UNASSIGNED: Atrial septal defects (ASDs) lead to volume-loaded right ventricles (RVs). ASD closure does not always alleviate symptoms or improve exercise capacity, which is possibly explained by impaired left ventricular (LV) haemodynamics. This study evaluated the effect of ASD closure in children using non-invasive LV pressure-volume (PV) loops derived from cardiac magnetic resonance (CMR) imaging and brachial blood pressure, compared with controls.
UNASSIGNED: Twenty-three children with ASD underwent CMR, and 17 of them were re-examined 7 (6-9) months after ASD closure. Twelve controls were included. Haemodynamic variables were derived from PV loops by time-resolved LV volumes and brachial blood pressure. After ASD closure, LV volume increased [76 (70-86) vs. 63 (57-70) mL/m2, P = 0.0001]; however, it was still smaller than in controls [76 (70-86) vs. 82 (78-89) mL/m2, P = 0.048]. Compared with controls, children with ASD had higher contractility [2.6 (2.1-3.3) vs. 1.7 (1.5-2.2) mmHg/mL, P = 0.0076] and arterial elastance [2.1 (1.4-3.1) vs. 1.4 (1.2-2.0) mmHg/mL, P = 0.034]. After ASD closure, both contractility [2.0 (1.4-2.5) mmHg/mL, P = 0.0001] and arterial elastance [1.4 (1.3-2.0) mmHg/mL, P = 0.0002] decreased.
UNASSIGNED: Despite the left-to-right atrial shunt that leads to low LV filling and RV enlargement, the LV remains efficient and there is no evidence of impaired LV haemodynamics in children. Closure of ASD at young age while the ventricle is compliant is thus beneficial for LV function. LV volumes, however, remain small after ASD closure, which may impact long-term cardiovascular risk and exercise performance.

BACKGROUND: Percutaneous pulmonary valve implantation (PPVI) is a recognized alternative treatment to surgery for patients with dysfunctional right ventricular outflow tracts. Patient selection is essential to avoid serious complications from attempted treatment, such as rupture or dissection, especially of the calcified outflow tracts. We describe a case with an unexpected rupture of a calcified homograft valve and main pulmonary artery, which was treated successfully by emergency implantation of a self-expanding Venus P-Valve (Venus MedTech, Hangzhou, China) without the need for pre-stenting with a covered stent.
METHODS: A 13-year-old boy had two previous operations of tetralogy of Fallot, one a total repair and the other a homograft valved conduit for pulmonary regurgitation. He presented with dyspnea and severe right ventricular outflow tract obstruction (RVOTO) and had a calcified outflow tract and main pulmonary artery. In the catheter laboratory, a non-compliant balloon dilation resulted in a contained rupture of the conduit. The patient remained hemodynamically stable, and the rupture was treated with a self-expandable Venus P-Valve without the need for a covered stent combined with a balloon-expandable valve or a further surgical procedure.
CONCLUSIONS: Preprocedural evaluation with an inflating balloon is necessary to examine tissue compliance and determine suitability for PPVI. However, this condition is accompanied by a risk of conduit rupture. Risk factors of this complication are calcification and homograft use. These ruptures are mostly controlled with a prophylactic or therapeutic covered stent, with a low rate of requiring surgery. However, there are severe ruptures which lead to hemothorax and death. In the available literature, there was no similar reported case of conduit rupture, which a self-expandable Pulmonary valve stent has managed. It seems that fibrosis and collagen tissue around the heart, formed after open surgeries, can contribute to the control of bleeding in these cases.
UNASSIGNED: The suitability of patients for the PPVI procedure should be examined more carefully, specifically patients with homograft and calcification in their conduit. Furthermore, conduit rupture might be manageable with self-expandable artificial pulmonary valves, specifically in previously operated patients, and the applicability of this hypothesis is worth examining in future research.

OBJECTIVE: Advances in the management of congenital heart disease (CHD) have significantly decreased mortality rates, indicating a need for continuous care as a chronic condition throughout the child\'s lifespan. This study examined the association of nurse-mother partnerships with parenting stress and family resilience among South Korean mothers of children with CHD.
METHODS: This descriptive study involved 93 mothers of children aged six years or younger with CHD admitted to a hospital. Data were collected from September to November 2020 and analyzed using SPSS/WIN (version 29.0) for t-tests, analyses of variance, Pearson\'s correlation coefficient, and multiple regression analyses.
RESULTS: The means and standard deviations of the nurse-mother partnership, parenting stress, and family resilience were 4.13 ± 0.47, 76.98 ± 16.6, and 56.54 ± 7.86 points, respectively. Parenting stress increased as the number of hospitalizations and surgeries increased and with complex types of CHD. Nurse-mother partnerships were stronger with longer hospital stays. Family resilience was higher with younger children, fewer rehospitalizations, and shorter hospital stays. A positive correlation was found between nurse-mother partnerships and family resilience, and a negative correlation between parenting stress and family resilience. Factors influencing parenting stress included family resilience, rehospitalizations, and complex types of CHD, and those affecting family resilience were nurse-mother partnerships, parenting stress, and the child\'s age.
CONCLUSIONS: Nurse-mother partnerships significantly affect family resilience.
CONCLUSIONS: Enhancing nurse-mother partnerships can improve family resilience, which in turn can reduce parenting stress, thus offering guidance for future nursing interventions.

OBJECTIVE: A comprehensive survey of congenital heart disease (CHD) prevalence has not yet been conducted in South Korea. This study aimed to investigate the prevalence of CHDs in Korean children and lay the foundation for national CHD epidemiology.
METHODS: Target patients were infantile crucial CHDs, which include critical CHDs (requiring urgent procedures after birth with common hypoxemic defects) and diverse categorical defects excluding simple shunt defects. Data were obtained from the National Health Insurance Service over a 5-year period (2014-2018). Birth prevalence (new cases per 1,000 live births) of CHDs in Korea was analyzed and compared with that of other countries.
RESULTS: The birth prevalences of right heart obstructive defects (pulmonary valve stenosis and pulmonary atresia), conus anomalies (tetralogy of Fallot and double outlet right ventricle), and total anomalous pulmonary venous return showed significant increases in the East Asian group (P < 0.001), whereas those of left heart obstructive defects (coarctation of aorta, aortic stenosis, and hypoplastic left heart syndrome), truncus anomalies (D-transposition of great artery and persistent truncus arteriosus), atrioventricular septal defect, and hypoplastic right heart syndrome were significantly decreased in the East Asian group (P < 0.001).
CONCLUSIONS: The overall birth prevalence of crucial CHDs in Korea was similar to that of critical CHDs in previous studies from other countries. Some subtypes of right heart obstructive defects, left heart obstructive defects, and conotruncal anomalies showed significant differences between East Asian and Western populations. This study contributes to a foundation for national CHD epidemiology in Korean children.

All reported ABL1 gain of function and loss of function (LOF) variants, impact both isoforms 1a and 1b. Our findings suggest that LOF variants affecting solely ABL1 isoform 1b may lead to a distinct autosomal recessive new phenotype.

UNASSIGNED: Anomalous origin of right pulmonary artery from the ascending aorta is a rare congenital heart malformation that results in early infant mortality. These patients are at risk for the early development of significant pulmonary hypertension. The surgical management during the early period of life is imperative.
UNASSIGNED: Anomalous pulmonary artery originating from the ascending aorta (often called hemitruncus) is a rare congenital cardiac defect requiring immediate management in the neonatal period. We report a case of a rare variant of anomalous right pulmonary artery originating from the ascending aorta in combination with pulmonary atresia and tetralogy of Fallot. The above-mentioned combination makes the surgical management of such cardiac defect exceedingly demanding.

Coronary artery obstruction is an uncommon yet devastating complication of transcatheter aortic valve replacement (TAVR) and may necessitate leaflet modification. A 38-year-old man presented to our center with quadricuspid aortic valve with severe aortic regurgitation. Double leaflet modification was performed with the Bioprosthetic or native Aortic Scallop Intentional Laceration to prevent Iatrogenic Coronary Artery obstruction (BASILICA) technique prior to TAVR, creating 6 leaflets from 4. The patient tolerated the procedure well with symptomatic improvement. Follow-up transthoracic echocardiogram showed normal bioprosthetic aortic valve function. This case demonstrates feasibility of this procedure with comprehensive preprocedural analysis and intraprocedural imaging guidance.

暂无摘要。

BACKGROUND: Patients with hepatoblastoma (HB) have a higher risk of congenital heart defects (CHD). There is limited literature on the management and outcomes of these patients. The purpose of this study was to identify demographics and outcomes of these patients in a single tertiary referral center.
METHODS: An Institutional Review Board (IRB)-approved retrospective chart review of patients with newly diagnosed HB from October 2004 to January 2021 was performed. CHD was defined as the presence of a septal defect, patent ductus arteriosus, pulmonary atresia, or bicuspid aortic valve. Chi-square and t-test were utilized for statistical analyses.
RESULTS: Of the 151 patients diagnosed with HB during the study timeframe, 29 patients were found to have CHD. Five-year overall survival (OS) for non-CHD HB patients was 81.9% compared to 68.9% in the CHD cohort (p = .12). The 5-year OS for patients without surgically intervened CHD was 63.6% compared to 70.5% for those with surgically repaired CHD (p = .88). Pre-treatment extent of tumor IV was present more often in patients with HB and CHD who passed away (6/9, 66.7%) compared to those who survived (3/16,18.8%, p = .01).
CONCLUSIONS: Patients with HB and CHD have similar survival compared to those without CHD. Our data support that patients with HB and CHD should be treated with curative intent including cardiac surgical intervention, medical oncology therapy, and oncological surgery for their HB.

We report a case of an active-duty diver who developed severe decompression sickness with concomitant patent foramen ovale that was successfully closed contrary to standard guideline recommendations. This case should prompt evaluation of the role of cardiac screening in occupational divers, including tactical athletes, relative to recreational divers.