Pheochromocytoma storm presenting as cardiovascular collapse at term pregnancy

Pheochromocytomas are neuroendocrine tumors that typically present with paroxysms of hypertension, but occasionally can lead to marked hemodynamic instability, left ventricular dysfunction, and cardiovascular collapse. Although pheochromocytoma in pregnancy is rare, factors specific to pregnancy can precipitate catecholamine crisis, making diagnosis and treatment challenging. We present a case of acute cardiovascular collapse with transient left ventricular dysfunction due to catecholamine crisis in a healthy young woman at term pregnancy. Further clinical and genetic investigation revealed pheochromocytoma as part of multiple endocrine neoplasia IIa (Sipple syndrome). A discussion of diagnosis and treatment strategies for pheochromocytoma in pregnancy and acute catecholamine crisis accompanies this report.