Cardiac phaeochromocytoma presenting with severe hypertension and chest pain

Cardiac phaeochromocytoma is a rare cause of endocrine hypertension. We rep ort a case of a 25-year-old woman, who presented with severe hypertension a nd intermittent chest pain. The patient denied typical phaeochromocytoma sp ells 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 w ere normal. Computed tomography (CT) scan of the abdomen was normal. An I-1 31-labelled metaiodobenzylguanidine (MIBG) scan was falsely negative while the patient was taking labetalol. The cardiac phaeochromocytoma was localiz ed with indium-111-pentetreotide scintigraphy and chest magnetic resonance imaging scan. Repeat I-123-MIBG scintigraphy was positive after discontinui ng labetalol. The cardiac phaeochromocytoma was located in the right atrial groove, adjacent to the tricuspid valve, and contained multiple feeder art eries from the right coronary artery. After treatment with volume expansion , alpha -methyl-p-tyrosine, and alpha- and beta -adrenergic blockade, surgi cal resection was performed. While under cardiopulmonary bypass, coronary b ypass grafting and tricuspid annuloplasty were performed to facilitate the complete surgical resection of the 4.5-cm tumour. The surgical course was u ncomplicated, with complete cure of hypertension and normalization of catec holamine excretion. Post-operative cardiac function, as measured by echocar diogram, was normal. Although cardiac phaeochromocytoma may be highly vascu lar, invasive and difficult to resect, it can be cured.