A 51–year–old man with a past medical history significant for obesity, cirrhosis and COPD presented to an outside emergency department for evaluation of progressive edema. The patient was diagnosed with alcoholic cirrhosis 15 years prior but reported that he had not consumed alcohol since that diagnosis. He had recently returned from a trip where he had not been adherent to his low–sodium diet and had gained approximately 18 kilograms. He was put on a furosemide infusion and developed acute renal failure requiring hemodialyiss at which point he was transferred to our institution. On exam, he had massive anasarca with edema prominent up to his chest. He had an additional heart sound and marked JVD. A paracentesis was performed but returned minimal fluid. ECG was significant for low voltage and nonspecific ST abnormalities. Abdominal ultrasound with dopplers demonstrated a nodular cirrhotic liver however the splenic, portal, hepatic veins, as well as the IVC were all patent and had anterograde flow. There was no significant ascites. Chest x–ray was significant for pericardiac calcification. A transthoracic echocardiogram was performed and demonstrated severe ventricular septal shift to the left with inspiration, as well as marked diastolic ventricular septal bounce, which was concerning for constrictive pericarditis. A CT chest was performed which confirmed dense, calcific constrictive pericarditis. The patient was referred to thoracic surgery for definitive pericardiectomy.
Constrictive pericarditis is the physiological consequence of scarring and subsequent loss of elasticity of the pericardium. Resultant chronic hepatic congestion can lead to cirrhosis. A high index of suspicion is required to make this diagnosis in patients presenting with cirrhosis and volume overload, as they can otherwise go misdiagnosed for years. These patients are pre–load dependent and consequently diuresis, as would be appropriate in conventional heart failure or portal hypertension, leads to end–organ hypoperfusion and was the likely etiology of our patient’s acute renal failure and resultant need for hemodialysis. The physical examination findings are key to identifying this condition and include elevated jugular pressure with paradoxical inspiratory rise (Kussmaul’s sign) and a pericardial knock. As was the case with our patient, supportive findings on investigations include low voltages on ECG, subtle, pericardiac calcification on chest x–ray, ventricular septal shift on echo in the context of signs of severe right heart failure.
Congestive hepatopathy secondary to constrictive pericarditis is an uncommon cause of cirrhosis. Timely identification and definitive management of this condition by pericardiectomy has significant implications for patient outcomes.