Ambulatory sedation for children under 6 years with CHD in MRI and CT.

INTRODUCTION: In infants and young children, good image quality in MRI and CT requires sedation or general anesthesia to prevent motion artefacts. This study aims to determine the safety of ambulatory sedation for children with CHD in an outpatient setting as a feasible alternative to in-hospital management. METHODS: We recorded 91 consecutive MRI and CT examinations of patients with CHD younger than 6 years with ambulatory sedation. CHD diagnoses, vital signs, applied sedatives, and adverse events during or after ambulatory sedation were investigated. RESULTS: We analysed 91 patients under 72 months (6 years) of age (median 26.0, range 1-70 months; 36% female). Sixty-eight per cent were classified as ASA IV, 25% as ASA III, and 7% as ASA II (American Society of Anesthesiologists Physical Status Classification). Ambulatory sedation was performed by using midazolam, propofol, and/or S-ketamine. The median sedation time for MRI was 90 minutes (range 35-235 minutes) and 65 minutes for CT (range 40-280 minutes). Two male patients (age 1.5 months, ASA II, and age 17 months, ASA IV) were admitted for in-hospital observation due to unexpected severe airway obstruction. The patients were discharged without sequelae after 1 and 3 days, respectively. All other patients were sent home on the day of examination. CONCLUSION: In infants and young children with CHD, MRI or CT imaging can be performed under sedation in an outpatient setting by a well-experienced team. In-hospital backup should be available for unexpected events.

Improved Tricuspid Valve Function, Preload Recruitment and Ventricular Efficiency During Submaximal Exercise in Patients with Unoperated Ebstein's Anomaly: An MRI Study.

BACKGROUND: Adolescents and adults with native Ebstein's anomaly (EA) are at the benign part of the Ebstein spectrum, having survived infancy without surgery. In this population, surgical indication and timing remain objects of controversy and depend, among other factors, on exercise capacity. PURPOSE: To better understand the pathophysiology of exercise adaptation in native EA. STUDY TYPE: Retrospective. POPULATION: Ten patients with unoperated EA (age range 18-61 years) and 13 healthy subjects as controls. FIELD STRENGTH/SEQUENCE: Balanced steady-state free precession cine and phase contrast flow sequences at 1.5 T. ASSESSMENT: We measured volumes and flows at rest and during submaximal exercise. Hemodynamic parameters including stroke volume (SV), cardiac index (CI), ejection fraction (EF), and tricuspid regurgitation (TR) were calculated. STATISTICAL TESTS: We used nonparametric Mann-Whitney U-test and Wilcoxon signed-rank test. A P-value of <0.05 was considered statistically significant. RESULTS: Rest CI and SV were significantly higher in controls; rest heart rate (HR) was similar in the two groups (median 71 bpm by patients and 65 bpm by controls, P = 0.448). During exercise, CI increased significantly in both groups: from 2.40 to 3.35 L/min/m2 in the patient group and from 3.60 to 4.20 L/min/m2 in controls; HR increased significantly in both groups. SV increased significantly in the patient group, whereas it remained stable in controls (P = 0.5284). Patients' median TR decreased significantly: median 42% at rest and 30% during exercise; concomitantly, left ventricular (LV) preload increased significantly (+3% indexed LV end-diastolic volume) as did LVEF (median 59% at rest vs. 65% during exercise). DATA CONCLUSION: During submaximal exercise, patients with mild to moderate EA improved their cardiovascular system's total efficiency by increasing CI; this was obtained by an increase in HR and by the recruitment of volume, as shown by an increased LV end-diastolic volume and SV, with simultaneous decrease in TR. This was different from healthy subjects in which CI increased only due to HR increase. LEVEL OF EVIDENCE: 3 TECHNICAL EFFICACY STAGE: 3.

Different CMR Imaging Modalities for Native and Patch-Repaired Right Ventricular Outflow Tract Sizing: Impact on Percutaneous Pulmonary Valve Replacement Planning.

Percutaneous pulmonary valve replacement (PPVI) in native or patched right ventricular outflow tract (RVOT) has proven to be feasible. The procedure is highly dependent on the size of the RVOT. Several methods exist to evaluate the size of the RVOT by cardiovascular magnetic resonance (CMR). We evaluated different CMR modalities for measuring RVOT diameters. Thirty-one consecutive patients with native or patched RVOT were retrospectively evaluated. CMR was part of follow-up of patients with corrected Tetralogy of Fallot or pulmonary stenosis with significant pulmonary regurgitation (PR). CMR included 3D-SSFP whole-heart in systole, diastole, and contrast-enhanced MR angiography (ceMRA). Diameters of the RVOT were assessed by the three sequences. Additionally, in patients who underwent cardiac catheterization (n = 11) for PPVI, vessel diameters assessed by cine-angiography were compared to CMR. Systolic diameters of RVOT were significantly larger compared to the diameters taken in diastole and ceMRA (median difference 5.0 mm and 3.8 mm). Diastolic and ceMRA diameters did not differ significantly. CMR diameters taken in systole showed no statistical difference to systolic diameters taken by cine-angiography, while diastolic and ceMRA diameters were significantly smaller. PPVI was feasible to a maximal CMR diameter of 31 mm measured by SSFP whole-heart sequence in systole. Absolute diameters of native RVOT differ depending on the CMR sequence and timing of acquisition (systolic vs diastolic gating). Diameters taken during heart catheterization by cine-angiography best correlate to systolic CMR values. Data may help to select RVOTs suitable for PPVI.

Magnetic Resonance Imaging Risk Factors for Ventricular Arrhythmias in Tetralogy of Fallot.

Sudden cardiac death (SCD) is the most common cause of late mortality in tetralogy of Fallot (TOF). Pulmonary regurgitation (PR) was previously found to be the most common hemodynamic abnormality associated with ventricular arrhythmias (VA), but cardiovascular magnetic resonance (CMR)-based studies did not show this association. The aim of this study is to investigate the risk factors for VA in TOF using CMR. Electronic records of TOF patients and their CMR studies between July 2006 and October 2018 in one center were retrospectively reviewed. Demographic, clinical and CMR data of patients were collected. Outcome was defined as sustained ventricular tachycardia (VT), aborted SCD and SCD. From a total of 434 TOF patients with complete CMR studies, 19 (4.4%) patients developed a positive outcome (12 sustained VT, 4 aborted SCD, 3 SCD) at a median age of 24 years. The number of surgical interventions was significantly greater in patients who developed VA. Right ventricular volumes were significantly larger in patients who suffered a positive outcome. Odds ratio for developing VA was 6.905 for RVEDVI ≥ 160 ml/m2 and 6.141 for RVESVI ≥ 80 ml/m2 (P = 0.0014 and 0.0012, respectively). Event-free survival was longer in patients with smaller right ventricular volumes. In conclusion, right ventricular dimensions are the most significant factors associated with the development of VA in TOF. The number of surgical interventions is also related to an increased risk.

Elevated diastolic wall shear stress in regurgitant semilunar valvular lesions.

BACKGROUND: Alterations in wall shear stress (WSS) assessed using 4D flow MRI have been shown to play a role in various vascular pathologies, such as bicuspid aortic valve aortopathy. Most studies have focused on systolic WSS, whereas altered diastolic hemodynamics in regurgitant semilunar valvular lesions have not so far been well characterized. PURPOSE: To investigate diastolic WSS in aortic and pulmonary regurgitation. STUDY TYPE: Retrospective data analysis. POPULATION: Thirty tetralogy of Fallot patients, 19 bicuspid aortic valve patients, 11 healthy volunteers. FIELD STRENGTH/SEQUENCE: 5 T, 3D time-resolved phase-contrast MRI with 3D velocity encoding. ASSESSMENT: Estimation of WSS and its axial and circumferential vector components along cardiac cycle timeframes in the proximal main pulmonary artery in pulmonary regurgitation (PR) and in the proximal ascending aorta in aortic regurgitation (AR) as well as in healthy volunteers. STATISTICAL TESTS: Wilcoxon matched pairs test was used for intra-group comparisons and Mann-Whitney test for intergroup comparisons. Correlations were assessed using Spearman correlation. RESULTS: WSS along the entire cardiac cycle was higher in PR and AR in comparison with controls (mean WSS 0.381 ± 0.070 vs. 0.220 ± 0.018, P < 0.0001; 0.361 ± 0.099 vs. 0.212 ± 0.030, P < 0.0001; respectively). Peak diastolic WSS was significantly higher than the mean WSS in AR and PR (P < 0.0001-0.005). The severity of PR correlated with the peak diastolic axial WSS (Spearman's r s = 0.454, P = 0.018), whereas the severity of AR correlated with both peak systolic and diastolic tangential WSS (Spearman's r s = 0.458, P = 0.049; r s = 0.539, P = 0.017, respectively). DATA CONCLUSION: Elevated diastolic WSS is a component of the altered flow hemodynamics in AR and PR. This may give more insight into the pathophysiologic role of WSS in vascular remodeling in AR and PR. LEVEL OF EVIDENCE: 4 Technical Efficacy Stage: 1 J. Magn. Reson. Imaging 2019;50:763-770.

Non-invasive Hemodynamic CMR Parameters Predicting Maximal Exercise Capacity in 54 Patients with Ebstein's Anomaly.

BACKGROUND: Exercise capacity is a well-defined marker of outcome in congenital heart disease. We analyzed seventeen cardiovascular magnetic resonance (CMR) derived parameters and their correlation to exercise capacity in patients with Ebstein's anomaly (EA). METHODS: Fifty-four surgery free patients, age 5 to 69 years (median 30 years) prospectively underwent CMR examination and cardiopulmonary exercise testing (CPET). The following volume/flow parameters were compared with peak oxygen uptake as the percentage of normal (peakVO2%) using univariate and multivariate analysis: right and left ventricular ejection fraction (RVEF and LVEF), the indexed end-diastolic and end-systolic volumes (RVEDVi, RVESVi, LVEDVi, and LVESVi), the indexed stroke volumes (RVSVi and LVSVi), the total normalized right and left heart volumes; the total right to left heart volume ratio (R/L-ratio). The indexed antegrade flow (ante), indexed net flow (net) as well as cardiac index (CI) in the aorta (Ao) and pulmonary artery (PA) were used. RESULTS: RVEF (R2 0.2788), indexed flow PA net (R2 0.2330), and PA ante (R2 0.1912) showed the best correlation with peakVO2% (all p < 0.001) in the univariate model. Further significant correlation could also be demonstrated with CI-PA, LVEF, LVSVi, Aorta net, RVESVi, and Aorta ante. Multivariate analysis for RVEF and indexed net flow PA revealed a R2 of 0.4350. CONCLUSION: Functional CMR parameters as RVEF and LVEF and flow data of cardiac forward flow correlate to peakVO2%. Evaluation of the indexed net flow in the pulmonary artery and the overall function of the right ventricle best predicts the maximal exercise capacity in patients with EA.

Quantifying aortic valve regurgitation in patients with congenital aortic valve disease by 2D and 4D flow magnetic resonance analysis.

BACKGROUND: In congenital aortic valve disease, quantifying aortic regurgitation (AR) varies by the measurement site. Our study aimed to identify the optimal site for AR assessment using 2D and 4D MR flow measurements, with a focus on vortices. METHODS: We retrospectively analysed 31 patients with congenital aortic valve disease, performing 2D and 4D MR flow measurements at the aortic valve, sinotubular junction (STJ), ascending aorta (AAo), and using midpulmonary artery measurements as a reference. We assessed percentage AR and net forward volumes, calculated linear correlations, and plotted Bland-Altman plots. Net forward flow at all aortic sites were correlated with the main pulmonary artery. Differences in AR between 2D and 4D flows were linked to vortices detected by 4D streamlines. RESULTS: The best agreement in % AR between 2D and 4D flows was at the aortic valve (mean difference 4D2D -2.9%, limits of agreement 8.7% to -14.3%; r2 = 0.7). Correlations weakened at STJ and AAo. Vortices in the ascending aorta led to AR overestimation in 2D measurements. Net forward flow at the aortic valve by 4D flow correlated closer with main pulmonary artery than did 2D flow. (Mean difference for 2D and 4D MR flow 7.5 ml and 4.2 ml, respectively). CONCLUSIONS: For congenital aortic valve disease, the most accurate AR quantification occurs at the aortic valve using 2D and 4D MR flow. Notably, vortices in the ascending aorta can result in AR overestimation with 2D MR flow.

Junctional rhythm produces retrograde flow in the whole Fontan system: cardiac magnetic resonance-derived flow documentation-a case report.

Background: Arrhythmias after palliation of congenital malformations with functional monoventricle by different Fontan modifications are very common. Sinus node dysfunction and junctional rhythm are known to have a high prevalence and a detrimental impact on the optimal functioning of Fontan circulations. Maintaining sinus node function has high prognostic significance, and some cases have even been described where atrial pacing with restoring of atrioventricular synchrony was able to reverse protein-losing enteropathy with overt failure of the Fontan. Case summary: A 12-year-old boy with a complex congenital malformation (double outlet right ventricle, transposition of the great arteries, pulmonary stenosis, and straddling atrioventricular valve) palliated through a modified Fontan (total cavopulmonary connection with a fenestrated extracardiac 18 mm Gore-Tex conduit) presented for cardiac magnetic resonance evaluation for mild asthenia and worsening of exercise tolerance. Flow profiles in all the regions of the Fontan (both caval veins and right and left pulmonary arteries) showed a small amount of retrograde flow; a four-chamber cine sequence clearly showed contraction of the atria against closed atrioventricular valve; this haemodynamic condition can be caused either by retro-conducted junctional rhythm (previously demonstrated in our patient) or by isorhythmic dissociation of sinus rhythm. Discussion: Our finding directly demonstrates the profound impact of retro-conducted junctional rhythm on the haemodynamic of a Fontan circulation in which, with each cardiac beat, the pressure rise in the atria and pulmonary veins due to atrial contraction with closed atrioventricular valves is able to stop and invert the passive flow of the systemic venous return towards the lungs.

Aortic wall shear stress in bicuspid aortic valve disease-10-year follow-up.

Background: Bicuspid aortic valve (BAV) disease leads to deviant helical flow patterns especially in the mid-ascending aorta (AAo), potentially causing wall alterations such as aortic dilation and dissection. Among others, wall shear stress (WSS) could contribute to the prediction of long-term outcome of patients with BAV. 4D flow in cardiovascular magnetic resonance (CMR) has been established as a valid method for flow visualization and WSS estimation. The aim of this study is to reevaluate flow patterns and WSS in patients with BAV 10 years after the initial evaluation. Methods: Fifteen patients (median age 34.0 years) with BAV were re-evaluated 10 years after the initial study from 2008/2009 using 4D flow by CMR. Our particular patient cohort met the same inclusion criteria as in 2008/2009, all without enlargement of the aorta or valvular impairment at that time. Flow patterns, aortic diameters, WSS and distensibility were calculated in different aortic regions of interest (ROI) with dedicated software tools. Results: Indexed aortic diameters in the descending aorta (DAo), but especially in the AAo did not change in the 10-year period. Median difference 0.05 cm/m2 (95% CI: 0.01 to 0.22; P=0.06) for AAo and median difference -0.08 cm/m2 (95% CI: -0.12 to 0.01; P=0.07) for DAo. WSS values were lower in 2018/2019 at all measured levels. Aortic distensibility decreased by median 25.6% in the AAo, while stiffness increased concordantly (median +23.6%). Conclusions: After a ten years' follow-up of patients with isolated BAV disease, indexed aortic diameters did not change in this patient cohort. WSS was lower compared to values generated 10 years earlier. Possibly a drop of WSS in BAV could serve as a marker for a benign long-term course and implementation of more conservative treatment strategies.

Training intensity influences left ventricular dimensions in young competitive athletes.

Background: In young athletes, exercise causes changes in the heart that include growth in wall thickness and mass of the left ventricle and expansion of the heart's chambers. The heart's function is either preserved or enhanced, but this may change to the opposite over time. Objective: This study aimed to assess structural and functional cardiac adaptations in relation to exercise training time, intensity, and performance in young competitive athletes. Methods: A total of 404 children and adolescents (14.23 ± 2.0 years, 97 females) were enrolled in the Munich Cardiovascular Adaptations in Young Athletes Study (MuCAYA-Study). Eighty-five participants were examined two times a year. Two-dimensional echocardiography was performed to assess left ventricular structure and function. Training time and intensity was measured with the MoMo physical activity questionnaire, maximum aerobic capacity by cardiopulmonary exercise testing, and strength with the handgrip strength test. Results: Maximum aerobic capacity significantly influenced interventricular septal thickness in diastole. Training intensity significantly influenced left ventricular internal diameter in diastole and systole, and left ventricular mass indexed to body surface area. Within one year, interventricular wall thickness, relative wall thickness and left ventricular mass, indexed to body surface area and height, increased significantly. Training intensity and aerobic capacity contributed to cardiac adaptations in young competitive athletes, as represented by altered structural parameters but preserved cardiac function. Within a year, however, structural changes and a decline in diastolic performance were observed within the longitudinal sub-sample. Conclusion: Our results confirm the hypothesis that cardiac adaptations to exercise occur at a young age. Cardiac adaptation in our cohort was influenced by exercise intensity and maximum aerobic capacity.

Safety of intrahospital transport for MR or CT scans in ventilated pediatric intensive care patients with congenital heart disease.

Background: MR or CT scans are often required in the treatment of pediatric intensive care patients. During the therefore needed intrahospital transport continuous ventilation of the patient must be maintained. Intrahospital transport is considered safe regarding changes in hemodynamics or adverse events (AEs). As those studies cover inhomogeneous patient groups, we analyzed the safety for ventilated pediatric patients with congenital heart disease (CHD) focusing on differences between manual and mechanical ventilation during transport and examination. Methods: Retrospective monocentric case-control study covering a 10 years' period in a tertiary cardiac center for CHD. Sixty-three critically ill ventilator-dependent patients, median 2 (0-37) months, were included. Fifty-one patients got ventilated manually and 12 patients got ventilated with a mobile ventilator. The data include vital parameters and blood gas data, as well as catecholamine support, occurrence of AEs and total duration of transport and examination. Results: In both groups we found minor changes of vital parameters or blood gas data. Regarding the HR we found a drop from median 135/min before leaving the ICU to 130/min after returning to the ICU (P=0.0072) in the manually ventilated group and a drop from median 133/min to 123/min (P=0.0703) in the mechanically ventilated group. The median transport time including scan was higher in the manually ventilated group (P=0.0098) with a median duration of transport and scan time of 100 minutes (30-245 minutes) in the manually ventilated group and of 97.5 minutes (60-224 minutes) in the mechanically ventilated group. A total of 9 AEs was recorded, 7 (13.7%) of them in the manually and 2 (16.7%) in the mechanically ventilated group with a drop of the mean arterial pressure (MAP) and an increase in catecholamine support. Conclusions: We consider both manual ventilation and mechanical ventilation for intrahospital transport safe for pediatric intensive care patients with CHD. Using a mechanical ventilator might have the advantage to react faster to changes in hemodynamics.

Detection of early signs of right ventricular systolic impairment in unoperated Ebstein's anomaly by cardiac magnetic resonance feature tracking.

Background: Cardiovascular magnetic resonance feature-tracking analysis (CMR-FT) provides a quantitative assessment of myocardial contraction with potential for diagnostic and prognostic ability in a wide spectrum of diseases. Ebstein's anomaly (EA) is a rare congenital heart disease characterized by apical displacement of the tricuspid valve. However, it is also considered a disorder of development affecting the global right ventricular myocardium. Aim of our study is to describe the complex contractile mechanics of the functional right ventricle (RV) in patients affected by EA through CMR-FT. Methods: Fifty surgery-free EA patients who had undergone a complete CMR protocol at our institution between January 2017 and December 2020 were selected for the retrospective study. A historical control group of twenty-five healthy subjects was also included. CMR-FT analysis was performed at a dedicated workstation by manually tracing RV endo- end epicardial borders on steady-state-free-precession (SSFP) cine images. Strain values were calculated. Apical displacement of the tricuspid valve (TV) was measured on a 4-chamber cine image from the right atrio-ventricular junction to the functional annulus of the TV. Results: EA patients presented significantly impaired RV global radial strain (GRS) and global circumferential strain (GCS) compared to controls (P<0.0001 and P=0.0008, respectively). In a subgroup analysis, GRS was significantly compromised in patients with a severely displaced TV (>16 mm/m2) compared to milder forms (P=0.03) and to controls (P<0.0001). Among EA patients with a preserved ejection fraction, 12 (48%) vs. 6 (24%) controls had reduced both GRS and GCS. Conclusions: The contractile pattern of the functional RV in EA is characterised by prevalent alterations in the short-axis direction as indicated by reduced GRS and GCS. Strain values might be reduced prior to routine used functional parameters like RV ejection fraction (RVEF) and can possibly serve as an early predictor of myocardial dysfunction in EA patients.

Favorable Atrial Remodeling After Percutaneous Pulmonary Valve Implantation and Its Association With Changes in Exercise Capacity and Right Ventricular Function.

Background Right atrial (RA) dilatation and impaired right ventricular (RV) filling are common in patients with RV outflow tract dysfunction. We aimed to study potential correlations between atrial function with clinically relevant hemodynamic parameters and to assess the predictive impact of atrial performance on the recovery of exercise capacity and RV pump function after percutaneous pulmonary valve implantation (PPVI). Methods and Results Altogether, 105 patients with right ventricular outflow tract dysfunction (median age at PPVI, 19.2 years; range, 6.2-53.4 years) who underwent cardiac magnetic resonance imaging before and 6 months after PPVI were included. RA and left atrial maximal and minimal volumes as well as atrial passive and active emptying function were assessed from axial cine slices. RA emptying function was inversely related to invasive RV end-diastolic pressure, and RA passive emptying correlated significantly with peak oxygen uptake. After PPVI, a significant decrease in RA minimum volume was observed, whereas RA passive emptying function improved, and RA active emptying function decreased significantly. Patients with predominant right ventricular outflow tract stenosis showed more favorable changes in RA active and left atrial passive emptying than those with primary volume overload. None of the RA and left atrial emptying parameters was predictive for recovery of peak oxygen uptake or RV ejection fraction. Conclusions In patients with right ventricular outflow tract dysfunction, impaired RA emptying assessed by cardiac magnetic resonance imaging was associated with increased RV filling pressures and lower exercise capacity. PPVI leads to a reduction in RA size and improved passive RA emptying function. However, RA function was not associated with improved exercise performance and RV pump function.

Real-time CMR guidance for intracardiac and great vessel pressure mapping in patients with congenital heart disease using an MR conditional guidewire-results of 25 patients.

BACKGROUND: The aim of this study was to test a CE-certified MR-conditional guidewire to facilitate blood pressure measurement in cardiovascular magnetic resonance (CMR) using fluid-filled catheters in patients with congenital heart disease (CHD). The main purpose was to determine procedural success in a post market clinical follow-up (PMCF) for routine procedure in a diagnostic and interventional workflow. Real-time CMR provides high quality imaging without the risk of exposing the patient to X-rays, especially for patients with irregular heart anatomy and patients who are susceptible to radiation and iodinated contrast media. To date, the assessment of blood pressure gradients is not a common feature of CMR, as these gradients cannot be accurately evaluated in routine CMR. METHODS: Twenty-five CHD patients who were planned for combined clinical CMR and diagnostic and/or interventional catheterization were enrolled in the trial. Prior to inclusion, a specific procedure for catheterization in CMR was defined, encompassing the assessment of pressure and pressure gradients in the heart and great vessels. RESULTS: By the use of an MR-conditional guidewire we successfully measured specific pressure and pressure gradients in up to 92% of cases with liquid-filled catheters which were guided exclusively under CMR guidance. There were no guidewire-related adverse events, and guidewire guidance and manipulation of catheters were successful. CONCLUSIONS: Using a MR-conditional guidewire assists in easily reaching targets in the heart and great vessels and makes the catheter itself visible, so that invasive blood pressure assessment by CMR guidance with liquid-filled catheters can be improved. KEYWORDS: Cardiovascular magnetic resonance (CMR); congenital heart disease (CHD); cardiac catheterization; magnetic resonance; pressure; guidewire.

Aortic diameter assessment by cardiovascular magnetic resonance: do we really need contrast enhanced images?

BACKGROUND: Cardiovascular magnetic resonance (CMR) is widely used for aortic diameter assessment but there is no consensus on the sequence or cardiac cycle phase in which the measures should be taken. The most used sequence is contrast-enhanced-magnetic-resonance-angiography (angiography), usually non-ECG-triggered. An alternative is a navigated 3D-whole-heart-steady-state-free-precession sequence which is contrast-free and breath- and ECG-gated (mostly diastolic gating), producing very sharp anatomical rendering. Nonetheless, its routine use has not yet spread. Our aim was evaluating aortic diameters by a systolic-gated 3D and put additional effort in the validation of diastolic-gated 3D as alternative to angiography. METHODS: We retrospectively analysed 30 patients scheduled for routine Angiography. We measured the aorta at 9 standard positions by three different sequences (angiography, 3D-diastole and 3D-systole) and compared the diameters obtained by calculating the differences and by paired t-test analysis. RESULTS: Diameters by 3D-systole were larger than by 3D-diastole and angiography (P<0.01). In the ascending aorta we found the maximal differences between systole and diastole and between systole and angiography which were 1.7± SD 1.02 mm and 1.5± SD 1.07 mm respectively. There was no significant difference between diastolic and angiography measurements (mean difference 0.2± SD 0.16 mm, P not significant). CONCLUSIONS: Our results support the use of navigated 3D-whole-heart CMR to evaluate aortic diameters. Systolic-gated 3D produces larger diameter, especially in the ascending aorta.

Long-term outcomes of childhood onset Noonan compared to sarcomere hypertrophic cardiomyopathy.

BACKGROUND: To compare outcome and cardiac pathology between patients with Noonan syndrome (N-HCM) and sarcomere protein-associated (S-HCM) childhood onset hypertrophic cardiomyopathy (HCM). METHODS: Clinical data were recorded from medical charts. Primary endpoint was survival. Secondary endpoints were survival without hospitalization, without intervention or without arrhythmic events. Functional clinical status and results from genetic testing, imaging, electrocardiographic (ECG) studies, cardiopulmonary exercise testing (CPET) and histopathology were compared between groups. RESULTS: Childhood HCM was diagnosed in 29 N-HCM and 34 S-HCM patients. Follow-up time was greater than 10 years in more than half of all patients. Mortality was below 7% and not different between groups. Children with N-HCM presented at a younger age and there was less time of survival without hospitalization for heart failure or intervention in N-HCM compared to S-HCM patients. Clinical functional status improved over time in N-HCM patients. On long-term follow-up, left ventricular posterior wall thickness indexed to body surface area decreased in N-HCM and increased in S-HCM patients. There was a trend to lower risk for severe arrhythmic events in N-HCM patients and only S-HCM individuals received an implantable cardioverter-defibrillator. There were no differences between groups in ventricular function, ECG and CPET parameters. Myocardial fibrosis as assessed by histopathology of myocardial specimens and cardiovascular magnetic resonance with late gadolinium enhancement or T1 mapping was present in both groups. CONCLUSIONS: When compared to S-HCM patients, children with N-HCM have increased morbidity during early disease course, but favorable long-term outcome with low mortality, stagnation of myocardial hypertrophy, and low risk for malignant arrhythmias.

Diffuse fibrosis is common in the left, but not in the right ventricle in patients with transposition of the great arteries late after atrial switch operation.

In adult patients with transposition of the great arteries (TGA) late after atrial switch operation (AtSO), each of the ventricles is faced with a profoundly different pressure regimen from the one they are meant to support in normal conditions. The extent of diffuse fibrosis of the right ventricle (RV) and left ventricle (LV) in these patients remains incompletely investigated. Aim of this study was to quantify the degree of fibrosis of the unloaded LV and of the overloaded RV by determining the myocardial extracellular volume (ECV) with non-invasive techniques as T1 mapping. We determined ECV by cardiac magnetic resonance (CMR) in 10 patients (36.8 ± 5.3 years old) with TGA late after AtSO, without relevant pulmonary stenosis, by acquiring T1-maps of the myocardium before and 10 min after injection of Gadolinium-based contrast agent. ECV of the inferior wall (36% (33-41%)) and of the lateral wall (37% (35-39%)) of the LV was significantly increased compared to the ECV of the RV (27% (25-29%)), in both comparisons P < 0.0001. Long-time LV unloading following atrial switch procedures leads to severe myocardial fibrosis of the subpulmonary LV. T1 mapping CMR might be useful for selection of patients with atrial switch operation, in whom reestablishment of the LV as a systemic ventricle by staged arterial switch operation is planned. However larger studies and newer higher resolution methods for T1-mapping are needed to determine the role of ECV in the decision of a surgical intervention in this kind of population.

Clinical long-term outcome of septal myectomy for obstructive hypertrophic cardiomyopathy in infants.

OBJECTIVES: Surgical septal myectomy is performed to relieve left ventricular outflow tract narrowing in severe drug-refractory obstructive hypertrophic cardiomyopathy. The objective of this study was to assess the perioperative and long-term clinical outcome of this procedure performed during infancy. METHODS: Clinical, transthoracic echocardiographic, electrocardiographic, 24-h Holter, cardiopulmonary exercise test and genetic data were extracted by medical record review. A subset of patients underwent additional prospective detailed clinical evaluation including cardiac magnetic resonance imaging with contrast. RESULTS: Surgery was performed in 23 paediatric patients between 1978 and 2015 at the German Heart Centre Munich. Twelve patients had undergone surgery during infancy (≤ 1 year) (Group A), 11 between 1 and 18 years of age (Group B). The underlying genetic diagnosis was Noonan syndrome spectrum and non-syndromic hypertrophic cardiomyopathy. As compared to Group B, patients in Group A showed more concomitant cardiac procedures and received more homologous transfusions. One perioperative death occurred in Group A, and none in Group B. Two patients in Group A but no patient in Group B required redo septal myectomy. The long-term clinical outcome was similar between the 2 groups. One patient in Group B required cardioverter-defibrillator/pacemaker implantation for higher degree atrioventricular block and none in Group A. There was no evidence of differences in myocardial fibrosis between groups on long-term follow-up magnetic resonance imaging. CONCLUSIONS: Surgical septal myectomy can be performed safely during infancy with favourable perioperative and long-term clinical outcome but with a trend towards a higher reoperation rate later in life.