Acute cardiomyopathy and multiorgan failure in a patient with pheochromocytoma and neurofibromatosis type 1

Pheochromocytomas are catecholamine secreting tumours of the adrenal gland, discovered in 0.1% of patients with hypertension. Our case highlights an atypical presentation of pheochromocytoma in a patient with Neurofibromatosis type 1 who developed cardiogenic shock with multi-organ failure. The pati...

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Veröffentlicht in:Journal of the Pakistan Medical Association 2014-02, Vol.64 (2), p.214-216
Hauptverfasser: Ahmed, Rezwan, Darrat, Yousef, Hamoudeh, Eyad, Elhamdani, Mehair Omar, Yaqub, Abid
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Sprache:eng
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Zusammenfassung:Pheochromocytomas are catecholamine secreting tumours of the adrenal gland, discovered in 0.1% of patients with hypertension. Our case highlights an atypical presentation of pheochromocytoma in a patient with Neurofibromatosis type 1 who developed cardiogenic shock with multi-organ failure. The patient demonstrated reversible dilated cardiomyopathy during her hospital stay, and her blood pressure fluctuated widely. Discovery of right adrenal mass followed by biochemical testing facilitated the diagnosis. Judicious medical management led to an uneventful surgical removal of the tumour followed by marked stabilization of her blood pressure. We discuss the characteristics of pheochromocytoma associated with Neurofibromatosis type 1 via reversible cardiac dysfunction.
ISSN:0030-9982