. Restenosis was defined as narrowing >70%. All patients reported clinical improvement of symptoms at follow-up. CONCLUSION: Even in the era of wide circumferential lesions, PVS still occurs. While surgical PV patch plasty represents a valuable treatment option, restenosis remains an issue during follow-up. Nevertheless, surgical repair achieves highly acceptable long-term results for RFA-acquired PVS. Hence, it should be routinely discussed as a therapeutic option in cases with multiple PVS.

Symptomatic myocardial bridging: a frequently occurring coronary variation can cause severe myocardial ischaemia in affected children with underlying cardiac conditions.

Myocardial bridging is a congenital coronary artery anomaly in which the coronary artery has a partly "tunnelled" intramyocardial course. This tunnelling leads to compression of the affected vessel segment during ventricular systole. It is considered to be a benign variation of the norm in about 25% of the population caused by an aberrancy of embryologic coronary development. The bridging is also thought to cause severe cardiac conditions in a few of those affected. The series of six young patients presented here is the largest series so far to report on symptomatic myocardial bridging in children with different underlying heart diseases. All patients recently presented to our centre with signs of myocardial ischaemia. They subsequently underwent coronary angiography, which revealed myocardial bridging of the ramus interventricularis anterior. In all patients, therapy with ß blockers was started to reduce heart rate and myocardial contractility. ß Blocker treatment was also given in order to prolong diastole and improve coronary artery blood flow. Two patients underwent surgical exposure of the involved coronary segment: a 2-year-old boy because of recurrent, severe myocardial ischaemia in combination with a reduction of general health, changes in ST-segments, and the presence of a dilative cardiomyopathy; and a 13-year-old girl because of evidence of myocardial ischaemia during exercise testing after surviving sudden cardiac death. Surgery was successful and recovery was complete and uneventful. The presented series shows that myocardial bridging can be symptomatic and may require urgent treatment and even surgical intervention in early childhood in rare cases.

Retrospective study of complete atrioventricular canal defects: Anesthetic and perioperative challenges.

OBJECTIVE: The objective of this study was to highlight anesthetic and perioperative management and the outcomes of infants with complete atrioventricular (AV) canal defects. DESIGN: This retrospective descriptive study included children who underwent staged and primary biventricular repair for complete AV canal defects from 1999 to 2013. SETTING: A single-center study at a university affiliated heart center. PARTICIPANTS: One hundred and fifty-seven patients with a mean age at surgery of 125 ± 56.9 days were included in the study. About 63.6% of them were diagnosed as Down syndrome. Mean body weight at surgery was 5.6 ± 6.3 kg. METHODS: Primary and staged biventricular repair of complete AV canal defects. MEASUREMENTS AND MAIN RESULTS: A predefined protocol including timing of surgery, management of induction and maintenance of anesthesia, cardiopulmonary bypass, and perioperative intensive care treatment was used throughout the study. Demographic data as well as intraoperative and perioperative Intensive Care Unit (ICU) data, such as length of stay in ICU, total duration of ventilation including reintubations, and total length of stay in hospital and in hospital mortality, were collected from the clinical information system. Pulmonary hypertension was noted in 60% of patients from which 30% needed nitric oxide therapy. Nearly 2.5% of patients needed permanent pacemaker implantation. Thorax was closed secondarily in 7% of patients. In 3.8% of patients, reoperations due to residual defects were undertaken. Duration of hospital stay was 14.5 ± 4.7 days. The in-hospital mortality was 0%. CONCLUSION: Protocolized perioperative management leads to excellent outcome in AV canal defect repair surgery.

Calcification or Not. This Is the Question. A 1-Year Study of Bovine Pericardial Vascular Patches (CardioCel) in Minipigs.

A main problem with bioprosthesis used for surgical correction of congenital cardiac malformation is its tendency to shrink and to calcify. Recently, a new material, that is, decellularized bovine pericardium (CardioCel), was introduced in clinics. It was proposed that this new patch material should not calcify and should be particularly suitable for the correction of vascular defects in inborn cardiac diseases. The aim of our chronic minipig study was to evaluate the performance of CardioCel patches implanted in aortic and pulmonary artery position, respectively. Ten minipigs aged 3 months were operated on. A CardioCel patch was implanted in the aorta ascendens and arteria pulmonalis, respectively. Seven minipigs completed the 12 months' follow-up. Angiography of both vessels, measurement of pressure gradients, and histologic evaluation of the implanted patches were carried out. Angiography of both great vessels revealed a good clinical outcome without stenosis. However, histologic examination of the patches showed calcification and neo-formation of hyaline cartilage in both vessel types. Staining of collagen and elastic fibers as well as α-smooth muscle actin demonstrated that the patches did not remodel into an anatomic vascular structure during the 1 year of implantation. In our chronic piglet model, CardioCel patches, when implanted in the ascending aorta and the pulmonary artery, led to calcification and neo-formation of hyaline cartilage in both vessel types 1 year after implantation. The present study indicates that the ideal patch biomaterial for repair of inborn cardiac diseases is still a goal not achieved yet.

Cross-sectional Areas of the Thoracic Aorta in Children and Adolescents With Repaired Tetralogy of Fallot Obtained by Cardiac Magnetic Resonance Angiography.

PURPOSE: Dilatation of the thoracic aorta is a well-known finding in corrected Tetralogy of Fallot. Complications are rare but can be life-threatening. Standard 1-dimensional (1D) measurements have several limitations. We sought to establish contrast-enhanced magnetic resonance angiography cross-sectional areas of the aorta that could serve as reference values and to identify parameters that are associated with aortic dilatation. MATERIALS AND METHODS: We enrolled 101 children and adolescents. The aortic areas were measured at the level of the aortic sinus (AS), the sinotubular junction (STJ), the ascending aorta (AA), the brachiocephalic trunk (TBC), and the descending aorta (DA). Sex-specific aortic dimensions were presented as percentile curves as well as regression equations. Furthermore volumetric and functional parameters as well as clinical data were analyzed to identify parameters that are associated with aortic dilatation. RESULTS: Aortic areas (mm) for female subjects were 139+366×body surface area (BSA) for the AS, 134+255×BSA for the STJ, 113+239×BSA for the AA, 88+185×BSA proximal to the TBC, and 2.9+88×BSA for the DA. Aortic areas (mm) for male subjects were 162+403×BSA for the AS, 171+258×BSA for the STJ, 151+233×BSA for the AA, 73+206×BSA proximal to the TBC, and 21+80×BSA for the DA. The postoperative interval and age at examination were parameters associated with aortic size. CONCLUSIONS: We provide aortic areas in children and adolescents after correction of Tetralogy of Fallot measured by contrast-enhanced magnetic resonance angiography. Our 2D data may better depict the geometry of enlarged aortae than standard 1D diameters and serve as reference values for evaluating aortic disease in these patients.

Epigallocatechin gallate attenuates cardiopulmonary bypass-associated lung injury.

BACKGROUND: Lung dysfunction constitutes a severe complication after major cardiac surgery with cardiopulmonary bypass (CPB), substantially contributing to postoperative morbidity and mortality. The current possibilities of preventive and therapeutic interventions, however, remain insufficient. We, therefore, investigated the effects of intraoperative application of the antioxidant and anti-inflammatory green tea polyphenol (-)-epigallocatechin-3-gallate (EGCG) on CPB-associated lung injury. MATERIALS AND METHODS: Thirty piglets (8-15 kg) were divided into four groups: sham-operated and saline-treated control group (n = 7); sham-operated and EGCG-treated control group (EGCG-control group; n = 7); CPB group (n = 10); and CPB + EGCG group (n = 6). The CPB groups underwent 120 min of CPB followed by 90 min of recovery time. In the CPB + EGCG group, EGCG (10 mg/kg body weight) was administered intravenously before and after CPB. Hemodynamic monitoring, blood gas analysis, hematoxylin-eosin staining, and immunohistochemistry of lung tissue were performed. RESULTS: Histologic examination revealed thickening of the alveolar wall and enhanced alveolar neutrophil infiltration in the CPB group (P < 0.05) compared with those in the control group, which was prevented by EGCG (P < 0.05). In the CPB group, higher formation of poly(ADP-ribose) and nuclear translocation of apoptosis-inducing factor was detected in comparison with those in the control group (P < 0.001), which were both reduced in the CPB + EGCG group (P < 0.001). Compared with the control group, the EGCG-control group showed thickening of the alveolar wall and increased neutrophil infiltration (P < 0.05). CONCLUSIONS: CPB leads to lung edema, pulmonary neutrophil infiltration, and presumably initiation of poly(ADP-ribose) polymerase-dependent cell death signaling in the lung. EGCG appears to attenuate CPB-associated lung injury, suggesting that this may provide a novel pharmacologic approach.

Dimensions of the ascending aorta in children and adolescents with repaired Tetralogy of Fallot obtained by cardiac magnetic resonance angiography.

INTRODUCTION: Dilatation of the ascending aorta is a common finding in Tetralogy of fallot (TOF). We sought to provide aortic dimensions in children and adolescents after corrected TOF obtained by contrast-enhanced cardiac-magnetic-resonance angiography (CE-CMRA) that could serve as reference values. MATERIALS AND METHODS: We enrolled 101 children and adolescents (56 male) with a median age of 10.9 years. All patients underwent CE-CMRA imaging using a 3-dimensional spoiled gradient-echo-sequence. Aortic diameters were measured at the level of the aortic valve (AV), aortic sinus (AS), sino-tubular junction (STJ) and the ascending aorta (AA) and compared with normal values obtained from literature. Sex-specific aortic dimensions are given as percentile curves as well as z scores. Furthermore CMR volumetric and functional parameters as well as clinical and anamnestic data were analyzed to identify parameters that are associated with aortic dilatation. RESULTS: Diameters for aortic size for males were 3.6 + 16.6*BSA(0.5) at the AV level, 7.0 + 19.5*BSA(0.5) at the AS level, 7.0 + 14.4*BSA(0.5) at the STJ level and 7.3 + 15.5*BSA(0.5) at the AA level. Diameters for females were 5.8 + 14.1*BSA(0.5) at the AV level, 7.2 + 17.6*BSA(0.5) at the AS level, 5.2 + 15.4*BSA(0.5) at the STJ level and 2.0 + 17.8*BSA(0.5) at the AA level. All diameters in TOF patients were larger compared with normal values. The postoperative interval and age at examination were the only parameters associated with aortic size at all measured levels. CONCLUSION: We provide CE-CMRA data of aortic dimensions in children and adolescents after correction of TOF. Our data might be useful for an estimation of the "normal" aortic size in this patient cohort and can serve as a basis for future longitudinal studies adding prognostic data.

Rescue surgery for neonate with huge rhabdomyoma and left outflow obstruction.

We describe a rescue operation in a neonate with a large left ventricular tumor obstructing the left ventricular outflow tract. Surgical resection was performed through an aortotomy and transseptal approach. We excised the main part of the tumor, which was obstructing the outflow tract, leaving a portion that was strongly attached to posterior wall of the left ventricle and mitral valve annulus, which was not feasible to remove. Histological examination showed a rhabdomyoma with benign features. The girl was doing well after 3 months, with no residual tumor growth and no signs of obstruction of the left ventricular outflow tract.