A case of Seckel syndrome with tricuspid atresia.

Seckel syndrome is an autosomal recessive disease presenting with marked growth retardation, microcephalic dwarfism, some facial and skeletal abnormalities. Tricuspid atresia is a rare and life threatening cyanotic congenital heart diseases, with an incidence of 1% to 3%. It is feature of the anatomically normally related great arteries with a large ventricular septum defect and stenosis of right ventricular outflow tract. Tricuspid atresia has never been reported in patients with Seckel syndrome. Here we report a 15-day-old girl baby diagnosed as having Seckel syndrome with tricuspid atresia.

The role of neutrophil-lymphocyte platelet ratio in predicting in-hospital mortality after acute Type A aortic dissection operations.

OBJECTIVE: Acute Type A aortic dissection (ATAAD) is an emergency cardiovascular condition. In this current study, we aimed to investigate the prognostic importance of preoperative neutrophil-lymphocyte platelet ratio (NLPR) value in predicting in-hospital mortality, after surgical treatment of ATAAD. PATIENTS AND METHODS: Consecutive patients who underwent an emergency operation as a result of ATAAD between August 2012 and August 2021 in our hospital, were retrospectively included in this study. Patients who survived the operation and were released were recorded as Group 1 and those who died in the hospital, as Group 2. RESULTS: Mortality (in-hospital) occurred in 44 (22.5%) patients (Group 2). The median age of the 151 patients included in Group 1 and 44 patients in Group 2 were 55 (37 to 81) and 59 (33 to 72) years, respectively (p = 0.191). In multivariate analysis Model 1, malperfusion (OR: 3.764, 95% CI: 2.140-4.152, p < 0.001), total perfusion time (OR: 1.156, 95% CI: 1.040-1.469, p = 0.012), low platelet counts (OR: 0.894, 95% CI: 0.685-0.954, p = 0.035) and NLR (OR: 1.944, 95% CI: 1.230-2.390, p < 0.001) were determined as independent predictors for mortality. In Model 2, malperfusion (OR: 3.391, 95% CI: 2.426-3.965, p < 0.001) and NLPR (OR: 2.371, 95% CI: 1.892-3.519, p < 0.001) were determined as independent predictors for mortality. CONCLUSIONS: According to our study, the NLPR value obtained preoperatively can be used to predict the risk of in-hospital mortality, after ATAAD surgery.

The Relationship between Metabolic Parameters, Cardiac Parameters and MIC-1/GDF15 in Obese Children.

Objective: Childhood obesity which is a predictor of adulthood obesity is associated with type 2 diabetes, metabolic syndrome, dyslipidemia, coronary artery diseases and subclinical inflammation. Growth differentiation factor-15, also known as macrophage inhibitory cytokine-1 (MIC-1/GDF15) is a member of the transforming growth factor- ß super family and increases during inflammatory states. Adults with obesity have increased serum concentrations of MIC-1/GDF15. MIC-1/GDF15 is associated with cardio metabolic risk in adults. Design: Identification of obese children at higher risk for cardio metabolic complications is important. The relations of MIC-1/GDF15 to parameters of obesity in children have never been studied. Methods: We measured serum MIC-1/GDF15 concentration in obese children by ELISA and evaluated its relationship with anthropometric, biochemical and echocardiographic parameters. Results: No significant difference in serum MIC-1/GDF15 between normal-weight and obese children was observed. MIC-1/GDF15 was positively correlated with CRP, ALT and aortic dimension when normal-weight and obese children were analyzed together. Conclusions: MIC-1/GDF15 may provide a link between childhood obesity and cardio metabolic complications that will occur in time course. Further work would be required to find out if MIC-1/GDF15 has any role in the early inflammatory, cardiac and metabolic changes in childhood obesity.

Prospective ECG-gated high-pitch dual-source cardiac CT angiography in the diagnosis of congenital cardiovascular abnormalities: Radiation dose and diagnostic efficacy in a pediatric population.

PURPOSE: The goal of this study was to investigate the radiation dose and diagnostic efficacy of cardiac computed tomography angiography (CCTA) using prospective ECG-gated high-pitch dual-source computed tomography (DSCT) in the diagnosis of congenital cardiovascular abnormalities in pediatric population. MATERIALS AND METHODS: One hundred five pediatric patients who were clinically diagnosed with congenital heart disease with suspected extracardiac vascular abnormalities were included in the study. All CCTAs were performed on a 128×2-section DSCT scanner. CCTA findings were compared with surgical and/or conventional cardiac angiography findings. Dose-length product (DLP) and effective doses (ED) were calculated for each patient. Patients were divided into 4 groups by age, and ED and DLP values were compared among groups. The image quality was evaluated using a five-point scale. RESULTS: CCTA showed 173 abnormalities in 105 patients. There were 2 patients with false positive and 3 with false negative findings. The sensitivity and specificity of CCTA were 98.3% and 99.9%, respectively. The positive predictive value and negative predictive value of CCT were 98.9% and 99.9%, respectively. The average DLP and ED values were 15.6±9.6 (SD) mGy.cm and 0.34±0.10 (SD) mSv, respectively. The mean image quality score was 4.8±0.5 (SD) in all patients. The inter-observer agreement for the image quality scores was good (κ=0.80). CONCLUSION: CCTA is an excellent imaging modality for evaluation of cardiovascular abnormalities and provides excellent image quality with very low radiation exposure when low-dose prospective ECG-triggered high-pitch DSCT is used.