Junctional ectopic tachycardia is a serious arrhythmia that frequently occurs after tetralogy of Fallot repair. Arrhythmia prophylaxis is not feasible for all pediatric cardiac surgery patients and identification of high risk patients is required. The objectives of this study were to characterize patients with JET, identify its predictors and subsequent complications and the effect of various treatment strategies on the outcomes in selected TOF patients undergoing total repair before 2 years of age. From 2003 to 2017, 609 patients had Tetralogy of Fallot repair, 322 were included in our study. We excluded patients above 2 years and patients with preoperative arrhythmia. 29.8% of the patients (n = 96) had postoperative JET. JET patients were younger and had higher preoperative heart rate. Independent predictors of JET were younger age, higher preoperative heart rate, cyanotic spells, non-use of B-blockers and low Mg and Ca (p = 0.011, 0.018, 0.024, 0.001, 0.004 and 0.001; respectively). JET didn’t affect the duration of mechanical ventilation nor hospital stay (p = 0.12 and 0.2 respectively) but prolonged the ICU stay (p = 0.011). JET resolved in 39.5% (n = 38) of patients responding to conventional measures. Amiodarone was used in 31.25% (n = 30) of patients and its use was associated with longer ICU stay (p = 0.017). Ventricular pacing was required in 4 patients (5.2%). Median duration of JET was 30.5 h and 5 patients had recurrent JET episode. Timing of JET onset didn’t affect ICU (p = 0.43) or hospital stay (p = 0.14) however, long duration of JET increased ICU and hospital stay (p = 0.02 and 0.009; respectively). JET increases ICU stay after TOF repair. Preoperative B-blockers significantly reduced JET. Patients with preoperative risk factors could benefit from preoperative arrhythmia prophylaxis and aggressive management of postoperative electrolyte disturbance is essential.

Cutis laxa syndrome is an uncommon connective tissue disorder affecting the major ultrastructure of the skin by progressive loss of elasticity. The results of this syndrome lead to the appearance of premature aging, which might also affect the internal organs. The disorder can be either congenital or acquired. The congenital form consists of autosomal dominant, autosomal recessive, and X-linked recessive patterns. The autosomal recessive pattern is the most common and severe one. Different systemic complications have been described in congenital cutis laxa syndrome, but the most serious and lethal one is cardiopulmonary abnormalities. In this report, we discuss the presentation of congenital cutis laxa syndrome with successful cardiovascular surgical management of multiple valvular heart diseases.

Background: Right ventricular outflow tract (RVOT) reconstruction is frequently performed for pediatric patients with pulmonary valve anomalies, yet optimal techniques remain debated. The equine pericardium offers a promising substrate for pulmonary valve reconstruction but has been understudied in pulmonary valve reconstruction. This study evaluated a novel technique using the equine pericardium to create a cylinder bicuspid pulmonary valve for RVOT reconstruction. Methods: In this retrospective cohort study, 17 pediatric patients (median age: 10 months; 53% male) underwent RVOT reconstruction with equine pericardium between 2023 and 2024. The valve was fashioned from a patch of equine pericardium into a cylinder to create a functionally bicuspid valve. The height of the cylinder ranged from 1.5–2 cm. The diameter was measured around a Hegar dilator corresponding to a valve size z-score of +3. The outcomes included the degree of postoperative pulmonary regurgitation, RVOT pressure gradients, postoperative complications, and reinterventions. Results: Postoperatively, the median peak RVOT pressure gradient decreased significantly from 70 mmHg (IQR: 65–90) to 25 mmHg (IQR: 20–40; p < 0.001). Mild pulmonary regurgitation persisted in one patient (5.9%). Five patients had mild right ventricular dysfunction (29.41%). At a median 3-month follow-up (IQR: 1–8), 17.7% (n = 3) underwent cardiac catheterization. There was no postoperative mortality. Conclusions: Cylinder bicuspid pulmonary valve reconstruction using the equine pericardium effectively reduces RVOT obstruction while maintaining pulmonary valve competence and demonstrates acceptable short-term safety. Having a competent pulmonary valve after repairing the hypoplastic pulmonary valve annulus is very promising; however, the small cohort and limited follow-up preclude definitive conclusions about long-term durability. Larger prospective studies with longer follow-up periods are needed to validate this technique for RVOT reconstruction.