Pericardial Cyst in Birt-Hogg-Dubé Syndrome: An Unexpected Discovery

Birt-Hogg-Dubé syndrome (BHD) is a genetic disorder typically characterized by pulmonary cysts, cutaneous fibrofolliculomas, and renal tumors. We report a case of an 87-year-old male patient with a known diagnosis of BHD and a large pericardial cyst who presented to the emergency room with chest pain. BHD is classically associated with pulmonary cysts and not pericardial cysts. In this report, we highlight the potential of pericardial cysts to independently cause retrosternal pain resembling angina, while also mentioning that BHD too can cause chest pain through the rupture of a pulmonary cyst leading to spontaneous pneumothorax. In our case, coronary angiography revealed non-obstructive coronary arteries, so the cause of chest pain was attributed to myocardial infarction with non-obstructive coronary arteries (MINOCA). Atypical causes of chest pain should be considered, especially in patients with diagnosed genetic syndromes.