Background:Development of fibrinous pericarditis after pericardiotomy is a well-recognized reaction. Within a few post-operative days, the inflammated surface of pericardium begins to fuse to the overlying sternum.Objectives:Our aim was to assess the prevalence, risk factors, time course and therapy response of pericardial effusion (PE) after cardiac surgeries in children.Patients and Methods:PE occurrence was assessed prospectively in 486 children who underwent cardiac surgery for congenital heart diseases by serial echocardiography. Clinical manifestations were observed and response to different therapies was analyzed.Results:The prevalence of PE was about 10% for all cardiac surgeries. Symptoms were exclusively seen in patients who had moderate to large effusions. The mean onset of pericardial effusion was 11 (± 8) days after surgery procedure, with 87 % (42 of 48) of cases being diagnosed on or before day 13 after operation. The prevalence of effusion after Fontan-type procedures and AVSD repair (29 %, 5 of 17 for both) was significantly higher than other types of cardiac surgeries. Aspirin administration was effective in 77 % and prednisone in 90 % of the cases.Conclusions:PE may be developed as late as weeks after cardiac surgeries. PE after palliative cardiac surgeries is not uncommon. Low doses of aspirin and corticosteroids are usually effective for treating this complication.
This study was conducted to evaluate the accuracy of exercise testing for predicting accessory pathway characteristics in children with Wolff-Parkinson-White (WPW) syndrome. The study enrolled 37 children with WPW syndrome and candidates for invasive electrophysiologic study (EPS). Exercise testing was performed for all the study participants before the invasive study. Data from the invasive EPS were compared with findings from the exercise testing. The sudden disappearance of the delta (Δ) wave was seen in 10 cases (27 %). No significant correlation was found between the Δ wave disappearance and the antegrade effective refractory period of the accessory pathway (AERP-AP) or the shortest pre-excited RR interval (SPERRI). The sensitivity, specificity, and positive and negative predictive values of Δ wave disappearance, based on AERP-AP as gold standard, were respectively 29.4, 80, 71.4, and 40 %. The corresponding values with SPERRI as the gold standard were respectively 23.8, 71.4, 71.4 and 23.8 %. Exercise testing has a medium to low rate of accuracy in detecting low-risk WPW syndrome patients in the pediatric age group.
TRDN mutations cause catecholaminergic polymorphic ventricular tachycardia (CPVT) but may present with abnormal electrocardiogram (ECG) findings provoking a diagnosis of long QT syndrome (LQTS). We report two novel cases of sudden cardiac death in children due to mutations of TRDN, providing further insight into this rare and aggressive inherited arrhythmia syndrome. Whole exome sequencing (WES) was performed in two unrelated children who experienced cardiac arrest during exercise and were negative for targeted testing of LQTS. WES identified a novel homozygous splice‐site mutation in both patients, denoted c.22+1G>T, absent from gnomAD and suggesting a founder variant in the Iranian population. We now summarize the genetic architecture of all reported TRDN‐related patients, including 27 patients from 21 families. The average age‐onset was 30 months (range 1–10) for all cases. Adrenergic‐mediated cardiac events were common, occurring in 23 of 27 cases (85%). LQTS was diagnosed in 10 cases (37%), CPVT in 10 (37%) cases, and in 7 cases. No phenotypic diagnosis was provided. Five cases (15%) had evidence for associated skeletal myopathy. Four missense TRDN variants (24%) were observed in diseased cases, while the remaining variants reflect putative loss‐of‐function (LOF) mutations. No disease phenotype was reported in 26 heterozygous carriers. In conclusion, TRDN mutations cause a rare autosomal recessive arrhythmia syndrome presenting with adrenergic‐mediated arrhythmic events, but with ECG abnormalities leading to a diagnosis of LQTS in a proportion of cases. Heterozygous carriers are free of disease manifestations.
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