THE IMPORTANCE OF SEVERE CYTOLYSIS SYNDROME IN HYPERTROPHIC CARDIOMYOPATHY IN CHILDREN

Hypertrophic cardiomyopathy is the second most frequent cardiomyopathy in children and it counts as a genetic disorder for a high number of cases, with widely variable clinical presentation. The disease can be recognized in all age groups and has an unpredictable prognosis, especially if undiagnosed on time. We present the case of a two and a half years old male toddler diagnosed at territorial level with systolic heart murmur, who manifested mild fatigue, whose echocardiographic examination showed obstructive hypertrophic cardiomyopathy and restrictive atrial septal defect. Laboratory findings revealed a severe, progressive cytolysis syndrome, with negative genetic tests for Duchenne muscular dystrophy and type II glycogen storage disease – Pompe disease. Haematological tests excluded a pathology in this section and the serology for viruses with liver tropism was negative. The particularity of this case lies in the discrepancy between severe cytolysis syndrome and clinical oligosymptomatic presentation

Fetal Tachyarrhythmia Management from Digoxin to Amiodarone—A Review

Sustained fetal tachycardias are rare but represent a high risk of mortality and morbidity. Consensus has yet to be found regarding their optimal management. The aim of this narrative review is to summarize the data available in the current literature regarding the efficacy and safety of medications used in the management of intrauterine tachyarrhythmias and to provide possible treatment protocols. In this review, we would like to emphasize the importance of a thorough evaluation of both the fetus and the mother, prior to transplacental antiarrhythmic drug initiation. Factors such as the hemodynamic status of the fetus, possible mechanisms of fetal arrhythmia, and concomitant maternal conditions are of primordial importance. As a possible treatment protocol, we would like to recommend the following: due to the risk of sustained supraventricular tachycardia (SVT), fetuses with frequent premature atrial beats should be evaluated more frequently by echocardiography. A careful hemodynamic evaluation of a fetus with tachycardia is primordial in forestalling the appearance of hydrops. In the case of atrial flutter (AFL), sotalol therapy could represent a first choice, whereas when dealing with SVT patients, flecainide should be considered, especially for hydropic patients. These data require consolidation through larger scale, non-randomized studies and should be handled with caution

Impact of Early Surfactant Administration on Ductus Arteriosus Assessed at 24 h in Preterm Neonates Less than 32 Weeks of Gestational Age

Background and Objectives: The purpose of this study was to investigate whether early surfactant administration affects the status of ductus arteriosus (DA) in preterm infants ≤ 32 weeks of gestational age (GA) within 24 h of birth. Materials and Methods: It is a prospective study conducted from 1 March 2022 to 31 December 2023 in a tertiary academic center. In-born infants ≤ 32 weeks of gestation (n = 88) were enrolled. The study group was further divided into surfactant (n = 44) and non-surfactant (n = 44) subgroups. Results: A total of 76% of the preterm infants who received surfactant therapy (RRR = 0.839) recorded an increase in Kindler score at 24 h of life (1 − RR = 1 − 0.24 = 76%). Surfactant administration was significantly associated with decreased pre-ductal diastolic pressure (29.9 mmHg vs. 34.8 mmHg, p = 0.0231), post-ductal diastolic pressure (28.7 mmHg vs. 32.2 mmHg, p = 0.0178), pre-ductal MAP (41.6 mmHg vs. 46.5 mmHg, p = 0.0210), and post-ductal MAP (41.0 mmHg vs. 45.3 mmHg, p = 0.0336). There were no significant changes in ductus arteriosus parameters at 24 h of life. Conclusions: Early surfactant administration does not affect the status of ductus arteriosus in preterm infants ≤ 32 weeks of gestational age at 24 h of life