Medium-Term Cardiac Outcomes in Young People with Multi-system Inflammatory Syndrome: The Era of COVID-19.

Multi-system inflammatory syndrome in children (MIS-C) causes widespread inflammation including a pancarditis in the weeks following a COVID infection. As we prepare for further coronavirus surges, understanding the medium-term cardiac impacts of this condition is important for allocating healthcare resources. A retrospective single-center study of 67 consecutive patients with MIS-C was performed evaluating echocardiographic and electrocardiographic (ECG) findings to determine the point of worst cardiac dysfunction during the admission, then at intervals of 6-8 weeks and 6-8 months. Worst cardiac function occurred 6.8 ± 2.4 days after the onset of fever with mean 3D left ventricle (LV) ejection fraction (EF) 50.5 ± 9.8%. A pancarditis was typically present: 46.3% had cardiac impairment; 31.3% had pericardial effusion; 26.8% demonstrated moderate (or worse) valvar regurgitation; and 26.8% had coronary dilatation. Cardiac function normalized in all patients by 6-8 weeks (mean 3D LV EF 61.3 ± 4.4%, p < 0.001 compared to presentation). Coronary dilatation resolved in all but one patient who initially developed large aneurysms at presentation, which persisted 6 months later. ECG changes predominantly featured T-wave changes resolving at follow-up. Adverse events included need for ECMO (n = 2), death as an ECMO-related complication (n = 1), LV thrombus formation (n = 1), and subendocardial infarction (n = 1). MIS-C causes a pancarditis. In the majority, discharge from long-term follow-up can be considered as full cardiac recovery is expected by 8 weeks. The exception includes patients with medium sized aneurysms or greater as these may persist and require on-going surveillance.

Transcatheter creation of a pulmonary artery to left atrial fenestration in a failing Fontan circulation using the Atrial Flow Regulator (AFR).

Transcatheter creation of an interatrial communication using the Occlutech Atrial Flow Regulator Device for pulmonary hypertension or heart failure is well described. We report a case of an 8-year-old boy with a failing Fontan circulation, in whom the Atrial Flow Regulator was used to successfully create a fenestration between the pulmonary artery and left atrium, improving his clinical condition.

Initial experience with a novel ePTFE-covered balloon expandable stent in patients with near-atretic or severe aortic coarctation and small femoral arterial access.

OBJECTIVES: We report our experience of using the Bentley BeGraft Aortic stent in patients with severe or near-atretic aortic coarctation and small femoral arterial access. BACKGROUND: Use of covered stent is recommended in some settings such as aortic coarctation with associated aneurysm, Turner syndrome, and coarctation with aortic atresia. However, currently available covered stents need larger sheaths that may limit their use in children and patients with smaller arterial access. Newer stents may overcome this limitation. METHODS: Single-centre retrospective study of patients with severe or near-atretic aortic coarctation and small femoral arterial access. RESULTS: Between July and October, 2019, five patients (median age 15 years) with near-atretic or severe coarctation were treated with a Bentley BeGraft Aortic stent. Long sheaths between 9 and 11 Fr were used to implant stents, which were dilated up to 12-16 mm. None of the patients had residual coarctation (gradient >20 mm of mercury) after stenting. None of the patients developed acute vascular injuries or local access related complications at the end of the procedure or during follow-up (range 6-10 months). CONCLUSIONS: Bentley BeGraft aortic stents are important to consider in patients with severe coarctation and provided acute procedural success in patients with small femoral arterial access and widen the applicability in this patient population.

Multimodality cardiac evaluation in children and young adults with multisystem inflammation associated with COVID-19.

AIMS: Following the peak of the UK COVID-19 epidemic, a new multisystem inflammatory condition with significant cardiovascular effects emerged in young people. We utilized multimodality imaging to provide a detailed sequential description of the cardiac involvement. METHODS AND RESULTS: Twenty consecutive patients (mean age 10.6 ± 3.8 years) presenting to our institution underwent serial echocardiographic evaluation on admission (median day 5 of illness), the day coinciding with worst cardiac function (median day 7), and the day of discharge (median day 15). We performed cardiac computed tomography (CT) to assess coronary anatomy (median day 15) and cardiac magnetic resonance imaging (CMR) to assess dysfunction (median day 20). On admission, almost all patients displayed abnormal strain and tissue Doppler indices. Three-dimensional (3D) echocardiographic ejection fraction (EF) was <55% in half of the patients. Valvular regurgitation (75%) and small pericardial effusions (10%) were detected. Serial echocardiography demonstrated that the mean 3D EF deteriorated (54.7 ± 8.3% vs. 46.4 ± 8.6%, P = 0.017) before improving at discharge (P = 0.008). Left main coronary artery (LMCA) dimensions were significantly larger at discharge than at admission (Z score -0.11 ± 0.87 vs. 0.78 ± 1.23, P = 0.007). CT showed uniform coronary artery dilatation commonly affecting the LMCA (9/12). CMR detected abnormal strain in all patients with global dysfunction (EF <55%) in 35%, myocardial oedema in 50%, and subendocardial infarct in 5% (1/20) patients. CONCLUSIONS: Pancarditis with cardiac dysfunction is common and associated with myocardial oedema. Patients require close monitoring due to coronary artery dilatation and the risk of thrombotic myocardial infarction.

Pulmonary hypertension in congenital heart disease.

Pulmonary hypertension is defined as a mean pulmonary arterial pressure ≥25 mmHg. We focus on its relevance in congenital heart disease, reviewing pathophysiology, diagnosis and management. Pulmonary hypertension is a relatively common complication of congenital heart disease, with adult prevalence between 5 and 10%. A multifactorial cause is recognized, relating to the size and nature of cardiac defect as well as environmental and genetic factors. More complex disease is increasingly recognized rather than pure Eisenmenger complex. Remodeling of the pulmonary vascular bed causes increased pulmonary vascular resistance diagnosed by a collection of investigations including echocardiography, exercise testing, cardiac catheterization, MRI and CT scanning. Management employs disease-modifying medications which are now used with increasing benefit.