Case Report: Remote magnetic navigation and accessory pathways ablation in a single ventricle young adult with complex corrective surgeries

Supraventricular arrhythmias have become an increasingly significant contributor to the risk of mortality and morbidity in adults with complex congenital heart disease (CHD), especially in light of recent advances in palliative corrective surgeries. Because of their unique characteristics, they demand specific treatment approaches. While pharmaco-logical interventions are an option, they have limited effectiveness and may lead to side effects. Although performing radiofrequency ablation (RFA) can be exceptionally challenging in patients with complex CHD, due to particular vascular access and also modified anatomy, it has paved the way to enhance comprehension of the underlying mechanisms of supraventricular arrhythmias. This, in turn, enables the provision of improved therapies and, ultimately, an enhancement in the quality of life and symptom management for these patients. The purpose of this case report is to highlight the benefits of utilizing advanced technologies such as three-dimensional electro-anatomical mapping systems, remote magnetic navigation, and highly flexible mapping and ablation catheters during RFA in a young adult with complex congenital heart disease. Although he lacked venous connections to the right atrium (RA) due to multiple corrective surgeries we, remarkably, were capable to advance a decapolar deflectable diagnostic catheter inside the Fontan tunnel and from there to record and stimulate the RA. Successful ablation of two accessory pathways was achieved with no arrhythmia recurrence during follow-up

Management of macrovascular diabetic complications: a single-center case series analysis of consecutively attending patients in primary care

Introduction. The prevalence of diabetes mellitus is increasing, with type 2 diabetes (T2DM) being a major health priority for any public health system. Increased arterial stiffness in patients with diabetes will lead to the appearance of vascular complications. Increased arterial stiffness in patients with diabetes usually leads to vascular complications. Any earlier diagnosis of impaired macrovascular evidence may lead to improved outcomes in patient care. The objective of our study was to assess and evaluate the finger-toe pulse wave velocity (ftPWV), as a measure of arterial stiffness, in order to assist with early detection of macrovascular diabetic complications. Materials and Methods. The observational case series included 140 patients who are registered in a primary care office, of whom 73 were previously diagnosed with diabetes mellitus (study group). The age-matched non-diabetic group included 67 consecutive registered patients who visited the practice for other reasons. Results. The mean age of all patients was 51.42±11.57 years, with DM patients being with 4.5 years older than the non-DM patients (CI 95% and CI 95%). There was a significantly higher mean value of ftPWV in the DM group (p = 0.0039) although the study presented some limitations. Conclusions. The mean value of ftPWV was statistically significant higher in diabetic patients. The assessment of ftPWV is a non-invasive test, and the data can be used as a useful marker of vascular stiffness in primary care, thus providing an early diagnosis of macrovascular complications during the monitoring and care of the diabetic patient

Challenging Diagnosis of Anomalous Origin of the Right Coronary Artery from the Pulmonary Artery

Anomalous origin of the right coronary artery (RCA) from the pulmonary artery, ARCAPA, is an extremely rare congenital heart disease. Only 200 cases were reported from 1885 to the present. Patients diagnosed with ARCAPA can be either asymptomatic or can experience symptoms, such as heart murmur, dyspnea, or angina, shortly after birth or around 40–60 years of life. Usually, those with isolated ARCAPA are diagnosed later in life compared to those who associate other structural cardiac defects. We report two cases of anomalous origin of the right coronary artery at the level of the pulmonary artery trunk (ARCAPA) that were diagnosed by invasive coronary angiography. Although asymptomatic, general recommendations suggest an early corrective intervention to prevent complications such as myocardial ischemia and cardiac dysfunction, which can lead to sudden cardiac death

Myocardial Infarction in Children after COVID-19 and Risk Factors for Thrombosis

Acute myocardial infarction (AMI) in children is rather anecdotic. However, following COVID-19, some conditions may develop which may favor thrombosis, myocardial infarction, and death. Such a condition is Kawasaki-like disease (K-lD). K-lD appears in children as a subgroup of the multisystem inflammatory syndrome (MIS-C). In some cases, K-lD patients may develop giant coronary aneurysms. The evolution and characteristics of coronary aneurysms from K-lD appear to be different from classical Kawasaki disease (KD) aneurysms. Differences include a lower percentage of aneurysm formation than in non-COVID-19 KD, a smaller number of giant forms, a tendency towards aneurysm regression, and fewer thrombotic events associated with AMI. We present here a review of the literature on the thrombotic risks of post-COVID-19 coronary aneurysms, starting from a unique clinical case of a 2-year-old boy who developed multiple coronary aneurysms, followed by AMI. In dehydration conditions, 6 months after COVID-19, the boy developed anterior descending artery occlusion and a slow favorable outcome of the AMI after thrombolysis. This review establishes severity criteria and risk factors that predispose to thrombosis and AMI in post-COVID-19 patients. These may include dehydration, thrombophilia, congenital malformations, chronic inflammatory conditions, chronic kidney impairment, acute cardiac failure, and others. All these possible complications should be monitored during acute illness. Ischemic heart disease prevalence in children may increase in the post-COVID-19 era, due to an association between coronary aneurysm formation, thrombophilia, and other risk factors whose presence will make a difference in long-term prognosis

Acute Myocarditis-Like Episode in a Curly-Haired Young Boy—Red Flags for Familial Arrhythmogenic Cardiomyopathy

The present case report describes a mother and son with arrhythmogenic cardiomyopathy (ACM) with early and greater left ventricle (LV) involvement. The presence of curly hair in both, together with the resuscitated sudden cardiac death of the mother, allowed timely genetic testing, which found a pathogenic nonsense mutation of the desmoplakin gene. While asymptomatic from an arrhythmic point of view, the son’s evolution was characterized by a well-documented exercise-induced myocarditis-like stage

Phospholamban p.Leu39* Cardiomyopathy Compared with Other Sarcomeric Cardiomyopathies: Age-Matched Patient Cohorts and Literature Review

Hypertrophic cardiomyopathy (HCM) is a heterogeneous genetic disorder, most often caused by sarcomeric gene mutations, with a small proportion due to variants in non-sarcomeric loci. Phospholamban (PLN) is a phosphoprotein associated with the cardiac sarcoplasmic reticulum, a major determinant of cardiac contractility and relaxation. We conducted a retrospective study to determine the prevalence, phenotypical spectrum and clinical course of patients carrying the PLN p.Leu39* variant. A cohort including 11 PLN patients was identified among all patients with HCM (9/189, 4.8%) and DCM (2/62, 3.2%) who underwent genetic testing from two tertiary centers and five more were detected through cascade screening. Complete phenotyping was performed. PLN p.Leu39* variant-driven cardiomyopathy presented mostly as hypertrophic, with frequent progression to end-stage dilated HCM. We proceeded to compare these results to a similar analysis of a control cohort consisting of age-matched individuals that inherited pathogenic or likely pathogenic variants in common sarcomeric genes (MYBPC3/MYH7). Overall, the clinical characteristics and examination findings of patients carrying PLN p.Leu39* were not different from patients with cardiomyopathy related to sarcomeric mutations except for the presence of pathological Q waves and the incidence of non-sustained ventricular arrhythmias, which were higher in PLN patients than in those with MYBPC3/MYH7-related diseases

An Up-to-Date Literature Review on Ventricular Assist Devices Experience in Pediatric Hearts

Ventricular assist devices (VAD) have gained popularity in the pediatric population during recent years, as more and more children require a heart transplant due to improved palliation methods, allowing congenital heart defect patients and children with cardiomyopathies to live longer. Eventually, these children may require heart transplantation, and ventricular assist devices provide a bridge to transplantation in these cases. The FDA has so far approved two types of device: pulsatile and continuous flow (non-pulsatile), which can be axial and centrifugal. Potential eligible studies were searched in three databases: Medline, Embase, and ScienceDirect. Our endeavor retrieved 16 eligible studies focusing on five ventricular assist devices in children. We critically reviewed ventricular assist devices approved for pediatric use in terms of implant indication, main adverse effects, and outcomes. The main adverse effects associated with these devices have been noted to be thromboembolism, infection, bleeding, and hemolysis. However, utilizing left VAD early on, before end-organ dysfunction and deterioration of heart function, may give the patient enough time to recuperate before considering a more long-term solution for ventricular support