Pheochromocytoma Presenting As Acute Cardiomyopathy and Multiorgan Failure in a Young Lady – Think Twice When One Sends a Patient for Heart-Transplantation

Presentation Number: LB SUN 51
Date of Presentation: April 2nd, 2017

Kitty Kit Ting Cheung*1 and Francis CC Chow2
1Prince of Wales Hospital, N.T., Hong Kong, 2The Chinese University of Hong Kong

Abstract

Background:

Pheochromocytoma often poses as a diagnostic challenge to physicians.

Clinical case:

A 29-year-old woman was admitted for cardiogenic shock with multiorgan failure and was started on extracorporeal membrane oxygenation (ECMO) as life sustaining measure. Significant past medical history was an episode of myocarditis few years ago labeled to be caused by viral infection, from which she regained full cardiac function. The initial diagnosis this time was “mumps-related myocarditis” based on positive mumps immunoglobulin M antibodies. The patient was therefore initially planned to be put onto the heart-transplantation waiting list.

Upon enrollment of the patient with the heart-transplantation team, a request to rule-out pheochromocytoma as a prerequisite to have her enlisted was made based on her young age and recurrent cardiomyopathy history. Endocrinologists were consulted. Plasma metanephrine levels were markedly high. An abdominal computer-tomography with contrast revealed a well-defined 7cm right adrenal mass with heterogeneous density with mild contrast enhancement. The diagnosis of right “pheochromocytoma-related cardiomyopathy” was made based on the above investigation results. The patient was started on alpha- and then beta-blockade.

Since the patient was extremely frail, relying on ECMO for life maintenance, and would unlikely survive any hypertensive crisis during adrenalectomy, the clinical plan was to first reduce the blood supply, in order to reduce the risk of hemodynamic instability intraoperatively, to the very vascular right pheochromocytoma by embolizing the supplying vessels. Uneventful right adrenal and accessory right renal arteries embolization was completed followed by successfully right adrenalectomy one day later. There was no hypertensive crisis during the operation. The patient then gradually regained near-normal cardiac and renal function, with ECMO weaned off. Histology revealed an encapsulated tumor measuring 7cm in diameter, with most of the tumor being necrotic, compatible with previous embolization. Immunostains confirmed the diagnosis of pheochromocytoma.

Pre-operative embolization has been employed to lower circulating catecholamine levels and help wean patients off from ECMO before proceeding to adrenalectomies. Our case is the first reported case to have post-embolization adrenalectomy done with ECMO on board due to the very ill status of our patient. Successful operation and normalization of her cardiac function prove that this is a feasible management pathway in critically ill patients with pheocrhomocytoma-related cardiomyopathy.

Conclusion:

High clinical suspicion is needed in the setting of unusual recurrent cardiomyopathy in a young patient. Post-embolization adrenalectomy done with ECMO on board is a feasible option for operation of a patient with pheocrhomocytoma-related cardiomyopathy.

 

Nothing to Disclose: KKTC, FCC