Annals of Pediatric Cardiology最新文献

A 10-year-old girl underwent successful device closure of a 15-mm atrial septal defect with a short and thick retroaortic rim using a 16-mm atrial septal occluder from the conventional approach under transesophageal echocardiographic guidance. There were no periprocedural complications. The aortic valve was noted to be trileaflet and competent. Transthoracic echocardiography at a routine 2-year follow-up visit demonstrated new-onset moderate aortic regurgitation. There was no pericardial effusion.

Cardiac strangulation is a rare phenomenon in children following epicardial pacemaker implantation, caused by compression of the heart and great vessels by the epicardial pacemaker wires. We report a rare case of cardiac strangulation presenting after 8 years of epicardial pacemaker implantation. On routine follow-up, computed tomography angiography showed significant extrinsic compression of the mid-left anterior descending (LAD) artery by the epicardial pacing wire. She was referred to our department for myocardial perfusion imaging (MPI), which showed significant inducible ischemia in the LAD territory. Following this, she underwent a successful pacemaker reimplantation. MPI can, thus, act as a good tool to assess the functional significance of the compression caused by strangulation of the heart by pacemaker leads in asymptomatic patients.

Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart disease, presenting either in isolation or with other cardiac anomalies. The most common presentation is congestive heart failure in infancy, though some may remain asymptomatic till adulthood. A 5-month-old infant with congestive heart failure was diagnosed to have ALCAPA with severe Left ventricular (LV) dysfunction. Unlike the more common variants where the left coronary artery (LCA) arose from the facing sinuses, the LCA arose from the left anterior nonfacing sinus of the pulmonary artery at a considerable distance from the left aortic sinus. This made direct reimplantation of LCA to the aorta challenging. LCA was reimplanted to the aorta using an extrapulmonary baffle made of an autologous pulmonary flap. The postoperative period was uneventful. The LV contractility improved during the follow-up. Early diagnosis and intervention with a novel surgical technique prevented irreversible cardiac complications and mortality.

Association of Ebstein's anomaly of tricuspid valve (TV) with tetralogy of Fallot (TOF) is extremely rare. Their coexistence is unique as they modify each other's physiology. We report a case of a girl child having this rare combination along with Diamond-Blackfan syndrome awaiting a bone marrow transplant. She underwent intracardiac repair for TOF along with cone reconstruction of the TV, as tricuspid regurgitation of Ebstein's anomaly would worsen with TOF correction alone. Through this surgery, we aimed to achieve a competent pulmonary valve using a transannular patch with a monocusp and adequate relief of the right ventricular tract obstruction, thus preserving the right ventricular function, as both can affect the repaired TV function in the long term. The child had an uneventful postoperative course and is asymptomatic on follow-up.

We hypothesized that a double-balloon technique would be associated with a lower likelihood of aortic regurgitation (AR) following balloon aortic valvotomy (BAV). We present the short- and mid-term outcomes of the double-balloon technique for BAV. Fifty consecutive patients (median age: 6.5 years; interquartile range (IQR): 3.8-13.2) underwent BAV using double balloons. Prior AR was present in 17 (34%). Following BAV, the peak-to-peak gradient reduced from 80 (66.5-100) mm Hg to 24 (16-35) mm Hg (P < 0.0001); 10 (20%) had trivial AR, 18 (36%) had mild AR, 10 (20%) had moderate AR and none had severe AR. Four (8%) had transient arterial occlusion requiring anticoagulation. There was no procedure-related mortality or requirement for emergency open-heart surgery. The double-balloon technique offers a safe and effective alternative option for BAV in patients beyond infancy with relatively large annulus sizes. There is a low likelihood of postprocedural severe AR and the need for repeat procedures. Further long-term follow-up of this cohort is needed to ascertain long-term outcomes.

Background: The management of ventricular septal defect (VSD) alongside mitral regurgitation (MR) in pediatric patients remains a contentious issue due to the intricacies of cardiac surgery and the need to minimize ischemic time. Despite observations of MR regression following VSD closure, definitive guidelines for this patient subset are lacking, particularly concerning the management of the subgroup of patients with moderate MR. The objective of the study is to explore the factors influencing the choice between surgical intervention and conservative management for the mitral valve (MV) in VSD patients with moderate MR.

Materials and methods: A retrospective cohort study from January 2000 to December 2015, we analyzed management trends and focused on 53 patients with both VSD and moderate MR. This cohort was subdivided into four groups: first by their MV pathology, and then stratified by the receipt of intervention towards the diseased MV. Our primary goal was to identify correlations, especially concerning surgical outcomes such as mortality and need for re-operation. Statistical significance is determined when the P value is lower than 0.05.

Results: There were no notable differences in preoperative variables across four cohort groups, apart from the cross-clamp duration which was longest in Group B intervention. Outcome analysis showed survival rates that were higher in cohort groups that underwent intervention toward the disease MV regardless of existing MV morphology, although results were not statistically significant. Cox model analysis found no correlation between the cohort groups and postoperative outcomes, but cross-clamp duration significantly correlated with mortality.

Conclusions: In cases of VSD with moderate MR with associated pathologies of the valvular apparatus, opting for MV repair (MVr) appears to improve survival outcomes without significantly increasing postoperative morbidity. Similarly, for patients with moderate MR and isolated annular dilatation, surgical MVr is observed to have better survival trend compared to the control group.

Uhl's disease is a rare disorder secondary to the uncontrolled destruction of right ventricular myocytes during the perinatal period. We present here the case of a 1-month-old child who died suddenly of Uhl's disease, which was only diagnosed at autopsy and histological examination. From an anamnestic point of view, the child's sister had also died at about 1 month of age from the same pathology. In both cases, genetic tests showed a heterozygous mutation in the KCNH2 gene. The case that we presented is particularly significant as very few familial cases of Uhl disease have been described in the literature, and genetic analyses have been conducted in very few cases. It is possible that the observed mutation played a role in the onset of the disease process. However, further scientific studies with larger case series are needed to confirm our results.

Background: Surgical intervention is the definitive treatment for Ebstein anomaly, offering both biventricular and nonbiventricular repair options. The objective of this study is to identify a specific cutoff value for tricuspid septal leaflet displacement, which will be a crucial factor in determining the selection of a surgical approach with lower mortality risk in biventricular repair.

Methods and results: This is a retrospective cohort study of consecutive patients with Ebstein anomaly undergoing surgical intervention at the National Cardiovascular Center Harapan Kita from January 2010 to December 2023. A total of 83 patients with Ebstein anomaly were treated surgically; 43 of those underwent biventricular repair, whereas the remaining underwent nonbiventricular repair. Echocardiography was performed, and the Great Ormond Street Echocardiography score was calculated. Several risk factors were identified and stratified for patients with biventricular repair (n = 43). Tricuspid septal leaflet displacement was measured for each patient, and there was a statistically significant higher mortality risk directly proportional to higher displacement in patients with biventricular repair (P < 0.05). A cutoff value of 43.5 mm/m² for the tricuspid septal leaflet displacement is the best predictor of mortality risk in biventricular repair with 83.3% sensitivity and 93.3% specificity.

Conclusions: In patients with Ebstein anomaly undergoing a biventricular repair, mortality rates are significantly elevated in patients with a higher tricuspid septal leaflet displacement distance. The results of the study indicated that the mortality risk in biventricular repair can be predicted based on the tricuspid septal leaflet displacement distance using a cutoff value of 43.5 mm/m².