Prognostic value of CMR-derived extracellular volume in AL amyloidosis: a multicenter study.

BACKGROUND: This study aimed to assess the prognostic value of cardiac magnetic resonance (CMR) variables and compare them with biological and echocardiographic markers in patients with AL cardiac amyloidosis (CA). METHODS: We conducted a prospective study across three tertiary centres, where patients underwent clinical examination, blood tests, echocardiography, and CMR. The primary endpoint was all-cause mortality. RESULTS: A total of 176 patients with AL CA were included, with a median age of 68 years (IQR 58-75). According to the 2004 Mayo Clinic staging, 121 patients (69%) were in stage 3. During a median follow-up of 22 months (IQR 8-48), 45 patients died, and 55 were hospitalized for heart failure. Patients who died had higher NT-proBNP and troponin levels, and lower LVEF, cardiac output, and longitudinal strain. Among CMR variables, extracellular volume (ECV) was most strongly associated with all-cause mortality. In multivariate Cox models, including Mayo Clinic staging, ECV ≥ 0.45 was independently associated with mortality (HR 2.36, CI 95% 1.47-5.60) and also with heart failure hospitalizations (HR 4.10, 95%CI 2.15-8.8). CONCLUSION: ECV is a powerful predictor of outcomes in AL CA, providing additional prognostic value on top of Mayo Clinic staging.

Fatty acid composition MRI of epicardial adipose tissue: Methods and detection of proinflammatory biomarkers in ST-segment elevation myocardial infarction patients.

PURPOSE: To develop a method for quantifying the fatty acid composition (FAC) of human epicardial adipose tissue (EAT) using accelerated MRI and identify its potential for detecting proinflammatory biomarkers in patients with ST-segment elevation myocardial infarction (STEMI). METHODS: A multi-echo radial gradient-echo sequence was developed for accelerated imaging during a breath hold using a locally low-rank denoising technique to reconstruct undersampled images. FAC mapping was achieved by fitting the multi-echo images to a multi-resonance complex signal model based on triglyceride characterization. Validation of the method was assessed using a phantom comprised of multiple oils. In vivo imaging was performed in STEMI patients (n = 21; 14 males/seven females). FAC was quantified in EAT, subcutaneous AT, and abdominal visceral AT. RESULTS: Phantom validation demonstrated strong correlations (r > 0.97) and statistical significance (p < 0.0001) between measured and reference proton density fat fraction and FAC values. In vivo imaging of STEMI patients revealed a distinct EAT FAC profile compared to subcutaneous AT and abdominal visceral AT. EAT FAC parameters had significant correlations with left ventricular (LV) end-diastolic volume index (p < 0.05), LV end-systolic volume index (p < 0.05), and LV mass index (p < 0.05). CONCLUSIONS: Accelerated MRI enabled accurate quantification of human EAT FAC. The relationships between the EAT FAC profile and LV structure and function in STEMI patients suggest the potential of EAT FAC MRI as a biomarker for adipose tissue quality and inflammatory status in cardiovascular disease.

Free-Running Cardiac and Respiratory Motion-Resolved Imaging: A Paradigm Shift for Managing Motion in Cardiac MRI?

Cardiac magnetic resonance imaging (MRI) is widely used for non-invasive assessment of cardiac morphology, function, and tissue characteristics due to its exquisite soft-tissue contrast. However, it remains time-consuming and requires proficiency, making it costly and limiting its widespread use. Traditional cardiac MRI is inefficient as signal acquisition is often limited to specific cardiac phases and requires complex view planning, parameter adjustments, and management of both respiratory and cardiac motion. Recent efforts have aimed to make cardiac MRI more efficient and accessible. Among these innovations, the free-running framework enables 5D whole-heart imaging without the need for an electrocardiogram signal, respiratory breath-holding, or complex planning. It uses a fully self-gated approach to extract cardiac and respiratory signals directly from the acquired image data, allowing for more efficient coverage in time and space without the need for electrocardiogram gating, triggering, navigators, or breath-holds. This review provides a comprehensive overview of the free-running framework, detailing its history, concepts, recent improvements, and clinical applications.

Mesalazine-induced myocarditis in a patient with ulcerative colitis: a case report.

Background: Mesalazine is an established first-line therapy for inflammatory bowel disease (IBD) and remains the mainstay of treatment for mild to moderate ulcerative colitis (UC). Both mesalazine and UC are rare but recognized causes of myopericarditis. Cardiac magnetic resonance (CMR) is a non-invasive method of assessing for myopericarditis. This case reports highlights the importance of early CMR in diagnosis, and management of myocarditis in a patient with IBD. Case summary: A 28-year-old male was admitted with a 2-day history of chest pain. Three weeks prior to this presentation, the patient was initiated on mesalazine for UC. Serum troponin T and C-reactive protein were elevated. An echocardiogram showed borderline low left ventricular systolic function (LVEF = 50-55%). A CMR showed extensive patchy late gadolinium enhancement (LGE) in the mid to epicardial basal and mid lateral wall. The findings were consistent with acute myocarditis, and a working diagnosis of mesalazine-induced myocarditis was made. Mesalazine was stopped and guideline-directed anti-inflammatories initiated. Oral prednisolone was also introduced for IBD control. Follow-up CMR at four months showed near complete resolution of LGE. Discussion: Myocarditis in the context of IBD may be infective, immune-mediated or due to mesalazine hypersensitivity. Histological conformation was not available in this case. This case report highlights the importance of access to early CMR in order establish the diagnosis and withdrawal of the culprit medication. In the majority of cases, CMR will replace the need for endomyocardial biopsy; however, this may still be required in the most severe cases.

Imaging for the diagnosis of acute myocarditis: can artificial intelligence improve diagnostic performance?

Myocarditis is a cardiovascular disease characterised by inflammation of the heart muscle which can lead to heart failure. There is heterogeneity in the mode of presentation, underlying aetiologies, and clinical outcome with impact on a wide range of age groups which lead to diagnostic challenges. Cardiovascular magnetic resonance (CMR) is the preferred imaging modality in the diagnostic work-up of those with acute myocarditis. There is a need for systematic analytical approaches to improve diagnosis. Artificial intelligence (AI) and machine learning (ML) are increasingly used in CMR and has been shown to match human diagnostic performance in multiple disease categories. In this review article, we will describe the role of CMR in the diagnosis of acute myocarditis followed by a literature review on the applications of AI and ML to diagnose acute myocarditis. Only a few papers were identified with limitations in cases and control size and a lack of detail regarding cohort characteristics in addition to the absence of relevant cardiovascular disease controls. Furthermore, often CMR datasets did not include contemporary tissue characterisation parameters such as T1 and T2 mapping techniques, which are central to the diagnosis of acute myocarditis. Future work may include the use of explainability tools to enhance our confidence and understanding of the machine learning models with large, better characterised cohorts and clinical context improving the diagnosis of acute myocarditis.

Multi-modality imaging to assess rheumatic mitral stenosis severity.

Multi-modality imaging is the recommended approach to assess the severity of valvular heart diseases. Rheumatic mitral stenosis (MS), however, has yet to benefit from this approach. The aim of this study is to assess the added value of cardiac MRI (CMR) and computed tomography (CT) calcium score in assessing severity of MS when compared to 3D echocardiography. Patients with MS in sinus rhythm were included. Both CMR and 3D echo assessments of the mitral valve were performed. Subsequently, three radiologists and three cardiologists independently measured mitral valve area (MVA). In addition, CT of the mitral valve was conducted. Mitral calcium score was calculated according the Agatston method. A total of 41 patients were included. CMR significantly overestimated MVA when compared to 3D echo MVA regardless of the investigator [F (1, 40) = 23.3, p < 0.001, η2 = 0.36]. The more severe the MS, the greater the overestimation by CMR compared to 3D echo. Regarding CT of the mitral valve, 25 (61%) patients had an undetectable calcium. There was no significant difference in CT calcium scores between severe and non-severe MS (74 ± 282 HU vs. 65 ± 210 HU, p = 0.9). MVA measurement by CMR is overestimated when compared to 3D echo. Additionally, mitral valve calcium score is not correlated to MS severity.

Flow quantification within the aortic ejection tract using 4D flow cardiac MRI in patients with bicuspid aortic valve: Implications for the assessment of aortic regurgitation.

PURPOSE: The purpose of this study was to evaluate the performance of four-dimensional (4D) flow cardiac MRI in quantifying aortic flow in patients with bicuspid aortic valve (BAV). MATERIALS AND METHODS: Patients with BAV who underwent transthoracic echocardiography (TTE) and 4D flow cardiac MRI were prospectively included. Aortic flow was quantified using two-dimensional phase contrast velocimetry at the sinotubular junction and in the ascending aorta and using 4D flow in the regurgitant jet, in the left ventricular outflow tract, at the aortic annulus, the sinotubular junction, and the ascending aorta, with or without anatomical tracking. Flow quantification was compared with ventricular volumes, pulmonary flow using Pearson correlation test, bias and limits of agreement (LOA) using Bland Altman method, and with multiparametric transthoracic echocardiography quantification using weighted kappa test. RESULTS: Eighty-eight patients (63 men, 25 women) with a mean age of 50.5 ± 14.8 (standard deviation) years (age range: 20.8-78.3) were included. Changes in flow with or without tracking were modest (< 5 mL). The best correlation was obtained at the aortic annulus for forward volume (r = 0.84; LOA [-28.4; 25.3] mL) and at the regurgitant jet and sinotubular junction for regurgitant volume (r = 0.68; LOA [-27.8; 33.8] and r = 0.69; LOA [-28.6; 24.2] mL). A combined approach for regurgitant fraction and net volume calculations using forward volume measured at ANN and regurgitant volume at sinotubular junction performed better than each level taken separately (r = 0.90; LOA [-20.7; 10.0] mL and r = 0.48, LOA [-33.8; 33.4] %). The agreement between transthoracic echocardiography and 4D flow cardiac MRI for aortic regurgitation grading was poor (kappa, 0.13 to 0.42). CONCLUSION: In patients with BAV, aortic flow quantification by 4D flow cardiac MRI is the most accurate at the annulus for the forward volume, and at the sinotubular junction or directly in the jet for the regurgitant volume.

Hydroxychloroquine-induced cardiomyopathy: role of cardiac magnetic resonance for the diagnosis and follow-up of a very rare entity-a case report.

Background: Hydroxychloroquine (HCQ) is a disease-modifying antirheumatic used in rheumatological diseases such as systemic lupus erythematosus. Long-term exposure to HCQ results in drug accumulation and predisposes to adverse effects. Case summary: We present the case of a 45-year-old woman with long-term treatment with HCQ who presented to the Emergency Department with acute heart failure. Transthoracic echocardiogram, previously normal, showed severe biventricular hypertrophy and biventricular systolic dysfunction. Cardiac magnetic resonance (CMR) confirmed the previous findings and showed elevated native T1 and T2 values, elevated extracellular volume, and extensive mid-wall late gadolinium enhancement (LGE). Infiltrative cardiomyopathy was suspected, and endomyocardial biopsy performed. Light microscopy showed myocyte hypertrophy and vacuolar change and absence of lymphocytic inflammatory infiltrates. The diagnosis of HCQ-induced cardiomyopathy was established, and the drug was withdrawn. A CMR performed 1 year later showed normal systolic function of both ventricles and normalization of T2 values, reflecting resolution of myocardial oedema. However, severe hypertrophy, elevated native T1 values, and LGE persisted. Discussion: Our case shows that although discontinuation of the drug stops the progression of the disease, established myocardial structural damage persists. Early diagnosis of this entity is therefore essential to improve prognosis.

Comparison of left ventricular mass and wall thickness between cardiac computed tomography angiography and cardiac magnetic resonance imaging using machine learning algorithms.

Aims: Cardiac magnetic resonance imaging (MRI) is the gold standard in the assessment of left ventricle (LV) mass and wall thickness. In recent years, cardiac computed tomography angiography (CCTA) has gained widespread usage as an imaging modality. Despite this, limited previous investigations have specifically addressed the potential of CCTA as an alternative modality for quantitative LV assessment. The aim of this study was to compare CCTA derived LV mass and wall thickness with cardiac MRI utilizing machine learning algorithms. Methods and results: Fifty-seven participants who underwent both CCTA and cardiac MRI were identified. LV mass and wall thickness was calculated using LV contours which were automatically placed using in-house developed machine learning models. Pearson's correlation coefficients were calculated along with Bland-Altman plots to assess the agreement between the LV mass and wall thickness per region on CCTA and cardiac MRI. Inter-observer correlations were tested using Pearson's correlation coefficient. Average LV mass and wall thickness for CCTA and cardiac MRI were 127 g, 128 g, 7, and 8 mm, respectively. Bland-Altman plots demonstrated mean differences and corresponding 95% limits of agreement of -1.26 (25.06; -27.58) and -0.57 (1.78; -2.92), for LV mass and average LV wall thickness, respectively. Mean differences and corresponding 95% limits of agreement for wall thickness per region were -0.75 (1.34; -2.83), -0.58 (2.14; -3.30), and -0.29 (3.21; -3.79) for the basal, mid, and apical regions, respectively. Inter-observer correlations were excellent. Conclusion: Quantitative assessment of LV mass and wall thickness on CCTA using machine learning algorithms seems feasible and shows good agreement with cardiac MRI.

Diagnostic performance and relationships of structural parameters and strain components for the diagnosis of cardiac amyloidosis with MRI.

PURPOSE: The purpose of this study was to evaluate the diagnostic performance and relationships of cardiac MRI structural parameters and strain components in patients with cardiac amyloidosis (CA) and to estimate the capabilities of these variables to discriminate between CA and non-amyloid cardiac hypertrophy (NACH). MATERIALS AND METHODS: Seventy patients with CA (56 men; mean age, 76 ± 10 [standard deviation] years) and 32 patients (19 men; mean age, 63 ± 10 [standard deviation] years) with NACH underwent cardiac MRI. Feature tracking (FT) global longitudinal strain (GLS), radial strain (GRS), circumferential strain (GCS), strain AB ratio (apical strain divided by basal strain), myocardial T1, myocardial T2 and extracellular volume (ECV) were calculated. Comparisons between patients with CA and those with NACH were made using Mann-Whitney rank sum test. The ability of each variable to discriminate between CA and NACH was estimated using area under the receiver operating characteristic curve (AUC). RESULTS: Patients with CA had higher median GLS (-7.0% [Q1, -9.0; Q3, -5.0]), higher median GCS (-12.0% [Q1, -15.0; Q3, -9.0]), and lower median GRS (16.5% [Q1, 13.0; Q3, 23.0]) than those with NACH (-9.0% [Q1, -11.0; Q3, -8.0]; -17.0% [Q1, -20.0; Q3, -14.0]; and 25.5% [Q1, 16.0; Q3, 31.5], respectively) (P < 0.001 for all). Median myocardial T1 and ECV were significantly higher in patients with CA (1112 ms [Q1, 1074; Q3, 1146] and 47% [Q1, 41; Q3, 55], respectively) than in those with NACH (1056 ms [Q1, 1011; Q3, 1071] and 28% [Q1, 26; Q3, 30], respectively) (P < 0.001). Basal ECV showed the best performance for the diagnosis of CA (AUC = 0.975; 95% confidence interval [CI]: 0.947-1). No differences in AUC were found between AB ratio of GRS (0.843; 95% CI: 0.768-0.918) and basal myocardial T1 (0.834; 95% CI: 0.741-0.928) for the diagnosis of CA (P = 0.81). The combination of the AB ratio of FT-GRS and basal myocardial T1 had a diagnostic performance not different from that of basal ECV (P = 0.06). CONCLUSION: ECV outperforms FT-strain for the diagnosis of CA with cardiac MRI. The AB ratio of FT-GRS associated with myocardial T1 provides diagnostic performance similar to that achieved by ECV.

Fat-free noncontrast whole-heart CMR with fast and power-optimized off-resonant water excitation pulses.

BACKGROUND: Cardiovascular MRI (CMR) faces challenges due to the interference of bright fat signals in visualizing structures like coronary arteries. Effective fat suppression is crucial, especially when using whole-heart CMR techniques. Conventional methods often fall short due to rapid fat signal recovery, leading to residual fat content hindering visualization. Water-selective off-resonant radiofrequency (RF) pulses have been proposed but come with tradeoffs between pulse duration, which increases scan time, and increased RF energy deposit, which limits their applicability due to specific absorption rate (SAR) constraints. The study introduces a lipid-insensitive binomial off-resonant (LIBOR) RF pulse, which addresses concerns about SAR and scan time, and aims to provide a comprehensive quantitative comparison with published off-resonant RF pulses for CMR at 3T. METHODS: A short (1ms) LIBOR pulse, with reduced RF power requirements, was developed and implemented in a free-breathing respiratory-self-navigated 3D radial whole-heart CMR sequence at 3T. A binomial off-resonant rectangular (BORR) pulse with matched duration, as well as previously published lipid-insensitive binomial off-resonant excitation (LIBRE) pulses (1ms and 2.2ms), were implemented and optimized for fat suppression in numerical simulations and validated in volunteers (n=3). Whole-heart CMR was performed in volunteers(n=10) with all four pulses. The signal-to-noise ratio (SNR) of ventricular blood, skeletal muscle, myocardium, and subcutaneous fat and the coronary vessel detection rates and sharpness were compared. RESULTS: Experimental results validated numerical findings and near homogeneous fat suppression was achieved with all four pulses. Comparing the short RF pulses (1ms), LIBOR reduced the RF power nearly two-fold compared with LIBRE, and three-fold compared with BORR, and LIBOR significantly decreased overall fat SNR from cardiac scans, compared to LIBRE and BORR. The reduction in RF pulse duration (from 2.2ms to 1ms) shortened the whole-heart acquisition from 8.5min to 7min. No significant differences in coronary arteries detection and sharpness were found when comparing all four pulses. CONCLUSION: LIBOR pulses enabled whole-heart CMR under 7minutes at 3T, with large volume fat signal suppression, while reducing RF power compared with LIBRE and BORR pulses. LIBOR is an excellent candidate to address SAR problems encountered in CMR sequences where fat suppression remains challenging and short RF pulses are required. AVAILABILITY OF DATA AND MATERIALS: An online repository containing the anonymized human MRI raw data, as well as RF pulse shapes used in this study is publicly available at: https://zenodo.org/records/8338079(PART 1: KNEE V1-V3, HEART V1-V5) https://zenodo.org/records/10715769 (PART 2: HEART V6-V10) Matlab code to 1) simulate the different RF pulses within a GRE sequence and 2) to read and display the anonymized raw data is available from: https://github.com/QIS-MRI/LIBOR_LIBRE_BORR_SimulationCode The compiled research sequence can be requested through the Teamplay platform of Siemens Healthineers.

Cardiac manifestations and outcomes of COVID-19 vaccine-associated myocarditis in the young in the USA: longitudinal results from the Myocarditis After COVID Vaccination (MACiV) multicenter study.

Background: We aimed to study the clinical characteristics, myocardial injury, and longitudinal outcomes of COVID-19 vaccine-associated myocarditis (C-VAM). Methods: In this longitudinal retrospective observational cohort multicenter study across 38 hospitals in the United States, 333 patients with C-VAM were compared with 100 patients with multisystem inflammatory syndrome in children (MIS-C). We included patients ≤30 years of age with a clinical diagnosis of acute myocarditis after COVID-19 vaccination based on clinical presentation, abnormal biomarkers and/or cardiovascular imaging findings. Demographics, past medical history, hospital course, biochemistry results, cardiovascular imaging, and follow-up information from April 2021 to November 2022 were collected. The primary outcome was presence of myocardial injury as evidenced by late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) imaging. Findings: Patients with C-VAM were predominantly white (67%) adolescent males (91%, 15.7 ± 2.8 years). Their initial clinical course was more likely to be mild (80% vs. 23%, p < 0.001) and cardiac dysfunction was less common (17% vs. 68%, p < 0.0001), compared to MIS-C. In contrast, LGE on CMR was more prevalent in C-VAM (82% vs. 16%, p < 0.001). The probability of LGE was higher in males (OR 3.28 [95% CI: 0.99, 10.6, p = 0.052]), in older patients (>15 years, OR 2.74 [95% CI: 1.28, 5.83, p = 0.009]) and when C-VAM occurred after the first or second dose as compared to the third dose of mRNA vaccine. Mid-term clinical outcomes of C-VAM at a median follow-up of 178 days (IQR 114-285 days) were reassuring. No cardiac deaths or heart transplantations were reported until the time of submission of this report. LGE persisted in 60% of the patients at follow up. Interpretation: Myocardial injury at initial presentation and its persistence at follow up, despite a mild initial course and favorable mid-term clinical outcome, warrants continued clinical surveillance and long-term studies in affected patients with C-VAM. Funding: The U.S. Food and Drug Administration.

Large left ventricular outflow tract mass in a young patient: uncommon presentation of a common disease! A case report.

Background: Infective endocarditis (IE) predominantly involves the cardiac valves. Timely diagnosis and initiation of therapy significantly reduce morbidity and mortality. Infective endocarditis presenting as a large left ventricular outflow tract (LVOT) mass is an atypical manifestation that provides significant challenges to the treating team. Case summary: A 19-year-young male presented with exertional shortness of breath, palpitations, and presyncope for 4 months with constitutional symptoms for the last 6 months. Two-dimensional echocardiogram showed a large LVOT mass arising from the mitral aortic intervalvular fibrosa causing dynamic severe aortic valve obstruction, moderate aortic regurgitation, and severe mitral regurgitation. He was managed on lines of IE and received intravenous antibiotics. In view of worsening heart failure and cardiogenic shock, he underwent mass excision, mechanical aortic valve replacement, and mitral valve repair. Histopathology confirmed it as vegetation. He was discharged and is doing well at 2-month follow-up. Discussion: An atypical presentation of IE as a large LVOT mass was observed in this young male. Sound clinical judgement, judicious use of ancillary imaging, and a multidisciplinary approach ensured timely diagnosis and appropriate treatment. Management included appropriate intravenous antibiotics followed by surgery.

Monitoring the Efficacy of Tafamidis in ATTR Cardiac Amyloidosis by MRI-ECV: A Systematic Review and Meta-Analysis.

BACKGROUND: The usefulness of monitoring treatment effect of tafamidis using magnetic resonance imaging (MRI) extracellular volume fraction (ECV) has been reported. OBJECTIVE: we conducted a meta-analysis to evaluate the usefulness of this method. METHODS: Data from 246 ATTR-CMs from six studies were extracted and included in the analysis. An inverse variance meta-analysis using a random effects model was performed to evaluate the change in MRI-ECV before and after tafamidis treatment. The analysis was also performed by classifying the patients into ATTR-CM types (wild-type or hereditary). RESULTS: ECV change before and after tafamidis treatment was 0.33% (95% CI: -1.83-2.49, I2 = 0%, p = 0.76 for heterogeneity) in the treatment group and 4.23% (95% CI: 0.44-8.02, I2 = 0%, p = 0.18 for heterogeneity) in the non-treatment group. The change in ECV before and after treatment was not significant in the treated group (p = 0.76), but there was a significant increase in the non-treated group (p = 0.03). There was no difference in the change in ECV between wild-type (95% CI: -2.65-3.40) and hereditary-type (95% CI: -9.28-4.28) (p = 0.45). CONCLUSIONS: The results of this meta-analysis suggest that MRI-ECV measurement is a useful imaging method for noninvasively evaluating the efficacy of tafamidis treatment for ATTR-CM.

Mitral Valve Prolapse With Mitral Annular Disjunction Causing Ventricular Tachycardia During a Stress Test: A Case Report.

Mitral valve prolapse (MVP) is a relatively common valvular disorder characterized by displacement of one or both mitral valve leaflets into the left atrium (LA) during systole. Mitral annular disjunction (MAD) is an associated abnormality where a portion of the mitral valve annulus attaches superiorly in the left atrial wall. Although MVP is often considered benign, it can rarely lead to serious complications such as ventricular arrhythmias, especially when MAD is present. Herein, we present a case of a 63-year-old male with MVP and MAD who experienced sustained ventricular tachycardia (VT) during cardiac stress testing. This case underscores the importance of recognizing MVP with MAD as a potential substrate for ventricular arrhythmias, notably under heightened physiological or induced periods of stress.

Left atrial strain by cardiac MRI feature tracking in mitral stenosis before and after balloon valvuloplasty.

The aim was to investigate LA strain by feature tracking cardiac MRI in mitral stenosis (MS) patients before and after percutaneous balloon mitral valvuloplasty (PBMV). Patients underwent cardiac MRI before and after successful PBMV (n = 18). Mitral valve area, transmitral velocity and gradients, LA volumes and ejection fraction (LAEF) were measured. LA strain feature tracking analysis was used to calculate LA reservoir, conduit, and booster strain. LA strain, LA volumes, LAEF and mitral valve severity indices were compared before and after PBMV. Correlations between LA strain and other cardiac MRI parameters were assessed. After PBMV, mitral valve area increased from 1.18 ± 0.25 cm2 to 2.26 ± 0.27 cm2, p < 0.001. Transmitral peak velocity decreased from 1.7 ± 0.37 m/s to 1.3 ± 0.27 m/s, p < 0.001. Transmitral peak gradient decreased from 12.4 ± 4.8 mmHg to 6.8 ± 2.9 mmHg, p < 0.001, and mean gradient decreased from 6.9 ± 3.8 mmHg to 2.9 ± 1.4 mmHg, p < 0.001. Maximal LA volume decreased from 73.1 ± 14.2 ml/m2 to 62.7 ± 16.3 ml/m2, p = 0.018; corrected p value = 0.054. LAEF increased from 36.3 ± 8.7% to 44.4 ± 9.5%, p = 0.010. Reservoir strain increased from 11.7 ± 3.1% to 14.9 ± 3.6% after PBMV, p = 0.009, and conduit strain from 3.8 ± 2% to 6 ± 2.3%, p = 0.005. Booster strain insignificantly increased after PBMV. Cardiac MRI feature tracking provides information on the 3 LA functional phases. Significant improvement was observed in reservoir and conduit functions after successful PBMV.

Characterizing left ventricular systolic dysfunction in sickle cell disease: three contrasting case reports.

Background: Left ventricular systolic dysfunction (LVSD) is an uncommon but life-threatening complication of sickle cell disease (SCD), with poorly characterized aetiology. We present three SCD patients with LVSD due to different underlying mechanisms. Case summary: The first case describes rapid deterioration in LV function secondary to severe cardiac iron overload in a 37-year-old female with poor chelation compliance after 10 years of top-up transfusions for SCD. The second case is a severe non-ischaemic dilated cardiomyopathy (DCM) in a 42-year-old SCD patient with longstanding sickle nephropathy and hypertension. The final case demonstrates severe LVSD with large transmural infarcts (ischaemic DCM) in the absence of epicardial coronary disease in a 52-year-old SCD patient. Discussion: This case series presents the first attempt to characterize the aetiology of LVSD in SCD. We identified three phenotypes: iron-overload cardiomyopathy, non-ischaemic DCM, and ischaemic DCM. These contrasting cases highlight the significance of understanding the underlying pathology in determining individualized treatment plans for these high-risk patients. We discuss the role of cardiac MRI (CMR) in characterizing LV dysfunction, and we believe that this case series will form the basis of prospective studies to further delineate the pathophysiology of LVSD in SCD.