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Despite improvement in survival, patients with a Fontan circulation are at risk for numerous complications, and exercise capacity and quality of life are usually reduced compared with healthy controls. However, only a few studies have assessed the impact of the amount of sporting activity on exercise capacity and health-related quality of life. We analyzed cardiopulmonary exercise tests (CPET) in a large cohort of patients with a Fontan circulation. Questionnaires were used to assess health-related quality of life and sporting activity. A total of 79 patients with a median age of 13.0 (6.5 to 34.4) years at CPET were included (female, n = 31). Questionnaires revealed that 80% of patients (n = 63) do leisure sports with 43% (n = 27) exercising more than 2 hours per /week. In a subgroup analysis on pediatric patients (n = 52) we found that nearly all participate in school sports (n = 51) and report good subjective health (n = 48). In the pediatric subgroup, oxygen uptake at the anaerobic threshold and peak oxygen uptake correlated with subjective health (p 0.05) and the amount of leisure sports activity (p 0.01). In the overall cohort, running time and running distance were significantly associated with the hours of sports per week (p0.01). CPET results did not significantly differ between single right and single left ventricle patients. Furthermore, an open fenestration was not associated with reduced exercise capacity. In conclusion, most patients with a Fontan circulation participate in leisure sports and report good subjective healthiness. In pediatric patients, increased sports activity is associated with better exercise capacity and subjective healthiness.

Fontan associated liver disease (FALD) has been recognized as a potentially serious sequela of the Fontan circulation. Prevalence of FALD among different age groups and risk factors for advanced changes were assessed. FALD screening included abdominal ultrasound and laboratory tests. A "liver disease score (LDS)" incorporating items from ultrasound and blood testing was calculated to grade FALD severity (5 items each, maximum score 10 points). 240 patients (male: n = 139, female: n = 101, systemic right ventricle: n = 160) underwent FALD screening 10 (IQR 7-15) years after Fontan surgery. Ultrasound was abnormal in 184 (76.6%) patients (surface nodularity / blunted liver edge: n = 133, 55.4%; heterogeneous parenchyma: n = 93, 38.8%; splenomegaly: n = 68, 28.3%; ascites: n = 23, 9.6%). At least one abnormal laboratory test was detected in 218 (90.8%) patients. Gamma-glutamyl-transpeptidase was elevated in the majority of patients (n = 206, 85.8%). Median LDS was 3 (2-4). Scores ≥5 were observed in 32 (13.3%) patients. Longer follow-up (15 (11-20) vs 9 (6-14) years, P0.001), higher central venous (13 (11-15) vs 10 (9-12) mmHg, P0.001) and end-diastolic pressure (8 (5-10) vs 6 (5-7) mmHg, P = 0.001), impaired ventricular function and absence of sinus rhythm were associated with LDS ≥5. Longer follow-up (OR 1.2 (1.1-1.3), P0.001) and higher central venous pressure (OR 1.6 (1.3-2.1), p0.001) were the only independent predictors of advanced FALD. Abdominal ultrasound and laboratory abnormalities suggestive of FALD are common during routine follow-up already in childhood and adolescence irrespective of ventricular morphology. More advanced findings are associated with longer follow-up and higher central venous pressure.

BACKGROUND: The Norwood procedure is the first stage of correction for patients with hypoplastic left heart syndrome (HLHS) and may lead to an abnormal neoaortic anatomy. We prospectively studied the neoaorta’s fluid dynamics and the abnormal twist of the neoaorta by MRI examinations of HLHS patients in Fontan circulation. This study for the first time investigates the hypothesis that the neoaorta twist is associated with increased helical flow patterns, which may lead to an increased workload for the systemic right ventricle (RV) and ultimately to RV hypertrophy. METHODS: A group of forty-two HLHS patients with a median age of 4.9 (2.9–17.0) years, at NYHA I was studied along with a control group of eleven subjects with healthy hearts and a median age of 12.1 (4.0–41.6). All subjects underwent MRI of the thoracic aorta including ECG-gated 2D balanced SSFP cine for an axial slice stack and 4D-flow MRI for a sagittal volume slab covering the thoracic aorta. The twist of the neoaortic arch was quantified by the effective geometric torsion, defined as the product of curvature and geometric torsion. Fluid dynamics and geometry in the neoaorta, including the flow helicity index, were evaluated using an in-house analysis software (MeVisLab-based). Myocardial mass of the systemic ventricle at end-diastole was estimated by planimetry of the short-axis stack. RESULTS: Compared to the control group, the neoaorta in the HLHS patients shows an increased twist (P=0.04) and higher peak helicity density (P=0.03). The maximum helicity density was correlated with maximum effective torsion of the ascending neoaorta (P

OBJECTIVES The aim of this study was to assess single right ventricular (RV) function in a large cohort of hypoplastic left heart syndrome (HLHS) patients after the completion of total cavopulmonary connection by analysing serial cardiovascular magnetic resonance (CMR) studies. METHODS CMR studies from 95 HLHS patients were analysed. RV end-diastolic and end-systolic volumes (RVEDV, RVESV), ejection fraction (RVEF) and long-axis strain (LAS) were measured from cine images. RESULTS All 95 patients had at least 2 CMR scans and 35 patients had 3 CMR scans. The median age (first quartile–third quartile) at the 3 examinations was 4.2 (3.3–6.1), 9.4 (6.1–11.4) and 14.6 (11.8–16.8) years. RV indexed volumes (RVEDVi and RVESVi) increased from first to the second and from the first and second examination to the third examination in patients with >10 years of age (P CONCLUSIONS Serial assessment of CMR studies in HLHS patients after total cavopulmonary connection completion demonstrate an increase in indexed RV volumes in older HLHS patients but only mild reduction in RVEF and LAS. The correlation of indexed RV volumes with RVEF and LAS together with the significant increase in RV volumes over time suggests that indexed RV volumes might be superior to RV functional markers to monitor the RV in HLHS patients.

Background Right ventricular outflow tract (RVOT) stenosis after repair of tetralogy of Fallot has been linked with favorable right ventricular remodeling but adverse outcomes. The aim of our study was to assess the hemodynamic impact and prognostic relevance of right ventricular pressure load in this population. Methods and Results A total of 296 patients with repaired tetralogy of Fallot (mean age, 17.8±7.9 years) were included in a prospective cardiovascular magnetic resonance multicenter study. Myocardial strain was quantified by feature tracking technique at study entry. Follow‐up, including the need for pulmonary valve replacement, was assessed. The combined end point consisted of ventricular tachycardia and cardiac death. A higher echocardiographic RVOT peak gradient was significantly associated with smaller right ventricular volumes and less pulmonary regurgitation, but lower biventricular longitudinal strain. During a follow‐up of 10.1 (0.1–12.9) years, the primary end point was reached in 19 of 296 patients (cardiac death, n=6; sustained ventricular tachycardia, n=2; and nonsustained ventricular tachycardia, n=11). A higher RVOT gradient was associated with the combined outcome (hazard ratio [HR], 1.03; 95% CI, 1.00–1.06; P =0.026), and a cutoff gradient of ≥25 mm Hg was predictive for cardiovascular events (HR, 3.69; 95% CI, 1.47–9.27; P =0.005). In patients with pulmonary regurgitation ≥25%, a mild residual RVOT gradient (15–30 mm Hg) was not associated with a lower risk for pulmonary valve replacement. Conclusions Higher RVOT gradients were associated with less pulmonary regurgitation and smaller right ventricular dimensions but were related to reduced biventricular strain and emerged as univariate predictors of adverse events. Mild residual pressure gradients did not protect from pulmonary valve replacement. These results may have implications for the indication for RVOT reintervention in this population.

Background: The status of the systemic right ventricular coronary microcirculation in hypoplastic left heart syndrome (HLHS) is largely unknown. It is presumed that the systemic right ventricle’s coronary microcirculation exhibits unique pathophysiological characteristics of HLHS in Fontan circulation. The present study sought to quantify myocardial blood flow by cardiac magnetic resonance imaging and evaluate the determinants of microvascular coronary dysfunction and myocardial ischemia in HLHS. Methods: One hundred nineteen HLHS patients (median age, 4.80 years) and 34 healthy volunteers (median age, 5.50 years) underwent follow-up cardiac magnetic resonance imaging ≈1.8 years after total cavopulmonary connection. Right ventricle volumes and function, myocardial perfusion, diffuse fibrosis, and late gadolinium enhancement were assessed in 4 anatomic HLHS subtypes. Myocardial blood flow (MBF) was quantified at rest and during adenosine-induced hyperemia. Coronary conductance was estimated from MBF at rest and catheter-based measurements of mean aortic pressure (n=99). Results: Hyperemic MBF in the systemic ventricle was lower in HLHS compared with controls (1.89±0.57 versus 2.70±0.84 mL/g per min; P P =0.446). Independent risk factors for a reduced hyperemic MBF were an HLHS subtype with mitral stenosis and aortic atresia ( P =0.017), late gadolinium enhancement ( P =0.042), right ventricular diastolic dysfunction ( P =0.005), and increasing age at total cavopulmonary connection ( P =0.022). The coronary conductance correlated negatively with systemic blood oxygen saturation (r, −0.29; P =0.02). The frequency of late gadolinium enhancement increased with age at total cavopulmonary connection ( P =0.014). Conclusions: The coronary microcirculation of the systemic ventricle in young HLHS patients shows significant differences compared with controls. These hypothesis-generating findings on HLHS-specific risk factors for microvascular dysfunction suggest a potential benefit from early relief of frank cyanosis by total cavopulmonary connection.

Summary Right ventricular hypertrophy (RVH) occurs in high pressure afterload, e.g., tetralogy of Fallot/pulmonary stenosis (TOF/PS). Such RVH is associated with alterations in energy metabolism, neurohormonal and epigenetic dysregulation (e.g., microRNA), and fetal gene reprogramming in animal models. However, comprehensive expression profiling of competing endogenous RNA in human RVH has not been performed. Here, we unravel several previously unknown circular, long non-coding, and microRNAs, predicted to regulate expression of genes specific to human RVH in the non-failing heart (TOF/PS). These genes are significantly overrepresented in pathways related to regulation of glucose and lipid metabolism (SIK1, FABP4), cell surface interactions (THBS2, FN1), apoptosis (PIK3IP1, SIK1), extracellular matrix composition (CTGF, IGF1), and other biological events. This is the first unbiased RNA sequencing study of human compensated RVH encompassing coding and non-coding RNA expression and predicted sponging of miRNAs by non-coding RNAs. These findings advance our understanding of adaptive RVH and highlight future therapeutic targets., Graphical abstract, Highlights • First comprehensive transcriptomic study of human RVH via RNA expression and network analysis • First human RVH study using exclusively freshly isolated myocardium • Known hypertrophy genes are regulated the strongest by competing endogenous RNA networks in RVH • Epigenetic mRNA regulation in RVH by ncRNAs is dependent on sex and age, Molecular Biology; Omics

Objectives Neonates and infants undergoing surgery for congenital heart disease are at risk for developmental impairment. Hypoxic-ischemic brain injury might be one contributing factor. We aimed to investigate the perioperative release of the astrocyte protein S100B and its relation to cerebral oxygenation. Methods Serum S100B was measured before and 0, 12, 24, and 48 hours after surgery. Cerebral oxygen saturation was derived by near-infrared spectroscopy. S100B reference values based on preoperative samples; concentrations above the 75th percentile were defined as elevated. Patients with elevated S100B at 24 or 48 hours were compared to cases with S100B in the normal range. Neonates (≤28 days) and infants (>28 and ≤365 days) were analyzed separately due to age-dependent release of S100B. Results Seventy-four patients underwent 94 surgical procedures (neonates, n = 38; infants, n = 56). S100B concentrations were higher in neonates before and after surgery at all time points (P ≤ .015). Highest values were noticed immediately after surgery. Postoperative S100B was elevated after 15 (40.5%) surgeries in neonates. There was no difference in pre-, intra-, or postoperative cerebral oxygenation. In infants, postoperative S100B was elevated after 23 (41.8%) procedures. Preoperative cerebral oxygen saturations tended to be lower (53 ± 12% vs 59 ± 12%, P = .069) and arterial-cerebral oxygen saturation difference was higher (35 ± 11% vs 28 ± 11%, P = .018) in infants with elevated postoperative S100B. In the early postoperative course, cerebral oxygen saturation was lower (54 ± 13% vs 63 ± 12%, P = .011) and arterial-cerebral oxygen saturation difference was wider (38 ± 11% vs 30 ± 10%, P = .008). Cerebral oxygen saturation was also lower for the entire postoperative course (62 ± 18% vs 67 ± 9%, P = .047). Conclusions Postoperative S100B was elevated in about 40% of neonates and infants undergoing cardiac surgery. Infants with elevated postoperative S100B had impaired perioperative cerebral tissue oxygenation. No relation between S100B and cerebral oxygenation could be demonstrated in neonates.

Introduction The descending aorta (DAo) in patients with hypoplastic left heart syndrome (HLHS) after Norwood procedure is frequenty enlarged and exhibits increased stiffness. Such findings of previous studies still remain unexplained given the fact that the DAo is not involved in the Norwood operation. Methods We studied five HLHS patients with DAo dilatation (aged: 2.9–15.1 years (y), median 9.0 y), four HLHS patients without DAo dilatation (aged: 3.5–9.7 years, median 6.45 y) and 7 healthy controls (aged 6.3–41.6 y, median 26.0 y) using cardiovascular magnetic resonance imaging with acquisition of cine images, contrast-enhanced angiograms and 4D flow. 4D flow data were analyzed based on in-house developed analysis software to quantify vortical flow patterns in terms of vorticity. Results All patients with DAo dilatation presented with a caliber reduction between the proximal and distal aortic arch of more than 40% (median reduction 71%, range 43%–79%) and with increased z-scores of the DAo. Vorticity in the DAo of patients with DAo dilatation (median: −24 s−1, range: −26 s−1 - −8 s−1) was significantly increased in magnitude compared to controls (median: 0 s−1, range: −2 – +2) (p Conclusions The findings of signficiantly increased vorticity and its association with increased DAo z-scores can potentially explain vascular alterations in the DAo of HLHS patients. This study gives motivation for further investigations and may ultimately lead to future Norwood procedure modifications.

OBJECTIVES Since 1996, our centre performs restrictive enlargement of the pulmonary annulus at surgical repair of tetralogy of Fallot. A transannular patch is only used if the z-score of the pulmonary annulus is smaller than -2. We sought to determine whether this strategy reduces pulmonary insufficiency (PI) and reoperation rate compared to a nationwide contemporary cohort that has not been operated using a uniform strategy. METHODS Eighty-seven tetralogy of Fallot patients were included in the study (Group 1). One hundred sixty-seven tetralogy of Fallot patients from the Competence Network for Congenital Heart Disease served as controls (Group 2). Clinical, echocardiographic, electrocardiogram, cardiovascular magnetic resonance and outcome data were analysed. RESULTS Follow-up time since repair was not different between groups [12.9 (7.8-18.8) vs 13.1 (5.2-16.9) years, P = 0.96] while transannular patch rate was significantly lower in Group 1 (32.2% vs 64.7%, P

Background Neonates undergoing surgery for complex congenital heart disease are at risk of developmental impairment. Hypoxic–ischemic brain injury might be a contributing factor. We aimed to investigate the perioperative release of the astrocyte cytoskeleton component glial fibrillary acid protein and its relation to cerebral oxygenation. Methods Serum glial fibrillary acid protein levels were measured before and 0, 12, 24, and 48 hours after surgery. Reference values were based on preoperative samples; concentrations above the 95th percentile were defined as elevated. Cerebral oxygenation was derived by near-infrared spectroscopy. Results Thirty-six neonates undergoing 38 surgeries utilizing cardiopulmonary bypass were enrolled (complete data available for 35 procedures). Glial fibrillary acid protein was elevated after 18 surgeries (arterial switch: 7/12; Norwood: 5/15; others: 6/8; p = 0.144). Age at surgery was higher in cases with elevated serum levels (6 [4–7] vs. 4 [2–5] days, p = 0.009) and intraoperative cerebral oxygen saturation was lower (70 ± 10% vs. 77 ± 7%, p = 0.029). In cases with elevated postoperative glial fibrillary acid protein, preoperative cerebral oxygen saturation was lower for neonates undergoing the arterial switch operation (55 ± 9% vs. 64 ± 4%, p = 0.048) and age at surgery was higher for neonates with a Norwood procedure (7 [6–8] vs. 5 [4–6] days, p = 0.028). Conclusions Glial fibrillary acid protein was elevated after ∼50% of neonatal cardiac surgeries and was related to cerebral oxygenation and older age at surgery. The potential value as a biomarker for cerebral injury after neonatal cardiac surgery warrants further investigation; in particular, the association with neurodevelopmental outcome needs to be determined.

OBJECTIVES The right ventricular outflow tract reconstruction is a common necessity in congenital cardiac surgery. As homograft availability is limited, alternatives need to be evaluated. The Labcor® conduit consists of a porcine tricomposite valve assembled inside a bovine pericardium tube. This study presents intermediate-term results for its utilization for right ventricular outflow tract reconstruction. METHODS Labcor conduits were implanted in 53 patients (February 2009–July 2016). We analysed perioperative data, freedom from conduit failure and risk factors for conduit dysfunction. RESULTS The most common diagnosis was Tetralogy of Fallot (n = 20, 37.7%). The median age at surgery was 10.0 [interquartile range (IQR) 4.9–14.3] years. Pulmonary artery plasty (n = 37, 69.8%) and augmentation of the right ventricular outflow tract (n = 16, 30.2%) were often part of the procedure. The median conduit size was 21 (range 11–25) mm. There was no in-hospital death. The median follow-up after surgery was 4.6 (IQR 3.4–5.6) years. Fourteen patients (27.5%) developed conduit failure with stenosis being the main cause. Freedom from conduit failure was 98.0% at 2 and 80.5% at 5 years. The median longevity of the conduit was 7.4 years (95% confidence interval 5.1–9.8 years). Younger age and smaller conduit size were related to conduit failure. CONCLUSIONS Utilization of the Labcor conduit revealed acceptable intermediate-term results. The conduit appeared to be functioning sufficiently well within the first 5 years in the majority of patients. The higher rate of failure concerning smaller conduits might be associated with somatic outgrowth; however, conduit degeneration as common and long-term outcome still needs to be evaluated.

Objectives Patients with hypoplastic left heart syndrome are at risk for neurodevelopmental impairment. Hypoxic-ischemic brain injury during neonatal treatment might be a relevant cause. We evaluated the association between cerebral oxygenation in the perioperative course of the Norwood procedure and neurodevelopmental outcome. Methods Cerebral tissue oxygen saturation (ScO 2 ) was obtained by near-infrared spectroscopy for 24 hours before and 48 hours after surgery in 43 patients. Full-scale, verbal, and performance IQ scores were evaluated with the Wechsler Preschool and Primary Scale of Intelligence at a median of 4.5 years (range, 3.5-6.8 years). Cognitive functions were assessed with the German Kognitiver Entwicklungstest fur das Kindergartenalter (KET-KID). Results Mean IQ scores and KET-KID percentile ranks were in the lower-normal range (full-scale IQ, 94 ± 11; verbal IQ, 97 ± 13; performance IQ, 93 ± 9; KET-KID global, 42 ± 27; verbal, 48 ± 29; nonverbal, 37 ± 23). Scores were below average (full scale IQ 2 was lower in patients scoring below average (56.8% ± 7.1% vs 61.7% ± 5.8%; P = .028) and was correlated with full-scale IQ ( r = 0.495; P = .001), verbal IQ ( r = 0.524; P = .001), and performance IQ ( r = 0.386; P = .012) scores, and with global ( r = 0.360; P = .018) and verbal ( r = 0.395, P = .009) KET-KID scores. A relationship between IQ or KET-KID scores and postoperative ScO 2 was not found. Gestational age, head circumference z -score, age at surgery, and postoperative length of stay were associated with IQ and KET-KID scores. Conclusions Neurodevelopmental outcome was in the lower-normal range. Along with innate patient factors, preoperative cerebral tissue oxygenation is likely an important determinant of cognitive development.

We report a rare association of common arterial trunk with left pulmonary artery sling and highlight the importance of cross-sectional imaging in complex congenital cardiac lesions. The patient was antenatally diagnosed with common arterial trunk and underwent surgical repair in the neonatal period. At the age of 20 months, the patient presented with respiratory symptoms and increased right ventricular pressure. Multislice computed tomography demonstrated a pulmonary sling with compression of the distal trachea. Surgical correction of the pulmonary sling and change of the right ventricular to pulmonary artery conduit to a bigger size was performed.

The Fontan procedure provides relief from cyanosis in patients with univentricular hearts. A major clinical unmet need is to understand whether the venous flow patterns of the Fontan circulation lead to the development of congestive hepatopathy and other life-threatening complications. Currently, there is no consensus on whether heart beat or respiration is the main driving force of venous return and which one affects the periodic flow changes for the most (i. e., pulsatility). The present study, for the first time, quantified respiratory and cardiac components of the venous flow in the inferior vena cava (IVC) of 14 Fontan patients and 11 normal controls using a novel approach (“physio-matrix”). We found that in contrast to the normal controls, respiration in Fontan patients had a significant effect on venous flow pulsatility, and the ratio of respiration-dependent to the cardiac-dependent pulsatility was positively associated with the retrograde flow. Nevertheless, the main driving force of net IVC flow was the heart beat and not respiration. The separate analysis of the effects of respiration and heart beat provides new insights into the abnormal venous return patterns that may be responsible for adverse effects on liver and bowel of the patients with Fontan circulation.

Background Cardiac disease modelling using human-induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CM) requires thorough insight into cardiac cell type differentiation processes. However, current methods to discriminate different cardiac cell types are mostly time-consuming, are costly and often provide imprecise phenotypic evaluation. DNA methylation plays a critical role during early heart development and cardiac cellular specification. We therefore investigated the DNA methylation pattern in different cardiac tissues to identify CpG loci for further cardiac cell type characterization. Results An array-based genome-wide DNA methylation analysis using Illumina Infinium HumanMethylation450 BeadChips led to the identification of 168 differentially methylated CpG loci in atrial and ventricular human heart tissue samples (n = 49) from different patients with congenital heart defects (CHD). Systematic evaluation of atrial-ventricular DNA methylation pattern in cardiac tissues in an independent sample cohort of non-failing donor hearts and cardiac patients using bisulfite pyrosequencing helped us to define a subset of 16 differentially methylated CpG loci enabling precise characterization of human atrial and ventricular cardiac tissue samples. This defined set of reproducible cardiac tissue-specific DNA methylation sites allowed us to consistently detect the cellular identity of hiPSC-CM subtypes. Conclusion Testing DNA methylation of only a small set of defined CpG sites thus makes it possible to distinguish atrial and ventricular cardiac tissues and cardiac atrial and ventricular subtypes of hiPSC-CMs. This method represents a rapid and reliable system for phenotypic characterization of in vitro-generated cardiomyocytes and opens new opportunities for cardiovascular research and patient-specific therapy. Electronic supplementary material The online version of this article (10.1186/s13148-019-0679-0) contains supplementary material, which is available to authorized users.

Author(s): Rickers, Carsten; Kheradvar, Arash; Sievers, Hans-Hinrich; Falahatpisheh, Ahmad; Wegner, Philip; Gabbert, Dominik; Jerosch-Herold, Michael; Hart, Chris; Voges, Inga; Putman, Leon M; Kristo, Ines; Fischer, Gunther; Scheewe, Jens; Kramer, Hans-Heiner | Abstract: ObjectivesTo compare the Lecompte technique and the spiral anastomosis (complete anatomic correction) two decades after arterial switch operation (ASO).MethodsNine patients after primary ASO with Lecompte and 6 selected patients after spiral anastomosis were evaluated 20.8 ± 2.1 years after ASO versus matched controls. Blood flow dynamics and flow profiles (e.g. vorticity, helicity) in the great arteries were quantified from time-resolved 3D magnetic resonance imaging (MRI) phase contrast flow measurements (4D flow MR) in addition to a comprehensive anatomical and functional cardiovascular MRI analysis.ResultsCompared with spiral reconstruction, patients with Lecompte showed more vortex formation, supranatural helical blood flow (relative helicity in aorta: 0.036 vs 0.089; P l 0.01), a reduced indexed cross-sectional area of the left pulmonary artery (155 vs 85 mm²/m²; P l 0.001) and more semilunar valve dysfunctions (n = 5 vs 1). There was no difference in elastic aortic wall properties, ventricular function, myocardial perfusion and myocardial fibrosis between the two groups. Cross-sectional area of the aortic sinus was larger in patients than in controls (669 vs 411 mm²/m²; P l 0.01). In the spiral group, the pulmonary root was rotated after ASO more towards the normal left position (P l 0.01).ConclusionsIn this study, selected patients with spiral anastomoses showed, two decades after ASO, better physiologically adapted blood flow dynamics, and attained a closer to normal anatomical position of their great arteries, as well as less valve dysfunction. Considering the limitations related to the small number of patients and the novel MRI imaging techniques, these data may provoke reconsidering the optimal surgical approaches to transposition of the great arteries repair.

Objectives Somatic development is impaired in children with congenital heart defects (CHDs), and head circumference seems to be a strong predictor of neurodevelopmental prognosis. The aim of this study was to generate up-to-date reference values for the somatic development (head circumference, body weight, and length/height) of children with CHDs. Study design Our study population consisted of all patients included in the PAN study (Pravalenz angeborener Herzfehler bei Neugeborenen in Deutschland), which was conducted prospectively over a 3-year study period by the Competence Network for Congenital Heart Defects. All children with mild, moderate, and severe CHDs born in 2006-2009 in Germany were enrolled. For computing of z-scores, only children with the following characteristics were included: appropriate for gestational age, nonsyndromic disease, term or post-term delivery, and no cardiac surgery. Results There were 2818 patients included. New z-scores for the described somatic measures of children with mild, moderate, and severe CHDs were computed. Comparisons with the KiGGS study (Gesundheit von Kindern und Jugendlichen in Deutschland) and the Berlin Longitudinal Study revealed significantly lower measurements for all measures—most notably in children with severe CHDs and/or cardiac surgery. In our cohort, no catch-up growth was seen after cardiac surgery. Conclusion Children with severe CHDs demonstrated the most abnormal pattern in growth, including head circumference before and after cardiac surgery, which is indicative of accompanying brain pathology unrelated to operative injury.

Background Optimal pulmonary perfusion is crucial for a well-functioning Fontan circulation in patients with hypoplastic left heart syndrome (HLHS). To obtain an adequate size of the left pulmonary artery (LPA), patch enlargement is a routine part of the hemi-Fontan procedure in our center. However, LPA patch enlargement at the time of the modified Norwood procedure may have surgical advantages. Therefore, the aim of this study was to evaluate whether anatomic and functional effects of the new approach are superior. Methods A total of 51 consecutive HLHS patients underwent a cardiovascular magnetic resonance imaging study including assessment of LPA anatomy and lung perfusion. The LPA of 20 patients was enlarged during the modified Norwood procedure (group N) and of 31 patients during the hemi-Fontan procedure (group HF). Results The median indexed cross-sectional area of the LPA in group N was significantly higher than in group HF (49.5 versus 27.9 mm2/m2, p Conclusions These first magnetic resonance imaging data show that HLHS patients after LPA patch enlargement during the modified Norwood procedure have significantly higher LPA cross-sectional areas and show improved lung perfusion and shorter overall procedure time as compared with LPA patching during second stage (hemi-Fontan). Therefore, this promising surgical technique may improve blood flow dynamics of the Fontan circulation in the long run.

The Fontan procedure provides relief from cyanosis in patients with univentricular hearts. A major clinical unmet need is to understand whether the venous flow patterns of the Fontan circulation lead to the development of congestive hepatopathy and other life-threatening complications. Currently, there is no consensus on whether heart beat or respiration is the main driving force of venous return and which one affects the periodic flow changes for the most (i. e., pulsatility). The present study, for the first time, quantified respiratory and cardiac components of the venous flow in the inferior vena cava (IVC) of 14 Fontan patients and 11 normal controls using a novel approach ("physio-matrix"). We found that in contrast to the normal controls, respiration in Fontan patients had a significant effect on venous flow pulsatility, and the ratio of respiration-dependent to the cardiac-dependent pulsatility was positively associated with the retrograde flow. Nevertheless, the main driving force of net IVC flow was the heart beat and not respiration. The separate analysis of the effects of respiration and heart beat provides new insights into the abnormal venous return patterns that may be responsible for adverse effects on liver and bowel of the patients with Fontan circulation.

Background An intrapericardial bronchogenic cyst (IBC) is a rare congenital malformation seemingly asymptomatic in most subjects. Case Description A 9-year-old boy presented with persistent chest pain and fever. Imaging revealed a large pericardial effusion with a tumor located at the left atrial appendage extending behind the great vessels. Mass rupture with scattered mucoid debris was found intra-operatively. The tumor was excised and the IBC was confirmed by immunohistochemistry. A postcardiotomy syndrome occurred four weeks post-operative. It was treated conservatively. Since then, follow-up visits have remained uneventful. Conclusion IBCs are a scarce, but serious cause of persistent inflammation in children.

BACKGROUND: The size of the remnant left ventricle (LV) may influence right ventricular function and thus long-term outcome in palliated hypoplastic left heart syndrome (HLHS). We therefore sought to assess the impact of the size of the hypoplastic LV on intrinsic RV function in HLHS patients after Fontan surgery. METHODS: Fifty-seven HLHS patients were studied 2.5 (range: 0.8-12.6) years after Fontan-type palliation with the pressure-volume conductance system. The patient cohort was divided into two groups according to the median LV area index (group 1: LV area index≤1.33cm(2)/m(2), n=29; group 2: LV area index>1.33cm(2)/m(2), n=28). RESULTS: The slopes of the end systolic elastance (Ees) and the preload recruitable stroke work relation (Mw) were not different between group 1 and 2 (Ees: 2.70±1.92 vs. 3.68±2.68mmHg/ml; Mw: 52.75±14.98 vs. 51.09±16.63mmHg x ml; P=NS for all). Furthermore, the systolic responses to dobutamine were not statistically different between groups. However, the slope of the end diastolic stiffness (Eed) was higher in group 2 and catecholaminergic stimulation resulted in a decrease in Eed in group 2 (group 1: 0.40±0.26 vs. 0.52±0.45; group 2: 0.68±0.44 vs. 0.47±0.38mmHg/ml, P

Background— The elastic function of the aorta in patients with transposition of the great arteries after arterial switch operation (ASO) is suspected to be important for long-term prognosis. Methods and Results— Fifty-one patients and 34 controls were studied at 3.0 Tesla with MRI. Forty-three patients (12.8±6.9 years) underwent 1-stage ASO, 8 patients (23.8±6.9 years) had prior pulmonary artery banding (2-stage ASO). Aortic dimensions, distensibility, pulse wave velocity, aortic arch angle, left ventricular (LV) mass, LV systolic function and left atrial (LA) volumes, and LA passive emptying function as marker of LV diastolic function were assessed. Compared with controls, patients had increased aortic root areas (602.6±240.5 versus 356.8±113.4 mm 2 /m; P −3 mm Hg −1 ; P P P P =0.04 to P =0.02). LA volumes correlated negatively with aortic root and ascending aortic distensibility and positively with pulse wave velocity ( P P P =0.004). Conclusions— Reduced aortic bioelasticity and aortic root dilatation are present in transposition of the great artery patients post ASO and are likely to contribute to LV diastolic dysfunction. Impaired aortic bioelasticity was strongly associated with age, suggesting the usefulness of follow-up studies for early onset of degenerative cardiovascular disease.

OBJECTIVES Complex neonatal surgery is considered a risk factor for neuro-developmental impairment in single-ventricle patients. Neuro-developmental outcome was compared between preschool-aged Fontan patients who underwent a Norwood procedure and single-ventricle patients not requiring neonatal surgery with cardiopulmonary bypass. METHODS Verbal, performance and full-scale intelligence quotient (IQ) were evaluated with the Wechsler Preschool and Primary Scale of Intelligence. Cognitive functions were assessed with the German 'Kognitiver Entwicklungstest fur das Kindergartenalter' (KET-KID). Risk factors for impaired neuro-development were evaluated. RESULTS Neuro-developmental assessment was completed in 95 patients (Norwood: n = 69; non-Norwood: n = 26). Median (interquartile range) IQ and KET-KID scores were in the normal range. Except for verbal KET-KID, scores did not differ between Norwood and non-Norwood patients (verbal IQ: 98 (86-105) vs 93 (85-102), P = 0.312; performance IQ: 91 (86-100) vs 96 (86-100), P = 0.932; full-scale IQ: 93 (86-101) vs 89 (84-98), P = 0.314; KET-KID verbal: 48 (17-72) vs 25 (2-54), P = 0.020; KET-KID non-verbal: 33 (18-62) vs 45 (15-54), P = 0.771; KET-KID global: 42 (14-65) vs 28 (6-63), P = 0.208). Full-scale IQ was below average (

Transposition of the great arteries (TGA) is caused by discordance between the great arteries andthe ventricles. If left untreated, this anomaly has a disastrous perspective. More recent surgical approach for correction includes the Lecompte technique in which the pulmonary bifurcation is transposed anterior to the aorta, which may be less physiologic. Although the early results are excellent, there is potential for future problems involving the great arteries and semilunar valves. These potential problems necessitate the development of other improved surgical techniques . Here we report an MRI 4D flow study related to a case of simple TGA whose primary surgical correction – direct spiral arterial switch operation (DSASO) – was performed twenty years ago in an attempt to restore physiologic arrangement among the great arteries and semilunar valves.

OBJECTIVES: Near-infrared spectroscopy (NIRS) offers continuous non-invasive monitoring of regional tissue oxygenation. We evaluated NIRS monitoring during the postoperative course after superior cavopulmonary anastomosis in patients with hypoplastic left heart syndrome and anatomically related malformations. METHODS: Cerebral (cSO2) and somatic (sSO2) tissue oxygenations were recorded for 48 h and compared with routine measures of intensive care monitoring. Changes in parameters in the case of postoperative complications were evaluated. RESULTS: Data were obtained from 32 patients. Median age at operation was 2.9 (1.5–10.0) months and weight was 5.3 ± 1.0 kg. Postoperative complications occurred in 7 patients ( pulmonary artery thrombus n = 4, pneumothorax n = 1, cardiopulmonary resuscitation n = 1 and low-cardiac output n = 1). cSO2 was 44 ± 14% at the end of the operation and reached its minimum of 40 ± 11% 2 h later (P = 0.018). Overall, cSO2 was depressed early after surgery and increased from a mean of 42 ± 11% during the first 4 postoperative hours to 57 ± 8% in the last 4 h of the study period (P < 0.001). The sSO2 decreased from 77 ± 11% during the early postoperative course to 68 ± 9% within the later course (P < 0.001). The cSO2 correlated with the arterial partial pressure of oxygen ( pO2, r = 0.364, P < 0.001), with the arterial oxygen saturation (SaO2, r = 0.547, P < 0.001) and with the central venous oxygen saturation providing the strongest correlation (SvO2, r = 0.686, P < 0.001). Analysis of agreement between cSO2 and SvO2 measurements revealed a mean bias of 0.97 with limits of agreement between 19.8 and �17.9%. Inclusion of both cSO2 and sSO2 into a linear regression model slightly improved the prediction of SvO2 from NIRS values (r = 0.706, P < 0.001). The mean values of cSO2, sSO2, SaO2 and SvO2 during the early postoperative period were lower in patients with complications (cSO2: 45 ± 9 vs 29 ± 5%, P < 0.001; sSO2: 80 ± 11 vs 70 ± 6%, P = 0.004; SaO2: 76 ± 8 vs 66 ± 6%, P = 0.004; SvO2: 48 ± 14 vs 32 ± 6%, P < 0.001). CONCLUSIONS: NIRS technology allows inferring the global oxygenation from continuous non-invasive measurements of regional tissue oxygenation. The cSO2 is lowered in the early postoperative course. Lower cSO2 values in the early postoperative course may be predictive of postoperative complications.

Background. Cardiac catheterization and interventional procedures are an important part of staged surgical palliation for hypoplastic left heart syndrome (HLHS). Methods. We reviewed our experience of interventional procedures for HLHS patients treated between 01/1996 and 12/2010. Results. Overall, 222 neonates received a Norwood operation. Of them, 181 underwent 554 catheterizations with 243 interventions. Recoarctation was treated by balloon angioplasty (BA) in 46 patients. The pressure gradient dropped from 29 ± 14 mm Hg to 6 ± 7 mm Hg (P

Please cite this paper as: Bierbach B, Scheewe J, Derfuss T, Krug A, Schramm R, Dahm M, Kuroczynski W, Kempski O, Horstick G. Continuous regional myocardial blood flow measurement: validation of a near-infrared laser Doppler device in a porcine model. Microcirculation 19: 485–493, 2012. Abstract Objective: RMBF measurement is a major concern in various clinical and experimental settings, but no validated device for RMBF is currently available. Methods: An LVP-triggered laser Doppler to measure RMBF was validated by simultaneous fluorescent MS RMBF in a porcine LAD flow reduction model (n = 10 pigs). The laser probe was positioned on the left ventricle’s anterior wall. LAD blood flow reduction was achieved by a shaft-driven occluder positioned proximal to the transit-time flow meter measuring coronary blood flow. RMBF was measured at baseline; after the reduction of LAD blood flow to 70% and 30% of baseline; at 20 and 120 minutes of reperfusion; and, finally, 15 minutes after LAD occlusion. Results: Laser Doppler RMBF (LDU) correlated strongly with MS RMBF under all tested conditions: baseline (epicardial 194.7 ± 41.9, endocardial 130.2 ± 29.2); 70% baseline-flow (epicardial 160.4 ± 27.7, endocardial 112.1 ± 15.1); 30% baseline-flow (epicardial 44.3 ± 5.5, endocardial 32.9 ± 9); 20 minutes reperfusion (epicardial 175.8 ± 33.6, endocardial 126.5 ± 30); 120 minutes reperfusion (epicardial 146.3 ± 31.1, endocardial 107.1 ± 29.7); and complete LAD occlusion (epicardial 10.5 ± 5.8 endocardial 1.4 ± 0.3) (r = 0.986–0.962, p

Objective. Two-dimensional speckle tracking can assess ventricular deformation independent of ventricular geometry. Using this technique, the aim of our study was therefore to compare global and regional longitudinal deformation and intraventricular dyssynchrony between children with left and right ventricular morphology after Fontan operation. Design and Patients. In this retrospective study, we examined 29 children with systemic right (group 1: age 7.7 ± 2.7 years, time after Fontan 5.3 ± 3.0 years) and 22 children with systemic left (group 2: age 7.8 ± 4.8 years, time after Fontan 4.6 ± 4.2 years) ventricles after Fontan surgery using two-dimensional speckle tracking. We compared global and regional longitudinal strain and strain rate (SR) as well as time to peak strain in basal lateral and septal segments. Results. Global strain (−18.5 ± 3.5 vs. −17.9 ± 3.2%, P= NS) and global SR (−1.0 ± 0.2 vs. −1.0 ± 0.2/s, P= NS) did not differ between groups. Regional strain (−8.7 ± 8.6 vs. −14.7 ± 6.7%, P= .008) and SR (−0.7 ± 0.4 vs. −1.0 ± 0.3%, P= .002) in the basal septal segment were lower in group 1, while regional strain was higher in group 1 in the apical septal segment (−23.5 ± 8.0 vs. −18.4 ± 5.9%, P= .02). Time to peak strain was higher in the basal septal segment in group 1 (410 ± 78 vs. 338 ± 90 ms, P= .004) but not different in the basal lateral segment. Conclusions. Despite minor regional differences in longitudinal deformation and dyssynchrony, overall ventricular longitudinal deformation was not different between morphologic right and left ventricles. These findings may reflect similar adaptation of longitudinal function of both ventricular morphologies to the single-ventricle circulation in our cohort, albeit relatively early after Fontan surgery.

The purpose of this study was to investigate changes in right ventricular (RV) function and deformation parameters before and at steady state after the Norwood operation in neonates with hypoplastic left heart syndrome. A further aim was to delineate factors that affected these changes.On echocardiograms before and 21 days (range, 10-35 days) after the Norwood operation, the two-dimensional speckle-tracking parameters global and regional peak systolic longitudinal strain and strain rate were retrospectively compared in 33 patients with hypoplastic left heart syndrome. In addition, RV functional assessment included RV fractional area change and tricuspid annular plane systolic excursion. The associations between postoperative echocardiographic findings and preoperative or postoperative complications, prenatal diagnosis, postoperative heart rate, oxygen saturation, and medication use as well as cardiopulmonary bypass and aortic cross-clamp times were tested.Global strain (-18.3 ± 3.6% vs -16.8 ± 3.8%, P = .02) and global strain rate (-1.6 ± 0.3 vs -1.2 ± 0.3 sec(-1), P.0001) decreased significantly. Regional strain decreased significantly in the apical and mid lateral segments, while regional strain rate decreased significantly in all but the basal septal segments. Tricuspid annular plane systolic excursion of the lateral annulus decreased significantly, while RV fractional area change remained the same. No significant associations were found between postoperative RV function and potential impact factors.Two-dimensional global and regional longitudinal strain and strain rate as well as tricuspid annular plane systolic excursion were reduced in patients with hypoplastic left heart syndrome after the Norwood operation. None of the examined preoperative and postoperative patient or surgical factors was found to explain this decrease.

ObjectiveSignificant depression in cerebral oxygen saturation has been observed in patients with hypoplastic left heart syndrome (HLHS) undergoing Norwood operations. We monitored cerebral oxygen saturation with near-infrared spectroscopy before and after this procedure. Patients with transposition of great arteries (TGA) before and after arterial switch operation were also studied to elucidate whether post–cardiopulmonary bypass (CPB) changes in cerebral oxygen saturation are related to CPB or hemodynamic alterations inherent in single-ventricle physiology.MethodsWe monitored 33 patients with HLHS and 20 with TGA 24 hours before and 48 hours after CPB. In addition to cerebral oxygen saturation, routine measurements of oxygen transport and delivery were performed.ResultsPreoperatively, cerebral oxygen saturation was higher in patients with HLHS than with TGA (61% ± 7% vs 56% ± 8%, P = .04). After CPB, cerebral oxygen saturation was markedly depressed in both groups but increased toward end of monitoring (HLHS vs TGA minimal value 42% ± 12% vs 54% ± 11%, P

We report on the rare case of prenatally detected Taussig-Bing anomaly complicated by a coarctation of the aorta and a singular coronary artery in an otherwise healthy boy. After initially successful arterial switch, a high-grade stenosis of the singular coronary artery leads to a severe biventricular heart failure 5 weeks after the procedure. Although immediate surgical intervention was carried out, the boy died due to already severely impaired myocardial function. This review discusses the perinatal management, typical diagnostic features, and frequent additional anomalies, as well as surgical strategies in complex Taussig-Bing anomaly.

BackgroundThe left ventricle in patients with hypoplastic left heart syndrome may influence right ventricular function and outcome. We aimed to investigate differences in right ventricular deformation and intraventricular dyssynchrony between hypoplastic left heart syndrome patients with different anatomical subtypes and left ventricle sizes after Fontan surgery using two-dimensional speckle tracking.Patients and methodsWe examined 29 hypoplastic left heart syndrome patients aged 5.4 plus or minus 2.8 years after Fontan surgery and compared 15 patients with mitral and aortic atresia with the remaining 14 patients with other anatomic subtypes. We used two-dimensional speckle tracking to measure the global and regional systolic longitudinal strain and strain rate as well as intraventricular dyssynchrony.ResultsGlobal strain (−19.5, 2.8% versus −17.4, 3.9%) and global strain rate (−1.0, 0.2 per second versus −0.9, 0.3 per second) were not different between groups. The mitral and aortic atresia group had higher strain in the basal septal (−13.0, 5.0% versus −3.9, 9.3%, p = 0.003) and mid-septal (−19.4, 4.7% versus −13.2, 6.5%, p = 0.009) segments, and higher strain rates in the mid-septal segment (−1.14, 0.3 per second versus −0.95, 0.4 per second, p = 0.047), smaller left ventricle area (0.18, 0.41 square centimetre versus 2.83, 2.07 square centimetre, p = 0.0001), and shorter wall-to-wall delay (38, 29 milliseconds versus 81, 57 milliseconds, p = 0.02).ConclusionSignificant differences in regional deformation and intraventricular dyssynchrony exist between the mitral and aortic atresia subtype with small left ventricles and the other anatomic subtypes with larger left ventricles after Fontan surgery.

Objective: Outcome of staged palliation for hypoplastic left heart syndrome has improved over the past decades. However, only little is known about the exercise capacity of children with palliated hypoplastic left heart syndrome where a systemic right ventricle supports the systemic circulation. The aim of the study was to assess exercise capacity in a contemporary cohort of children with hypoplastic left heart syndrome palliated in a single centre according to a uniform surgical strategy. Methods: Standardised cardiopulmonary exercise testing on a treadmill was performed in 46 consecutive hypoplastic left heart patients (median age: 6.0 (4.1—11.4) years). All but one patient reached the anaerobic threshold. Exercise data were compared to normal values obtained with a similar exercise protocol in a large cohort of paediatric volunteers. Results: Oxygen uptake at anaerobic threshold (26.9 6.0 ml kg 1 min 1 ; 74.5 18.2% of predicted) and maximal oxygen uptake (31.0 6.8 ml kg 1 min 1 ; 60.8 15.0% of predicted) were significantly reduced compared with controls (P < 0.0001 for both). The limitation in exercise capacity was due to an impaired rise in heart rate (158 23 bpm; 79.7 11.5% of predicted; P < 0.0001) and oxygen pulse (4.5 1.6 ml per beat; 85.5 22.0% of predicted; P < 0.0001). Furthermore, respiration during exercise was inefficient with an elevated respiratory rate and reduced maximal tidal volume and minute ventilation at maximal exercise. Conclusions: The exercise capacity of children with hypoplastic left heart syndrome is markedly reduced. Limitations in heart rate increase and stroke volume augmentation are the major contributors to this. An abnormal ventilatory response to exercise also adds to their limitation in exercise tolerance. However, the degree of physical disability does not justify discouraging these patients from school and leisure sports. # 2009 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Objectives: Retrospective data suggest that a wide pulmonary annulus after Fallot repair aggravates pulmonary regurgitation. Therefore, since 1997, in our institution transannular patch enlargement was only intended for patients with a native pulmonary annulus z-score less than -4. If transannular patching was needed, enlargement was aimed to diameters within the range of a z-score of -2. We sought to determine whether this strategy of restrictive enlargement of the pulmonary annulus was adequate to reduce transannular patch rate and to limit pulmonary annulus width without increased right ventricular pressure load. Methods: Two-hundred-and-sixteen Fallot patients were retrospectively analysed. Ninety-eight patients underwent repair between 1997 and 2006 adhering to our uniform strategy (Group 1). One hundred and eighteen patients were operated between 1977 and 1996 without a uniform strategy (Group 2). Transannular patch rate, native and postoperative pulmonary annulus z-score, postoperative right ventricular outflow tract velocity on echocardiography and early reoperation rate for right ventricular outflow tract obstruction were analysed in both groups. Results: Compared to Group 2, patients in Group 1 were younger at repair, transannular patch rate was significantly reduced (32 vs 68%, p < 0.0001) and postoperative pulmonary annulus diameters were smaller (z-score -2.1 ± 1.5 vs 0.0 ± 3.1, p < 0.0001). However, no difference in right ventricular outflow tract velocity (2.4 ± 0.8 vs 2.2 ± 0.8 m/s; p = NS) or the incidence of early reoperation for right ventricular outflow tract obstruction was found between the groups (3/98 vs 1/118; p = NS). Conclusion: Restrictive enlargement of the pulmonary annulus at Fallot repair lowers transannular patch rate, limits the postoperative width of the pulmonary annulus but does not result in increased right ventricular pressure load or reoperation rate for residual right ventricular outflow tract obstruction. A limitation of postoperative pulmonary regurgitation can be expected when the extent of pulmonary annulus enlargement at repair is limited.

Anesthesiologists involved in the care of children with congenital heart disease (CHD) have to understand the pathophysiology of each cardiac lesion and anticipate the impact of the planned procedure. Often the dimished margin of hemodynamic stability determines the guidelines of care. In children with CHD maintainance of the homeostasis during anesthesia for a wide variety of procedures is the anesthesiologist's challenge. Left-to-right shunting means a volume load to the left ventricle frequently combined with pulmonary hypertension. Right-to-left shunting leads to a central cyanosis.

Objective: The objective of the study was to estimate the capacity of pressure volume (PV) loop analysis to assess right ventricular (RV) function after Fallot (TOF) repair.Patients: Fifty six patients were examined after TOF repair. PV loops were constructed from RV angiocardiography and simultaneous pressure measurement. Patients were divided in three groups according to RV size and pressure (Group I: normal RV size and pressure; group II: enlarged RV, near normal pressure; group III: normal RV size, elevated pressure).Main outcome measures: Systolic stroke work corrected for body surface area (W/BSA) and for RV enddiastolic volume (W/EDV), peak RV filling (PFR) and emptying rates (PER) corrected for RV stroke volume, cycle efficiency (CE), RV ejection fraction (RVEF).Results: W/BSA was significantly higher in group II than in group I (0.19 ± 0.05 vs. 0.11 ± 0.04 J/m2, p < 0.001) and was similar between groups II and III (0.19 ± 0.05 vs. 0.17 ± 0.05 J/m2 ; NS). W/EDV was similar in groups I and II (12.4 ± 5.4 vs. 12.4 ± 2.9 mmHg; NS). CE was smallest in group II. The difference was significant between groups II and III (0.62 ± 0.08 vs. 0.73 ± 0.09; p < 0.05). RVEF was negatively correlated to RV end systolic volume (RVESV) in the patients of groups I and II (r = −0.32, p < 0.05). A similar correlation was found between PFR and RVESV (r = −0.28, p < 0.05).Conclusions: Analysis of a single PV loop allows quantification of RV load after TOF repair. W/BSA is increased to the same extent under volume and pressure load. The lack of decrease in W/EDV in patients with enlarged RV indicates that RV is capable to perform adequate work in a wide range. RVESV is a useful measure for estimating RV function after TOF repair depicting parameters of systolic and diastolic RV function.

Objective Cardiac surgery for complex congenital malformations with use of extracorporeal circulation predisposes to an excessive systemic inflammatory response and a consecutive capillary leak syndrome. In a prospective randomized study the influence of 2 oxygenators especially designed for pediatric use on inflammatory markers and clinical outcome was investigated. Methods Forty neonates and infants (body surface area, 2 ) undergoing cardiac surgery with extracorporeal circulation were randomized into one of 3 groups: in the first group (n = 14) the Medtronic Minimax Oxygenator and in the second group (n = 12) the Dideco Lilliput 1 Oxygenator, both with a 750-mL priming volume, were used. In the third group the Dideco Lilliput 1 Oxygenator was filled with a reduced priming volume of 450 mL. Parameters of interest for evaluation of a systemic inflammatory response after extracorporeal circulation were interleukin 6, tumor necrosis factor α, neutrophil elastase, complement C3, and free hemoglobin. In addition, erythrocyte, leukocyte, and thrombocyte counts and hemoglobin and C-reactive protein values were determined at different measurement points before, during, and after the operation. Results In all 3 groups peak values for tumor necrosis factor α were observed during the operation, whereas interleukin 6, elastase, and free hemoglobin values peaked in the first 4 hours. The highest values for leukocytes and C-reactive protein were obtained between 24 and 72 hours after the operation. Erythrocyte and thrombocyte counts, as well as hemoglobin values, were lowest at extracorporeal circulation onset, normalizing under substitution in the first 4 hours after the operation. By using the Lilliput/750 oxygenator, higher interleukin 6 values 1 and 4 hours after the operation and higher tumor necrosis factor α values during and 1 hour after the operation could be observed compared with results with the Minimax and Lilliput/450 oxygenators. In spite of our randomization protocol, patients in the Lilliput/750 group were significantly smaller and younger than those in the Minimax group. However, the statistical analysis showed no correlation between age and interleukin 6 or tumor necrosis factor α values, but it did show a correlation between younger age and the occurrence of capillary leak syndrome. Accordingly, the number of children with clinically complicated course (capillary leak, longer duration of catecholamine therapy, and ventilation) was higher in the Lilliput/750 group than in the Minimax group. Conclusion By using an adequate priming volume, the systemic inflammatory response is similar after use of the Dideco Lilliput 1 Oxygenator and the Medtronic Minimax Oxygenator. Tip-to-tip surface coating of the extracorporeal circulation with either heparin or phosphorylcholine seems to have similar biologic effects in neonates and infants undergoing cardiac surgery.

Objective: Long-term angiographic evaluation of left ventricular performance and size of the great arteries after one-stage neonatal versus two-stage arterial switch operation (ASO) of simple transposition. Methods: Analysis of cineangiographic studies obtained during the process of two-stage ASO for 34 patients and after neonatal repair for 52 patients. Results: At early follow-up after two-stage ASO the left ventricular enddiastolic volume (LVEDV) was þ 1.8 standard deviations (S.D.) larger than LVEDV of control patients, but normalized completely (0.0 S.D.) at late follow-up. In contrast, after neonatal repair the LVEDV was always normal, and the median EF was significantly higher than after two-stage ASO (73 vs. 68%). The diameters of the native pulmonary annulus and sinus increased significantly after pulmonary artery banding to þ4.5 and þ4.8 S.D., respectively. After ASO, a significant decrease of the respective sizes occurred from early to late follow-up (annulus: þ6.0 to þ2.1 S.D.; sinus: þ 7.1 to þ 4.1 S.D.). After neonatal ASO the neoaortic annulus and sinus were only þ 1.5 and þ 2.7 S.D. larger than the comparable normal structures. The differences to the two-stage group were significant. In both groups, the neoaortic anastomosis had no diameters significantly different from normal. After one- and two-stage repair, the size of the neopulmonary annulus and sinus decreased similarly in both groups from early to late follow-up (annulus þ0.9 to 22.4 S.D.; þ0.3 to 22.8 S.D.; sinus: 2 0.7 to 2 1.6 S.D.; 20.7 to 2 1.8 S.D.). Conclusions: Neonatal ASO has definite advantages over two-stage repair concerning LV-performance and the degree of dilation of the neoaortic root. The significantly reduced size of the neopulmonary root after both procedures is remarkable, but fortunately mostly without clinical significance. q 2003 Elsevier B.V. All rights reserved.

Background: The optimal choice of treatment for aortic valve (AV) pathologies in children is still unclear. Compared with valve replacement, AV reconstruction may provide advantages, especially in cases of isolated valve stenosis. However, as the interplay between anatomical prerequisites and reconstructive techniques is complex, durable valve competence after AV repair remains difficult to predict. Here we present the outcome of patients after AV reconstruction at our department. Methods and Results: We retrospectively reviewed the data of 32 patients that had undergone AV reconstruction between 1993 and 2013. Median age at operation was 8 years (range 11 days-17 years). 12 (38%) patients suffered from isolated AV pathologies, 20 (63%) patients had concomitant cardiac defects. 9 (28%) patients had previously undergone ballon valvuloplasty, 15 (47%) patients had had prior cardiac operations. Combined AV defects were present in 19 (59%) patients, isolated AV stenosis in 4 (13%) and isolated AV insufficiency in 9 (28%) patients. 2 (6%) patients presented with neo-aortic insufficiency after switch procedures. Operation duration was 263 ± 67 minute, bypass time 124 ± 60 minute, cross-clamp time 78 ± 40 minute. 17 (53%) patients underwent isolated AV reconstruction, 15 (47%) patients had combination procedures. Reconstruction techniques involved commissurotomy in 12 (38%) patients, a pericardial patch was used in 20 (63%) of cases. Intra-operative and 30-day mortality was 0%. 1 (3%) patient with congenital aortic stenosis died during follow-up. 5 (16%) patients were in need of a second AV procedure. Median time to re-intervention was 34 months (range 7 days to 7 years). 2 (6%) of these patients had to undergo repeated operation for AV insufficiency, 1 (3%) patient for recurrent AV stenosis. 1 (3%) patient underwent two AV reconstructions with subvalvular resection due to recurrent stenosis. 1 (3%) patient underwent repeated ballon valvuloplasty. Summary and Conclusion: Although the validity of this study is limited by the small number of patients presented here, we show that AV reconstruction in children, including both- mainly insufficient as well as stenotic defects can be performed with an acceptable risk for re-intervention.