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ObjectiveThere were reports of cardiac dysfunction that led to sudden unexpected death in epilepsy (SUDEP) in patients with epilepsy. Early detection of cardiac dysfunction can lead to early management to prevent sudden cardiac death in these patients. The objective of our study is to assess cardiac functions in children with drug-resistant epilepsy (DRE) compared with the normal population by using a standard echocardiogram (SE), tissue Doppler imaging (TDI) and myocardial strain evaluations (MSE).MethodTwenty-seven children who have been diagnosed with DRE based on the International League against Epilepsy (ILAE) were included in the study, along with 27 children whose ages match those of the normal control group.ResultsSeventeen children, median age 12 years old, were using more than four anti-seizure medications. Structural brain lesions were the most common cause of epilepsy, 55.6% (15). Generalized tonic–clonic seizures were the most common seizure type, 55.6% (15). Children with DRE had a lower early mitral valve E wave inflow velocity compared with the control group (p

Introduction/objectiveExtubation failure in pediatric patients with congenital or acquired heart diseases increases morbidity and mortality. This study aimed to develop a clinical risk score for predicting extubation failure to guide proper clinical decision-making and management.MethodsWe conducted a retrospective study. This clinical prediction score was developed using data from the Pediatric Cardiac Intensive Care Unit (PCICU) of the Faculty of Medicine, Chiang Mai University, Thailand, from July 2016 to May 2022. Extubation failure was defined as the requirement for re-intubation within 48 h after extubation. Multivariable logistic regression was used for modeling. The score was evaluated in terms of discrimination and calibration.ResultsA total of 352 extubation events from 270 patients were documented. Among these, 40 events (11.36%) were extubation failure. Factors associated with extubation failure included history of pneumonia (OR: 4.14, 95% CI: 1.83–9.37, p = 0.001), history of re-intubation (OR: 5.99, 95% CI: 2.12–16.98, p = 0.001), and high saturation in physiologic cyanosis (OR: 5.94, 95% CI: 1.87–18.84, p = 0.003). These three factors were utilized to develop the risk score. The score showed acceptable discrimination with an area under the curve (AUC) of 0.77 (95% CI: 0.69–0.86), and good calibration.ConclusionThe derived Pediatric CMU Extubation Failure Prediction Score (Ped-CMU ExFPS) could satisfactorily predict extubation failure in pediatric cardiac patients. Employing this score could promote proper personalized care. We suggest conducting further external validation studies before considering implementation in practice.

Pheochromocytomas are catecholamine-producing tumors derived from the adrenomedullary chromaffin cells. The presentation is a classic triad of episodic headaches, sweating, and tachycardia. Hypertensive crisis can occur due to profuse catecholamine excess. Unusual manifestations mimicking cardiogenic shock, arrhythmia, and myocarditis have been rarely reported in children. We present a case with uncommon manifestations of pheochromocytoma in a child, including the episodes of exercised-induced presyncope with QT prolongation, and subsequently cardiogenic shock due to fulminant myocarditis. He later developed hypertensive crisis. The adrenal mass on abdominal computed tomography with an increased chromogranin A level and elevated plasma normetanephrine, and the histological study confirmed the diagnosis of pheochromocytoma. Cardiac functions completely recovered after adrenalectomy. Genetic testing was positive for von Hippel-Lindau syndrome. We describe pheochromocytoma crisis presenting with prolonged QT and catecholamine-induced myocarditis. We discuss the clues to assist in the diagnosis of this condition and its appropriate treatment.

Introduction/objectiveExtubation failure increases morbidity and mortality in pediatric cardiac patients, a unique population including those with congenital heart disease or acquired heart disease. This study aimed to evaluate the predictive factors of extubation failure in pediatric cardiac patients and to determine the association between extubation failure and clinical outcomes.MethodsWe conducted a retrospective study in the pediatric cardiac intensive care unit (PCICU) of the Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand, from July 2016 to June 2021. Extubation failure was defined as the re-insertion of the endotracheal tube within 48 hours after extubation. Multivariable log-binomial regression with generalized estimating equations (GEE) was performed to explore the predictive factors associated with extubation failure.ResultsWe collected 318 extubation events from 246 patients. Of these, 35 (11%) events were extubation failures. In physiologic cyanosis, the extubation failure group had significantly higher SpO2 than the extubation success group (P

CHARGE syndrome is a rare autosomal dominant disorder, associated with coloboma (C), heart defects (H), choanal atresia (A), retardation of growth and/or central nervous system (R), genitourinary anomalies (G) and ear abnormalities (E). Prenatal diagnosis of the syndrome is very rare but may be suspected when a combination of such abnormalities is identified. We describe a prenatally suspected case of CHARGE syndrome due to unique findings of cardiac defects (DORV) in combination with minor clues, including a structurally malformed ear with persistent non-response to an acoustic stimulation (which has never been prenatally described elsewhere), renal malrotation and growth restriction. Postnatal diagnosis was made based on confirmation of the prenatal findings and additional specific findings of bilateral coloboma, choanal atresia and ear canal stenosis. Finally, molecular genetic testing by whole exome sequencing of the neonate and her parents revealed a novel de novo heterozygous frameshift c.3506_3509dup variant in the CHD7 gene, confirming the clinical diagnosis of CHARGE syndrome. In conclusion, we describe unique prenatal features of CHARGE syndrome. Educationally, this is one of the rare examples of CHARGE syndrome, comprising all of the six specific anomalies as originally described; it is also supported by the identification of a specific genetic mutation. The identified genetic variant has never been previously reported, thereby expanding the mutational spectrum of CHD7. Finally, this case can inspire prenatal sonographers to increase awareness of subtle or minor abnormalities as genetic sonomarkers.

Objective: To compare fetal and neonatal cardiac morphology in fetuses of mothers with gestational diabetes mellitus (GDM) with the controls Patients and methods: Pregnant women at average risk of GDM underwent 100-g, 3-h-OGTT at 24-28 weeks of gestation for diagnosis of GDM. BothGDMgroup and the control group underwent fetal echocardiography at 32-36 weeks to assess cardiac dimensions. The neonates underwent echocardiography within 48 h after birth to assess cardiac morphology. Results: A total of 154 pregnant women were recruited, including 60 in the GDM group and 94 in the control group. All of the study group were well controlled for GDM. Most baseline characteristics of both groups were comparable. All obstetric outcomes were not significantly different between the two groups. Morphological cardiac dimensions in the fetuses and newborns of both groups were also not significant different. Subgroup analysis in the study group showed no significantly different in cardiac morphology between the group with diet control and that of insulin control. Conclusion: Fetal and neonatal cardiac morphologic changes among mothers with well-controlled GDM are not significantly different from those in the controls. It is possible that good control of maternal blood glucose can prevent fetal and neonatal cardiac abnormalities. [ABSTRACT FROM AUTHOR]