Catecholamine-induced cardiomyopathy in a pediatric patient with paraganglioma: A case report

: Introduction Pheochromocytomas and paragangliomas (PPGL) are neuroendocrine tumors that arise from the adrenal medulla and extra adrenal paraganglia. Catecholamine excess is characterized by episodic or persistent hypertension, tachycardia, and rarely, catecholamine-induced cardiomyopathy. Case Presentation A 6-year-old girl presented with anxiety, palpitations, diaphoresis, and worsening dyspnea without orthopnea . Diagnostic work-up was notable for family history of VHL, elevated catecholamines, and imaging that demonstrated a retroperitoneal mass near the left adrenal gland consistent with a paraganglioma. Echocardiogram showed dilated left that included initiating an alpha-adrenergic-blockade for blood pressure (BP) control, careful volume expansion, and with an ACE-inhibitor for supplemental BP control. An inotropic agent was given prior to for beta-blockade. A tyrosine hydroxylase inhibitor treated paroxysmal increases in pressure. The patient had severe anxiety requiring intensive psychological management . The medical team selected a window of physiologic stability for surgery accepting a low EF. The paraganglioma was removed with intraoperative stabilization of heart rate and BP. Post-operatively, she stayed in the ICU for 4 days, with a slow wean of cardiotropic medications. EF had recovered to near-normal levels ten months later. She will undergo lifetime surveillance for PPGL recurrence and other manifestations of VHL. Conclusion Preoperative optimization of patients with catecholamine-induced cardiomyopathy may require alpha and beta blockade, adjuncts for BP and tyrosine hydroxylase. While BP and HR may normalize, full cardiac function recovery may not occur in the presence of a secreting tumor. Preparation for catecholamine surges and cardiac failure during tumor removal is mandatory. Proactive management of anxiety is essential.