Multiple endocrine neoplasia 2A syndrome presenting as peripartum cardiomyopathy due to catecholamine excess

Kim, Jaime; Reutrakul, Sirimon; Davis, Dawn Belt; Kaplan, Edwin L.; Refetoff, Samuel

doi:10.1530/eje.0.1510771

Cited by 29 publications

(24 citation statements)

References 33 publications

(42 reference statements)

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“…The reversibility of the cardiomyopathy in pheochromocytoma indicates that it is catecholamine induced, and removal of the adrenal mass can lead to resolution of the cardiomyopathy. 8 The effects of catecholamines in pregnancy were demonstrated when dobutamine, a sympathomimetic agent, produced a reduction of contractile cardiac reserve and suboptimal response to hemodynamic stresses. 26 This study demonstrated that patients with previous cardiomyopathy during pregnancy and seemingly normal cardiac function after delivery showed decreased cardiac reserve when treated with an amount of dobutamine that normally would mimic the increased cardiac output of pregnancy.…”

Section: Discussionmentioning

confidence: 99%

Pheochromocytoma in Pregnancy

et al. 2010

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Section: Discussionmentioning

confidence: 99%

Pheochromocytoma in Pregnancy

et al. 2010

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“…Rarely, the patient may present with non-cardiac pulmonary edema or aortic dissection (26)(27)(28). Patients who develop unexplained peripartum cardiomyopathy should also be examined for the existence of a pheochromocytoma (29)(30)(31). The initially reported high maternal mortality in undiagnosed and untreated patients was as high as 50% (5,6,8,9).…”

Section: Introductionmentioning

confidence: 99%

ENDOCRINE DISORDERS IN PREGNANCY: Pheochromocytoma and pregnancy: a deceptive connection

Lenders¹

2012

European Journal of Endocrinology

132

139

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A pheochromocytoma in a pregnant patient is one of the most threatening medical conditions for mother, fetus, and physician. Although extraordinarily rare with a frequency of 0.002% of all pregnancies, this tumor is notorious for its devastating consequences. As in non-pregnant patients, the signs and symptoms are quite variable but not specific, with hypertension being one of the most prominent signs. Confusion with the much more prevalent forms of pregnancy-related hypertension is the main cause of overlooking the diagnosis. If undiagnosed, maternal and fetal mortality is around 50%. Conversely, early detection and proper treatment during pregnancy decrease the maternal and fetal mortality to !5 and 15% respectively. For the biochemical diagnosis, plasma or urinary metanephrines are the tests of first choice since they have a nearly maximal negative predictive value. For reliable localization, only magnetic resonance imaging is suitable, with a sensitivity of more than 90%. When the tumor is diagnosed in the first 24 weeks of gestation, it should be removed by laparoscopic adrenalectomy after 10-14 days of medical preparation with the same drugs as in non-pregnant patients. If the tumor is diagnosed in the third trimester, the patient should be managed until the fetus is viable using the same drug regimen as for regular surgical preparation. Cesarean section with tumor removal in the same session or at a later stage is then preferred since vaginal delivery is possibly associated with higher mortality. Despite all technical diagnostic and therapeutic progress over the last decades, the key factor for further reduction of maternal and fetal mortality is early awareness and recognition of the potential presence of a pheochromocytoma in a pregnant patient with hypertension.

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“…It has also been associated with the development of dilated cardiomyopathy, myocarditis, and heart failure. [1][2][3][4][5][6][7][8][9][10][11] Pheochromocytoma as a cause of systemic hypertension occurs in less than 1% of hypertensive patients. Surgical removal is curative in greater than 85% of cases.…”

Section: Discussionmentioning

confidence: 99%

Presentation of an Unsuspected Pheochromocytoma as Acute Aortic Valvular Insufficiency and Diabetes Mellitus Type 2

Sinnott

Sarne

et al. 2006

The Endocrinologist

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We report the uncommon presentation of an unsuspected pheochromocytoma in a 76-year-old woman. Two years after surgery for acute aortic valve insufficiency and a diagnosis of diabetes, the patient presented with postural headaches, orthostatic dizziness, and palpitations. Physical examination confirmed intractable hypertension, orthostatic hypotension, and congestive heart failure secondary to progressive dilated cardiomyopathy. Based on her symptomatology, examination findings, and progressive dilated cardiomyopathy despite aortic valve replacement, a diagnosis of a pheochromocytoma was entertained. Twenty-four-hour urinary studies confirmed the presence of a catecholamine producing tumor. A magnetic resonance image revealed the presence of a 4.6-cm left adrenal mass, which characteristically enhanced on T2 imaging. After medical stabilization with phenoxybenzamine, she underwent a left robotic adrenalectomy. Histology confirmed a diagnosis of a pheochromocytoma. Two weeks later, she was normoglycemic and normotensive off all medications. The pheochromocytoma is a rare catecholamine-producing tumor, which can be lethal unless recognized and appropriately treated. A high degree of clinical suspicion is necessary, especially in the presence of confounding disease processes. A pheochromocytoma should be considered in the differential diagnosis of acute aortic valvular insufficiency in the setting of orthostatic hypotension, intractable hypertension, and newly diagnosed diabetes. Learning Objectives• Recall the presenting clinical features of this patient when seen 2 years after replacement of an insufficient aortic valve, emphasizing cardiovascular abnormalities. • Point out the lethality of unrecognized pheochromocytoma and diagnostic measures that may permit timely surgical treatment. • Explain relationships among hypersecretion of catecholamines by a pheochromocytoma, cardiovascular disease, and type 2 diabetes. P heochromocytoma presenting as aortic valve insufficiency, progressive dilated cardiomyopathy, orthostatic hypotension, and diabetes is uncommon. We report an unusual case of a 76-year-old woman with a recent history of acute aortic valvular insufficiency and diabetes mellitus type 2 who presented with congestive heart failure, orthostatic hypotension, and was subsequently found to have an unsuspected pheochromocytoma. She had rapid resolution of her intractable orthostatic hypotension, congestive heart failure, and diabetes after the surgical removal of the tumor. CASE REPORTA 76-year-old woman was first hospitalized 2 years before the present illness for the surgical repair of acute aortic valve insufficiency. At that time, she was simultaneously diagnosed with type 2 diabetes requiring oral medications. Her medical history was notable for uncontrolled hypertension. An echocardiogram revealed severe acute aortic valvular insufficiency with evidence of normal left ventricular size and function. Cardiac catheterization demonstrated no evidence of coronary artery disease.

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Multiple endocrine neoplasia 2A syndrome presenting as peripartum cardiomyopathy due to catecholamine excess

Cited by 29 publications

References 33 publications

Pheochromocytoma in Pregnancy

Pheochromocytoma in Pregnancy

ENDOCRINE DISORDERS IN PREGNANCY: Pheochromocytoma and pregnancy: a deceptive connection

Presentation of an Unsuspected Pheochromocytoma as Acute Aortic Valvular Insufficiency and Diabetes Mellitus Type 2

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