Case Session 07: Cardiac Masses (2018)-Growing from within: dyspnea due to progressive stenosis of multiple heart vessels

Growing from within: dyspnea due to progressive stenosis of multiple heart vessels

Video Transcription

Video Summary

A 42-year-old woman presented with recurrent exertional dyspnea and brief rest chest pain. Exam and labs were largely normal except for a systolic murmur and echocardiographic severe pulmonary hypertension with right ventricular dilation and a moderate pericardial effusion. CT and cardiac MRI revealed a large vascularized pericardiac mass invading the aortic root, pulmonary artery, and coronary arteries, causing severe pulmonary stenosis and left main/coronary occlusion. Initial biopsy was nondiagnostic. After surgical biopsy, histology showed lymphoplasmacytic infiltration with abundant IgG4-positive cells, confirming IgG4-related disease involving the great vessels. She deteriorated with recurrent myocardial infarction and cardiac arrest.

Keywords

IgG4-related disease

pericardiac mass

pulmonary hypertension

coronary artery occlusion

aortic root invasion