Pediatric Cardiology最新文献

Pulmonary atresia/critical stenosis with intact ventricular septum is a heterogeneous disease in which perinatal management is not exempt from controversies. We aim to understand which areas are the ones with the highest and least consensus. An online questionnaire including 30 questions was administered through a REDCap link. Invitations were sent out through the Fetal Heart Society and personal emails to the heads of fetal cardiology programs. Results were analyzed in a descriptive manner and compared among the regions with most responses (North America and Europe). A total of 72 specialists in fetal and pediatric cardiology responded, most from North-America (n = 31) and Europe (n = 29). Antenatal counselling was mainly based on the tricuspid valve z-score (43.7%), although among European responders, multivariable models were preferred (44.8%). There was a wide regional variation in the nomograms used for prenatal assessment of the tricuspid valve. More than half did not consider prenatal valvuloplasty (54.3%), and those who did diverged in selection criteria. Postnatally, in mild-moderately hypoplastic right ventricle, most opted to first assess the tricuspid valve z-score (60%) whereas others would always attempt a biventricular pathway (30.8%). The majority opted to perform cardiac catheterization in all cases to assess ventriculo-coronary connections (60.6%). If right ventricle-dependent coronary circulation was confirmed, 66.7% would opt for single ventricle palliation, whereas 15.9% would directly list for transplant. There is great heterogeneity in the management of pulmonary atresia-critical stenosis with intact ventricular septum, particularly in prenatal assessment and consideration for prenatal intervention.

Thrombotic complications post-Fontan operation remain a significant clinical concern and preventing these events is critical to improve patient outcomes. While the optimal strategy for thromboprophylaxis post-Fontan is unclear, recent randomized studies have shown comparable outcomes in patients started on Direct Oral Anticoagulants (DOACs) as compared to either antiplatelet medications (e.g. aspirin) or anticoagulants (e.g. warfarin). Based on this increasing data, the Stollery Children's Hospital (primary pediatric cardiac surgical site for Western Canada) changed the standard thromboprophylaxis strategy in May 2024 from anticoagulation with warfarin (target international normalized ratio (INR) 2-3) to treatment dose rivaroxaban (adult equivalent dose 20 mg daily). The goal of this pilot study was to compare outcomes of the first 20 patients during the rivaroxaban period to the most recent 20 patients prior to the practice change. All patients who underwent Fontan procedure March/2023-April/2024 (warfarin cohort) and May/2024-September/2025 (rivaroxaban cohort) were identified and data on bleeding/thrombotic outcomes, length of hospital stay, and laboratory monitoring was collected through retrospective review of the electronic medical record including from surgical admission until 30 days post-discharge. All patients started on rivaroxaban had peak-rivaroxaban-calibrated-anti-Xa used to monitor dosing. During the study period, no significant bleeding or thrombotic events occurred post initiation of rivaroxaban, in the warfarin cohort there was one episode of clinically relevant non-major bleeding. Results suggest that thromboprophylaxis post-Fontan procedure with treatment dose rivaroxaban had similar early outcomes to warfarin. Future work is needed to validate these results in a larger cohort and include longer-term patient outcomes.

Pediatric intraluminal pulmonary vein stenosis (PVS) is a challenging condition where the diagnosis is confirmed via cardiac catheterization. The patient population is complex with inherent hemodynamic vulnerabilities, and the high rate of restenosis necessitates recurrent, technically demanding transcatheter interventions. Furthermore, decisions made during each procedure can have a lasting impact on future care. To address these challenges, we present a guideline for transcatheter interventions for PVS, informed by both published literature and the Authors' clinical experience.

Rare malformations of the aortic arch system may be of minor relevance for general medical practice. Their abnormal arterial patterns, however, sometimes can represent a challenge not only for experts in cardiovascular medicine but also for the validity of well-established concepts traditionally used to explain the embryology of normal and abnormal patterning of the aortic arch system. The present article provides a review of (1) the pathomorphological features, (2) associated cardiac and extracardiac malformations, and (3) embryology of a very rare malformation of the aortic arch system, called isolation of a common carotid artery. It is shown that this diagnosis does not characterize a single pathomorphological entity. Among 28 previously documented cases we can distinguish two different subsets. One of these subsets is of general interest since it challenges the traditional concept of the embryology of congenital malformations of the aortic arch system. It is shown that malseptation of the embryonic aortic sac and cardiac outflow tract may play a fundamental role in the pathogenesis of this malformation.

Cardiopulmonary exercise testing (CPET) is a cornerstone of functional assessment in congenital heart disease (CHD), yet there are additional important contributors to exercise limitation that are not directly measured by CPET. This narrative review highlights the complementary utility of spirometry, body composition analysis, and evaluation of handgrip strength in augmenting our understanding of functional capacity in CHD. Spirometry provides insights into restrictive lung disease patterns prevalent in post-surgical CHD populations; body composition analysis, especially lean mass assessment, can reveal sarcopenia not apparent using BMI alone; and handgrip strength serves as a validated surrogate for overall muscular function and frailty. We propose routine integration of all three testing techniques as complements to CPET protocols to enhance clinical assessment, guide rehabilitation, and support prognostication in pediatric CHD patients. This review advocates for broader adoption and standardization of these measures in clinical settings to improve risk stratification and long-term outcomes.