Acute Cardiovascular Manifestations in 286 Children With Multisystem Inflammatory Syndrome Associated With COVID-19 Infection in Europe.

BACKGROUND: The aim of the study was to document cardiovascular clinical findings, cardiac imaging, and laboratory markers in children presenting with the novel multisystem inflammatory syndrome associated with coronavirus disease 2019 (COVID-19) infection. METHODS: This real-time internet-based survey has been endorsed by the Association for European Paediatric and Congenital Cardiologists Working Groups for Cardiac Imaging and Cardiovascular Intensive Care. Children 0 to 18 years of age admitted to a hospital between February 1 and June 6, 2020, with a diagnosis of an inflammatory syndrome and acute cardiovascular complications were included. RESULTS: A total of 286 children from 55 centers in 17 European countries were included. The median age was 8.4 years (interquartile range, 3.8-12.4 years) and 67% were boys. The most common cardiovascular complications were shock, cardiac arrhythmias, pericardial effusion, and coronary artery dilatation. Reduced left ventricular ejection fraction was present in over half of the patients, and a vast majority of children had raised cardiac troponin when checked. The biochemical markers of inflammation were raised in most patients on admission: elevated C-reactive protein, serum ferritin, procalcitonin, N-terminal pro B-type natriuretic peptide, interleukin-6 level, and D-dimers. There was a statistically significant correlation between degree of elevation in cardiac and biochemical parameters and the need for intensive care support (P<0.05). Polymerase chain reaction for severe acute respiratory syndrome coronavirus 2 was positive in 33.6%, whereas immunoglobulin M and immunoglobulin G antibodies were positive in 15.7% cases and immunoglobulin G in 43.6% cases, respectively, when checked. One child in the study cohort died. CONCLUSIONS: Cardiac involvement is common in children with multisystem inflammatory syndrome associated with the Covid-19 pandemic. The majority of children have significantly raised levels of N-terminal pro B-type natriuretic peptide, ferritin, D-dimers, and cardiac troponin in addition to high C-reactive protein and procalcitonin levels. In comparison with adults with COVID-19, mortality in children with multisystem inflammatory syndrome associated with COVID-19 is uncommon despite multisystem involvement, very elevated inflammatory markers, and the need for intensive care support.

[Absent right superior vena cava with left superior vena cava draining to an unroofed coronary sinus]. / Ausencia de vena cava superior derecha y vena cava superior izquierda con drenaje en el seno coronario sin techo.

We describe the case of a 1-month-old infant with a complete atrioventricular septal defect with right dominance, situs solitus, and drainage from the persistent left superior vena cava to the coronary sinus. Corrective surgery was carried out without previous cardiac catheterization. During the operation, the right superior vena cava was found to be absent. Cyanosis and head-and-neck edema were observed in the immediate postoperative period. Transthoracic echocardiography carried out after injection of a small volume of stirred saline into an epicranial vein demonstrated the presence of microbubbles in the left cardiac cavities. A second operation was performed to prevent drainage from the left superior vena cava to the left atrium (via the unroofed coronary sinus) and to insert a PTFE conduit between the innominate vein and the right atrial appendage. The outcome was excellent. In this report, the embryological, clinical, diagnostic and therapeutic characteristics of this entity are discussed.

Right ventricular dysfunction in adolescents with mild cystic fibrosis.

BACKGROUND: In cystic fibrosis (CF) patients the right ventricle (RV) suffers a progressive deterioration, but it is not clear when these changes begin. The aim of this study was to analyze the RV function in CF patients with mild respiratory disease. METHODS: Color-Doppler-Echocardiographic studies were prospectively performed in CF adolescent patients and an age-matched control group. Findings were correlated with pulmonary function tests (PFT), genotype, chronic bacterial colonization, pancreatic status and clinical scores. Only patients with mild CF were selected. RESULTS: Thirty seven CF patients and 40 healthy controls were recruited. In CF patients all echocardiographic parameters were abnormal compared to controls. Doppler analysis showed slightly elevated pulmonary artery pressure values, and abnormal relaxation and systolic function for all indexes. No correlation was found with any of the features studied. CONCLUSIONS: In CF patients, abnormalities in the structure and function of the RV may be present at early stages of the disease. These abnormalities are subclinical and do not correlate with clinical scores, PFT, genotype, chronic bacterial colonization or pancreatic insufficiency.

[Syncope in the debut of a mediastinal mass]. / Síncope como forma de presentación de masa mediastínica.

Pediatric syncope accounts for 3% of the consultations in emergency department. It is frequent in pediatrics, particularly in teenager girls between 15 and 19 years old, and up to a 25% of them have suffered from a syncopal episode. Most causes are benign, but the aim in its initial evaluation is ruling out those which mean any danger. Heart disease can lead to syncope in a 10-28% of cases. A detailed medical history and an exhaustive exploration are enough to rule out organic pathology in most situations. We present the case of a diffuse large B cell lymphoma located in the mediastinum diagnosed after repetitive syncopal episodes.

[Clinical observations on propranolol use for paediatric airway hemangiomas]. / Observaciones clínicas sobre el uso de propranolol en hemangiomas de vía aérea infantil.

Treatment of subglottic hemangiomas in children has traditionally been characterized as a challenging situation with multiple therapeutic options without consensus as to which one is the best and with risks of severe side effects. Recent reports on the experience of propranolol use in the treatment of paediatric airway hemangiomas suggest favourable reasons for this use due to the rapid improvement and its lack of severe side effects. In this paper we report the experience with 6 children having symptomatic airway hemangiomas treated with propranolol. All children improved their respiratory symptoms dramatically and did not need additional interventions.

Síncope como forma de presentación de masa mediastínica / Syncope in the debut of a mediastinal mass

El síncope supone el 3 por ciento de las consultas en los servicios deurgencias. En pediatría es frecuente, especialmente en niñas adolescentes de entre 15 y 19 años, quienes hasta un 25 por ciento hansufrido un episodio sincopal. La mayoría de las causas son benignas, pero el objetivo en la evaluación inicial de un paciente con síncope es descartar aquellas que supongan compromiso vital; una enfermedad cardíaca puede debutar en forma de síncope en un 10-28 por ciento de los casos. La historia clínica detallada y una exploración física exhaustiva son suficientes en la mayoría de las situaciones para descartar patología orgánica. Presentamos el caso de un paciente con linfoma B difuso de células grandes localizado en el mediastino cuyo diagnóstico se realizó tras episodios sincopales de repetición.