Title : Cardiac cirrhosis and portal hypertension in an undiagnosed adult atrial septal defect: A rare presentation
Abstract:
Background: Atrial Septal Defect (ASD) is a congenital cardiac anomaly that may remain clinically silent until adulthood, potentially progressing to serious complications if undiagnosed.
Case Summary: We report a rare case of a 36-year-old female who presented with anemia, generalized edema, and melena, ultimately found to have cardiac cirrhosis secondary to chronic right heart failure from undiagnosed ASD with Eisenmenger physiology. Diagnostic evaluation revealed a large ostium secundum ASD with severe tricuspid regurgitation, right atrial and ventricular dilation, severe pulmonary arterial hypertension, and signs of portal hypertension. Multisystem involvement, including hepatic dysfunction and thrombocytopenia, was evident. Management included blood transfusions, diuretics, beta-blockers, antiviral therapy for hepatitis B, and pulmonary vasodilators.
Discussion: This case underscores the critical need for early recognition and appropriate intervention in congenital heart defects to prevent irreversible multisystem complication such as cardiac cirrhosis and portal hypertension. It highlights the complex interplay between cardiac and hepatic pathology in long-standing intracardiac shunts.
Keywords: Atrial Septal Defect (ASD), Eisenmenger Syndrome, Cardiac Cirrhosis, Portal Hypertension, Right Heart Failure, Congenital Heart Disease, Multisystem Involvement.
