Paroxysmal Hypertension, Pheochromocytoma, and Pregnancy

David J. Lyman, MD, MPH

Disclosures

J Am Board Fam Med. 2002;15(2) 

In This Article

Abstract and Introduction

Background: Hypertension is the most common medical complication of pregnancy. Pheochromocytoma in pregnancy is rare, and if unrecognized, can cause serious perinatal morbidity and mortality.
Methods: A patient with severe hypertension, postpartum pulmonary edema, and a recognized pheochromocytoma is described.
Results: Abdominal palpation after vaginal childbirth reproduced the diagnostic triad of hypertension, headaches, and palpitations. Magnetic resonance imaging established the correct diagnosis before biochemical confirmation of excess catecholamine production. The patient responded to -adrenergic receptor blockade with control of her severe hypertension and clearing of pulmonary edema. The best time to diagnose a pheochromocytoma is before delivery because vaginal childbirth stimulates the release of lethal amounts of catecholamines.
Conclusions: The physician who delivers babies must distinguish between labile hypertension and paroxysmal hypertension. Most experts believe that a spontaneous vaginal delivery is contraindicated when the patient has a pheochromocytoma. Postpartum pulmonary edema associated with a pheochromocytoma is unusual. The profound pressor response elicited by palpation of the postpartum abdomen, the failure of medications usually effective in the treatment of a hypertensive crisis, and the use of magnetic resonance imaging to confirm a functioning adrenal adenoma are the features unique to this case.

Pheochromocytoma during pregnancy is rare, with an estimated prevalence in full-term pregnancies of 1 in 50,000 to 54,000.[1,2] Recent authors have reviewed the various clinical signs and symptoms, antenatal diagnostic features, and perioperative management of these rare endocrine tumors.[1,2,3,4,5,6] Most patients have hypertension, either sustained or paroxysmal. The classic triad of headaches, palpitations, and excessive sweating is common, but less so in the pregnant than in the nonpregnant state.[4] A wide variety of other signs and symptoms can also occur, including chest pain, dyspnea, visual disturbances, abdominal pain, nausea, vomiting, dizziness, dependent edema, postural hypotension, congestive heart failure, arrhythmias, seizures, and sudden death from hemorrhage into the tumor. Antenatal diagnosis is imperative to avoid maternal and fetal morbidity and mortality.

Before 1970 only 25% of cases were diagnosed during pregnancy. Since 1990, 85% of cases have been diagnosed antenatally, suggesting an increased awareness of this rare but lethal condition.[3,4] In the case report that follows, the diagnosis of a pheochromocytoma was not suspected until an examination of a postpartum patient's abdomen induced paroxysmal attacks of chest pain and hypertension. Magnetic resonance imaging (MRI) confirmed the diagnosis, and her hypertensive crisis and acute pulmonary edema responded to appropriate treatment.

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