Pramod Kumar, Kumar Ratnjeet, Baiju Sasi Dharan, Rajalakshmi Poyuran, Arun Gopalakrishnan
Sree Chitra Thirunal Institute for Medical Sciences and Technology.
India
Indian Journal of Thoracic and Cardiovascular Surgery
Indian J Thorac Cardiovasc Surg 2026; 42: 806-811
DOI: 10.1007/s12055-026-02179-8
Abstract
Autoimmune carditis in children is uncommon and often overlooked, particularly in regions where rheumatic fever is endemic. We report the case of a 12-year-old girl who presented with progressive dyspnea, orthopnea, and intermittent fever, ultimately found to have a superior vena cava (SVC)-type sinus venosus atrial septal defect (SV-ASD) with partial anomalous pulmonary venous connection (PAPVC). Her condition was compounded by severe mitral, aortic, and tricuspid regurgitation; moderate pulmonary regurgitation; and moderate pericardial effusion. Laboratory testing suggested an autoimmune inflammatory process, and rapid clinical decline with impending cardiac tamponade necessitated urgent pericardial drainage. Immunosuppressive therapy with corticosteroids, hydroxychloroquine, and mycophenolate mofetil was initiated for presumed autoimmune pancarditis, followed by surgical repair consisting of SV-ASD closure with the Warden technique, rerouting of anomalous pulmonary veins, and multivalvular repair. Histopathology confirmed fibrinous pericarditis with chronic inflammation. The patient improved significantly after surgery, with resolution of pulmonary hypertension and stable mild residual valvular disease. At 1 year, she remained asymptomatic with normalized inflammatory markers. This case highlights how autoimmune inflammation superimposed on congenital heart disease can accelerate hemodynamic compromise and underscores the importance of early recognition, timely immunosuppression, and individualized surgical planning to prevent irreversible cardiovascular damage.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Class II. Pulmonary Hypertension Associated with Valvular Disease of the Left Side of the Heart
Medical Therapy. Efficacy or Lack of Efficacy
Surgical and Catheter-mediated Interventions for Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes (available after June 1, 2027)