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Libman-Sacks endocarditis (LSE) is a rare complication of systemic lupus erythematosus (SLE), characterized by noninfectious vegetation on normal heart valves. We present the case of a 20-year-old woman with SLE and a congenital valve malformation. Despite a year of effective SLE treatment, she later developed stage IV dyspnea, chest pain, and signs of right heart failure. Investigations revealed active lupus, mitral valve vegetation, agenesis of the posterior mitral leaflet, and severe mitral insufficiency. The patient was treated with corticosteroids, antibiotics, anticoagulants, and symptomatic heart failure management. Despite initial improvement, her condition deteriorated, and she did not respond to resuscitation. While LSE often responds well to treatment, severe valvulopathy, particularly with congenital valve defects, can result in fatal outcomes.
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http://dx.doi.org/10.7759/cureus.66536 | DOI Listing |
JACC Case Rep
September 2025
Department of Adult Cardiology, Jakaya Kikwete Cardiac Institute (JKCI), Dar es Salaam, Tanzania.
Background: Transcatheter pulmonary valve implantation (TPVI) has emerged as a viable alternative to surgical pulmonary valve replacement for patients with congenital heart disease and right ventricular outflow tract dysfunction. However, its adoption in low-resource settings has been limited.
Case Summary: We report the first successful TPVI procedures in Tanzania.
Case Rep Pediatr
September 2025
Department of Thoracic Surgery, Avicenna Tajik State Medical University, Dushanbe, Tajikistan.
Ectopia cordis is an exceptionally uncommon congenital condition where the heart develops outside its normal position due to incomplete closure of the ventral chest wall during embryogenesis. The anomaly may occur in isolation or with other structural defects, often resulting in a poor prognosis despite advancements in medical and surgical care. This report discusses a preterm neonate delivered at 33 weeks of gestation following an uneventful pregnancy in a dizygotic twin gestation.
View Article and Find Full Text PDFEClinicalMedicine
October 2025
Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, China.
Background: Paediatric patients who underwent surgery for mitral regurgitation (MR) have a high risk of recurrence or death; however, no prediction tool has been developed to risk-stratify this challenging subpopulation.
Methods: In this multicentre cohort study, paediatric patients undergoing surgery for congenital MR in Shanghai Children's Medical Center in January 1st, 2009-December 31st, 2022 were included for analysis while those had a combination with infective endocarditis, anomalous left coronary artery from the pulmonary artery, rheumatic valvular disease, connective tissue disease, or single ventricle were excluded. A Cox regression model predictive of the primary outcome (a composite of mortality or mitral valve [MV] re-operation) was derived and converted to a point-based risk score.
JTCVS Open
August 2025
Division of Cardiovascular Surgery, Children's National Heart Institute, Children's National Hospital, Washington, DC.
Objectives: We sought to review the outcomes of patients with Ebstein anomaly (EA) after the Fontan operation.
Methods: Patients with EA were identified from a large binational registry about the Fontan operation. Data were collected from hospital records, registry data, and clinical correspondence.
JTCVS Open
August 2025
Division of Cardiothoracic Surgery, Department of Surgery, University of Southern California, Los Angeles, Calif.
Objective: Patients with heterotaxy-associated congenital heart disease often require multiple operations, which may have a cumulative effect on their outcomes. This study aimed to define the cardiac surgical course in a large cohort and identify longitudinal risk factors for death/transplant.
Methods: All patients with heterotaxy-associated congenital heart disease who underwent cardiac surgery at one institution from 2005 to 2022 were retrospectively reviewed.