Acute aortic dissection related to bilateral pelvic paragangliomas.

Abstract

A female in late adolescence with severe chest pain, dyspnoea, diaphoresis and dizziness presented to the emergency department where she was found to have exceptionally high blood pressure (250/150 mm Hg) and a diastolic murmur. Initial examinations showed left ventricular hypertrophy, and urgent CT angiography confirmed a Stanford type A aortic dissection. Following successful surgical repair, further evaluations were prompted by her persistent, drug-resistant hypertension, revealing elevated normetanephrine and 3-methoxytyramine. Subsequent imaging identified bilateral pelvic paragangliomas, which were surgically removed, significantly reducing her hypertension. Her postoperative period showed biochemical remission, and genetic testing was positive for germline SDHB mutation. Monitoring and follow-up imaging are ongoing. This case highlights the rare association of pelvic paragangliomas with acute aortic dissection in young adults, emphasising the importance of possible endocrine hypertension in young people with hypertensive emergencies.