ESPN 54th Annual Meeting

ESPN 2022

Much tension without the adrenaline: orthostatic hypertension mimics pheochromocytoma


 In the presence of hypertension in children, physicians should strive to find a diagnosis. Orthostatic hypertension (OHT) and postural tachycardia syndrome (PoTS) signal an abnormality in cardiovascular autonomic control mechanisms and might mimic pheochromocytoma because of the paroxysms of hyperadrenergic symptoms.

Material and methods:

 A 14-year old boy with a congenital solitary kidney presented with fatigue and paroxysmal headache, light-headedness and palpitations for the past 6 months. Blood pressure (BP) was 160/80 mmHg. 


 Complete blood count, serum electrolytes and serum creatinine were within normal reference range. Renal Doppler ultrasound was normal. He had no target-organ damage: heart ultrasound and fundoscopy were normal, proteinuria was negative. 24-h ambulatory BP monitoring found normal daytime and nighttime averages and revealed diurnal spikes of the systolic BP of more than 160 mmHg. 24-h urinary normetanephrine level was 5 times the normal amount. The patient underwent PET scanning to detect and localize the suspected pheochromocytoma, but no hypermetabolic activity was found. Further clinical examination found orthostatic tachycardia with concomitant orthostatic hypertension. Noradrenaline was measured after drawing a blood sample in both a supine and a standing position. The patient had a massive elevation of plasma noradrenaline in the standing position. 10-minute tilt and stand tests induced symptoms as well as modifications in heart rate and BP.  The patient needed a triple hypotensive therapy (beta-blockers, α1-blockers, central sympatholytics) as well as non-pharmacological treatment (sophrology) in order to control symptoms. 


 PoTS and OHT are disorders of the autonomic nervous system that can produce substantial disability among otherwise healthy people. The clinical picture of PoTS and OHT can be confused with pheochromocytoma. While the latter requires urgent medical attention, PoTS and OHT are considered to be benign conditions. However, OHT in children might herald sustained arterial hypertension later in life, therefore close follow-up is needed.