The Neuroendocrine Heartbreak 💔 : Pheochromocytoma Induced Stress Cardiomyopathy (Takotsubo cardiomyopathy).

Dr. Zayed | Published : April 13, 2025.

Pheochromocytoma is a neuroendocrine neoplasm originating from chromaffin cells of the adrenal medulla or extra-adrenal paraganglia, characterized by excessive catecholamine secretion. A subset of these tumors exhibits a noradrenergic biochemical phenotype, in which norepinephrine predominates over epinephrine and dopamine in the secretory profile. This pattern is clinically significant due to its association with sustained hypertension and heightened cardiovascular morbidity, including stress-induced cardiomyopathy, notably Takotsubo cardiomyopathy.

Takotsubo cardiomyopathy, a transient systolic dysfunction of the left ventricle often precipitated by acute emotional or physiological stressors, has been increasingly recognized as a complication of catecholamine excess. In the context of pheochromocytoma, catecholamine-induced myocardial toxicity, microvascular dysfunction, and coronary vasospasm collectively contribute to regional wall motion abnormalities that resemble acute coronary syndrome. However, angiographic evaluation typically reveals 'patent epicardial coronary arteries'.

Evidence from recent literature, including data published in Hypertension, indicates that up to 28% of patients with pheochromocytoma or paraganglioma exhibit cardiovascular complications, with a notable prevalence of Takotsubo-like cardiomyopathy. The noradrenergic phenotype appears particularly prone to such outcomes, likely attributable to the potent alpha-adrenergic vasoconstrictive effects of norepinephrine, which increase afterload and myocardial oxygen demand, thereby exacerbating myocardial stress and injury.

Clinically, pheochromocytoma-induced TTC may manifest with acute-onset chest discomfort, elevated cardiac biomarkers (e.g., troponin I/T), dynamic electrocardiographic changes, and echocardiographic findings of 'apical or mid-ventricular hypokinesis besides this we has also seen a case of inverted takotsubo pattern resembling base hypokinesia with normal mid and apical left ventricular contractility'. Definitive diagnosis necessitates correlation of imaging findings with biochemical confirmation of catecholamine excess via plasma-free or 24-hour urinary metanephrines. Cardiac magnetic resonance imaging may further delineate the pattern of myocardial involvement and is the 'gold standard' compared to USG.

Therapeutic management is centered on hemodynamic stabilization, with initiation of alpha-adrenergic blockade (e.g., phenoxybenzamine) as the first-line approach to mitigate hypertensive episodes. Beta-adrenergic blockade is introduced cautiously following adequate alpha-blockade to avoid unopposed alpha stimulation. "Surgical adrenalectomy" remains the definitive treatment, with favorable prognostic outcomes and restoration of left ventricular function observed in most patients post-resection.

The intersection of pheochromocytoma and stress-induced cardiomyopathy underscores the necessity for heightened clinical suspicion in patients presenting with unexplained cardiomyopathy, particularly when accompanied by paroxysmal hypertension or adrenergic symptoms. Prompt recognition and targeted intervention are critical to reducing morbidity and optimizing cardiovascular outcomes.

Interesting to know : It is quite well known to us the profound effects of TTC seen in left ventricle. it is not uncommon in present day to see isolated cases of right ventricular involvement and/or Biventricular involvement of ttc for reasons unknown. It is found that right ventricular involvement has poor hospital outcome with prolonged stay Than left sided ttc.