Percutaneous rheolytic thrombectomy and cerebral embolic protection in a massive thrombosis of a fenestrated Fontan conduit: a case report.

Background: Clinical thromboembolism in Fontan patients is often a catastrophic event resulting in death and adverse long-term outcomes. The treatment of acute thromboembolic complications in these patients is very controversial. Case summary: We describe the use of rheolytic thrombectomy in a Fontan patient with life-threatening pulmonary embolism, employing a cerebral protection system to reduce the risk of stroke through the fenestration. Discussion: Rheolytic thrombectomy may be a successful alternative to systemic thrombolytic therapy and open surgical resection for the treatment of acute high-risk pulmonary embolism in the Fontan population. Embolic protection device to capture and remove thrombus/debris may be an innovative tool to reduce the risk of stroke through the fenestration while performing a percutaneous procedure in fenestrated Fontan patient.

Atrial Flutters in Adults with Congenital Heart Disease.

The macroreentrant atrial tachycardia is very frequent in the adults with congenital heart disease. The impact of the arrhythmias on this type of patients is related to several factors: the anatomy and physiopathology of the specific congenital heart disease (CHD), the sequelae of the corrective surgery or surgical palliation, the presence of residual lesions (shunt, regurgitation), and the age and the clinical status of the patient and the comorbidities. In turn, the mechanism of the MAT depends on the peculiar features of the conduction's system in the CHD and native and acquired (post-surgery) substrates.

Atrial Flutter in Particular Patient Populations.

"Despite being one of the best understood cardiac arrhythmias, the clinical meaning of atrial flutter varies according to the specific context, and its optimal treatment may be limited by both the suboptimal response to rate/rhythm control drugs and by the complexity of the underlying substrate. In this article, we present a state-of-the-art overview of mechanisms, prognostic impact, and medical/interventional management options for atrial flutter in several specific patient populations, including heart failure, cardiomyopathies, muscular dystrophies, posttransplant patients, patients with respiratory disorders, athletes, and subjects with preexcitation, aiming to stimulate further research in this challenging field and facilitate appropriate patient care."

SARS-CoV-2 infection in kidney transplant recipients: Experience of the italian marche region.

BACKGROUND: Infection related to Coronavirus-19 (CoV-2) is pandemic affecting more than 4 million people in 187 countries worldwide. By May 10, 2020, it caused more than 280 000 deaths all over the world. Preliminary data reported a high prevalence of CoV-2 infection and mortality due to severe acute respiratory syndrome related CoV-2 (SARS-CoV-2) in kidney-transplanted patients (KTRs). Nevertheless, the outcomes and the best treatments for SARS-CoV-2-affected KTRs remain unclear. METHODS: In this report, we describe the clinical data, the treatments, and the outcomes of 5 KTRs with SARS-CoV-2 admitted to our hospital in Ancona, Marche region, Italy, from March 17 to present. Due to the severity of SARS-CoV-2, immunosuppression with calcineurin inhibitors, antimetabolites, and mTOR-inhibitors were stopped at the admission. All KTRs were treated with low-dose steroids. 4/5 KTRs were treated with hydroxychloroquine. All KTRs received tocilizumab up to one dose. RESULTS: Overall, the incidence of SARS-CoV-2 in KTRs in the Marche region was 0.85%. 3/5 were admitted in ICU and intubated. One developed AKI with the need of CRRT with Cytosorb. At present, two patients died, two patients were discharged, and one is still inpatient in ICU. CONCLUSIONS: The critical evaluation of all cases suggests that the timing of the administration of tocilizumab, an interleukin-6 receptor antagonist, could be associated with a better efficacy when administered in concomitance to the drop of the oxygen saturation. Thus, in SARS-CoV-2-affected KTRs, a close biochemical and clinical monitoring should be set up to allow physicians to hit the virus in the right moment such as a sudden reduction of the oxygen saturation and/or a significant increase in the laboratory values such as D-dimer.

A case of co-existence of muscle bridge and anomalous origin of coronary artery.

INTRODUCTION: Coronary artery anomalies result from disturbances which may occur to the fetus in the third week of development. These disturbances may lead to anatomical variations which may affect the origin, trajectory, and termination of these arteries, or alterations in their intrinsic anatomy. PRESENTATION OF CASE: We report an unusual case in which both anomalous origin of the right coronary artery and myocardial bridge on left anterior descending artery were detected concurrently. DISCUSSION: An investigation for myocardial bridge should be performed in all congenital coronary artery anomalies. CONCLUSION: Invasive testing could be considered in patients with myocardial and equivocal non-invasive functional testing, in order to obtain better risk stratification and treatment.

Evolution of a rare ECG pattern in an aggressive case of neonatal tuberous sclerosis complex.

INTRODUCTION: Rhabdomyomas are the most frequent cardiac tumors in children. Furthermore, they are often associated to tuberous sclerosis complex, an autosomal dominant neurocutaneous disorder characterized by tumor-like malformations that involve many organ systems. PRESENTATION OF THE CASE: We describe a rare ECG pattern in a severe case of neonatal tuberous sclerosis complex. DISCUSSION: In the presence of significant rhabdomyomatosis related to tuberous sclerosis, multiple clusters of rhabdomyoma-like cells can infiltrate the myocardium, with increased fibrosis areas. CONCLUSION: Considering the fact that rhabdomyomas often show spontaneous regression, close follow-up is sufficient in hemodynamically stable cases. Destruction of the conduction system, with arrhythmias as consequence, can be the presenting feature of diffuse rhabdomyomatosis.

Obstructive neonatal atrial myxoma.

INTRODUCTION: Cardiac myxoma is a benign neoplasm representing the most common primary cardiac tumor in adults, however it is unusual in neonates. It is represented by an endocardial mass that occupies the cardiac chamber. Although the majority of myxomas are attached to the fossa ovalis of the interatrial septum, they also attach to the walls of the cardiac chambers and to valve leaflets surfaces. Approximately 75% of myxomas are found in the left atrium, 20% are located in the right atrium, and rarely in the ventricles. PRESENTATION OF CASE: We describe a rare case of neonatal cardiac myxoma arising from interatrial septum, causing significant mechanical obstruction to blood flow through tricuspid valve, in an otherwise normal newborn. The patient underwent successful excision of the myxoma with an uneventful recovery [1]. DISCUSSION: Cardiac tumors are rare in children, thus an understanding of the common types of benign and malignant paediatric cardiac tumors and their imaging features, is important because the epidemiology and tumor types differ from those encountered in adults. CONCLUSION: Large neonatal myxoma is exceptionally rare and even more infrequent is the surgical excision in the first day of life.

The 212A variant of the APJ receptor gene for the endogenous inotrope apelin is associated with slower heart failure progression in idiopathic dilated cardiomyopathy.

BACKGROUND: Idiopathic dilated cardiomyopathy (IDC) has multiple genetic and acquired causes. Apelin is an endogenous peptide that increases cardiac inotropism through his APJ receptor. No data are available concerning the APJ gene mutations responsible for IDC or on the role of APJ receptor gene variants in predicting heart failure (HF) progression. METHODS AND RESULTS: We prospectively evaluated 202 consecutive patients with IDC and 202 matched controls: 90 were screened for APJ gene mutations and all 202 were genotyped for G212A and A445C APJ receptor polymorphisms. No mutations were found within the coding or untranslated regions of the APJ receptor, and no differences in allelic or genotype frequencies were observed comparing patients with a healthy control population. The correlations between APJ receptor polymorphisms and HF progression were assessed. During a median follow-up of 37 months, 35 patients experienced HF progression. Univariate analysis showed that patients carrying at least 1 copy of 212A had a significantly lower risk for HF-related events than those who were homozygous for the G212 variant, and multivariate analysis confirmed that it was significantly related to a more favorable prognosis. CONCLUSIONS: APJ is unlikely to be a gene causing IDC, but the independent correlation between the 212A allele and a better prognosis suggests that it might act as a modifier gene.