Cardiac phaeochromocytoma presenting with severe hypertension and chest pain

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Abstract

Cardiac phaeochromocytoma is a rare cause of endocrine hypertension. We report a case of a 25-year-old woman, who presented with severe hypertension and intermittent chest pain. The patient denied typical phaeochromocytoma spells of palpitation, headache, and diaphoresis. The 24-hr urinary excretion of norepinephrine was increased sevenfold above the upper limit of normal; however, the excretion of total metanephrines, epinephrine, and dopamine were normal. Computed tomography (CT) scan of the abdomen was normal. An 131I-labelled metaiodobenzylguanidine (MIBG) scan was falsely negative while the patient was taking labetalol. The cardiac phaeochromocytoma was localized with indium-111-pentetreotide scintigraphy and chest magnetic resonance imaging scan. Repeat 123I-MIBG scintigraphy was positive after discontinuing labetalol. The cardiac phaeochromocytoma was located in the right atrial groove, adjacent to the tricuspid valve, and contained multiple feeder arteries from the right coronary artery. After treatment with volume expansion, α-methyl-p-tyrosine, and α- and β-adrenergic blockade, surgical resection was performed. While under cardiopulmonary bypass, coronary bypass grafting and tricuspid annuloplasty were performed to facilitate the complete surgical resection of the 4.5-cm tumour. The surgical course was uncomplicated, with complete cure of hypertension and normalization of catecholamine excretion. Post-operative cardiac function, as measured by echocardiogram, was normal. Although cardiac phaeochromocytoma may be highly vascular, invasive and difficult to resect, it can be cured.

Original languageEnglish (US)
Pages (from-to)689-692
Number of pages4
JournalClinical Endocrinology
Volume54
Issue number5
DOIs
StatePublished - 2001

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Pheochromocytoma
Chest Pain
Hypertension
Labetalol
Radionuclide Imaging
Metanephrine
Tricuspid Valve
Cardiopulmonary Bypass
Abdomen
Adrenergic Agents
Epinephrine
Catecholamines
Blood Vessels
Headache
Tyrosine
Dopamine
Coronary Vessels
Norepinephrine
Thorax
Arteries

ASJC Scopus subject areas

  • Endocrinology

Cite this

Cardiac phaeochromocytoma presenting with severe hypertension and chest pain. / Sawka, A. M.; Young, William Francis; Schaff, Hartzell V.

In: Clinical Endocrinology, Vol. 54, No. 5, 2001, p. 689-692.

Research output: Contribution to journalArticle

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