We aimed to evaluate the relationship between mitral annular disjunction (MAD) severity and myocardial interstitial fibrosis at the left ventricular (LV) base in patients with mitral valve prolapse (MVP), and to assess the association between severity of interstitial fibrosis and the occurrence of ventricular arrhythmic events. In MVP, MAD has been associated with myocardial replacement fibrosis and arrhythmia, but the importance of interstitial fibrosis remains unknown. In this retrospective study, 30 patients with MVP and MAD (MVP–MAD) underwent cardiovascular magnetic resonance (CMR) with assessment of MAD length, late gadolinium enhancement (LGE), and basal segments myocardial extracellular volume (ECVsyn). The control group included 14 patients with mitral regurgitation (MR) but no MAD (MR-NoMAD) and 10 patients with normal CMR (NoMR-NoMAD). Fifteen MVP–MAD patients underwent 24 h-Holter monitoring. LGE was observed in 47% of MVP–MAD patients and was absent in all controls. ECVsyn was higher in MVP–MAD (30 ± 3% vs 24 ± 3% MR-NoMAD, p < 0.001 and vs 24 ± 2% NoMR-NoMAD, p < 0.001), even in MVP–MAD patients without LGE (29 ± 3% vs 24 ± 3%, p < 0.001 and vs 24 ± 2%, p < 0.001, respectively). MAD length correlated with ECVsyn (rho = 0.61, p < 0.001), but not with LGE extent. Four patients had history of out-of-hospital cardiac arrest; LGE and ECVsyn were equally performant to identify those high-risk patients, area under the receiver operating characteristic (ROC) curve 0.81 vs 0.83, p = 0.84). Among patients with Holter, 87% had complex ventricular arrhythmia. ECVsyn was above the cut-off value in all while only 53% had LGE. Increase in ECVsyn, a marker of interstitial fibrosis, occurs in MVP–MAD even in the absence of LGE, and was correlated with MAD length and increased risk of out-of-hospital cardiac arrest. ECV should be includedin the CMR examination of MVP patients in an effort to better assess fibrous remodelling as it may provide additional value beyond the assessment of LGE in the arrhythmic risk stratification.

Aims: To investigate the incidence of major adverse ventricular arrhythmias and related events (MAREs) and to develop a stratification tool predicting MAREs in adults with a systemic right ventricle (sRV). Methods and results: In a multicentre approach, all adults (≥16 years old) with a sRV undergoing follow-up between 2000 and 2018 were identified. The incidence of MAREs, defined as sudden cardiac death, sustained ventricular tachycardia, and appropriate implantable cardioverter-defibrillator (ICD) therapy, was analysed. The association of MAREs with clinical, electrical, and echocardiographic parameters was evaluated. A total of 1184 patients (median age 27.1 years; interquartile range 19.9–34.9 years; 59% male; 70% with atrial switch repair for D-transposition of the great arteries) were included. The incidence of MAREs was 6.3 per 1000 patient-years. On multivariate analysis, age, history of heart failure, syncope, QRS duration, severe sRV dysfunction and at least moderate left ventricular outflow tract obstruction were retained in the final model with a C-index of 0.78 [95% confidence interval (CI) 0.72–0.83] and a calibration slope of 0.93 (95% CI 0.64–1.21). For every five ICDs implanted in patients with a 5-year MARE risk >10%, one patient may potentially be spared from a MARE. Conclusion: Sudden cardiac death remains a devastating cause of death in a contemporary adult cohort with a sRV. A prediction model based on clinical, electrocardiographic, and echocardiographic parameters was devised to estimate MARE risk and to identify high-risk patients who may benefit from primary prevention ICD implantation.

Purpose: In this work, we integrated the pilot tone (PT) navigation system into a reconstruction framework for respiratory and cardiac motion‐resolved 5D flow. We tested the hypotheses that PT would provide equivalent respiratory curves, cardiac triggers, and corresponding flow measurements to a previously established self‐gating (SG) technique while being independent from changes to the acquisition parameters.Methods: Fifteen volunteers and 9 patients were scanned with a free‐running 5D flow sequence, with PT integrated. Respiratory curves and cardiac triggers from PT and SG were compared across all subjects. Flow measurements from 5D flow reconstructions using both PT and SG were compared to each other and to a reference electrocardiogram‐gated and respiratory triggered 4D flow acquisition. Radial trajectories with variable readouts per interleave were also tested in 1 subject to compare cardiac trigger quality between PT and SG.Results: The correlation between PT and SG respiratory curves were 0.95 ± 0.06 for volunteers and 0.95 ± 0.04 for patients. Heartbeat duration measurements in volunteers and patients showed a bias to electrocardiogram measurements of, respectively, 0.16 ± 64.94 ms and 0.01 ± 39.29 ms for PT versus electrocardiogram and of 0.24 ± 63.68 ms and 0.09 ± 32.79 ms for SG versus electrocardiogram. No significant differences were reported for the flow measurements between 5D flow PT and from 5D flow SG. A decrease in the cardiac triggering quality of SG was observed for increasing readouts per interleave, whereas PT quality remained constant.Conclusion: PT has been successfully integrated in 5D flow MRI and has shown equivalent results to the previously described 5D flow SG technique, while being completely acquisition‐independent.

For free-breathing cardiovascular magnetic resonance (CMR), the self-navigation technique recently emerged, which is expected to deliver high-quality data with a high success rate. The purpose of this study was to test the hypothesis that self-navigated 3D-CMR enables the reliable assessment of cardiovascular anatomy in patients with congenital heart disease (CHD) and to define factors that affect image quality. CHD patients ≥2 years-old and referred for CMR for initial assessment or for a follow-up study were included to undergo a free-breathing self-navigated 3D CMR at 1.5T. Performance criteria were: correct description of cardiac segmental anatomy, overall image quality, coronary artery visibility, and reproducibility of great vessels diameter measurements. Factors associated with insufficient image quality were identified using multivariate logistic regression. Self-navigated CMR was performed in 105 patients (55 % male, 23 ± 12y). Correct segmental description was achieved in 93 % and 96 % for observer 1 and 2, respectively. Diagnostic quality was obtained in 90 % of examinations, and it increased to 94 % if contrast-enhanced. Left anterior descending, circumflex, and right coronary arteries were visualized in 93 %, 87 % and 98 %, respectively. Younger age, higher heart rate, lower ejection fraction, and lack of contrast medium were independently associated with reduced image quality. However, a similar rate of diagnostic image quality was obtained in children and adults. In patients with CHD, self-navigated free-breathing CMR provides high-resolution 3D visualization of the heart and great vessels with excellent robustness.

Hypertensive disorders of pregnancy (HDP) occur in 10% of pregnancies in the general population, pre-eclampsia specifically in 3–5%. Hypertensive disorders of pregnancy may have a high prevalence in, and be poorly tolerated by, women with heart disease. The prevalence and outcomes of HDP (chronic hypertension, gestational hypertension or pre-eclampsia) were assessed in the ESC EORP ROPAC (n = 5739), a worldwide prospective registry of pregnancies in women with heart disease. The overall prevalence of HDP was 10.3%, made up of chronic hypertension (5.9%), gestational hypertension (1.3%), and pre-eclampsia (3%), with significant differences between the types of underlying heart disease (P < 0.05). Pre-eclampsia rates were highest in women with pulmonary arterial hypertension (PAH) (11.1%), cardiomyopathy (CMP) (7.1%), and ischaemic heart disease (IHD) (6.3%). Maternal mortality was 1.4 and 0.6% in women with vs. without HDP (P = 0.04), and even 3.5% in those with pre-eclampsia. All pre-eclampsia-related deaths were post-partum and 50% were due to heart failure. Heart failure occurred in 18.5 vs. 10.6% of women with vs. without HDP (P < 0.001) and in 29.1% of those with pre-eclampsia. Perinatal mortality was 3.1 vs. 1.7% in women with vs. without HDP (P = 0.019) and 4.7% in those with pre-eclampsia. Hypertensive disorders of pregnancy and pre-eclampsia rates were higher in women with CMP, IHD, and PAH than in the general population. Adverse outcomes were increased in women with HDP, and maternal mortality was strikingly high in women with pre-eclampsia. The combination of HDP and heart disease should prompt close surveillance in a multidisciplinary context and the diagnosis of pre-eclampsia requires hospital admission and continued monitoring during the post-partum period. Hypertensive disorders of pregnancy (HDP) in women with heart disease.

BACKGROUND: Pericardial late gadolinium enhancement (LGE) is usually associated with active pericarditis, but it is not infrequently found in patients after cardiac surgery even a long time after the intervention. The clinical relevance of this finding and its histological correlates are unknown. We sought to determine the prevalence of chronic pericardial LGE in patients after cardiac surgery. METHODS: All consecutive patients with previous cardiac surgery, who were referred to cardiovascular magnetic resonance between January 2017 and December 2021 were enrolled in the study. Cardiovascular magnetic resonance examination protocol was adapted to clinical indication but always included standard LGE acquisitions. Two independent observers blinded to clinical data assessed the presence of pericardial enhancement on LGE sequences. Fifteen patients underwent cardiac reintervention and pericardial biopsies were obtained. The primary study end point was to assess the prevalence of pericardial enhancement after cardiac surgery and identify possible determinants. The secondary end point was to correlate pericardial enhancement with clinical symptoms and histopathology. RESULTS: Two hundred four patients were included in the study. The median time between surgery and cardiovascular magnetic resonance was 160 months (35–226 months). Pericardial LGE was observed in 90 patients (44%). All patients were asymptomatic, and no specific treatment for pericarditis was started. All patients remained asymptomatic at a 1-year clinical follow-up. Pericardial LGE was significantly correlated with the number of previous surgeries ( P =0.03). Pericardial fibrosis was detected in all 15 pericardial biopsy specimens; pericardial LGE was present in 7 patients (47%) who underwent biopsy. Histological signs of low-grade inflammation were detected in 6 patients (40%) with severe, circumferential pericardial LGE but in no patient without pericardial enhancement. CONCLUSIONS: Pericardial LGE is a frequent finding even several years after cardiac surgery. Its histological correlate is a chronic subclinical post–pericardiotomy inflammation.