Evaluation of T-wave memory after accessory pathway ablation in pediatric patients with Wolff-Parkinson-White syndrome.

BACKGROUND: T-wave memory (TWM) is a rare cause of T-wave inversion (TWI). Alterations in ventricular activation due to abnormal depolarization may cause repolarization abnormalities on the ECG, even if myocardial conduction returns to normal. These repolarization changes are defined as TWM. In our study, we aimed to determine the frequency of TWM development and the predictors affecting it in the pediatric population who underwent accessory pathway (AP) ablation due to Wolff-Parkinson-White (WPW) syndrome. METHODS: The data of patients with manifest AP who underwent electrophysiological studies and ablation between 2015 and 2021 were retrospectively analyzed. The study included 180 patients who were under 21 years of age and had at least one year of follow-up after ablation. Patients with structural heart disease, intermittent WPWs, recurrent ablation, other arrhythmia substrates, and those with less than one-year follow-up were excluded from the study. The ECG data of the patients before the procedure, in the first 24 h after the procedure, three months, and in the first year were recorded. The standard ablation technique was used in all patients. RESULTS: Postprocedure TWM was observed in 116 (64.4%) patients. Ninety-three patients (51.7%) had a right-sided AP, and 87 patients (48.3%) had a left-sided AP. The presence of posteroseptal AP was found to be significantly higher in the group that developed TWM. Of these patients, 107 (93.1%) patients showed improvement at the end of the first year. Preprocedural absolute QRS-T angle, postprocedural PR interval, and right posteroseptal pathway location were identified as predictors of TWM. CONCLUSION: The development of TWM is particularly associated with the right-sided pathway location, especially the right posteroseptal pathway location. The predictors of TWM are the preprocedural QRS-T angle, the postprocedural PR interval, and the presence of the right posteroseptal AP.

Successful ductal stent implantation for initial palliation in two patients with aortic atresia and interrupted aortic arch.

The coexistence of aortic valve atresia and interrupted aortic arch are an extremely rare condition. In this pathology, blood flow to the ascending aorta and coronary arteries should be provided through the ductus arteriosus or collaterals originating from the descending aorta. In rare cases where bilateral ductus arteriosus is present, they can provide circulation. Here, we report two cases in which coronary arteries and ascending aorta were supplied by one ductus arteriosus and distal systemic circulation is supplied by a second ductus arteriosus in one patient and a collateral artery in the other. Initial palliation was successfully performed by bilateral pulmonary artery banding and transcatheter ductal stent implantation in both cases.

Biventricular repair in an infant with transposition of the great arteries and straddling of the tricuspid valve.

Biventricular repair is challenging in patients with transposition of the great arteries and straddling of the atrioventricular valves. Biventricular repair is the preferred option because of its anatomical and physiological advantages. However, in cases where biventricular repair carries operative risks that are too high or cases with unsuitable intracardiac anatomy, univentricular heart repair may have to be chosen. We report a five-month-old male patient with transposition of the great arteries, an inlet ventricular septal defect and anomalous coronary anatomy who had previously undergone a pulmonary banding operation and balloon atrial septostomy. Successful biventricular repair was performed while the patient had straddling of the tricuspid valve.

A Rare Cause of Left Ventricular Dysfunction and Familial Dilated Cardiomyopathy in Children; Emery-Dreifuss Type 2: A Case Report.

Emery-Dreifuss muscular dystrophy is one of a group of muscular dystrophies caused by a deficiency in genes encoding nuclear proteins (emerin, lamin A/C, nesprin). It progresses with joint contractures, muscular dystrophy, and cardiac involvement. Cardiac findings include dilated cardiomyopathy, conduction defects, and an associated increased risk of sudden cardiac death. We report the case of a young boy, aged 16, with lamin A/C gene mutation and dilated cardiomyopathy. From the patient's history, it was learned that his father and sister also had dilated cardiomyopathy and both died of heart failure. Cardiac resynchronization therapy implantation was planned in the follow-up of the patient due to progressive left ventricular dysfunction and left ventricular dyssynchrony. But the family did not accept this treatment option. The patient was placed on the heart transplant list. While waiting for a suitable donor, he died as a result of sudden cardiac arrest while he was being treated in the intensive care unit.

Role of RDW in Prediction of Burn after Caustic Substance Ingestion.

A quantifiable, quick, inexpensive and reproducible predictor is needed to decide if caustic substance ingestion results in burn regardless of the symptoms. A multicenter cohort study was conducted to investigate the predictive value of red cell distribution width (RDW) in detecting the esophageal burns. The data of 174 patients were retrospectively analyzed. Eleven patients were excluded due to inability to define the substance ingested. Complete blood count (CBC) was taken at admission, and an esophagogastroduodenoscopy was performed within the first 12-24 h in all patients, regardless of their symptoms. The age and gender of the patients, the types of substances ingested, the parameters in the CBC and the severity of the esophageal injury were correlated. Esophageal burns were diagnosed in 38 of 163 patients (23.3%). The risk of esophageal burn with RDW values below 12.20 was significantly lower. Multivariate analysis showed that RDW was the most significant predictor of esophageal burn (p = 0.000, odds ratio (OR) 7.74 (95% confidence interval (CI), 3.02-19.9)). Receiver operating characteristic (ROC) curve analysis demonstrated 84.2% sensitivity at a cut-off value of 12.20 for RDW. The results showed that CBC parameters could avoid unnecessary esophagogastroduodenoscopy. The RDW values regardless of the symptomatology is a good predictor of esophageal burns, and an RDW value over 12.20 shows the increased risk of esophageal burn.

Is There a Causal Relationship between Intussusception and Food Allergy?

Although intussusception and food allergy are common health problems in childhood, the relation between these two diseases remain obscure. The aim of this study is to investigate the relationship between food allergy and intussusception, and the factors associated with both. Patients diagnosed with intussusception by the Brighton Collaboration Intussusception Working Group criteria were prospectively investigated for food allergy per the European Society for Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) Guideline. They were analyzed per demographic features, clinical, physical and laboratory findings. There were eight (38.1%) patients diagnosed with food allergy, while 13 (61.9%) patients were non-allergic. The mean number of days of presenting symptoms was 1.13 days in the allergy group and 7.85 days in the non-allergy group. The mean number of intussusception attacks was 1.63 in the allergy group while 1 in the non-allergy group (p < 0.05, relative risk (RR) = 2.6). In the allergy group, one (13%) patient was followed up, six (75%) patients were reduced with pneumatic and one (13%) patient reduced manually. In the non-allergy group, four (31%) patients were followed up, six (46%) patients were reduced with pneumotic, one (7%) patient was reduced manually, and resection anastomosis was performed in two (15%) patients. Food allergy is an unrecognized associated factor for intussusception patients, which increases the risk for recurrence. Due to the small patient population, these results should be interpreted with caution.

Long QT syndrome diagnosed in two sisters with propionic acidemia: a case report.

Propionic acidemia (PA) is a rare autosomal recessive metabolic disorder caused by deficiency of the mitochondrial enzyme propionyl-CoA carboxylase (PCC). This disorder mostly progresses with episodes of metabolic acidosis. Cardiomyopathy is among the cardiac complications known to occur during metabolic decompensation episodes. However, several recent papers emphasized the association of PA and long QT syndrome (LQTS) which may lead to extremely serious and fatal consequences. In this report, we describe two sisters with PA who have prolonged QT duration that were incidentally detected in an outpatient setting. LQTS was verified by electrocardiogram, stress test and 24 h rhythm holter monitoring. By this report, we want to emphasize the importance of early diagnosis of LQTS in asymptomatic patients with PA to prevent fatal complications.