Pheochromocytoma-induced cardiogenic shock: a systematic review
N Cahuapaza-Gutierrez, C Calderon-Hernandez, I Jordan-BeisagaAbstract
Background
Pheochromocytoma is a catecholamine-secreting adrenal tumour that is rarely associated with the development of cardiogenic shock. To date, significant gaps remain regarding timely diagnosis, optimal management, and clinical outcomes in affected patients.
Purpose
To synthesise and analyse the clinical profile, diagnostic findings, management strategies, and outcomes of patients with pheochromocytoma-induced cardiogenic shock (PICS).
Methods
We included studies reporting adult patients diagnosed with cardiogenic shock secondary to pheochromocytoma. Letters, editorials, and reviews were excluded. A systematic literature search was conducted in PubMed, Scopus, EMBASE, and Web of Science from inception to January 2026. Descriptive statistical analyses, including frequencies and percentages, were performed using STATA Now version 19 SE.
Results
A total of 119 patients were identified from the included studies. There was a predominance of female patients (58.8%), with a mean age of 44.1 ± 13.15 years. The most frequently reported cardiovascular risk factors were hypertension (28.4%), coronary artery disease (17%), and diabetes mellitus (5%). The most common clinical manifestations included tachycardia (55%), chest pain (34%), headache (28%), and palpitations (27%), often accompanied by fluctuating episodes of hypertension and hypotension. Laboratory findings commonly demonstrated elevated cardiac troponins (I or T) in 80% of cases, metanephrine in 63%, and normetanephrine in 52%. Echocardiographic assessment revealed severe left ventricular systolic dysfunction, with left ventricular ejection fraction <10% in 17%, 11–20% in 48%, 21–30% in 32%, and >30% in 13% of patients. Wall motion abnormalities included global left ventricular hypokinesia (56%), diffuse left ventricular hypokinesia (19%), and biventricular involvement (16%). A relevant subgroup exhibited a Takotsubo-like cardiomyopathy phenotype. Adrenal imaging, primarily computed tomography, identified tumour localisation predominantly in the left adrenal gland (62.5%), followed by right-sided and bilateral involvement. Surgical adrenalectomy was performed in 58% of cases, frequently complemented by extracorporeal membrane oxygenation (ECMO) support (37.5%) and cardiovascular pharmacotherapy, including alpha-blockers, beta-blockers, and dobutamine. Overall, 95% of patients experienced a favourable clinical outcome following timely intervention.
Conclusions
This review delineates a characteristic clinical and diagnostic pattern in patients with PICS. Clinical suspicion should be heightened in patients presenting with fluctuating hypertensive and hypotensive episodes accompanied by tachycardia, chest pain, and headache. Elevated troponins, metanephrines, and distinctive echocardiographic findings represent key diagnostic features. Prompt recognition, rapid haemodynamic stabilisation, and definitive tumour management are critical and substantially improve clinical outcomes.