The paramount of three-dimensional echocardiography in percutaneous closure of large oval perimembranous ventricular septal defect: a case report.

Background: Ventricular septal defect (VSD) is the most common type of congenital heart abnormality with perimembranous VSD (pmVSD) accounting for ∼70% of all VSD. Nowadays, transcatheter closure is the first choice for suitable pmVSD. However, there was no report about closing the large oval-shaped VSD percutaneously. Case summary: A 34-year-old male with known VSD was referred for transcatheter closure after failed attempts in other hospital. Patient had been diagnosed with VSD at a young age, but he was lost to follow-up. He presented with shortness of breath due to heart failure and pulmonary hypertension. The initial measurement of the defect was 6-7 mm by transthoracic echocardiography (TTE), transoesophageal echocardiography (TEE), and LV angiography. However, re-measurement using TEE and 3D echocardiography revealed that the VSD is oval with diameters of 18 mm × 6 mm. Initially, device No. 12/14 was used, but it was dislodged on two attempts. The operator then decided to upsize the device size to No. 16/18 that was successful. The patient's condition was good, and 6 months follow-up after the procedure showed good outcomes without any residual defect or arrhythmia. Discussion: In this study, we would like to highlight the rarity of large oval pmVSD that almost failed to be closed with the conventional measurement with echocardiography and fluoroscopy. Transoesophageal echocardiography especially 3D can be the new modality of choice that might be superior to fluoroscopy to decide the right device size in some cases such as oval-shaped pmVSD.

Right ventricular outflow tract stenting for late presenter unrepaired Fallot physiology: a single-center experience.

Objectives: The purpose of this study was to assess the clinical outcome after right ventricular outflow tract (RVOT) stenting in late presenter patient with unrepaired Fallot physiology. Background: In younger patients, RVOT stenting is an alternative to mBTT shunt; however, there have been few reports of this palliative technique in late presenter population, including adults. Methods: This was a single-center, retrospective study of nonrandomized, palliated Fallot patients. Clinical outcomes such as left ventricular ejection fraction and saturation were measured in 32 individuals following RVOT stenting in adults (n = 10) and children (n = 22). The Statistical Package for Social Science (SPSS) 26.0 software was used to analyze the statistical data. Results: During the procedure, the average stent diameter and length were 8.84 ± 1.64 mm and 35.46 ± 11.23 mm, respectively. Adult patients received slightly longer stents than pediatric patients (43.60 ± 11.64 mm vs. 31.77 ± 9.07 mm). Overall, patients' saturation increased from 58.56 ± 19.03% to 91.03 ± 8.98% (p < 0.001), as did their left ventricular ejection fraction (LVEF) from 64.00 ± 18.21% to 75.09 ± 12.98% (p = 0.001). Three patients improved their LVEF from 31 to 55%, 31 to 67%, and 26 to 50%. The median length of stay was 8 (2-35) days, with an ICU stay of 2 (0-30) days. The median time from RVOT stent palliation to total repair was 3 months (range: 1 month-12 months). Conclusions: RVOT stenting is a safe and effective method for increasing saturation and ejection fraction not only in newborn infants but also in late presenters, including adults with unrepaired Fallot physiology.

Ductal stenting vs. surgical shunting in late presenting duct-dependent pulmonary circulation: a single-center experience.

Introduction: PDA stenting is an option to mBTT shunt for younger patients; nevertheless, few reports of this palliative approach have been made for the late presenter population, especially for patients who are older than 30 days but under 5 years. This study aimed to evaluate the clinical result and intra-hospital costs of ductal stenting in late-presenting patients in comparison to surgical shunting. Methods: A single-center, retrospective cohort study was conducted from August 2016 to August 2022. This study included patients with pulmonary duct dependent CHD who were hospitalized for palliative therapy. The extracted data were baseline characteristics, clinical findings, supportive examination findings, complications, outcomes, and length of stay of the patients. Monitoring was carried out during treatment up to 30 days after the procedure. Results: A total of 143 patients were included in the analysis; 43 patients underwent PDA stent and 100 patients underwent mBTT shunt with median age of PDA stent group 110 (31-1,498) days and mBTT shunt group 174.5 (30-1,651) days. Primary outcome composite was not significant in both groups including 30 days mortality [6 (14%) vs. 14 (14%), p = 1.000], reintervention [1 (2.3%) vs. 7 (7%), p = 0.436], and 30 days rehospitalization [0 (0%) vs. 2 (2%), p = 0.319]. Secondary outcome analysis showed shorter ICU length of stay in the PDA stent group [2 (0-16) days vs. 4 (1-63) days, p = 0.002]. Conclusions: PDA stent has an outcome that is non inferior from the mBTT shunt procedure in the composite outcome including 30 days mortality, reintervention, and 30 days rehospitalization but significantly lower in ICU length of stay.

Posterior Annulus Elevation Technique in Reducing Residual Regurgitation During Mitral Valve Repair in Children: A Randomized Controlled Trial.

Background: The current technique used in severe mitral regurgitation in children can occasionally lead to residual regurgitation. To address this issue, the posterior annulus elevation technique was developed to enhance coaptation and reduce residual lesions. This study aims to evaluate the effectiveness of this technique in reducing residual regurgitation during mitral valve repair in children. Methods: A total of 64 patients aged <18 years old undergoing mitral valve repair were randomized into two groups: the intervention (with posterior annulus elevation) group and the control group, which underwent conventional repair techniques. Various parameters, including coaptation area, residual mitral regurgitation, clinical outcomes, metabolic, and hemolytic markers, were measured on days 0, 5, 2 weeks, and 3 months after surgery. Results: The intervention group (n = 32) showed a significant reduction in residual mitral regurgitation compared with the control group (n = 32) on each evaluation. At three months after surgery, we found that the posterior annulus elevation technique could be a protective factor that reduces the chance of residual regurgitation compared with the control group (RR = 0.31; confidence interval: 0.18-0.54; P ≤ .001). Coaptation length and index were also found to be significantly higher in the intervention group (P ≤ .001). Clinical outcomes, metabolic markers, and hemolysis marker did not show any significant differences between the two groups. Conclusions: The posterior annulus elevation technique demonstrated effectiveness in reducing residual mitral regurgitation and improving coaptation area in pediatric mitral valve repair. This technique showed better short-term surgical outcomes in children with mitral regurgitation compared with the conventional technique.

Case Series of Berry syndrome: A rare constellation of fatal cardiac anomalies.

Berry syndrome is an extremely rare constellation of several congenital cardiac anomalies consisting of aortopulmonary window, aortic origin of the right pulmonary artery (AORPA), interrupted aortic arch or hypoplastic aortic arch or coarctation of the aorta, and an intact ventricular septum with high neonatal mortality rates. The disease is fatal with high mortality (90%) in the neonatal period with surviving patients mostly developing pulmonary hypertension. We describe the clinical presentation and diagnostic clues in two patients with Berry syndrome.