COULD PHEOCHROMOCYTOMA INDUCED CARDIOMYOPATHY BE INFLUENCED BY AN EXCESSIVE DELAY BEFORE DIAGNOSIS? DISCUSSION ABOUT TWO CASE-REPORTS

Abstract

Objective: Different types of pheochromocytoma-related cardiomyopathies have been described in literature, but predictive factors of their occurrence and evolution are not yet defined. Here are described two successive clinical cases concerning this issue. Design and method: Case-report 1: A 26 year-old woman was admitted for abdominal pain lasting for ten days. Blood pressure (BP) was 150/100 mmHg. The EKG presented negative T waves on the precordial leads, and the trans-thoracic echocardiography (TTE) revealed a severe left heart dysfunction (Left Ventricular Ejection Fraction (LVEF) 20%). There was no significant coronary artery stenosis but left ventricular apical ballooning resembling Takotsubo cardiomyopathy on the cardiac angiography. Plasma concentration of catecholamines was increased to over 26 times their normal values. A 60x58x58 mm right adrenal mass was detected on computer tomography (CT). A laparoscopic adrenalectomy was performed. The diagnosis was confirmed by histology. Thereafter, BP normalized and TTE control assessed complete recovery. Case-report 2: A 35 years old man presented clinical heart failure, his BP measured at 150/100 mmHg. Medical history revealed high blood pressure, which had been detected for a year, without specific therapy thus far. He also described the classic triad of head-aches, palpitations, and diaphoresis. Hypertrophic cardiomyopathy, evocated by negative T waves on anterior leads on the EKG, was confirmed on TTE with an altered ejection fraction of 30%. Coronary catheterization was normal. Explorations found elevated plasma catecholamines level and a right adrenal tumor of 65X55X50 mm on CT. Pathologic examination of the resection specimen affirmed the diagnosis of pheochromocytoma. The clinical follow-up showed BP normalization, but persistence of an altered LVEF. Results: The severe acute clinical presentation of the first patient led to early diagnosis and treatment, with complete recovery. For the second one, the long term evolution of the disease before treatment may explain the poor improvement of the myocardiopathy. Conclusions: These two case-reports suggest that the form and evolution of pheochromocytoma-related cardiomyopathies depend on duration of catecholamine exposure, possibly in close relation with the length of diagnosis delay. This hypothesis could be tested by a larger follow-up of multiple cases of pheochromocytoma-related cardiomyopathies.