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[Pheochromocytoma-induced dilated cardiomyopathy]
1Medicinska kliniken i Torsby.
Abstract:
Pheochromocytoma may mimic a variety of clinical syndromes which present a diagnostic challenge when blood pressure is normal. Occasionally the predominant manifestation of such a tumour is congestive heart failure. The article describes a 19 year-old adolescent with an advanced dilated cardiomyopathy (ejection fraction 0.20) which was finally proven to be induced by a noradrenaline-secreting tumour (urinary noradrenaline: 88,130 nmol/24 hours, ref. interval: less than 350) localized in his right adrenal medulla. In spite of normalization of urinary noradrenaline levels postoperatively the patient died within two weeks due to intractable biventricular failure. Even though pheochromocytoma-induced cardiomyopathy is regarded as potentially reversible after tumour resection, careful consideration is imperative to optimize the patients preoperative status. The article reviews the mechanisms involved in the development of the myocardial changes induced by pheochromocytoma, as well as therapeutic principles relating to the pre- and intraoperative stages of the disease.