Khalifah A. Aldawsari

Restrictive right ventricular physiology (RRVP) occurs due to impaired right ventricular compliance and associated diastolic dysfunction. This results in reduced cardiac output and elevated central venous pressure with downstream consequences for the liver, kidneys, gut, and lymphatic system. RRVP is frequently seen after surgical repair of tetralogy of Fallot (TOF), though it occurs across a range of congenital heart defects with long‐standing RV pressure overload. This review provides a clinically oriented description of the pathophysiology, patients at risk, end‐organ consequences, and management of RRVP in the immediate post‐operative period after TOF repair.

Neonates and infants with FOXF1 mutation develop alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV), which is typically considered a uniformly fatal neonatal lung disorder due to marked hypoxemic respiratory failure with severe pulmonary hypertension (PH). Survival beyond infancy is exceedingly rare, with limited reports of FOXF1 genetic abnormalities in older children or adult cases of severe PH. Here, we report the case of a male patient with a pathogenic FOXF1 variant who presented with severe PH in early infancy whose disease stabilized and improved with early and aggressive PH therapy. The patient subsequently died at the age of 30 years, however, due to severe right ventricular failure due to the acute onset of pneumonia. This case highlights that survival into adulthood is feasible in patients with FOXF1‐related pulmonary vascular disease and supports the inclusion of FOXF1 in pulmonary hypertension genetic panels for subjects who develop PH across the lifespan.

Generative artificial intelligence (AI) has rapidly become embedded in academic writing, assisting with tasks ranging from language editing to drafting text and producing evidence. Despite the wide range of AI use, the expectations for disclosure remain inconsistent. Several journals use binary disclosure statements that fail to distinguish minor language assistance from uses that have a significant impact on the manuscript structure and data interpretation. This article proposes a risk-proportional approach separating disclosure by risk.

Eisenmenger syndrome (ES) represents an advanced stage of pulmonary arterial hypertension (PAH) associated with congenital heart disease. It is characterized by elevated pulmonary vascular resistance, with a right‐to‐left shunt and resting hypoxemia. ES is associated with high morbidity and mortality. Sotatercept, an activin‐signaling inhibitor recently approved for PAH, has demonstrated meaningful hemodynamic and clinical benefits; however, post‐approval experience has raised concern for an association with pericardial effusion. We report a 21‐year‐old woman with ES secondary to an unrepaired patent ductus arteriosus, who developed a hemodynamically significant pericardial effusion 84 days after initiating sotatercept. She presented with tamponade physiology requiring urgent pericardiocentesis and drain placement. An extensive evaluation revealed no obvious alternative etiology. A review of the published literature identified 39 cases of pericardial effusion associated with sotatercept, with more than half occurring in patients with connective tissue–associated PAH. This case highlights potential susceptibility in complex physiologic states such as ES and emphasizes the importance of close surveillance.

Background: Despite the role of cardiac catheterization for hemodynamic assessment and endomyocardial biopsy in children with cardiomyopathy, data on procedure‐related major adverse events (MAE) in this population are lacking. We aim to describe the rate of MAE in children with cardiomyopathy undergoing cardiac catheterization. Our secondary objective is to compare the rate of MAE among patient subsets within this population. Methods: We included patients with cardiomyopathy from the National Cardiovascular Data Registry IMPACT (Improving Pediatric and Adult Congenital Treatment) who were 18 years old or younger and underwent cardiac catheterization between April 2016 and December 2024. Data collected included demographic, preprocedural, procedural, and outcome‐related variables. We performed univariate and multivariate analyses to identify variables independently associated with MAE. Results: A total of 4932 procedures were evaluated, with a median age of 10 years. Dilated cardiomyopathy was the most prevalent cardiomyopathy in 53.4%. The rate of MAE was 3.17%. The most common MAE was arrhythmia, representing 42.3% of all MAE. The multivariate analysis also revealed that age <1 year, hypertrophic cardiomyopathy compared with dilated cardiomyopathy, preprocedural inotropic support, left femoral venous access, and systemic heparinization were associated with an increased rate of MAE. In contrast, procedures performed electively were associated with a lower risk of MAE. Conclusions: Cardiac catheterization in pediatric patients with cardiomyopathy was associated with low incidence of MAE as compared with the general pediatric population. Factors associated with an increased rate of MAE include age <1 year, hypertrophic cardiomyopathy, inotropic support, left femoral venous access, and systemic heparinization.

Summary: Dumping syndrome is a rare but potentially serious condition that causes inappropriate postprandial hyperinsulinemia leading to hypoglycemia in children following gastrointestinal surgeries. While dietary modifications are often the first line of treatment, severe cases may require pharmacological intervention to prevent severe hypoglycemia. We present a case of successful treatment of dumping syndrome with diazoxide. A 2-month-old infant with left hypoplastic heart syndrome who underwent single ventricle palliation pathway and developed feeding intolerance that required Nissen fundoplication. Postprandial hypoglycemia was detected following the procedure, with glucose level down to 12 mg/dL, and the diagnosis of dumping syndrome was established. The patient was successfully managed with diazoxide, which effectively resolved postprandial hypoglycemia without any major adverse events. The patient was eventfully weaned off the medication at the age of 5 months. This case highlights the potential role of diazoxide in the management of pediatric patients with postprandial hyperinsulinemic hypoglycemia secondary to dumping syndrome. Learning points: Dumping syndrome is a possible complication of gastrointestinal surgeries and should be suspected in children with abnormal glucose levels. Postprandial hyperglycemia should be monitored closely for significant subsequent hypoglycemia. Diazoxide might be considered as part of the treatment plan for dumping syndrome.

While still considered a high-risk procedure, cardiac catheterization during the early postoperative period is being performed more frequently in the current era. Limited data are currently available concerning the acute hemodynamic consequences of these procedures. Therefore, the purpose of this study was to evaluate the safety/efficacy of cardiac catheterization performed within thirty days of congenital heart surgery. We completed a retrospective review of all catheterizations within 30 days of congenital heart surgery. Procedures were performed due to failure to progress or hemodynamic deterioration. There were 1873 congenital heart surgeries during the study period. One hundred and three (6.2%) patients with a median age of 124 days underwent catheterization. Sixty-three cases received interventions, and forty patients underwent diagnostic catheterization. Early cardiac catheterization did not show a significant immediate change in the hemodynamics or inotrope score. Survival for patients undergoing diagnostic Cath (81%) did not differ significantly from the intervention group (89%). Although cardiac catheterization was performed on patients at the highest risk for death in the postoperative period, catheter intervention did not increase the risk of death. Those patients undergoing catheter intervention did not seem to experience major adverse events but achieved mild improvement in tissue perfusion.