The atrial and ventricular myocardial proteome of end-stage lamin heart disease.

Lamins A/C (encoded by gene) can lead to dilated cardiomyopathy (DCM). This pilot study sought to explore the postgenomic phenotype of end-stage lamin heart disease. Consecutive patients with end-stage lamin heart disease (LMNA-group, n = 7) and ischaemic DCM (ICM-group, n = 7) undergoing heart transplantation were prospectively enrolled.

Saturation-pulse prepared heart-rate independent inversion-recovery (SAPPHIRE) biventricular T1 mapping: inter-field strength, head-to-head comparison of diastolic, systolic and dark-blood measurements.

Background: To assess the feasibility of biventricular SAPPHIRE T mapping in vivo across field strengths using diastolic, systolic and dark-blood (DB) approaches.

Methods: 10 healthy volunteers underwent same-day non-contrast cardiovascular magnetic resonance at 1.5 Tesla (T) and 3 T.

Diagnosis and risk stratification in hypertrophic cardiomyopathy using machine learning wall thickness measurement: a comparison with human test-retest performance.

Background: Left ventricular maximum wall thickness (MWT) is central to diagnosis and risk stratification of hypertrophic cardiomyopathy, but human measurement is prone to variability. We developed an automated machine learning algorithm for MWT measurement and compared precision (reproducibility) with that of 11 international experts, using a dataset of patients with hypertrophic cardiomyopathy.

Methods: 60 adult patients with hypertrophic cardiomyopathy, including those carrying hypertrophic cardiomyopathy gene mutations, were recruited at three institutes in the UK from August, 2018, to September, 2019: Barts Heart Centre, University College London Hospital (The Heart Hospital), and Leeds Teaching Hospitals NHS Trust.

Healthcare Workers Bioresource: Study outline and baseline characteristics of a prospective healthcare worker cohort to study immune protection and pathogenesis in COVID-19.

: Most biomedical research has focused on sampling COVID-19 patients presenting to hospital with advanced disease, with less focus on the asymptomatic or paucisymptomatic. We established a bioresource with serial sampling of health care workers (HCWs) designed to obtain samples before and during mainly mild disease, with follow-up sampling to evaluate the quality and duration of immune memory. : We conducted a prospective study on HCWs from three hospital sites in London, initially at a single centre (recruited just prior to first peak community transmission in London), but then extended to multiple sites 3 weeks later (recruitment still ongoing, target n=1,000).

An unusual cause of polymorphic ventricular tachycardia: Acquired long QT syndrome from atypical variant of stress-induced cardiomyopathy.

A 55-year-old woman with a recent history of surgically and radioiodine treated thyroid cancer experienced a run of polymorphic ventricular tachycardia with hemodynamic perturbation during anaesthetic induction with propofol, fentanyl and rocuronium for elective surgical excision of right hip metastasis. Electrocardiography showed new T-wave inversion and QT prolongation that subsequently resolved. Cardiac enzymes were elevated but invasive coronary angiography showed unobstructed epicardial coronary arteries.

The myocardial phenotype of Fabry disease pre-hypertrophy and pre-detectable storage.

Aims: Cardiac involvement in Fabry disease (FD) occurs prior to left ventricular hypertrophy (LVH) and is characterized by low myocardial native T1 with sphingolipid storage reflected by cardiovascular magnetic resonance (CMR) and electrocardiogram (ECG) changes. We hypothesize that a pre-storage myocardial phenotype might occur even earlier, prior to T1 lowering.

Methods And Results: FD patients and age-, sex-, and heart rate-matched healthy controls underwent same-day ECG with advanced analysis and multiparametric CMR [cines, global longitudinal strain (GLS), T1 and T2 mapping, stress perfusion (myocardial blood flow, MBF), and late gadolinium enhancement (LGE)].

Myocardial Edema, Myocyte Injury, and Disease Severity in Fabry Disease.

Background Cardiovascular magnetic resonance can demonstrate myocardial processes in Fabry disease (FD), such as low native T1 (sphingolipid storage) and late gadolinium enhancement (LGE, scar). Recently, high T2 (edema) has been observed in the basal inferolateral wall along with troponin elevation. We hypothesized that edema and myocyte injury would be chronically associated and have electrical, mechanical, and disease associations in FD.

Familial cardiomyopathy caused by a novel heterozygous mutation in the gene (c.1434dupG): a cardiac MRI-augmented segregation study.

In a five-generation family carrying a novel frameshift variant (c.1434dupG, p.Leu479AlafsX72), imaging-augmented segregation analysis supports its association with lamin heart disease.