Diagnostic & Therapeutic Center of Athens Hygeia SA

Tropomyosin receptor kinase (TRK) fusions are detected in less than 2% of central nervous system tumors. There are limited data on the clinical course of affected patients.

We conducted an international retrospective cohort study of patients with TRK fusion–driven central nervous system tumors.

A total of 119 patients were identified. The median age at the time of diagnosis was 4.5 years. The majority were reported to have a histology consistent with a diagnosis of high-grade glioma (HGG; 57.1%) followed by low-grade glioma (LGG; 27.7%). Pediatric patients had a better prognosis, with a median overall survival of 185.5 months compared with 24.8 months in adults (P < 0.0001). Patients with LGG also had a better outcome when compared with HGG (P = 0.0012). The objective response was 68.8% with larotrectinib compared with 38.1% for nontargeted treatment.

Children with LGG had a favorable outcome compared with adult glioma and HGG. TRK inhibitors seem to improve tumor control.

Atrial fibrillation (AF) is the most common sustained cardiac arrhythmia in patients with cardiomyopathies and its clinical management presents a significant challenge. The prevalence of AF varies among cardiomyopathies, with hypertrophic (HCM) and dilated cardiomyopathy (DCM) associated with higher rates of AF. Presence of AF portends increased risk for thromboembolism, heart failure, and cardiovascular morbidity and mortality in patients with cardiomyopathy. The complex genetic substrate in DCM and non-dilated left ventricular cardiomyopathy (NDLVC) contribute to the heterogeneity of AF burden and its sequelae among cardiomyopathy genotypes, necessitating genotype-tailored approach in AF screening and management. Given the lack of validation of traditional risk scores for AF in cardiomyopathies, current clinical recommendations emphasize the importance of comprehensive risk stratification for AF, monitoring for AF, and early initiation of oral anticoagulation for brief AF episodes in specific cardiomyopathy subtypes such as hypertrophic or amyloid cardiomyopathy. AF management includes antiarrhythmic drugs, interventional therapies such as catheter ablation, mitral valve replacement when necessary, and lifestyle modifications to attenuate AF burden and improve quality of life. This review summarizes the current knowledge on the clinical significance, prognostic implications, and treatment of AF among different cardiomyopathy subtypes. We underscore the paradigm shift in AF management advocating for an individualized, subtype-specific, and genotype-aware approach to AF in cardiomyopathies, which is instrumental in improving prognosis and patient-centric care.

It is estimated that the number of patients with a cardiac implantable electronic device (CIED) in Greece exceeds 120,000, and this population is expected to further rise by 5% annually. The importance of adequate monitoring and follow-up management of these devices is well-recognized. However, the increasing complexity and growing number of CIEDs makes their management a demanding medical service. Traditionally, interrogation and programming of CIEDS is performed through the use of a portable programmer by qualified personnel, requiring patient physical presence. During the last decade, remote monitoring (RM) of CIEDs tends to become more and more popular given the advantages and improved outcomes in many groups of patients. Currently, RM represents the standard of care for CIEDs follow-up, and it is recommended by major cardiology societies worldwide, including the European Society of Cardiology. The objective of this statement is to summarize current management of patients with CIED in Greece and the available evidence about clinical efficacy and safety of RM of CIEDs, present the most recent guideline recommendations, and finally, to propose actions to move towards widespread adoption of RM of CIEDs in Greece.