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Background: Congenital cardiovascular malformations (CCVM) may cause infrequently airway pathologies (AP) in children and are of prognostic and therapeutic relevance. While computed tomography (CT) is considered first-line imaging modality in many centres, we started using magnetic resonance imaging (MRI) more and more in the last years to detect CCVM and AP to avoid radiation in this patient group. Objective: The aim of this retrospective study was to determine and to compare the diagnostic accuracy of CT and MRI when used to detect CCVM and/or AP. Methods: All patients suspected to have CCVM and/or AP and examined either by CT or MRI between 2000 and 2013 in our hospital were included. Extension and type of CCVM, as well as their relationship to esophagus, trachea or bronchi were assessed and related to findings of tracheobronchoscopy, cardiac catheterization or surgery if available. Results: One hundred six patients (median [range] 4 years [2 days to 66 years]) were examined by CT (n = 27) or MRI (n = 79). In 78 patients (74%), CCVM and/or AP were found with either of the imaging methods. CCVM were found in 63 subjects. Forty-six of 63 subjects had both, CCVM and AP. The presence of CCVM was always detected correctly by CT or MRI, although both techniques had a weakness detecting atretic segments directly. AP (n = 61) were correctly diagnosed in all patients not intubated for artificial ventilation by CT (n = 17) and in all but 2 patients by MRI (39 out of 41). Conclusions: MRI is sensitive to detect CCVM associated with AP equally to CT without any radiation exposure.

Background Vancomycin is an extensively used anti-infective drug in neonatal ICUs. However, exposure–toxicity relationships have not been clearly defined. Objectives To evaluate the risk profile for hearing deficits in vancomycin-exposed very-low-birthweight infants (VLBWI). Methods In a large cohort study of the German Neonatal Network (GNN; n = 16 967 VLBWI) we assessed the association of vancomycin treatment and pathological hearing tests at discharge and at 5 year follow-up. We performed audits on vancomycin exposure, drug levels, dose adjustments and exposure to other ototoxic drugs in a subgroup of 1042 vancomycin-treated VLBWI. Results In the GNN cohort, 28% (n = 4739) were exposed to IV vancomycin therapy. In multivariable logistic regression analysis, vancomycin exposure proved to be independently associated with pathological hearing test at discharge (OR 1.18, 95% CI 1.03–1.34, P = 0.016). Among vancomycin-treated infants, a cumulative vancomycin dose above the upper quartile (>314 mg/kg bodyweight) was associated with pathological hearing test at discharge (OR 2.1, 95% CI 1.21–3.64, P = 0.009), whereas a vancomycin cumulative dose below the upper quartile was associated with a reduced risk of pathological tone audiometry results at 5 years of age (OR 0.29, 95% CI 0.1–0.8, P = 0.02, n = 147). Conclusions Vancomycin exposure in VLBWI is associated with an increased, dose-dependent risk of pathological hearing test results at discharge and at 5 years of age. Prospective studies on long-term hearing impairment are needed.

Background Even after successful aortic coarctation (CoA) repair, hypertension causes premature morbidity and mortality. The mechanisms are not clear. The aim was to evaluate elastic wall properties and aortic morphology and to correlate these results with severity of restenosis, hypertension, aortic arch geometry, noninvasive pressure gradients, and time and kind of surgical procedure. Methods Eighty-nine patients (17 ± 6.3 years) and 20 controls (18 ± 4.9 years) were examined using magnetic resonance imaging (MRI). In addition to contrast-enhanced MR angiography and flow measurements, CINE MRI was performed to assess the relative change of aortic cross-sectional areas at diaphragm level to calculate aortic compliance (C). Results Fifty-four percent of all patients showed hypertension (> 95th percentile), but more than half of them had no significant stenosis (defined as ≥30%). C was lower in CoA than in controls (3.30 ± 2.43 vs. 4.67 ± 2.21 [10–5 Pa–1 m–2]; p = 0.024). Significant differences in compliance were found between hyper- and normotensive patients (2.61 ± 1.60 vs. 4.11 ± 2.95; p = 0.01), and gothic and Romanesque arch geometry (2.64 ± 1.58 vs. 3.78 ± 2.81; p = 0.027). There was a good correlation between C and hypertension (r = 0.671; p Conclusion The decreased compliance, a high rate of hypertension without restenosis, and independency of time and kind of repair confirm the hypothesis that CoA may not be limited to isthmus region but rather be a widespread (systemic) vascular anomaly at least in some of the CoA patients. Therefore, aortic compliance should be assessed in these patients to individually tailor treatment of CoA patients with restenosis and/or hypertension.

ObjectiveTo determine if survival rates of preterm infants receiving active perinatal care improve over time.DesignThe German Neonatal Network is a cohort study of preterm infants with birth weight Setting43 German level III neonatal intensive care units (NICUs).Patients8222 preterm infants with a gestational age between 22/0 and 28/6 weeks who received active perinatal care.InterventionsParticipating NICUs were grouped according to their specific survival rate from 2011 to 2013 to high (percentile >P75), intermediate (P25–P75) and low (Main outcome measuresDeath by any cause before discharge.ResultsTotal survival increased from 85.8% in 2011–2013 to 87.4% in 2014–2016. This increase was due to reduced mortality of NICUs with low survival rates in 2011–2013. Survival increased in these centres from 53% to 64% in the 22–24 weeks strata and from 73% to 84% in the 25–26 weeks strata.ConclusionsOur data support previous reports that active perinatal care of very immature infants improves outcomes at the border of viability and survival rates at higher gestational ages. The high total number of surviving infants below 24 weeks of gestation challenges national recommendations exclusively referring to gestational age as the single criterion for providing active care. However, more data are needed before recommendations for parental counselling should be reconsidered.Trial registrationApproval by the local institutional review board for research in human subjects of the University of Lübeck (file number 08–022) and by the local ethic committees of all participating centres has been given.

Cardiac magnetic resonance imaging (MRI) and computed tomography (CT) have been developed rapidly in the last decade. Technical improvements and broad availability of modern CT and MRI scanners have led to an increasing and regular use of both diagnostic methods in clinical routine. Therefore, this German consensus document has been developed in collaboration by the German Cardiac Society, German Radiology Society, and the German Society for Pediatric Cardiology. It is not oriented on modalities and methods, but rather on disease entities. This consensus document deals with coronary artery disease, cardiomyopathies, arrhythmias, valvular diseases, pericardial diseases and structural changes, as well as with congenital heart defects. For different clinical scenarios both imaging modalities CT and MRI are compared and evaluated in the specific context.

BACKGROUND Ventricular dysfunction represents one of the major problems in the long-term follow-up of patients after atrial repair for dextrotransposition of the great arteries. We aimed to study the role of tissue Doppler derived isovolumic acceleration (IVA) to detect early myocardial dysfunction in these patients. METHODS Twenty-four patients with dextrotransposition of the great arteries (D-TGA) that underwent atrial repair (Senning procedure: n = 12; Mustard procedure: n = 12) in infancy were examined at the age of 21 [12-33] years (median [range]) using tissue Doppler analysis of IVA and peak systolic myocardial velocity at rest and during exercise. 12 age-matched healthy subjects served as controls. RESULTS At rest, IVA and peak systolic myocardial velocity were reduced in the systemic ventricle (SV) of patients. IVA correlated with peak systolic myocardial velocity (r = 0.76, P < 0.001). During exercise, IVA, but not peak systolic myocardial velocity, increased significantly in the SV of patients (rest: 1.03 +/- 0.44 cm/sec(2); 1 W/kg: 1.80 +/- 1.22 cm/sec(2); 2 W/kg: 2.85 +/- 1.26 cm/sec(2)). In the subpulmonary ventricle, IVA was significantly lower in patients compared to the controls (patients: 1.45 +/- 0.49 cm/sec(2) vs. controls: 2.31 +/- 0.43 cm/sec(2), P < 0.05). IVA but not peak systolic myocardial velocity was able to discriminate between patients and healthy subjects. CONCLUSIONS IVA is superior to peak systolic myocardial velocity to assess a reduction in functional reserve of both ventricles in patients after atrial repair for D-TGA.

After childhood Kawasaki syndrome (KS) the coronary arteries undergo a lifelong dynamic pathological change, and follow-up coronary artery imaging is essential. At present, conventional coronary catheterization (CCC) and angiography is still regarded as the gold standard. Less-invasive methods such as multidetector CT angiography (MDCT-A) and MRI have been used sporadically. To compare the diagnostic quality of MDCT-A and MRI with that of CCC for coronary imaging in a group of patients with coronary artery pathology after childhood KS. A total of 16 patients (aged 5–27 years) underwent CCC and 16-row MDCT-A and 14 patients MRI (1.5 T). There was 100% agreement between MDCT-A and CCC in the detection of coronary aneurysms and stenoses. MDCT-A was superior for the visualization of calcified lesions. MRI and CCC showed 93% agreement for the detection of aneurysms. Visualization of coronary artery stenoses was difficult using MRI—one stenosis was missed. MDCT-A has excellent correlation with CCC regarding all changes affecting the coronary arteries in the follow-up of childhood KS. In comparison to MDCT-A and CCC, MRI is less precise in the detection of stenotic lesions. Due to its high image quality and ease of performance MDCT-A should be the primary diagnostic modality in patients following childhood KS.

BACKGROUND: Tracheal stenosis in combination with vascular and/or cardiac anomalies is a life-threatening condition in infants and children presenting with severe symptoms of airway obstruction. The optimal surgical treatment of these cases remains controversial. OBJECTIVES: We present here a group of infants and children with combined tracheal malformations and vascular and/or cardiac anomalies. More than 30 % of the stenotic trachea was resected in a subgroup of the patients. A reconstruction with end-to-end anastomosis was achieved on the basis of extensive mobilization of the whole tracheobronchial tree and use of CPB. METHODS: The clinical outcome in 37 children with a median age of 8 (1 - 72) months was analyzed retrospectively. The patients presented with severe airway obstruction in combination with congenital heart defects and/or vascular anomalies. Cardiac catheterization, bronchoscopy and thoracic computer tomography were performed prior to operation. The operations were performed under CPB and consisted of tracheal resection with end-to-end anastomosis or external stabilization. Associated intracardiac and vascular anomalies were repaired simultaneously. RESULTS: All but 1 patient survived and had a straightforward recovery. The patients were extubated under bronchoscopic control with a median intubation time after airway repair of 12.2 days. The average follow-up was 8.4 years (1 - 14 years) and the surviving patients did not show signs of restenosis clinically. A segment longer than 30 % of the tracheal length was resected and reconstructed with end-to-end anastomosis in 57 % of the patients (12 of 21 patients). CONCLUSIONS: Our experience demonstrates that resection of tracheal stenosis and end-to-end anastomosis can be achieved successfully even in cases with stenosis of more than 30 % of the total tracheal length. The use of CBP allowed extensive mobilization of the tracheobronchial tree and resection with end-to-end tension-free anastomotic reconstruction.

PURPOSE To compare the aortic compliance of the normal-sized aorta of adolescents with Marfan syndrome and healthy controls using MR measurements of the aortic distensibility and pulse wave velocity. MATERIALS AND METHODS Fourteen patients (median age: 15 years) and 11 healthy subjects (23 [12 - 32] years) were examined at 1.5 T. The MR protocol included 2D steady-state free precession (SSFP)-CINE MRI of the aortic distensibility and PC-MRI of the pulse wave velocity. All measurements were positioned perpendicular to the descending aorta at the level of the diaphragm for assessing the changes in the aortic cross-sectional areas and additionally above and below this plane for assessing the pulse wave velocity. In addition contrast-enhanced 3D-MR angiography was performed in adolescents with Marfan syndrome to exclude morphologic changes and to prove normal-sized aorta. RESULTS Compared with control subjects, adolescents with Marfan syndrome had significantly decreased distensibility and significantly increased pulse wave velocity (chi (2)-test, p = 0.0002) using an age-related non-linear regression analysis. The related aortic compliance was significantly decreased (chi (2)-test, p = 0.0002). There was a good correlation between the two methods (r = 0.86). A low intraobserver variability was found for both methods (< or = 2 %). CONCLUSIONS MRI allows detecting abnormal elastic aortic wall properties already in the normal-sized aorta of adolescents with Marfan syndrome. Monitoring of these properties could be relevant for evaluating disease onset and progression. MRI has the potential value of compliance measurements for the follow-up and to guide therapy indications.

The study was done to determine the clinical feasibility and reproducibility of tissue Doppler echocardiography (TDE) with strain rate imaging (SRI) at rest and under physical exercise in healthy young individuals and to report normal values for parameters of regional myocardial function under exercise. Forty-five young volunteers (age 9–29 years) underwent echocardiography with TDE/SRI at rest and during a bicycle exercise test (2 W/kg body weight). Velocities could be obtained in 93% of segments, whereas strain rate was measurable at least in 80% of segments. Inter- and intraobserver variability for measurement of velocities under exercise was 14% and 9%, respectively for strain rate 28% and 20%. Except for peak strain, values for all other parameters were higher during exercise with the clearest response in the left lateral and the right ventricular wall. (1) Tissue Doppler with strain rate imaging is a practical and robust method for assessment of regional function of both ventricles under exercise. (2) Systolic motion, local myocardial relaxation and contractility increased significantly under physical exercise. These normal values obtained from healthy young subjects can serve as a reference database for further clinical studies.

Purpose To assess the value of multidetector CT (MDCT) for evaluation of vascular anomalies (VA) and associated complications in newborns and infants. Materials and methods Seventy-five children (mean age: 9 +/- 6 months, range: 2 weeks to 24 months) with VA were examined using MDCT (4-, 8- or 16-row; collimation 0.5-1.25 mm; scan time 7-30 s), which was performed under controlled ventilation or free breathing. Image quality was rated using a 5-point scale. Image findings were correlated to echocardiography, conventional catheter angiography (CCA), bronchoscopy, and intraoperative findings. Results High quality MDCT data were almost free of cardiac and respiratory motion. In all cases, VA morphology and topography in relation to adjacent structures, e. g. tracheal and esophageal compression caused by an aortic ring, could be assessed exactly and allowed the final diagnosis. Even aberrant vessels, such as aorto-pulmonary collaterals (MAPCA) with a diameter of less than 1 mm, could be identified and excellently visualized. Eighty percent (60/75) of all patients had benefited from the MDCT: in 31 patients CCA was neither necessary to perform surgical planning nor to exclude a VA; in an additional 29 patients radiation doses and sedation time due to interventional procedures could be reduced markedly. Conclusions MDCT can now be regarded as the modality of choice as a minimally invasive, robust, and accurate technique for the diagnosis of complex VA, their potentially life-threatening complications and preoperative planning even in newborns and infants. Its accuracy for detecting VA appears equivalent to CCA while it is more accurate in delineating potential life-threatening complications.

Background— Operator-independent isotropic 3D MRI may greatly simplify the assessment of complex morphology in congenital heart disease. We sought to evaluate the reliability of this new approach. Methods and Results— In 31 adolescent and adult patients (age, 6 to 42 years; median, 16 years) with congenital heart disease, cardiac morphology was determined with free-breathing (navigator-gated), isotropic, 3D steady-state free-precession (3D SSFP) MRI and independently evaluated by 2 observers. Cardiac diagnoses and multiple distance measurements were compared with conventional MR reference sequences (ie, spin-echo, cine gradient-echo, contrast-enhanced MR angiography) and with echocardiography/cine cardioangiography or surgery. Of the 31 patients, 24 had native congenital heart defects or residual defects after repair that warranted immediate treatment. None of these defects was missed by 3D SSFP. Novel diagnostic issues were discovered in 4 of 31 patients (coronary anomalies, n=3; left juxtaposition of the right atrial appendage in double-outlet right ventricle and transposition of the great arteries, 1). For sizes of valves and vessels, we found minor mean differences of −1.1 to 1.6 mm, with SD ranging from 1.2 to 2.9 mm, demonstrating overall good agreement with standard MRI (Bland-Altman analysis). Interobserver variability of 3D SSFP distance measures was low; mean differences ranged from −1.5 to 1.0 mm, and SD ranged from 0.8 to 2.5 mm. Scatter was lower for extracardiac than intracardiac measures. Conclusions— In adolescents and adults, isotropic 3D SSFP MRI allows reliable assessment of complex cardiac morphology. Distance measurements are accurate and reproducible. Thus, a single operator-independent acquisition may substitute for conventional 2D MRI sequences to accelerate and simplify MR scanning in congenital heart disease.

Purpose: To evaluate time-resolved, multiphasic 3D MR angiography (MRA) for the non-invasive assessment of the pulmonary vascular system in children. Materials and Methods: 10 children aged 6 to 15 years (mean age 10 years) ware examined on a 1.5 T whole body MR system with time-resolved, multiphasic 3D MRA after injection of 0.2 mmol/kg body weight of Gd-DTPA. With the use of an ultrafast gradient echo pulse sequence with asymmetric k-space filling and very short echo and repetition times, a nominal spatial resolution of 1.4 × 1.4 × 2.0 mm 3 could be achieved with a scan time of 5.6 and 6.2 seconds for a single 3D data set. Two radiologists, who were blinded to the clinical diagnosis, analyzed the image data in consensus. The image analysis included the assessment of the image contrast and artifacts as well as a quantitative analysis of the signal-to-noise (SNR) and contrast-to-noise ratios (CNR) for central and peripheral lung vessel segments. Results: The children tolerated all examinations without any side effects. The referral diagnosis, which was based on echocardiography, catheter angiography and surgery, was confirmed by MRA in all cases. The image contrast was rated at least satisfactory in all but one case (19 of 20) and no artifacts were observed. The quantitative analysis of the SNR and CNR in the pulmonary arteries and veins confirmed the reader analysis of a high and uniform contrast throughout the entire pulmonary circulation. Conclusion: Time-resolved multiphasic 3D MRA allows a non-invasive diagnostic evaluation of the pulmonary circulation in children. In view of the excellent image quality, MRA may replace conventional diagnostic catheter angiography in the near future.

Die kardiale Schnittbilddiagnostik mit der Magnetresonanztomographie (MRT) und Computertomographie (CT) hat sich in der letzten Dekade technisch rasant weiterentwickelt. Diese Verbesserungen und die breite Verfugbarkeit moderner CT- und MRT-Systeme haben dazu gefuhrt, dass beide Verfahren regelmasig in der klinischen Routine eingesetzt werden. Dieses deutsche Konsensuspapier wurde daher gemeinsam von der Deutschen Gesellschaft fur Kardiologie – Herz- und Kreislaufforschung (DGK), der Deutschen Rontgengesellschaft (DRG) und der Deutschen Gesellschaft fur Padiatrische Kardiologie (DGPK) erarbeitet und orientiert sich nicht an Modalitaten und Methoden, sondern gliedert sich nach grosen Krankheitsgruppen. Behandelt werden die koronare Herzerkrankung, Kardiomyopathien, Herzrhythmusstorungen, Klappenvitien, Perikarderkrankungen, erworbene und strukturellen Veranderungen sowie angeborene Herzfehler. Fur unterschiedliche klinische Szenarien werden die beiden Schnittbildmodalitaten CT und MRT vergleichend gegenubergestellt und in einem kurzen Textfeld bewertet.

Patients with glutaric aciduria type I are at risk for acute striatal injury precipitated by catabolic stress. Here, we report the successful interdisciplinary anesthetic and perioperative management of a child with glutaric aciduria type I undergoing cardiac surgery with extracorporeal circulation. Given the central focus on prevention of acute striatal injury, our anesthetic strategy emphasized avoiding a high protein load, high-dose inotropics, especially epinephrine (associated with impaired glucose utilization), deliberate hyperventilation, and other interventions associated with systemic inflammatory response.

To assess spatial and temporal pressure characteristics in patients with repaired aortic coarctation compared to young healthy volunteers using time-resolved velocity-encoded three-dimensional phase-contrast magnetic resonance imaging (4D flow MRI) and derived 4D pressure difference maps. After in vitro validation against invasive catheterization as gold standard, 4D flow MRI of the thoracic aorta was performed at 1.5T in 13 consecutive patients after aortic coarctation repair without recoarctation and 13 healthy volunteers. Using in-house developed processing software, 4D pressure difference maps were computed based on the Navier-Stokes equation. Pressure difference amplitudes, maximum slope of pressure amplitudes and spatial pressure range at mid systole were retrospectively measured by three readers, and twice by one reader to assess inter- and intraobserver agreement. In vitro, pressure differences derived from 4D flow MRI showed excellent agreement to invasive catheter measurements. In vivo, pressure difference amplitudes, maximum slope of pressure difference amplitudes and spatial pressure range at mid systole were significantly increased in patients compared to volunteers in the aortic arch, the proximal descending and the distal descending thoracic aorta (p < 0.05). Greatest differences occurred in the proximal descending aorta with values of the three parameters for patients versus volunteers being 19.7 ± 7.5 versus 10.0 ± 2.0 (p < 0.001), 10.9 ± 10.4 versus 1.9 ± 0.4 (p = 0.002), and 8.7 ± 6.3 versus 1.6 ± 0.9 (p < 0.001). Inter- and intraobserver agreements were excellent (p < 0.001). Noninvasive 4D pressure difference mapping derived from 4D flow MRI enables detection of altered intraluminal aortic pressures and showed significant spatial and temporal changes in patients with repaired aortic coarctation.

Objective: In this paper we present an attempt at noninvasive imaging of distributed myocardial electrical activity in patients suffering from myocardial infarction and in healthy subjects. Although advances have been made, noninvasive three-dimensional imaging of cardiac electrophysiological activity is still in its infancy and extending our knowledge of cardiac electrophysiological properties may be a valuable guide in the treatment of patients with coronary artery disease. Methods: Magnetic field mapping data formed the input for an inverse solution that is based on a multiple dipole model. The lead field normalized minimum norm least square criterion was applied to predefined myocardial source geometry. Current density distributions were calculated for the left ventricle during ventricular depolarization. Images from two patients with previous myocardial infarction were compared to images from two healthy subjects. Results: Low regional and global current density was found in the infarction patients. Regions of low current density corresponded to infarcted segments. The images of the healthy subjects displayed less marked areas of low current density. Conclusion: The proposed multiple dipole model may be able to distinguish viable from scarred myocardium. A prospective clinical study should be undertaken to investigate the spatial resolution and the diagnostic performance of this method.

Purpose: To assess spatial and temporal pressure changes in patients with repaired aortic coarctation compared with young healthy volunteers using 4D flow MRI and derived 4D pressure difference maps. Methods: 4D flow MRI of the thoracic aorta was performed at 1.5 T in 13 patients after aortic coarctation repair without recoarctation (mean age: 18.8 years, 5 female, 8 male) and 13 healthy volunteers (mean age: 22.9 years, 4 female, 9 male). 4D pressure difference maps were computed based on the Navier-Stokes equation. The thoracic aorta was divided into four segments: ascending aorta, aortic arch, proximal descending aorta, and distal descending aorta. Results: Mean spatial pressure range at mid systole for patients/volunteers was (in mm Hg): ascending aorta 1.8/1.6 (p= ns), arch 4.8/1.7 (p= 0.02), proximal descending 8.9/1.6 (p < 0.001), and distal descending 2.8/1.6 (p= 0.002). Spatial pressure profiles along the aortic centerline demonstrated pressure gradients in patients affecting both aortic arch and proximal descending aorta. Mean maximum slope of local pressure amplitudes for patients/volunteers was (in mm Hg/cm): ascending aorta 7.4/2.5 (p= ns), arch 6.6/2.1 (p < 0.001), proximal descending 12.1/1.9 (p= 0.002), and distal descending 3.9/2.1 (p= 0.002). Conclusion: Noninvasive 4D pressure difference mapping derived from 4D flow MRI showed significant spatial and temporal changes in patients with repaired aortic coarctation compared with young healthy volunteers, particularly affecting aortic arch and proximal descending aorta, but also distal descending aorta. The technique can characterize such changes not only noninvasively but also in great detail.

AIM The aim of this study was to evaluate the morphology and elastic properties of the aorta in children and adolescents with Ullrich-Turner syndrome (UTS) treated with growth hormone, by using magnetic resonance imaging (MRI). METHODS Thirty-seven conscious UTS patients were examined using a 1.5-T whole-body MRI. Contrast-free three-dimensional (3D)-MR angiographies were performed, including 2D cine MRI, to calculate the aortic compliance (C) and cine of the aortic valve. RESULTS Changes of aortic morphology were evident in 40% of the patients, whereas six had more than one alteration. A bicuspid aortic valve was identified in three patients that were missed by previous echocardiography. The aortic compliances in UTS patients were similar to those in healthy persons. CONCLUSION This study shows that aortic morphology and compliance can be assessed by MRI without using contrast agents and without sedation in children and adolescents with UTS.

Recent advances in flow-sensitive magnetic resonance imaging (MRI) and data analysis allow for comprehensive noninvasive three-dimensional (3D) visualization of complex blood flow. Electrocardiogram (ECG)-gated three-directional (3dir) flow measurements were employed to assess and visualize time-resolved 3D blood flow in the pulmonary arteries (PA) and thoracic aorta. We present findings in a juvenile patient with surgically corrected transposition of the great arteries (d-TGA) and aortic coarctation. For the first time, the complex flow patterns in the PA following d-TGA were visualized. Morphologically, a slight asymmetry of the PA was found, with considerable impact on vascular hemodynamics, resulting in diastolic retrograde flow in the larger vessel and diastolic filling of the smaller PA. Additionally, increased flow to the supraaortic vessels was found due to aortic coarctation.

Endothelin-1 (ET-1) is an endogenous vasoconstrictive peptide hormone and asymmetric dimethylarginine (ADMA) acts as an endogenous inhibitor of nitric oxide synthase. We hypothesized that both could contribute to pulmonary hypertension in patients with left-to-right shunt after intracardiac repair.We prospectively analyzed ET-1 and ADMA plasma levels in 31 patients (m = 16; f = 15) at an age of 0.6 [0.2-27] years (median [range]) with left-to-right shunt (ASD II: n = 12; VSD: n = 11; AVSD: n = 8) presenting with a Qp/Qs of 2.7 [1.4-6.3] and a pulmonary arterial mean pressure (PAP) of 23 [13-57] mmHg. Blood specimens were taken prior to cardiopulmonary bypass (CPB), after weaning from CPB and at 3, 6, 12 and 24 h after CPB.12/31 patients were found to have pulmonary hypertension prior to intracardiac repair and 11/12 patients showed persistent pulmonary hypertension during the first 24 h after CPB. Patients with pulmonary hypertension at 12 h after CPB showed significant higher plasma ET-1 compared with patients with normal PAP (1.4 [0-7.9] versus 0.5 [0-2.5] pg/ml; P = 0.048 (Mann-Whitney)). Plasma ADMA decreased from 1.3 [0.75-2.3] micromol/l before CPB to 0.7 [0.4-2.1] micromol/l at 12 h (P0.05). However patients with pulmonary hypertension did not show different ADMA plasma levels.Increased plasma ET-1 but not inhibition of nitric oxide synthase by ADMA is associated with pulmonary hypertension after intracardiac repair.

Evaluation of the severity and the follow-up of aortic insufficiency (AI) are important tasks in paediatric cardiology. Assessment is based on clinical and echocardiographic (ECHO) findings such as the configuration of the valve and the regurgitation fraction (RF). The goal of this study was to evaluate MRI compared to ECHO for determination of clinical severity, valve morphology and RF. Thirty patients (age 3–27 years) with mild-to-severe AI were evaluated by clinical examination, ECHO (2-D and Doppler), and MRI at 1.5 T (2-D true-FISP cine short axis, phase-contrast flow in the ascending aorta). Both methods identified 13 bicuspid and 17 tricuspid valves. Good correlations between ECHO and cine MRI were found for ventricular mass, stroke volume, and ejection fraction. A good linear correlation was found for the RF determined by ECHO and phase-contrast MRI (r = 0.7). The RF was 6% in mild AI, 17% in moderate AI, and 30% in severe AI. The different severity groups showed significantly different RF and it was possible to discriminate between clinical severity grades (P = 0.01). ECHO and MRI showed good agreement in evaluating morphology and function of the left ventricle. The clinical severity of the disease can be evaluated correctly using MRI.

Congenital heart disease (CHD), including complex anomalies of the pulmonary arteries, are now earlier diagnosed and treated. Due to improvements in interventional and surgical therapy, the number of patients with the need for follow-up examinations is increasing. Pre- and postinterventional imaging should be done as gently as possible, avoiding invasive techniques if possible. With the technical improvement of multidetector-row computed tomography (MDCT) and magnetic resonance imaging (MRI), both techniques are increasingly used for noninvasive assessment of the pulmonary vasculature in children with CHD. Knowledge of the most common diseases affecting the pulmonary vasculature and the kind of surgical and interventional procedures is essential for optimal imaging planning. This is especially important because interventions can be positively influenced by high-quality imaging. Therefore, the most common diseases and procedures are described and imaging modality of choice and important image findings are discussed.

Previous studies have suggested the feasibility of a non-invasive quantification of vascular trans-stenotic pressure gradients (DeltaP) by phase-contrast MR imaging (PC-MRI). Our purpose was to assess the value of MRI estimated pressure gradients as a screening tool for assessing hemodynamically significant (re-)coarctation of the aorta (CoA) in pediatric patients.Forty-three patients (median age (range), 16 (5-25) years) with CoA (38 postoperative and 5 native) and clinically suspected hemodynamically significant stenosis underwent quantitative and semi-quantitative PC-MRI blood flow measurements and 3D MR-angiography, Doppler ultrasound (US) and conventional catheter angiography (CCA, n=20). Estimated DeltaP for each modality was correlated with percent stenosis.The percent stenosis correlated only moderately with DeltaP(MRI) (r=0.55, p0.001) and DeltaP(CCA) (r=0.48, p0.001). Only moderate correlations were observed between DeltaP(MRI) vs. DeltaP(CCA) (r=0.54, p=0.02) and vs. DeltaP(US) (r=0.40, p=0.01). In contrast, semi-quantitative analysis of PC-MRI flow profiles predicted with good sensitivity (88%) and specificity (88%) who would be operated on. Thirteen patients met hemodynamic and percent stenosis criteria by CCA for surgical intervention.Measured pressure gradients using PC-MRI should be used cautiously when assessing patients for recoarctation of the aorta. The analysis of blood flow profiles by PC-MRI might be a promising alternative in assessing the hemodynamic significance of CoA.

A 55-year-old woman with congenital heart disease and increasing dyspnea at low levels of exertion was referred to our department for imaging. Clinical findings were cyanosis of the lips and clubbing of the fingers. The past medical history included several hospitalizations for dyspnea, tachycardia, recurrent pulmonary embolism, and suspected cerebral embolism. Despite several conventional angiographic studies, the exact classification of the congenital heart disease and the vascular abnormalities had not been made …

An 8-year-old boy was referred to our department for evaluation of a heart murmur. There was no history of cardiac failure, cyanosis, or respiratory distress or illness. At echocardiography, an aberrant origin of the left pulmonary artery was demonstrated. The barium-enhanced esophagogram showed an anterior indentation at the level of the tracheal bifurcation. Bronchoscopy revealed a stenosis of the ventral and right lateral tracheal wall approximately 1 cm above the bifurcation, with no evidence of tracheomalacia. To assess the pulmonary vascular anatomy, we performed a time-resolved contrast-enhanced magnetic resonance angiography (MRA) using an ultrafast 3-dimensional gradient echo pulse sequence (3D FLASH, repetition time …

A 12 year old boy was referred to our department for magnetic resonance imaging (MRI) of a known pulmonary arteriovenous malformation. The pulmonary malformation had been previously diagnosed by catheter angiography at the age of 1 year after a murmur had been noted over the left lung during a routine examination. So far the boy had always been asymptomatic and healthy. Regular follow up examinations, including …

An isolated intestinal allograft transplant was performed in September 2000 in a patient with a history of ischemic necrosis of the whole small bowel including the ascending and transverse colon plus left kidney after an aortic dissection (Stanford B). The donor was a cadaveric source who was ABO identical with a random HLA match. The patient was not preimmunized. No attempts were made to alter the graft immunologenicity. Whole jejunum and ileum were transplanted anastomosing the superior mesenteric artery to the aorta and the portal vein to the vena cava. Lymphovenous anastomosis of a lymph node to the hypogastric vein was performed. Immunosuppression consisted of tacrolimus, given intravenously for 3 days and then orally. Blood levels 12 hours after the last dose were maintained at 15 to 20 ng/mL for the first 2 months and between 10 and 15 for the next 6 months. Intraoperatively, daclizumab, a monoclonal antibody against the interleukin-2 receptor, was administered at a dose of 1 mg/kg in addition to prednisolone at a dose of 500 mg intravenously (IV). Four further doses of IV daclizumab were delivered every weeks. Prednisolone doses were tapered to 20 mg/day on day 5 post-SBTx and stopped at 6 months. In addition, topical budesonide 9 mg/d was administered initially through the enterostoma and from the third week as an oral medication. The initial creatinine clearance value was 30 mL/min and blood creatinine concentration 1.8 mg%, leading to consideration of addition of add immunosuppression with MMF in order to reduce the dose of tacrolimus. However, in spite of 3 g/d MMF, the blood concentrations of MMF remained low. Therefore, MMF was discontinued and 2 mg/d sirolimus was started. The ileostomy was closed 10 months after the transplant operation. At that time, renal function had gradually deteriorated, particularly when amphotericin B treatment had to be given due to candidae colonization of the bowel. A further effort to spare calcineurin inhibitory agents was made by reducing the tacrolimus dose and reintroducing MMF. Protocol biopsies were performed, weekly for the first month after transplantation and thereafter only when clinically indicated. Immunoprophylaxis with IgG against CMV and ganciclovir was given for the first 3 months and CMV and EBV surveillance performed thereafter.

Background The objective assessment of global and regional cardiac function in children has shown to be clinically relevant but is challenging to conduct. Cardiovascular magnetic resonance (CMR) has emerged as a valuable diagnostic modality especially in patients with cardiomyopathy or congenital heart disease. However, data on the normal cardiac deformation in children assessed by CMR is lacking at present. Thus, the aim of this study was to provide reference values for cardiac strain and strain rate in children and adolescents derived from CMR feature tracking (FT) measurements. Methods In this binational study, eighty children and adolescents (age 0.4–18.0 years, 41 male, 39 female) free from cardiac diseases from two centers underwent CMR in 1.5 T whole-body scanners in supine position. Global peak radial, circumferential and longitudinal systolic strains as well as the corresponding early peak diastolic strain rates were assessed applying FT on short axis as well as 3- and 4-chamber views of standard cine steady-state free precession images. Results The difference between genders yielded no significance for all assessed strains. Yet, all strains showed a significant parabolic relation to age and an even stronger one to body surface area (BSA). Therefore, BSA-specific reference values were determined using a polynomial regression model. The apical cardiac segments featured significant higher peak circumferential but lower peak radial systolic strains than the midventricular and basal segments (all p

Background Similar to speckle tracking echocardiography cardiac MR (CMR) feature tracking imaging (FTI) uses a 2-dimensional deforming analysis of the myocardium which allows quantitative evaluation of myocardial motion and contractility. This technique might provide important additional information in children, including those with congenital heart disease (CHD). Until now, there are no reference data from a healthy pediatric population. The availability of this type of data is a requirement for proper interpretation in pediatric heart disease. The aim of this study was to determine normal values of left ventricular (LV) longitudinal, circumferential and radial strain and strain rate in healthy children, using FTI in cardiovascular MR images. Methods Eighty children from 2 centers (mean age: 12±5.5 years, range: 1t o 17 years; 39 female; divided into 3g roups: 0-11y (n=24), 11-15y (n=32), 15-18y (n=24)) were examined at two different 1.5 T scanners (Siemens and GE) using a standard SSFP MRI protocol. LV longitudinal, circumferential and radial strain and strain rate were measured using FTI (2D CPA MR©, TomTec Imaging System, Fulda, Germany). FTI was applied to standard cine-images in the following orientations:3 LV short-axis slices (basal, middle, apical), 4- and 3-chamber views. The effects of age, gender, endo- and epicardial assessment, myocardial segments on strain and strain rate data were also evaluated. Results