Organisation of paediatric echocardiography laboratories and governance of echocardiography services and training in Europe: current status, disparities, and potential solutions. A survey from the Association for European Paediatric and Congenital Cardiology (AEPC) imaging working group.

BACKGROUND: There is limited data on the organisation of paediatric echocardiography laboratories in Europe. METHODS: A structured and approved questionnaire was circulated across all 95 Association for European Paediatric and Congenital Cardiology affiliated centres. The aims were to evaluate: (1) facilities in paediatric echocardiography laboratories across Europe, (2) accredited laboratories, (3) medical/paramedical staff employed, (4) time for echocardiographic studies and reporting, and (5) training, teaching, quality improvement, and research programs. RESULTS: Respondents from forty-three centres (45%) in 22 countries completed the survey. Thirty-six centres (84%) have a dedicated paediatric echocardiography laboratory, only five (12%) of which reported they were European Association of Cardiovascular Imaging accredited. The median number of echocardiography rooms was three (range 1-12), and echocardiography machines was four (range 1-12). Only half of all the centres have dedicated imaging physiologists and/or nursing staff, while the majority (79%) have specialist imaging cardiologist(s). The median (range) duration of time for a new examination was 45 (20-60) minutes, and for repeat examination was 20 (5-30) minutes. More than half of respondents (58%) have dedicated time for reporting. An organised training program was present in most centres (78%), 44% undertake quality assurance, and 79% perform research. Guidelines for performing echocardiography were available in 32 centres (74%). CONCLUSION: Facilities, staffing levels, study times, standards in teaching/training, and quality assurance vary widely across paediatric echocardiography laboratories in Europe. Greater support and investment to facilitate improvements in staffing levels, equipment, and governance would potentially improve European paediatric echocardiography laboratories.

Quantitative evaluation of aortic valve regurgitation in 4D flow cardiac magnetic resonance: at which level should we measure?

PURPOSE: To find the best level to measure aortic flow for quantification of aortic regurgitation (AR) in 4D flow CMR. METHODS: In 27 congenital heart disease patients with AR (67% male, 31 ± 16 years) two blinded observers measured antegrade, retrograde, net aortic flow volumes and regurgitant fractions at 6 levels in 4D flow: (1) below the aortic valve (AV), (2) at the AV, (3) at the aortic sinus, (4) at the sinotubular junction, (5) at the level of the pulmonary arteries (PA) and (6) below the brachiocephalic trunk. 2D phase contrast (2DPC) sequences were acquired at the level of PA. All patients received prior transthoracic echocardiography (TTE) with AR severity grading according to a recommended multiparametric approach. RESULTS: After assigning 2DPC measurements into AR grading, agreement between TTE AR grading and 2DPC was good (κ = 0.88). In 4D flow, antegrade flow was similar between the six levels (p = 0.87). Net flow was higher at level 1-2 than at levels 3-6 (p < 0.05). Retrograde flow and regurgitant fraction at level 1-2 were lower compared to levels 3-6 (p < 0.05). Reproducibility (inter-reader agreement: ICC 0.993, 95% CI 0.986-0.99; intra-reader agreement: ICC 0.982, 95%CI 0.943-0.994) as well as measurement agreement between 4D flow and 2DPC (ICC 0.994; 95%CI 0.989 - 0.998) was best at the level of PA. CONCLUSION: For estimating severity of AR in 4D flow, best reproducibility along with best agreement with 2DPC measurements can be expected at the level of PA. Measurements at AV or below AV might underestimate AR.

Accuracy of Cardiac Magnetic Resonance Imaging in Diagnosing Pediatric Cardiac Masses: A Multicenter Study.

BACKGROUND: After diagnosis of a cardiac mass, clinicians must weigh the benefits and risks of ascertaining a tissue diagnosis. Limited data are available on the accuracy of previously developed noninvasive pediatric cardiac magnetic resonance (CMR)-based diagnostic criteria. OBJECTIVES: The goals of this study were to: 1) evaluate the CMR characteristics of pediatric cardiac masses from a large international cohort; 2) test the accuracy of previously developed CMR-based diagnostic criteria; and 3) expand diagnostic criteria using new information. METHODS: CMR studies (children 0-18 years of age) with confirmatory histological and/or genetic diagnosis were analyzed by 2 reviewers, without knowledge of prior diagnosis. Diagnostic accuracy was graded as: 1) single correct diagnosis; 2) correct diagnosis among a differential; or 3) incorrect diagnosis. RESULTS: Of 213 cases, 174 (82%) had diagnoses that were represented in the previously published diagnostic criteria. In 70% of 174 cases, both reviewers achieved a single correct diagnosis (94% of fibromas, 71% of rhabdomyomas, and 50% of myxomas). When ≤2 differential diagnoses were included, both reviewers reached a correct diagnosis in 86% of cases. Of 29 malignant tumors, both reviewers indicated malignancy as a single diagnosis in 52% of cases. Including ≤2 differential diagnoses, both reviewers indicated malignancy in 83% of cases. Of 6 CMR sequences examined, acquisition of first-pass perfusion and late gadolinium enhancement were independently associated with a higher likelihood of a single correct diagnosis. CONCLUSIONS: CMR of cardiac masses in children leads to an accurate diagnosis in most cases. A comprehensive imaging protocol is associated with higher diagnostic accuracy.

In vivo MRI measurement of blood oxygen saturation in children with congenital heart disease.

OBJECTIVE: The purpose of this prospective study was to measure in vivo blood oxygen saturation (%O2) by MRI in children with congenital heart disease (CHD) using population-based values for T2O (T2 signal decay of fully oxygenated blood) and K (a parameter representing the deoxyhemoglobin effect) and compare the %O2 with direct cardiac catheterization measurements. BACKGROUND: MRI can determine %O2 using in vivo measurement of signal decay (T2) and an in vitro calibration curve relating T2 and %O2, based on the equation: 1/T2 = 1/T2O + K(1-%O2/100)2. Recent studies have correlated the T2/%O2 in children with CHD with the adult calibration statistics. METHODS: A total of ten children (five male, five female) with single ventricle CHD (median age 4.8 months, range 2 months to 4.4 years) undergoing cardiac catheterization were included in the study. The blood T2 measurements for each patient were performed in a 1.5 T GE CV scanner. The %O2 was then calculated based on the equation using values of T2O determined from individual hematocrits, and a population average value of K derived for children. The %O2 values were compared with direct %O2 measurements from cardiac catheterization. RESULTS: The %O2 values by MRI were strongly correlated with direct cardiac catheterization measurements (R = 0.825; P < 0.001). CONCLUSION: The study indicates that the noninvasive measurement of %O2 by MRI can accurately measure oxygen saturation in children with complex CHD.

Phase-contrast MR assessment of pulmonary venous blood flow in children with surgically repaired pulmonary veins.

BACKGROUND: Pulmonary venous (PV) obstruction may complicate surgical repair of PV abnormalities. By combining phase-contrast cine (PC) imaging and contrast-enhanced angiography, magnetic resonance (MR) imaging can provide physiological information complementing anatomical diagnosis. OBJECTIVES. To compare the PV flow pattern observed after surgical repair of PV abnormalities with normal PV flow pattern and to investigate the changes occurring in the presence of PV stenosis by using PC MR in children. MATERIALS AND METHODS: By using PC MR, PV flow was evaluated in 14 patients (3 months-14 years) who underwent surgical repair for PV abnormalities. Eleven children (8-18 years) were studied as normal controls. Peak flow velocities and patterns were compared among three groups: normal veins ( n=23), surgically repaired veins without ( n=44) and with stenosis ( n=10). RESULTS: Normal and unobstructed pulmonary veins after surgery showed a biphasic or triphasic flow pattern with one or two systolic peaks and a diastolic peak. Unobstructed surgically repaired veins showed decreased peak systolic velocity ( P =0.001) and an increased peak diastolic velocity ( P=0.005) when compared to normal values. Obstructed veins showed decreased systolic and diastolic velocities when measured upstream from the stenosis. CONCLUSION: PC MR shows different flow patterns among normal, surgically repaired pulmonary veins with and without stenosis.

Differential regurgitation in branch pulmonary arteries after repair of tetralogy of Fallot: a phase-contrast cine magnetic resonance study.

BACKGROUND: The importance of pulmonary regurgitation (PR) after repair of tetralogy of Fallot (TOF) is increasingly recognized, but little is known regarding its underlying mechanisms. This phase-contrast cine magnetic resonance (PC MR) study was performed to evaluate the relative contribution of each lung to total regurgitant volume. METHODS AND RESULTS: Twenty-two patients with significant PR underwent a PC MR 3 to 16 years after repair of TOF. Regurgitant fraction of the main pulmonary artery was 39+/-10%. Regurgitant fraction of the left pulmonary artery (LPA; 46+/-18%) was greater than that of the right pulmonary artery (34+/-16%; P=0.002). The average contribution of the LPA to the total regurgitant flow volume was 54+/-19%, whereas its average contribution to the total forward flow volume was 44+/-13% (P=0.002). In 4 patients, regurgitant flow in the LPA accounted for 75% to 100% of the total regurgitant flow. There was a linear relationship between regurgitant fraction and fraction of the regurgitant flow duration in the main pulmonary artery (P<0.001) and right pulmonary artery (P=0.001) but not in the LPA (P=0.129). CONCLUSIONS: PR after repair of TOF is commonly associated with differential regurgitation in the branch pulmonary arteries, which is usually greater in the LPA. Although the cause of this disparity requires further investigation, those patients with a significant unilateral contribution to total PR may be amenable to localized techniques to reduce regurgitation.

Assessment of the thymus at echocardiography in fetuses at risk for 22q11.2 deletion.

OBJECTIVES: An absent or hypoplastic thymus is common in patients with 22q11.2 deletion (del22q11.2). We sought to determine whether fetal echocardiography could identify absence of the thymus as a diagnostic tool in pregnancies at risk for fetal del22q11.2. METHODS: We evaluated the fetal thymus in 16 consecutive pregnancies at risk for fetal del22q11. Fourteen of the fetuses had a conotruncal cardiac lesion, one had a twin with a conotruncal lesion, and in one the mother had a diagnosis of del22q11.2. The fetal thymus assessment was performed by an individual who was not aware of the del22q11.2 status of the fetus. RESULTS: By 2D imaging, the thymus was identified in the anterosuperior mediastinum as a subtle hypoechogenic area. In nine cases, the thymus was demonstrated prenatally and none had del22q11.2. However, in one case the thymus was only seen on follow-up fetal echocardiography. In six cases, the thymus could not be identified and all six had del22q11.2. In one additional case, analyzed retrospectively, the thymus could not be assessed. The status of the thymus was confirmed on postnatal echocardiography or autopsy in 11 of the 15 cases assessed prenatally. CONCLUSIONS: Our study suggests that fetal echocardiography can assess the thymus in most cases at risk for del22q11.2. This information may be useful in counseling women/couples who decline amniocentesis or who are awaiting amniocentesis results.

Contrast-enhanced MR angiography of pulmonary venous abnormalities in children.

BACKGROUND: Echocardiography and X-ray angiography have been considered as gold standards for evaluation of pulmonary venous abnormalities. However, each technique has its own limitations, such as limitation in visualization of the pulmonary veins within the lungs by echocardiography, and the invasive nature of and use of ionizing radiation in X-ray angiography. Contrast-enhanced MR angiography (MRA) is a fast noninvasive method of visualization of the vessels including the pulmonary arteries and veins. OBJECTIVES: To evaluate the utility of contrast-enhanced MRA in the evaluation of pulmonary venous abnormalities in pediatric patients and to compare its diagnostic accuracy with that of transthoracic echocardiography. MATERIALS AND METHODS: In 30 pediatric patients 31 contrast-enhanced MRA studies were performed for evaluation of pulmonary venous abnormalities. Each of 124 pulmonary veins was evaluated for site of connection, course within the lung, presence of obstruction, and topographic relationship with the adjacent structures. The findings of MRA were compared with echocardiographic findings for 116 veins in 29 studies in 28 patients. RESULTS: Contrast-enhanced MRA visualized 99% (123 of 124) of the pulmonary veins investigated, while echocardiography visualized 89% (103 of 116). Exact agreement was found between the two methods in 72% of the veins with a weighted kappa of 0.60 (0.47-0.73, 95% CI). Echocardiography failed to diagnose an abnormal connection in 2 of 15 pulmonary veins, a discrete stenosis in 2 of 19 veins, and diffuse hypoplasia in 10 of 14 veins. In 29% of patients, MRA made the uncertain echocardiographic findings clear. In another 29%, MRA provided a new diagnosis. CONCLUSIONS: Contrast-enhanced MRA is a powerful, safe, and accurate fast-imaging technique for the anatomical evaluation of pulmonary venous abnormalities. MRA may obviate the need for conventional X-ray angiography. Cardiac catheterization may be reserved for those patients in whom pulmonary vascular resistance needs to be determined.

Cardiac rhythm disturbances in children on hemodialysis.

Cardiovascular complications are the leading causes of morbidity and mortality in adult dialysis patients. The aim of this study was to evaluate the cardiovascular system of children on hemodialysis (HD), with special focus on rhythm disturbances. Nine patients, aged 15.6+/-4.1 years, underwent electrocardiographic examination (ECG) including 12-lead ECG, Holter-ECG, QT dispersion, signal-averaged ECG, and exercise testing. Echocardiography and 24-h blood pressure measurement were also performed. Patients had been on HD for a median period of 7 months (range 1-29 months). Normal findings were obtained with 12-lead ECG, exercise testing, QT dispersion, and signal-averaged ECG. Holter-ECG showed short runs of slow monomorphic ventricular tachycardia in two patients. Echocardiography was normal except for ventricular hypertrophy in two patients. In conclusion, rhythm disturbances were rare, with slow monomorphic ventricular tachycardias being the only significant finding. The absence of late potentials and normal QT dispersion suggest that myocardial electrical excitability and recovery are preserved in children on HD.

Early postoperative arrhythmias after cardiac operation in children.

BACKGROUND: Arrhythmias are a recognized complication of cardiac operations. However, little is known about the incidence, treatment, and risk factors for early postoperative arrhythmias in children after cardiac operations. METHODS: Diagnosis and treatment of early postoperative arrhythmias were prospectively analyzed in an intensive care unit in 100 consecutive children with a median age of 17 months (range, 1 day to 191 months) who had undergone cardiac operation. Patients were grouped in three different categories of surgical complexity. RESULTS: During a median postoperative time of 1 day (range, 0 to 15 days), 64 critical arrhythmias occurred in 48 patients. Arrhythmias consisted of sinus bradycardia in 30, atrioventricular block II to III in 7, supraventricular tachyarrhythmias in 14, and premature complexes in 13 instances. Treatment of 52 arrhythmias was successful and included pacing in 41, intravenous amiodarone in 8, body cooling in 5, overdrive pacing in 3, and electrolyte correction in 2 cases, with more than one treatment modality in 8 cases. Risk factors for arrhythmias were lower body weight (p < 0.05), longer cardiopulmonary bypass duration (p < 0.05), and a category of higher surgical complexity (p < 0.001). CONCLUSIONS: Early postoperative arrhythmias occur frequently after cardiac operations in children. Sinus bradycardia, atrioventricular block II to III, and supraventricular tachyarrhythmias are the most frequent arrhythmias, which, however, can be treated effectively by means of temporary pacing, cooling, and antiarrhythmic drug therapy. Lower body weight, longer cardiopulmonary bypass duration, and a higher surgical complexity are risk factors for early postoperative arrhythmias.

MRI-based blood oxygen saturation measurements in infants and children with congenital heart disease.

BACKGROUND: Vessel oxygen saturation can be determined with MR oximetry using an in vivo measurement of signal decay (T2) and the calibration curve relating T2 to blood oxygen saturation (%HbO(2)), where: 1/T2=1/T2O+K (1-%HbO(2)/100)(2) and K is a constant parameter which correlates with measured fibrinogen levels. The ability to noninvasively measure %HbO(2) in cardiac chambers and vessels has enormous potential in children with congenital heart disease (CHD). OBJECTIVE: The purpose of the study was to prospectively characterize the T2-%HbO(2) relationship in infants where T2-%HbO(2) is the relationship between T2 and %HbO(2) (blood oxygen saturation) expressed by the equation given above, and to determine whether adult values for K and T2O (where T2O is the T2 of fully oxygenated blood) can be extrapolated to pediatric patients with CHD. A second objective was to apply this method to calculate the %HbO(2) in vivo using MR imaging in infants with CHD. MATERIALS AND METHODS: Fifteen patients with CHD undergoing cardiac catheterization (9 male; 6 female), median age 8 months, were recruited for the calibration study. T2O and K were measured directly from blood samples, compared with the values estimated from adult population statistics, and plotted against hematocrit and fibrinogen, respectively. In four studies of infants with CHD, T2 measurements were converted to %HbO(2) using the calibration curve. RESULTS: The T2-%HbO(2) relationship in infants correlated with the adult calibration statistics (1/T2O vs. hematocrit, r=0.77; K vs. fibrinogen, r=0.61). Our initial in vivo studies demonstrated that the MR oximetry reflected the expected oxygen saturations. CONCLUSION: Adult values for T2-%HbO(2) calibration can be used to measure blood oxygen saturation in vivo in children with CHD.