Abstract

A 41-year-old female was referred to our clinic with progressive dyspnea and a syncope, preceded by angina. On admission she was in cardiogenic shock. ECG showed diffuse repolarization changes and cardiac enzymes were elevated. The echocardiogram revealed severe left ventricular dysfunction with basal and medium walls hypokinesia. After stabilizing the patient, a coronary angiography was performed which revealed normal epicardial arteries. In the next days her clinical status was marked by severe hypertensive episodes with flash pulmonary edema and low responsiveness to therapy. Cardiovascular magnetic resonance showed myocardial edema and intramyocardial late gadolinium enhancement. An abdominal ultrasound raised suspicion of a pheochromocytoma due to an abnormal mass with cystic areas found on the right suprarenal gland. Elevated urinary free catecholamines and fractionated metanephrines confirmed the diagnosis. Further on, a CT scan better identified the heterogeneous tumor and the patient was referred for a right laparoscopic adrenalectomy. Follow-up at 1 month reported full recovery of the sistolic function. The particularity of the case is represented by the difficulty of diagnosis of adrenergic myocarditis, as well as the management of cardiogenic shock induced by it.

Highlights

  • Adrenergic myocarditis is a very rare presentation of pheochromocytoma and a particular cause of acute heart failure

  • Myocarditis, as a clinically significant condition, is extremely rare in pheochromocytoma, and most likely is provoked by a direct myocardial injury caused by catecholamines [1]

  • Adrenergic myocarditis complicated with cardiogenic shock is a therapeutic challenge due to the important limitations of disease pathogenesis

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Summary

Introduction

Adrenergic myocarditis is a very rare presentation of pheochromocytoma and a particular cause of acute heart failure. Cardiac auscultation revealed a systolic murmur in mitral area and pulmonary auscultation was specific with acute pulmonary edema – diffuse crackles in all lung fields. We observed a moderate improvement in the patient clinical status (remission of acute pulmonary edema: respiratory rate of 18/min, FiO2 =0.3, presence of diuresis, hemodynamic stability: MAP =65-70 mmHg, normal pulmonary auscultation) so it was decided to continue with a coronary angiogram to exclude or treat significant obstructive coronary artery disease. After 76 hours from admittance the patient was again in acute pulmonary edema but with very high blood pressure (250/130mmHg) which required i.v. nitroglycerin in high doses (50 micrograms/minute), furosemide (200mg over 100 minutes), morphine (single dose of 2 mg) and urapidil (intravenous bolus at a dose of 12.5 mg, followed by a continuous infusion at a rate of 10 mg/hr). Figure: Right adrenal gland with a massive tumor, surgically removed

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