Atypical presentation of pheochromocytoma as part of multiple endocrine neoplasia IIa in pregnancy.

BACKGROUND: Pheochromocytoma in pregnancy is extremely dangerous, especially when unrecognized, and can present alone or as part of a multineoplastic syndrome. Hypertension, its hallmark, is not present in all cases, particularly in women with multiple endocrine neoplasia type IIa.

CASE: We report a gravida with undiagnosed multiple endocrine neoplasia IIa who presented initially with peripartum cardiomyopathy but was diagnosed with an underlying pheochromocytoma. Once recognized and treated with appropriate alpha-adrenergic blockade, her condition reversed quickly.

CONCLUSION: Physicians should be aware that pheochromocytoma can present as cardiovascular collapse rather than just hypertension.