Long-term effects of physical training on cardiopulmonary exercise parameters in young patients with congenital heart diseases.

INTRODUCTION: Physical activity is recognised as an important intervention in patients with CHD. However, more data on the actual magnitude of physical training impact on functional capacity in this group of patients are still warranted. We aim to assess effort tolerance in a contemporary cohort of patients with congenital heart disease, regularly following a training programme, in comparison with a matched control group. METHODS: Patients with CHD followed at the sports medicine department, who had undergone cardiopulmonary exercise test between 2011 and 2019, were included. Variables recorded were maximum workload, absolute and indexed maximum oxygen consumption, maximum heart rate, absolute and indexed maximum O2 pulse, ventilatory equivalent of CO2 and oxygen consumption/Work. Trend of cardiopulmonary parameters was analysed over time. Maximal workload, maximum oxygen consumption and ventilatory equivalent of CO2 were compared with a control group of patients with a more sedentary lifestyle, matched for diagnosis, gender, age, and body mass index. RESULTS: Among one hundred and eleven patients, 73 males (66%) were analysed. Median age was 14 (12-17) years. Twenty-nine patients (27%) were practising sports at competitive level. Maximum oxygen consumption and oxygen consumption % of maximum predicted were not significantly different at follow-up as compared with baseline. Follow-up of maximum oxygen consumption was 38.2 ± 9 ml/kg/min versus 38.6 ± 9.2 ml/kg/min (p = NS) and follow-up of %oxygen consumption was 88 ± 20 versus 87 ± 15 (p = NS). Ventilatory equivalent of CO2 significantly improved in the last test as compared with the baseline: 30 ± 4 versus 33 ± 5 (p = 0.002). As compared with the control group, trained patients displayed a significantly higher maximum workload and oxygen consumption, while ventilatory equivalent of CO2 was not significantly different. CONCLUSIONS: In our cohort, patients following a regular training programme displayed a significantly higher functional capacity as compared with not trained control group, irrespective of NYHA class. Objective functional capacity was stable over a median follow-up of 3 years.

The diagnostic accuracy of carbon monoxide pulse oximetry in adults with suspected acute carbon monoxide poisoning: a systematic review and meta-analysis.

Introduction: 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. Methods: 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). Results: 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). Conclusion: 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.

Acute myocarditis as first presentation of severe ulcerative colitis in a young man. Multidisciplinary management and long-term follow-up.

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. Learning objective: •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.

Adherence of ticagrelOr in real world patients with aCute coronary syndrome: The AD-HOC study.

Background: Dual antiplatelet therapy (DAPT) with aspirin and a P2Y12 receptor antagonist is the cornerstone of therapy in patients with acute coronary syndrome (ACS). Adherence to medical therapy is an important issue, as premature DAPT discontinuation increases the risk of new ischemic events. The aim of the present observational prospective multicenter study was to evaluate in the real-world incidence and discontinuation patterns of ticagrelor during the first 12 months after ACS. Methods: We analyzed 431 ACS patients, discharged with ticagrelor, by 7 Italian centers. The primary end-point was the incidence of cessation of ticagrelor up to 12 months from the index event. Results: Definitive ticagrelor cessations occurred in 52 patients (12.1%), of which 35 were discontinuations (clinically driven) and 17 disruptions (due to acute events). Temporary cessation occurred in 14 cases (3.3%). Age ≥ 80 years and anticoagulant therapy were independent predictors of premature discontinuation. Bleeding occurred in 74 patients, of which 25 suffered a BARC ≥ 2 bleeding event. Bleeding were more frequent in female sex (27.0% vs 17.2%, p-value 0.049) and in patients with a history of bleeding (8.1% vs 2.9%, p-value 0.035). Conclusions: Our study found that the adherence to DAPT with ticagrelor after an ACS is still an important issue, premature discontinuation occurred mainly in fragile patients, like elderly, who suffered a previous bleeding or underwent previous percutaneous coronary intervention.

First case reported of COVID-19 infection in an adult patient with Ellis-van Creveld Syndrome.

Ellis-van Creveld syndrome (EVC) is a rare autosomal recessive disorder, the features of the syndrome are: chondral and ectodermal dysplasia characterized by short ribs, polydactyly, growth retardation resulting in dwarfism, teeth and craniofacial abnormalities and heart defects (mostly endocardial cushions and atrial septal defects). We describe the first case reported of COVID-19 infection in a 24-years-old girl, diagnosed with EVC syndrome. The patient suffered only from a mild illness, she remained stable with normal saturation without need of neither respiratory support nor specific therapy and she was rapidly discharged. This case appraises the pathophysiological interplay between different specific prognostic variable in a syndromic patient with congenital heart disease and COVID-19. In patients with congenital heart disease, comorbidities related to syndromic picture may affect the clinical course of COVID-19 infection regardless of the anatomic complexity.

Early Diagnosis of Acute Myocarditis in the ED: Proposal of a New ECG-Based Protocol.

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.

Oncogenesis in patients with congenital heart disease: A possible role of the neural crest.

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.

Cardio-thoracic imaging and COVID-19 in the pediatric population: A narrative review.

Worldwide experience about coronavirus disease 2019 (COVID-19) pandemics suggests that symptomatic disease is significantly less frequent in the pediatric age range. Nevertheless, multi-system inflammatory syndrome has been consistently reported in children and has been associated with severe acute respiratory syndrome coronavirus 2 exposure. In this paper we give an overview of the multimodality chest imaging of pediatric patients with suspected COVID-19, focusing on relevant differences with adults.

Catheter closure of a patent vertical vein after repair of an unusual total anomalous variant of pulmonary venous drainage.

Total anomalous pulmonary venous drainage (TAPVD) encompasses a wide spectrum of anatomical variants. The infradiaphragmatic type is almost always obstructive owing to the interposition of intrahepatic resistances; since almost invariable, the vertical vein inserts on the portal venous system. On correction of this variant, the vertical vein might not be ligated to avoid postoperative pulmonary hypertension. We hereby describe an unusual case of infradiaphragmatic TAPVD, with a vertical vein connected to ductus venosus. Since vertical vein was not ligated, it realized an unrestrictive pathway between the left atrium and the suprahepatic veins which resulted in persistent chylous peritoneal drainage. The patient successfully underwent catheter occlusion of the vertical vein which led to complete resolution of the clinical picture.

Patterns of myocardial involvement in children during COVID-19 pandemic: Early experience from northern Italy.

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.

QRS fragmentation as a possible electrocardiographic diagnostic marker in patients with acute myocarditis: preliminary histopathological validation.

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.

COVID-19 in adult patients with CHD: a matter of anatomy or comorbidities?

Little is know about COVID-19 outcome in specific populations such as Adult congenital heart disease (ACHD) patients. We report three cases of adult patients with similar underlying disease with completely different clinical severity at the time of COVID-19 infection. The patient with the most severe clinical course was obese and diabetic, suggesting that COVID-19 mortality and morbidity in Adult congenital heart disease patients might be independent of anatomic complexity.

QRS fragmentation in children with suspected myocarditis: a possible additional diagnostic sign.

AIMS: We aim to assess the diagnostic role of QRS fragmentation in children with suspected acute myocarditis. BACKGROUND: Diagnosis of myocarditis in the paediatric population is challenging. Clinical suspicion, electrocardiogram, and laboratory tests are the main diagnostic features at presentation. However, electrocardiogram in patients with myocarditis is usually considered aspecific. We have previously described QRS fragmentation in adult patients with acute myocarditis. METHODS: Patients aged less than 18 years, admitted between 2003 and 2019, and discharged with a diagnosis of acute myocarditis were included. Standard electrocardiogram, laboratory, and echocardiographic findings at admission and follow-up were reviewed. QRS fragmentation was defined by the presence of multiphasic R' spikes. Cardiac magnetic resonance and biopsy were performed in selected patients. RESULTS: Twenty-one patients were analysed, 16 males (76%), median age 9.5 (2.5-16) years. At presentation, 12 patients (57%) displayed QRS fragmentation. Median ejection fraction was 40% (27-60). Nine patients (43%) underwent cardiac magnetic resonance and displayed late gadolinium enhancement. One patient underwent biopsy that showed borderline findings. Electrocardiogram leads showing QRS fragmentation correlated with distribution of late gadolinium enhancement. Median follow-up was 600 (190-2343) days. All patients were alive at last follow-up. Six patients (33%) patients displayed persistence of QRS fragmentation. Median ejection fraction was 60% (60-65%). In three patients (14%), ejection fraction remained depressed, two of which showed persistence of QRS fragmentation. CONCLUSION: In this cohort of children with suspected myocarditis, QRS fragmentation was confirmed as a new additional diagnostic finding to look for at admission and during follow-up.