1. Pheochromocytoma-Related Cardiomyopathy: A Case Series
Poster Number: SAT-450
Pheochromocytoma-Related Cardiomyopathy: A Case Series
Iqra Javeed MD1, Roman Schumann MD2, Michael E Tarnoff MD3*, and Ronald M Lechan MD, PhD1
1Division of Endocrinology, Diabetes, and Metabolism, 2Department of Anesthesiology, 3Department of Surgery
Tufts Medical Center, Boston, Massachusetts
*Disclosure: Chief Medical Officer and full time employee, Covidien
Introduction
Case Series
Cardiovascular complications associated with pheochromocytoma (PCC) include arrhythmias, myocardial ischemia, and cardiomyopathy (CM), which can be takotsubo-like or hypertrophic/obstructive. These are more common than generally appreciated, even by cardiologists, and often manifest months or years before the diagnosis of PCC is made. To illustrate this association, we present four cases of PCC-related cardiomyopathy seen at our institution that resolved after anterior laparoscopic adrenalectomy of PCC.
Case 1: A 40 year old man with an indwelling ventriculo-peritoneal (VP) shunt was hospitalized for shunt revision. Postoperatively, he had an episode of supraventricular tachycardia and severe hypertension followed by cardiogenic shock and was diagnosed with takotsubo CM. His condition gradually improved and he was lost to follow up. Six years later, a diagnostic workup for new onset hypertension revealed elevated catecholamines and metanephrines in 24 hour urine collection (epinephrine 225 mcg (nl 2-24), norepinephrine 1018 mcg (nl ), metanephrine 2600 mcg (nl 58 – 203), normetanephrine 5074 mcg (nl )). Imaging demonstrated a 5.1cm left adrenal PCC (Figure 1) that was surgically resected, resulting in resolution of the hypertension.
Case 2: A 64 year old woman with a 12 year history of multiple atrial and ventricular arrhythmias and hypertrophic CM presented to the hospital with a hypertensive emergency. Workup including a 24 hour urine collection revealed elevated epinephrine (852 mcg), norepinephrine (421 mcg), metanephrine (23994 mcg,) and normetanephrine (5527 mcg). Imaging showed a 7.8cm left adrenal PCC (Figure 2) that was surgically resected with subsequent resolution of her hypertension and CM.
Case 3: A 21 year old woman with Turner syndrome was hospitalized after an episode of vomiting and syncope and found to be in hypoxic, respiratory failure from pulmonary edema. Echocardiogram revealed a reduced ejection fraction and distal septal and apical hypokinesis consistent with takotsubo CM. She had a history of a long QT syndrome and hypertension since childhood. Twenty-four hour urine collection confirmed elevated norepinephrine (2361 mcg) and normetanephrine (6235 mcg). Imaging identified a 2.9cm left sided paraganglioma (Figure 3), which after surgical removal, resulted in resolution of the hypertension and improvement of the ECG findings as well as her CM.
Case 4: A 21 year old woman with history of ADHD, and anxiety presented with hypertensive emergency and sinus tachycardia. Soon after admission, she developed altered mental status and had pulseless electrical activity. Echocardiogram revealed a low ejection fraction and severe global hypokinesis consistent with takotsubo CM. Workup including a 24 hour urine collection revealed elevated norepinephrine (>3300 mcg) and elevated serum free normetanephrine (9090 mcg). Imaging showed a 6 cm left adrenal mass (Figure 4) that was surgically resected with subsequent improvement of her hypertension and CM. 
Discussion
Figure 1
CT abdomen/ pelvis showing left pheochromocytoma (red arrow)
Figure 2
CT abdomen/ pelvis showing large left adrenal mass (red arrow)
Cardiovascular complications associated with PCC are a result of receptor-mediated effects, direct toxic effects, and/or excessive adrenergic stimulation attributable to elevated catecholamines. A high index of suspicion for the possibility of PCC, therefore, should be given to the patient presenting with CM who has a history of episodic hypertension. All patients diagnosed with takotsubo cardiomyopathy should be screened for pheochromocytoma
Figure 4
CT abdomen/ pelvis showing left pheochromocytoma (red arrow)
Figure 3
CT abdomen/ pelvis showing left paraganglioma (red arrow)