Background: Duodenal obstruction (DO) is a congenital anomaly that is highly associated with other anomalies, such as cardiac anomalies and trisomy 21. However, an overview of additional anomalies and patient-specific risk factors for cardiac anomalies is lacking. Potential association with the vertebral, anorectal, cardiac, trachea-esophageal, renal and limb anomalies (VACTERL) spectrum remains unknown. Therefore, we aim to examine the incidence of associated anomalies, a VACTERL-spectrum association and patient-specific risk factors for cardiac anomalies in patients with DO. Methods: A retrospective cohort study was performed between 1996 and 2021. Outcomes were the presence of any additional anomalies. Risk factors for cardiac anomalies were analyzed using multivariate logistic regression. Results: Of 112 neonates with DO, 47% (N = 53/112) had one associated anomaly and 38% (N = 20/53) had multiple anomalies. Cardiac anomalies (N = 35/112) and trisomy 21 (N = 35/112) were present in 31%. In four patients, VACTERL-spectrum was discovered, all with cardiac anomalies. Trisomy 21 was found to be a risk factor for cardiac anomalies (OR:6.5; CI-95%2.6–16.1). Conclusion: Associated anomalies were present in half of patients with DO, of which cardiac anomalies and trisomy 21 occurred most often, and the VACTERL-spectrum was present in four patients. Trisomy 21 was a significant risk factor for cardiac anomalies. Therefore, we recommend a preoperative echocardiogram in patients with DO. In case a cardiac anomaly is found without trisomy 21, VACTERL-screening should be performed.

Background: Congenital abdominal wall defects might be associated with other anomalies, such as atresia in gastroschisis and cardiac anomalies in omphalocele patients. However, in the current literature, an overview of these additional anomalies and potential patient-specific risk factors is missing. Therefore, we aimed to assess the prevalence of associated anomalies and their patient-specific risk factors in patients with gastroschisis and omphalocele. Methods: A mono-center retrospective cohort study between 1997 and 2023 was performed. Outcomes were the presence of any additional anomalies. Risk factors were analyzed via logistic regression analysis. Results: In total, 122 patients were included, of whom 82 (67.2%) had gastroschisis, and 40 (32.8%) had omphalocele. Additional anomalies were identified in 26 gastroschisis patients (31.7%) and in 27 omphalocele patients (67.5%). In patients with gastroschisis, intestinal anomalies were most identified (n = 13, 15.9%), whereas, in patients with omphalocele, cardiac anomalies were most identified (n = 15, 37.5%). Logistic regression showed that cardiac anomalies were associated with complex gastroschisis (OR: 8.5; CI-95%: 1.4–49.5). Conclusions: In patients with gastroschisis and omphalocele, intestinal and cardiac anomalies were most identified, respectively. Cardiac anomalies were found to be a risk factor for patients with complex gastroschisis. Therefore, regardless of the type of gastroschisis and/or omphalocele, postnatal cardiac screening remains important.

Introduction: Increasing survival of adult congenital heart disease (ACHD) patients comes at the price of a range of late complications—arrhythmias, heart failure, and valvular dysfunction. Transcatheter valve interventions have become a legitimate alternative to conventional surgical treatment in selected acquired heart disease patients. However, literature on technical aspects, hemodynamic effects, and clinical outcomes of percutaneous atrioventricular (AV) valve interventions in ACHD patients is scarce. Method: This is a descriptive cohort from CAHAL (Center of Congenital Heart Disease Amsterdam-Leiden). ACHD patients with severe AV valve regurgitation who underwent a transcatheter intervention in the period 2020–2022 were included. Demographic, clinical, procedural, and follow-up data were collected from patient records. Results: Five ACHD patients with severe or torrential AV valve regurgitation are described. Two patients underwent a transcatheter edge-to-edge repair (TEER), one patient underwent a valve-in-valve procedure, one patient received a Cardioband system, and one patient received both a Cardioband system and TEER. No periprocedural complications occurred. Post-procedural AV valve regurgitation as well as NYHA functional class improved in all patients. The median post-procedural NYHA functional class improved from 3.0 (IQR [2.5–4.0]) to 2.0 (IQR [1.5–2.5]). One patient died 9 months after the procedure due to advanced heart failure with multiorgan dysfunction. Conclusion: Transcatheter valve repair is feasible and safe in selected complex ACHD patients. A dedicated heart team is essential for determining an individualized treatment strategy as well as pre- and periprocedural imaging to address the underlying mechanism(s) of AV regurgitation and guide the transcatheter intervention. Long-term follow-up is essential to evaluate the clinical outcomes of transcatheter AV valve repair in ACHD patients.

(1) Background: Prevalence of patent foramen ovale (PFO) in the general population is estimated at around 24%. We hypothesized that right-to-left shunting (RLS) resulting from PFO might contribute to angina symptoms in patients with coronary artery spasm (CAS), potentially triggered by vasoactive metabolites. Therefore, the aim of this study was to investigate the prevalence of PFO-related RLS in patients with documented CAS. (2) Methods: This single-center prospective cohort study included patients with documented CAS undergoing transthoracic echocardiography (TTE), including a contrast bubble study between 2021 and 2023. The Seattle Angina Questionnaire (SAQ) and Migraine Disability Assessment (MIDAS) were used to survey patients. (3) Results: RLS (PFO group) was observed in 11 of the 48 patients included (23%). In the PFO group, 64% had epicardial spasm and 36% microvascular spasm. Furthermore, RLS was more prevalent in patients with CAS and concomitant migraine (29%). Remarkably, the density plot of the SAQ summary score showed a worse score for patients with RLS (median of 38 [Q1–Q3: 31–49]) than patients without RLS (median of 49 [Q1–Q3: 41–55]) (p = 0.0282). (4) Conclusions: The prevalence of RLS due to PFO in patients with CAS was in line with the PFO prevalence in the general population, and patients with RLS are more symptomatic according to the SAQ summary score. Whether PFO closure could be beneficial to patients with CAS and concomitant migraine requires further investigation.