Abstract / Summary
Atrial septal defect (ASD) is a congenital cardiac anomaly that may remain clinically silent until adulthood, potentially progressing to serious complications if undiagnosed. Cardiac cirrhosis and portal hypertension as initial manifestations of undiagnosed ASD with Eisenmenger physiology represent an exceptionally rare clinical presentation warranting documentation. We report the case of a 36-year-old Asian Indian female of Gujarati ethnicity, hailing from western India, who presented with generalized weakness, pitting edema of all four limbs, and melena for 15 days. Diagnostic evaluation revealed a large ostium secundum ASD with predominantly right-to-left shunt consistent with Eisenmenger physiology, severe tricuspid regurgitation, right atrial and ventricular dilation, and severe pulmonary arterial hypertension (RVSP 65 mmHg). Imaging demonstrated hepatic changes consistent with cardiac cirrhosis and portal hypertension with splenorenal and perigastric collaterals. Concurrent hepatitis B infection (HBV DNA 29,910 IU/ml) was identified. Laboratory findings included severe anemia (hemoglobin 6.5 g/dL), thrombocytopenia (platelets 50,000/cumm), elevated total bilirubin (9.8 mg/dL), and hypoalbuminemia. Management included packed cell volume transfusions, diuretics (furosemide and spironolactone), propranolol for portal hypertension, pulmonary vasodilators (tadalafil and ambrisentan), and antiviral therapy (tenofovir disoproxil) for hepatitis B. The patient showed clinical improvement with resolution of jaundice, reduced edema, and improved hemoglobin at discharge. This case highlights the need to consider congenital heart disease in patients with unexplained hepatic dysfunction and portal hypertension, particularly when common hepatic etiologies are absent. It illustrates a mixed-etiology liver disease in which long-standing congestive (cardiac) injury appears to have been the predominant process, with newly detected hepatitis B a probable superimposed insult; in the absence of liver histology, the relative contributions of the two cannot be partitioned with certainty. The case underscores the diagnostic and therapeutic challenges posed by such combined cardiac and viral hepatopathy.