Mohammad Shihata

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.

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.

Cardiac catheterization can affect clinical outcomes in patients on extracorporeal membrane oxygenation (ECMO) after congenital heart surgery; however, its effect in this group of patients remains unclear. This study aimed to evaluate the safety and outcome of cardiac catheterization in patients undergoing ECMO after congenital cardiac surgery and determine predictors that influence successful weaning. This retrospective cohort study included pediatric patients who underwent cardiac catheterization while on ECMO after congenital heart surgery in two cardiac centers between November 2012 and February 2020. Predictors of successful weaning from ECMO were studied using univariate and multivariate logistic regression analyses. Of 123 patients on ECMO support after congenital cardiac surgery, 60 patients underwent 60 cardiac catheterizations (31 diagnostic and 29 interventional). Thirty-four (56.7%) and 22 patients (36.7%) underwent successful decannulation from ECMO support and survived after hospital discharge, respectively. Patients who underwent earlier catheterization (within 24 h of ECMO initiation) had more successful weaning from ECMO and survival compared to others. Patients who underwent an interventional procedure (interventional catheterization or redo cardiac surgery after cardiac catheterization) had better survival than those who underwent only diagnostic catheterization (P = 0.038). Shorter durations of ECMO was the most important predictor of successful weaning from ECMO. Early cardiac catheterization greatly impacts successful weaning from ECMO and survival. Patients with correctable lesions amenable either by catheterization or redo surgery are more likely to survive. Shorter durations of ECMO could have a significant influence on successful weaning from ECMO and survival.