Vascular involvement in neurofibromatosis type 1 has been described, although coronary artery disease is rare. Data about clinical presentation and natural history are anecdotal. This is the first ...case of myocardial infarction due to coronary aneurysms in a 13-year-old boy with neurofibromatosis type 1. We discuss pathophysiology, diagnostic images, and therapeutic management of this rare association.
The diagnosis of acute myocarditis (AM) is based on a multi-parametric assessment including clinical presentation, ECG, imaging and biomarkers. Fragmented QRS (fQRS) might be an additional diagnostic ...sign in patients with proven AM. The main objective of this study was to assess the diagnostic yield of fQRS in patients with suspected AM presenting to the emergency department (ED). Patients admitted between January 2016 and March 2021 with a proven diagnosis of AM, according to clinical, cardiac magnetic resonance (CMR) and/or histologic criteria, were included in the analysis. In total, 51 patients were analyzed (41 men, 78%), with a median age of 36 (29-45) years. Thirty-three (65%) patients had prodromal flu-like symptoms. Patients presented to the ED mostly complaining of chest pain (68%) and palpitations (21%). Seven (14%) patients experienced cardiac arrest, one of whom died. At presentation, 40 patients (78%) displayed fQRS, and 10 (20%) presented ventricular arrhythmias. All the surviving patients underwent CMR and displayed late gadolinium enhancement (LGE). ECG leads showed that fQRS matched the LGE distribution in 38 patients (95%). The presence of fQRS is a simple clinical bedside tool to support the initial suspect of AM in the emergency department and to guide the most appropriate clinical workup.
There is limited information about coronavirus disease 2019 (COVID-19) in the pediatric population. Preliminary data suggest a not insignificant prevalence of cardiac involvement. Here, we report our ...early experience with COVID-19 in the pediatric population. These patients display exceptionally high levels of acute-phase reactants. The clinical syndrome in these patients is somewhat similar to Kawasaki disease with or without myocardial involvement. In some cases, the presentation mimics typical myocarditis. Severe myocardial involvement is associated with transient electrocardiographic and echocardiographic abnormalities. These findings may be due to the cardiotropic nature of the virus or may be the result of an immunologic response to the infection.
Patients with congenital heart disease (CHD) seem to have a higher risk for specific malignancies. We hypothesize a pathogenetic link between particular congenital heart defects and cancer ...originating from specific cellular lineages. We report a series of patients, followed in two high-volume referral centers, with CHD involving neural crest-derived structures who developed cancer later in life. Fourteen patients (five female) developed neoplasia with a cellular origin embryologically linked to the neural crest between 2010 and 2020. If confirmed on larger datasets, this observation might support the hypothesis of common embryogenetic pathway suggesting tailored surveillance of a specific subset of patients.
Myocarditis is a rare extra-intestinal complication of inflammatory bowel disease (IBD), in particular, ulcerative colitis.
We report a case of acute myocarditis as first manifestation of severe ...ulcerative colitis. A 22-year-old man was admitted with fever, bloody diarrhea, and fatigue. He had suffered from frequent bloody diarrhea, abdominal pain, fatigue, and weight loss for one month. A 12-lead-electrocardiogram showed sinus rhythm with QRS fragmentation and T waves inversion. High sensitivity troponin-I was elevated and the echocardiogram showed a mild pericardial effusion and inferior hypokinesia with normal ejection fraction. Cardiac magnetic resonance disclosed late enhancement in the inferior wall, corroborating the hypothesis of myocarditis. One week later, a colonoscopy revealed severe ulcerative extensive colitis (Mayo subscore 3). 5-aminosalicylic acid (mesalazine) and systemic steroid were started with good clinical and biochemical response. The following days the patient developed mesalazine hepatic and pancreatic induced toxicity requiring drug discontinuation and strict multi-disciplinary follow-up. At 7 months follow-up intestinal symptoms were well controlled with complete normalization of liver and pancreatic enzymes. Transthoracic echocardiography showed normal biventricular function and pericardial effusion resolution.
This case underscores the importance of a high suspicion for extra intestinal involvement in patients with IBD. These complications may be multifactorial and need multidisciplinary management.
•When a patient was first-time diagnosed with a severe form of inflammatory bowel disease or has a disease relapse, bear in mind myocarditis as possible extra intestinal manifestation.•Multidisciplinary management is crucial to ensure the best level of care and follow-up in a such challenging and insidious clinical picture.
Aims
We aim to assess the reproducibility of QRS fragmentation (fQRS) on a multi‐centre dataset of patients with acute myocarditis (AM), including a histopathological validation in a subgroup with ...biopsy‐proven disease. Electrocardiogram (ECG) in patients with myocarditis is usually considered aspecific. ST changes and conduction anomalies have been commonly reported so far. We have previously described fQRS in patients with AM.
Methods and results
Patients admitted between 2008 and 2019 in two centres with a diagnosis of AM were included. Standard ECG, echocardiography, and cardiac magnetic resonance (CMR) findings were recorded at baseline and at follow‐up (FU). Eighty patients were analysed, 66 men (82%), with median age of 34 (26–43) years. Twenty‐two patients had biopsy‐proven AM. At presentation, 61 patients (76%) displayed fQRS. Median ejection fraction (EF) was 55% (43–60). Seventy‐two patients (90%) underwent CMR and displayed late gadolinium enhancement (LGE). ECG leads showed that fQRS correlated with distribution of LGE. In patients with positive biopsy, fQRS was present in 18 (81%). Median FU was 419 days (224–956). Complete FU was available for 64 patients (80%), and 33 patients (52%) displayed persistence of fQRS. Median EF was 60% (57–64). Eleven patients underwent a repeated biopsy at FU, eight of whom had persistent inflammation and fQRS. Fifteen patients (23%) had ventricular tachycardia, 14 of whom still showed fQRS.
Conclusions
In this cohort fQRS was confirmed as an additional useful ECG sign. Persistence of fQRS was associated with ongoing inflammation and with a poorer outcome in terms of ventricular function and occurrence of arrhythmias.
Acute carbon monoxide poisoning (COP) is one of the leading causes of intoxication among patients presenting to the emergency department (ED). COP symptoms are not always specific and may vary from ...mild to critical. In the last few years, COHb pulse oximeters have been developed and applied to the setting of suspected COP. The aim of this systematic review is to assess the diagnostic accuracy of CO pulse oximetry (SpCO) with carboxyhemoglobin (COHb) levels measured by blood gas analysis, used as a reference standard, in patients with suspected COP.
We developed our search strategy according to the PICOS framework, population, index/intervention, comparison, outcome, and study, considering the diagnostic accuracy of SpCO compared to COHb levels measured by blood gas analysis, used as a reference standard, in patients with suspected COP enrolled in cross-sectional studies in English. The search was performed on MEDLINE/PubMed and EMBASE in February 2022. Quality assessment was performed using the QUADAS-2 methodology. A COHb cutoff of 10% was chosen to test the sensitivity and specificity of the index test. A bivariate model was used to perform the meta-analysis. The protocol was registered on PROSPERO (CRD42022359144).
A total of six studies (1734 patients) were included. The pooled sensitivity of the test was 0.65 (95% CI 0.44-0.81), and the pooled specificity was 0.93 (95% CI 0.83-0.98). The pooled LR+ was 9.4 (95% CI 4.4 to 20.1), and the pooled LR- was 0.38 (95% CI 0.24 to 0.62).
Our results show that SpCO cannot be used as a screening tool for COP in the ED due to its low sensitivity. Because of its high LR+, it would be interesting to evaluate, if SpCO could have a role in the prehospital setting as a tool to quickly identify COP patients and prioritize their transport to specialized hospitals on larger samples with a prospective design.
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