Pheochromocytoma presenting as acute decompensated heart failure reversed with medical therapy

Abstract

A 26-year-old woman presented to hospital with acute chest pain, hypertension, tachycardia and an elevated serum creatinine. She developed respiratory distress requiring endotracheal intubation and mechanical ventilation. She progressed to multiorgan failure due to decompensated congestive heart failure. Echocardiography demonstrated global hypokinesis and an ejection fraction of <10%. Her cardiac function improved with fluid resuscitation and β blockade, and she was eventually discharged home. She was readmitted a few days later with pancreatitis after presenting with nausea, abdominal pain and hypertension. During hospitalisation she had paroxysms of headache, nausea and diaphoresis associated with hypertension and tachycardia. A CT scan of her abdomen revealed an adrenal mass and serum metanephrine studies confirmed the diagnosis of pheochromocytoma. After fluid resuscitation and sympathetic blockade her ejection fraction improved to 55%. The patient underwent an uneventful adrenalectomy and made a complete recovery.

Pheochromocytoma, “the Great Masquerader,” Presenting as Severe Acute Decompensated Heart Failure in a Young Patient

“…Only around 10–20% of patients with pheochromocytoma present with catecholamine-induced cardiomyopathy [ 8 , 9 ]. When catecholamine-induced cardiomyopathy is present, most of the cases demonstrate a stress-induced cardiomyopathy, Takotsubo syndrome [ 7 , 10 ]. In those patients, an echocardiogram demonstrates transient left ventricular apical ballooning with midventricular dyskinesis that extends beyond the distribution of any single coronary artery.…”

“…However, there are some mechanisms that can explain the myocardial damage associated with catecholamines. These can cause a direct toxic effect on the myocardium through calcium overload due to altered sodium and calcium transporters, enhanced lipid mobility, free radical production, or increased sarcolemmal permeability [ 5 , 10 , 12 ]. In addition, myocardial damage may occur secondary to a decrease in myocardial oxygen supply combined with a sustained myocardial oxygen demand [ 12 , 13 ].…”

“…Nevertheless, in situations of increased stress with very high levels of catecholamines, beta1-adrenergic receptors' activation can induce apoptosis of the cardiac myocytes. Moreover, the binding of epinephrine to beta2-adrenergic receptors produces a negative inotropic effect [ 5 , 10 ]. Thus, catecholamines also have a dose-dependent cardiotoxic effect.…”

“…On the other hand, in chronic adrenergic exposure, it is hypothesized that the heart gets gradually used to the surge of catecholamines with desensitization of adrenergic receptors [ 7 ]. Unlike acute exposure, long-standing catecholaminergic burden leads to structural myocardial alterations such as interstitial fibrosis, inflammation, and a subsequent dysfunction [ 10 , 13 ]. This chronic exposure may explain the lower level of LVEF recovery in the catecholamine-induced cardiomyopathy subgroup described by Batisse-Lignier et al…”

An unexpected post-partum fulminant heart failure

“…There have been a few proposed mechanisms which include a catecholamine-induced cardiomyopathy by the direct toxic effect from norepinephrine and other oxidized products, downregulation of beta receptors and a reduction in myofibrils, alpha 1 adrenergic receptor stimulation (leading to vasospasm and subsequent hypoxia of the coronary arteries) [1][2][3]9]. Additionally, the increased inotropy, chronotropy and afterload may contribute to ischemia and tachyarrhythmias, which may lead to a rate related cardiomyopathy [4].…”