A rare case report of type 1 congenital disorders of glycosylation with acute decompensated heart failure and the incidental discovery of congenital disorders of glycosylation associated dilated cardiomyopathy and acute myocarditis.

Background: Congenital disorders of glycosylation (CDG) are rare genetically inherited defects leading to enzyme deficiency or malfunction in the glycosylation pathway. Normal glycosylation is essential to the development of normal cardiac anatomy and function. Congenital disorders of glycosylation-related cardiomyopathy are often the first manifestation detected in early life and may lead to sudden cardiac death.

Long-term clinical outcomes in patients with a working diagnosis of myocardial infarction with non-obstructed coronary arteries (MINOCA) assessed by cardiovascular magnetic resonance imaging.

Background: Myocardial infarction with non-obstructed coronary arteries (MINOCA) is a distinct entity among patients presenting with troponin-positive acute chest pain. We have previously reported on the incremental diagnostic capability of cardiovascular magnetic resonance (CMR) in this cohort. There is paucity of evidence on the long-term (> 5 years) clinical outcomes of these patients as graded by their acute CMR diagnosis.

Cardiotoxicity after cancer treatment: a process map of the patient treatment journey.

Background/aim: Cardiotoxicity is a potential complication of anticancer therapy. While guidelines have been developed to assist practitioners, an effective, evidence based clinical pathway for the treatment of cardiotoxicity has not yet been developed. The aim of this study was to describe the journey of patients who developed cardiotoxicity through the healthcare system in order to establish baseline data to inform the development and implementation of a patient-centred, evidence-based clinical pathway.

Assessment of myocardial oxygenation, strain, and diastology in MYBPC3-related hypertrophic cardiomyopathy: a cardiovascular magnetic resonance and echocardiography study.

Aims: Myocardial oxygenation is impaired in hypertrophic cardiomyopathy (HCM) patients with left ventricular hypertrophy (LVH), and possibly also in HCM gene carriers without LVH. Whether these oxygenation changes are also associated with abnormalities in diastolic function or left ventricular (LV) strain are unknown.

Methods And Results: We evaluated 60 subjects: 20 MYBPC3 gene positive patients with LVH (G+LVH+), 18 MYBPC3 gene positive without LVH (G+LVH-), 11 gene negative siblings (G-), and 11 normal controls (NC).

Late characterisation of cardiac effects following anthracycline and trastuzumab treatment in breast cancer patients.

Background: Anthracycline (A) and trastuzumab (T) chemotherapy have well-recognized cardiac toxicity, potentially leading to significant morbidity and mortality. Our previous work in 46 prospectively enrolled breast cancer patients showed early left ventricular (LV) and right ventricular (RV) function decline at 1 and 3 months, but only persistent RV dysfunction at 12 months which correlated with myocardial oedema observed early (1 and 3 months) after administration of chemotherapy regimes.

Method: To investigate late cardiac effects, the same cohort were re-imaged with advanced Cardiovascular Magnetic Resonance (CMR) imaging including T1 mapping 5 ± 1 year post chemotherapy.

Early Use of N-acetylcysteine With Nitrate Therapy in Patients Undergoing Primary Percutaneous Coronary Intervention for ST-Segment-Elevation Myocardial Infarction Reduces Myocardial Infarct Size (the NACIAM Trial [N-acetylcysteine in Acute Myocardial Infarction]).

Background: Contemporary ST-segment-elevation myocardial infarction management involves primary percutaneous coronary intervention, with ongoing studies focusing on infarct size reduction using ancillary therapies. N-acetylcysteine (NAC) is an antioxidant with reactive oxygen species scavenging properties that also potentiates the effects of nitroglycerin and thus represents a potentially beneficial ancillary therapy in primary percutaneous coronary intervention. The NACIAM trial (N-acetylcysteine in Acute Myocardial Infarction) examined the effects of NAC on infarct size in patients with ST-segment-elevation myocardial infarction undergoing percutaneous coronary intervention.

Cardiovascular magnetic resonance-GUIDEd management of mild to moderate left ventricular systolic dysfunction (CMR GUIDE): Study protocol for a randomized controlled trial.

Background: The majority of sudden cardiac death (SCD) in patients with heart failure occurs in those with mild-moderate left ventricular (LV) systolic dysfunction (LVEF 36-50%) who under current guidelines are ineligible for primary prevention implantable cardiac defibrillator (ICD) therapy. Recent data suggest that cardiac magnetic resonance (CMR) evidence of replacement fibrosis forms a substrate for malignant arrhythmia and therefore potentially identifies a subgroup at increased risk of SCD. Our hypothesis is that among patients with mild-moderate LV systolic dysfunction, a CMR-guided management strategy for ICD insertion based on the presence of scar or fibrosis is superior to a current strategy of standard care.

Troponin-positive chest pain with unobstructed coronary arteries: incremental diagnostic value of cardiovascular magnetic resonance imaging.

Aims: Troponin-positive chest pain patients with unobstructed coronaries represent a clinical dilemma. Cardiovascular magnetic resonance (CMR) imaging has an increasingly prominent role in the assessment of these patients; however, its utility in addition to expert clinical judgement is unclear. We sought to determine the incremental diagnostic value of CMR and the heterogeneity in diagnoses by experienced cardiologists when presented with blinded clinical and investigative data in this population.

Impaired Myocardial Oxygenation Response to Stress in Patients With Chronic Kidney Disease.

Background: Coronary artery disease and left ventricular hypertrophy are prevalent in the chronic kidney disease (CKD) and renal transplant (RT) population. Advances in cardiovascular magnetic resonance (CMR) with blood oxygen level-dependent (BOLD) technique provides capability to assess myocardial oxygenation as a measure of ischemia. We hypothesized that the myocardial oxygenation response to stress would be impaired in CKD and RT patients.

Myocardial perfusion is impaired in asymptomatic renal and liver transplant recipients: a cardiovascular magnetic resonance study.

Background: Myocardial ischemia is a major cause of death in chronic kidney disease (CKD) patients, which can be caused by either epicardial or microvascular coronary artery disease (CAD). Although renal transplantation improves survival, cardiovascular disease remains a major cause of mortality in post renal transplant recipients, including those with no significant epicardial CAD pre-transplant. We aim to utilize stress cardiovascular magnetic resonance (CMR) and MR coronary angiography (MRCA) to assess silent myocardial ischemia and epicardial CAD in renal transplant recipients.

Utility of CMR Markers of Myocardial Injury in Predicting LV Functional Recovery: Results from PROTECTION AMI CMR Sub-study.

Background: Adverse left ventricular (LV) remodelling following acute ST-segment elevation myocardial infarction (STEMI) has prognostic importance. We aimed to predict 90-day left ventricular (LV) function following acute STEMI using variables from clinical presentation, biomarkers, and cardiovascular magnetic resonance imaging (CMR).

Methods: Consecutive patients undergoing primary percutaneous coronary intervention for anterior STEMI as part of the Selective Inhibition of Delta-protein Kinase C for the Reduction of Infarct Size in Acute Myocardial Infarction (PROTECTION-AMI) trial were enrolled into the CMR sub-study at selected sites.

Cardiovascular magnetic resonance of total and atrial pericardial adipose tissue: a validation study and development of a 3 dimensional pericardial adipose tissue model.

Background: Recently pericardial adipose tissue (PAT) has been shown to be an independent predictor of atrial fibrillation (AF). Atrial PAT may influence underlying atrial musculature creating a substrate for AF. This study sought to validate the assessment of total and atrial PAT by standard cardiovascular magnetic resonance (CMR) measures and describe and validate a three dimensional atrial PAT model.

Mapping and ablation of the pulmonary veins and cavo-tricuspid isthmus with a magnetic resonance imaging-compatible externally irrigated ablation catheter and integrated electrophysiology system.

Background: Magnetic resonance imaging (MRI)-guided interventional electrophysiology (EP) has rapidly emerged as a promising alternative to x-ray-guided ablation. We aimed to evaluate an externally irrigated MRI-compatible ablation catheter and integrated EP pacing and recording system, testing the feasibility of pulmonary vein and cavo-tricuspid isthmus ablation.

Methods And Results: Externally irrigated MRI-compatible ablation and diagnostic EP catheters and an integrated EP recording system (Imricor Medical Systems, Burnsville, MN) were tested in n=11 sheep in a 1.

Myocardial viability imaging: does it still have a role in patient selection prior to coronary revascularisation?

Patients with severe left ventricular (LV) dysfunction and multi-vessel coronary artery disease (CAD) are at high risk during revascularisation, however they are also likely to derive the most benefit. Historically, the detection of dysfunctional but potentially viable myocardium ('stunned or hibernating myocardium') has been central to the decision-making regarding revascularisation. A number of recent studies have challenged this paradigm, questioning the role of viability testing in this population.

Nonvolumetric echocardiographic indices of right ventricular systolic function: validation with cardiovascular magnetic resonance and relationship with functional capacity.

Purpose: Right ventricular (RV) systolic function as measured by right ventricular ejection fraction (RVEF) has long been recognized as an important predictor of outcome in heart failure patients. The echocardiographic measurement of RV volumes and RVEF is challenging, however, owing to the unique geometry of the right ventricle. Several nonvolumetric echocardiographic indices of RV function have demonstrated prognostic value in heart failure.

Evaluation of myocardial viability with cardiac magnetic resonance imaging.

Assessment of myocardial viability is of clinical and scientific significance. Traditionally, the detection of myocardial viability (either stunning or hibernation) has been used in aiding diagnosis before revascularization, especially in high-risk patients. There is a considerable body of observational evidence showing substantial improvement after revascularization in patients with significant left ventricular dysfunction and myocardial viability.