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Background: Myocarditis is challenging to diagnose due to its varied presentations. Endomyocardial biopsy is the gold standard for diagnosis, but its invasive nature has led to alternative non-invasive modalities, notably cardiovascular magnetic resonance (CMR). Identifying the precise aetiology of myocarditis is crucial for effective treatment, yet extra-cardiac causes are often overlooked. In this paper, we spotlight the underexplored role of CMR in diagnosing extra-cardiac aetiologies, utilizing three insightful cases for illustration., Case Summary: The first case is a 31-year-old patient with myocarditis secondary to a pyogenic liver abscess, identified through CMR, who improved after abscess drainage. The second case involves a 54-year-old patient with myocarditis attributed to adult T-cell leukaemia-lymphoma, with the loco-regional thickening process identified thanks to CMR. This patient had an unfavourable disease progression due to the underlying malignancy. The third case concerns a 23-year-old patient suffering from myocarditis associated with pneumonia, again illustrated effectively through CMR imaging, who recovered after antibiotic treatment., Discussion: These cases underline the overlooked potential of CMR in diagnosing extra-cardiac aetiologies of myocarditis, even though such causes are rare. Despite current guidelines recognizing the importance of identifying the aetiology of myocarditis, they do not explicitly address the role of CMR in diagnosing extra-cardiac aetiologies. This article, therefore, proposes that future guidelines could emphasize the utility of CMR in exploring these causes, potentially leading to more accurate diagnoses and improved patient outcomes. It also advocates for a comprehensive, multidisciplinary approach to myocarditis diagnosis, encouraging vigilance for potential loco-regional causes, and calls for further research in this area., Competing Interests: Conflict of interest: None declared., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Objectives: To evaluate the precise dimensions of the normal aortic root, especially the true aortic annulus, during the cardiac cycle using an innovative reconstruction method based on multiphase cardiac computed tomography and to assess the feasibility and the reproducibility of this method for aortic root analysis., Methods: Between January 2019 and June 2021, 30 optimal consecutive ECG-gated multiphase cardiac computed tomography of patients with normal tricuspid aortic valve were analysed using an in-house software. Aortic annulus border was pinpointed on 9 reconstructed planes and the 3D coordinates of the 18 consecutive points were interpolated into a 3D curve using a cubic spline. Three additional planes were generated at the level of the left ventricular outflow tract, the level of the Valsalva sinus and the level of the sinotubular junction. This procedure was repeated for all the 10 temporal phases of the RR interval., Results: The aortic annulus mean 3D and 2D areas were 7.67 ± 1.51 and 5.16 ± 1.40 cm2, respectively. The mean 2D diameter was 2.51 ± 0.23 cm. The mean global area expansion was 11.8 ± 3.5% and the mean perimeter expansion of 7.1 ± 2.6%. During the cardiac cycle, the left ventricle outflow tract expands, reaching its maximum surface at the end of diastole, followed by the aortic annulus, the Valsalva sinuses and the sinotubular junction. The aorta changes from a clover-shaped cone during diastole to more cylindrical shape during systole. Compared to the 3D measurements, the analysis of the virtual basal ring significantly underestimates the annulus area, perimeter, and mean diameter., Conclusions: 4D morphometric analysis enables to have a precise and reproducible evaluation of the aortic annulus. The aortic annulus and root are deformable structures that undergo a unique expansion sequence during the cardiac cycle which should be considered for procedural planning., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: Patients with Cushing's syndrome have left ventricular (LV) hypertrophy and dysfunction on echocardiography, but echo-based measurements may have limited accuracy in obese patients. No data are available on right ventricular (RV) and left atrial (LA) size and function in these patients., Objectives: The objective of the study was to evaluate LV, RV, and LA structure and function in patients with Cushing's syndrome by means of cardiac magnetic resonance, currently the reference modality in assessment of cardiac geometry and function., Methods: Eighteen patients with active Cushing's syndrome and 18 volunteers matched for age, sex, and body mass index were studied by cardiac magnetic resonance. The imaging was repeated in the patients 6 months (range 2-12 mo) after the treatment of hypercortisolism., Results: Compared with controls, patients with Cushing's syndrome had lower LV, RV, and LA ejection fractions (P < .001 for all) and increased end-diastolic LV segmental thickness (P < .001). Treatment of hypercortisolism was associated with an improvement in ventricular and atrial systolic performance, as reflected by a 15% increase in the LV ejection fraction (P = .029), a 45% increase in the LA ejection fraction (P < .001), and an 11% increase in the RV ejection fraction (P = NS). After treatment, the LV mass index and end-diastolic LV mass to volume ratio decreased by 17% (P < .001) and 10% (P = .002), respectively. None of the patients had late gadolinium myocardial enhancement., Conclusion: Cushing's syndrome is associated with subclinical biventricular and LA systolic dysfunctions that are reversible after treatment. Despite skeletal muscle atrophy, Cushing's syndrome patients have an increased LV mass, reversible upon correction of hypercortisolism.

Aims: Fabry disease is a lysosomal storage disorder due to deficient alpha-galactosidase A activity, characterised by glycosphingolipids deposition in tissues. Patients have a common arterial involvement and contract progressive renal and cardiac disease. Although short-term effects of enzyme replacement therapy (ERT) on target organs have been established, no data are available on the long-term outcome., Methods and Results: We studied the effects of ERT (agalsidase beta, 1 mg/kg/14 days) on arterial and cardiac structure and function during a longitudinal study beginning in 1999, with 4.5 ± 0.4 years follow-up (four visits) in 30 patients (age: 33 ± 12 years). In addition, we studied 16 untreated Fabry patients during 2.6 ± 1.6 years (two visits). Aortic stiffness was determined by carotid-femoral pulse wave velocity, central pulse pressure by aplanation tonometry, and carotid and radial intima-media thickness and diameter by high definition echotracking device. Left ventricular mass was determined by MRI. A significant decrease in aortic stiffness (-0.56 ± 0.13 m/s/yr, p = 0.0002) was observed after ERT whereas central pulse pressure did not change. Carotid intima-media thickness (IMT) increased (+18 ± 6 µm/yr; p < 0.005) whereas radial IMT remained stable. Radial artery diameter decreased (-50 ± 20 µm/years, p < 0.05) whereas carotid diameter did not change. Carotid circumferential wall stress was reduced (-1.7 ± 0.6 kPa/yrs, p < 0.01). Left ventricular mass index significantly decreased (-7.8 ± 2.3 g/m(2)/yr, p < 0.005)., Conclusion: A sustained reduction in aortic stiffness and left ventricular hypertrophy, and a limited radial artery wall thickening were observed after long-term enzyme replacement therapy. There was no significant benefit of treatment on carotid hypertrophy.

Aims: To evaluate the feasibility and the incidence of complete heart block (CHB) after non-surgical septal myocardial reduction by coil embolization in hypertrophic obstructive cardiomyopathy (HOCM)., Methods and Results: Twenty patients with HOCM and drug-refractory symptoms underwent non-surgical myocardial septal reduction by coil embolization with detachable coils. Occlusion of septal perforator branches was successfully performed in all patients. We detected neither ventricular tachycardia nor CHB. One patient presented an interventricular septal defect after the procedure, and died 19 days later. Cardiac magnetic resonance imaging showed, in all patients, an increase in areas of hyperenhancement in the interventricular septum (IVS) compared with baseline. At 6-month follow-up, NYHA functional class and peak oxygen consumption were significantly improved compared with baseline (14.8 +/- 4.5 vs. 18.5 +/- 4.5 mL/kg/min; P = 0.001, respectively). Echocardiography showed a significant reduction of the IVS thickness and left ventricular outflow tract gradient (21 +/- 3 vs. 17 +/- 4 mm, P < 0.0001; 80 +/- 29 to 35 +/- 29 mmHg, P < 0.0001, respectively)., Conclusion: The results of this pilot non-randomized study suggest that non-surgical septal myocardial reduction by coil embolization in HOCM is feasible and does not induce CHB. Larger studies, ideally with a randomized comparison between coil embolization and alcohol septal ablation, are warranted.

Aims: To investigate the role of the geometry of the aortic arch in resting hypertension after successful repair of coarctation (CoA)., Methods and Results: 105 patients (15.3+/-6 years) with successful repair of aortic CoA underwent blood pressure (BP) measurements at rest and magnetic resonance imaging (MRI) of the aortic arch and left ventricle. Three categories of aortic arch shape were defined based on the global geometry of the aortic arch: gothic, crenel and normal. The ratio of the maximum height/transverse diameter of the aortic arch (A/T), the percentage of residual stenosis (RS), the growth index of the aortic arch segments (GIA), and the left ventricular mass index (LVMI) were calculated. Twenty-seven (25.7%) patients had hypertension (HT). HT was more frequent in patients with gothic arch geometry (20/44, 45.5%) than in crenel geometry (5/18, 27.8%) and in normal geometry (2/43, 4.6%) (P<0.001). Resting systolic BP and LVMI were significantly higher with gothic arch geometry. In multivariate analysis, the only correlates to HT, to level of systolic BP or to LVMI were A/T and gothic arch geometry., Conclusion: Gothic geometry of the aortic arch is associated with resting hypertension in patients having undergone successful repair of CoA. This raises concern about methods to harmoniously repair CoA in order to prevent or delay rest hypertension in adulthood.

Objective: 'Classical' repair of Ebstein's anomaly is usually performed with transverse plication of the atrialized chamber. However, the anterior leaflet has restricted motion which is an important factor of the tricuspid valve insufficiency. We studied the long term results of mobilization of the anterior leaflet associated with longitudinal plication of the right ventricule., Methods: From 1980 to July 2002, 191 patients (mean age 24.4+/-15 years (1-65)) were operated on. Anterior leaflet function was assessed on pre-op echocardiography and on surgical examination. Conservative surgery was possible in 187 patients (98%) and included mobilization of the anterior leaflet, longitudinal plication of the right ventricle and prosthetic annuloplasty in adults. Bidirectional cavo-pulmonary shunt was associated in 60 patients. Four patients had valve replacement., Results: Hospital mortality occurred in 18 patients: 9% (95%CL: 6-15%) due to right ventricle (RV) failure in nine patients. Mean follow-up was 6.4 years (0.07-22). Actuarial survival was 82% at 20 years. Tricuspid valve insufficiency was 1 or 2+ in 80% of the cases. Reoperation occurred in 8% (16 patients). A successful second repair was obtained in ten patients. Electron beam computerized tomography (20 patients) demonstrated improved left ventricle ejection fraction 56-66% (P<0.05). Supraventricular tachycardia and pre-excitation syndromes were reduced from 23 to 5%., Conclusion: Conservative surgery is indicated for all symptomatic patients. The incidence of valve repair is high when leaflet mobilization is performed. Valve replacement can be avoided in most cases. Functional and hemodynamic results are excellent.

This study firstly determined the accuracy of left ventricular mass (LVM) determination by electron beam CT in normal and distorted human cadaver hearts, and secondly the in vivo reproducibility of these measurements, in normal subjects and patients with various cardiac diseases, over a wide range of LVM (104-631 g). In cadaver hearts, there was a close correlation between electron beam CT and autopsy LVM values: r = 0.97, SEE = 16 g. Intra-observer variability: results of LVM measurements obtained in cadaver hearts by observer 1 and in in vivo studies by observer 2 were y = 1.001 x -1.34, r = 0.99, SEB = 6 g and y = 0.99 x +3.78, r = 0.99, SEE = 19 g, respectively. Inter-observer variability: results in cadaver and in vivo studies were y = 1.05 x -5.94, r = 0.98, SEE = 14 g and y = 1.13 x -23, r = 0.99, SEE = 28 g, respectively. Thus, these results over a wide LVM range provide extensive validation of electron beam CT measurements in hearts with marked left ventricular distortion. This method should play a valuable role, within the scope of clinical research, in assessing left ventricular remodelling in various conditions in which echocardiographic data are inaccurate.

An increasing number of patients with the acquired immunodeficiency syndrome (AIDS) and cardiac lymphoma have been documented. Antemortem diagnosis of cardiac non-Hodgkin lymphoma in AIDS is difficult because of the non-specificity of the clinical findings. Rapid progression of cardiac dysfunction is common after symptoms appear. We report the case of a patient with AIDS and cardiac lymphoma revealed by an atrial flutter.