The influence of maternal COVID-19 on cardiac functions: From fetal life to infancy.

OBJECTIVE: To evaluate both short and mid-term effects of maternal COVID-19 on cardiac functions of fetuses and children. METHODS: The present case-control study was conducted on 36 pregnant women who had COVID-19 infection in the second trimester of pregnancy and 30 pregnant women as healthy controls. Fetal, neonatal, and infant cardiac functions were compared between the groups. Assessment of fetal cardiac functions were performed in the last trimester of the pregnancy at least 6 weeks after the recovery of infection. The first postnatal echo was performed within the first 2 weeks and the follow-up (second) echo was performed in the 6-8 weeks of life. RESULTS: The demographic data were similar between groups. Interventricular septum and left ventricular posterior wall end-diastolic dimensions were significantly higher in the study group in both fetal, neonatal, and infant periods. Impaired diastolic functions of right and left ventricles were detected and myocardial performance indexes with tissue doppler imaging of both lateral walls and septum were significantly higher than controls at all periods. CONCLUSION: Maternal COVID-19 seems to have a global impact on the cardiac functions of babies in the short and mid-term periods after maternal recovery.

Admission and follow-up cardiac magnetic resonance imaging findings in BNT162b2 Vaccine-Related myocarditis in adolescents.

BACKGROUND: There is limited data on the pattern and severity of myocardial injury in patients with COVID-19 vaccination associated myocarditis. OBJECTIVE: We aimed to define the myocardial damage occurring after BNT162b2 vaccination, raise awareness about adverse reactions developing after vaccination, and determine the patterns and scope of Cardiac magnetic resonance imaging (MRI) findings. PATIENTS/METHODS: A total of 9 patients diagnosed with vaccine-associated myopericarditis were followed up. RESULTS: The mean age of the patient at diagnosis was 15.3 ± 1.0 (range: 14-17) years, and all patients were male. Seven patients presented with myocarditis symptoms after their second vaccine dose, one patient presented with pericarditis symptoms after his first dose, and the other patient presented with myocarditis symptoms after his booster dose. The median time at presenting to the hospital was 3 (range: 2-22) days. Seven (77.7%) patients had abnormal electrocardiography (ECG) findings, and the most prevalent finding was diffuse ST-segment elevation. Initial cardiac MRI results were abnormal in all patients, where 8 (88.8%) patients had late gadolinium enhancement, and 5 (55.5%) had myocardial edoema. Three patients showed local left ventricular wall-motion abnormalities. In their follow-up MRIs 3-6 months later, myocardial edoema was present in 2 (28.5%) patients, while late gadolinium enhancement was present in all patients (7/7, 100%, 2 patients did not have control MRI time). Hypokinetic segments were still present in one of the 3 patients. No negative cardiac events were observed in the short-term follow-up of any patient. CONCLUSION: Further follow-up evaluation and larger multicenter studies are needed to determine the clinical significance of persistent cardiac MRI abnormalities.

Primary CoQ10 deficiency with a severe phenotype due to the c.901 C > T (p.R301W) mutation in the COQ8A gene.

PURPOSE: A patient with primary CoQ10 deficiency associated with the c.901 C > T (p.R301W) (rs140246430) homozygous missense pathogenic variant in the COQ8A gene, who presented with recurrent status epilepticus, stroke-like lesions, and hypertrophic cardiomyopathy while being followed-up with early-onset autosomal recessive cerebellar ataxia will be reported in this article. CASE REPORT: A 16-year-old patient who was being followed up at an external center with a diagnosis of ataxia with cerebellar atrophy had been seen 3 different times within a year for status epilepticus. The cerebral MRI showed severe cerebellar atrophy, stroke like lesions, and an inverted double- lactate peak on spectroscopy. Her echocardiography revealed marked left ventricular hypertrophy. Mitochondrial cocktail therapy containing a standard dose of CoQ10 was started, considering mitochondrial disease. The patient died due to cardiomyopathy. Mitochondrial panel analysis revealed the presence of the c.901 C > T (p.R301W) homozygous missense mutation in the COQ8A gene. CONCLUSIONS: Primary Coenzyme Q10 deficiency should be considered in patients presenting with autosomal recessive stable-appearing progressive ataxia, emerging attacks of status epilepticus, stroke-like lesions on neuroimaging, and cardiomyopathy. Since there is a case with the same mutation with a similar fatal course in the literature, detection of c.901 C > T (p.R301W) mutation homozygously should be a warning for a severe prognosis and more aggressive treatment should be started without delay with a high dose of CoQ10 instead of the lower doses used in the treatment of mitochondrial disease.

Investigation of the prevalence of cardiovascular risk factors in obese patients diagnosed with metabolic syndrome in childhood and examination of left ventricular function by echocardiography.

OBJECTIVES: The objective of this study is to investigate the cardiovascular risk factors associated with metabolic syndrome (MetS), which is increasingly becoming prevalent in childhood obesity. METHODS: A total of 113 patients, 76 of whom were between the ages of 10 and 17 (mean age: 14.5 ± 1.8 years) and diagnosed with obesity (30 non-MetS and 46 MetS using IDF) and 37 of whom constituted the control group, participated in the study. Echocardiographic examination and atherogenicity parameters (Atherogenic index of plasma [AIP: logTG/HDL], total cholesterol/HDL, and TG/HDL ratio and non-HDL) were evaluated. RESULTS: The most common component accompanying obese MetS was found to be hypertension and low HDL. While obesity duration, body mass index (BMI), blood pressure, fasting insulin, insulin resistance, atherogenicity parameters were determined to be significantly higher in the obese-MetS group. Echocardiography showed that while the thickness, volume, and diameter of LV end-diastolic wall, left ventricular mass (LVM), LVM index (LVMI g/m2) and relative wall thickness (RWT) were significantly high in the MetS group, however, mitral E/A ratio was significantly lower (p<0.05). Change in LV geometry consistent with concentric remodeling (increased RWT, normal LVMI) was visible in obese groups. LVM were positively significantly related to BMI, waist circumference, insulin resistance, blood pressure, LDL level, and negative to mitral E/A ratio. In the obese-MetS group, LVMI was positively correlated to office systolic BP, left atrium end-diastolic volume/index. CONCLUSIONS: LVMI and atherogenicity parameters that were found to be significantly higher in obese MetS exhibit increased cardiovascular risk in childhood.

Therapeutic plasma exchange: A potential management strategy for critically ill MIS-C patients in the pediatric intensive care unit.

This study aimed to evaluate the effectiveness and the role of therapeutic plasma exchange (TPE) in treatment of children with severe MIS-C. In addition, we assessed demographic data, clinical features, laboratory abnormalities, underlying conditions, treatments, and outcomes. Patients with severe MIS-C who were admitted to the pediatric intensive care unit (PICU) between September 01 and October 05, 2020 were included in this observational, descriptive, retrospective study. The data collected included the patients' demographic data, presenting symptoms, clinical features, laboratory parameters, diagnostic investigations, and medications. Of 27 children with MIS-C, 63 % were male. The median age of the patients was nine years. Intravenous immunoglobulin and corticosteroids were used for treatment in 100 % of the patients, anakinra in 51.8 %, vasopressors in 85.1 %, noninvasive mechanical ventilation in 25.9 %, and invasive mechanical ventilation in 18.5 %. Ten of the 27 patients (37 %) underwent TPE. In the patients who underwent TPE, the median PELOD score was 21 (IQR: 11-30.25) before TPE and 10 (IQR: 10-11) after TPE (p < 0.001). Moreover, their median left ventricular ejection fraction (LVEF) was 52 % (IQR: 49.25 %-55 %) before TPE and median LVEF was 66.5 (IQR: 58 %-68.5 %) after TPE (p = 0.012). The median number of TPE sessions was three (IQR: 2-4.75). The mortality rate of the patients with severe MIS-C admitted to the PICU was 7.4 %. We suggest that TPE should be considered as a therapeutic option in children with severe MIS-C. Early initiation of TPE followed by immunomodulatory therapy in critically ill children with MIS-C may help improve clinical and laboratory outcomes.