Catecholamine-Induced Cardiomyopathy and Pheochromocytoma

Abstract

A previously asymptomatic 37-yr-old man presented to the hospital with acute hypertension of 220/120 mm Hg and unstable ventricular tachycardia requiring cardioversion. A transthoracic echocardiogram (TTE) 1 yr earlier had shown mitral valve prolapse with moderate mitral regurgitation (MR), normal left ventricular (LV) size and function and mild right ventricular (RV) dilation. His TTE after admission revealed dilated, hypokinetic RV with mild tricuspid regurgitation and flattening of the interventricular septum (IVS) (Video Clip 1; please see video clip available at www.anesthesia-analgesia.org). The estimated LV ejection fraction (EF) was 55% with moderate MR. The rest of the TTE was unremarkable. Further workup revealed elevated urine catecholamine levels and a 5 6 cm right adrenal mass. The patient was started on phenoxybenzamine and labetalol and was scheduled for adrenalectomy. A repeat, preoperative TTE 1 wk after admission demonstrated a deteriorated LVEF of 30% and an otherwise unchanged examination. Consequently, the diagnosis of pheochromocytoma and catecholamine-induced cardiomyopathy was made. A biventricular assist device was on standby in anticipation of intraoperative cardiac deterioration. Intraoperative transesophageal echocardiography (TEE) demonstrated a massively dilated and severely hypokinetic RV distorting the normal cardiac anatomy. Systolic and diastolic IVS flattening was present (Fig. 1, Video Clip 2; please see video clip available at www.anesthesia-analgesia.org). The right atrium was enlarged and tricuspid regurgitation remained mild. The LV was also significantly dilated with an estimated EF of 25%–30% (Video 1). The mitral valve annulus was widened at 5.1 cm. Mitral valve prolapse with severe, eccentric MR (vena contracta width 1 cm) was visualized (Video Clip 3; please see video clip available at www.anesthesiaanalgesia.org). The intraoperative anesthetic management was based on the stage of surgery, hemodynamic changes and TEE findings. His measured pulmonary artery pressures were mildly elevated with episodic increases to 80/40 mm Hg. The cardiac index was 2.6 L/m and the systemic vascular resistance was 1500 dynes s cm . Sodium nitroprusside, esmolol, and phentolamine were titrated to effect for intermittent hypertension of up to 240/130 mm Hg. Severe hypotension to 60/30mm Hg (cardiac index 3.2 L/m, systemic vascular resistance 500 dynes s cm ) followed adrenalectomy requiring treatment with epinephrine, norepinephrine, arginine vasopressin, and volume expansion. His arterial blood pressure slowly improved to 90/60 mm Hg. The estimated LVEF improved to 35% and ventricular assist device placement was deemed unnecessary. The patient had an uncomplicated postoperative course. TTE 1 mo later showed normalization of LV size and function. The mitral valve annulus was less dilated (4.2 cm) and the MR was moderate (vena contracta width of 0.55 cm). RV function improved slightly with less dilation and minimal IVS flattening (Video 1). This article has supplementary material on the Web site: www.anesthesia-analgesia.org.