Journal of Electrocardiology 58 (2020) 7–9

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Journal of Electrocardiology

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Pheochromocytoma – An ECG diagnosis?

Thomas Lindow, MD a,b,c,⁎, Olle Pahlm, MD, PhD c, Erik Ljungström, MD d
a
Department of Clinical Physiology, Växjö Central Hospital, Sweden
b
Department of Research and Development, Region Kronoberg, Sweden
c
Clinical Physiology, Skåne University Hospital, Department of Clinical Sciences, Lund University, Sweden
d
Arrythmia Section, Department of Cardiology, Skåne University Hospital, Sweden

a r t i c l e i n f o a b s t r a c t

Keywords: Pheochromocytoma is a rare catecholamine-secreting tumor in the adrenal medulla. In some cases, the first
Pheochromocytoma symptoms are cardiovascular. We report on two patients with pheochromocytoma, who both presented with bi-
Bidirectional ventricular tachycardia directional ventricular tachycardia (BDVT). We elaborate on the mechanisms of BDVT in the setting of
ECG
pheochromocytoma.
© 2019 Elsevier Inc. All rights reserved.

Introduction Case presentation #2

Pheochromocytoma is a rare catecholamine-secreting tumor in the
adrenal medulla. Patients present with headache, palpitations, diapho-
resis and hypertension [1]. In rare cases, the first symptoms are due to
severe cardiovascular complications [2–4].
Case presentation #1
A 44-year old woman was admitted to the cardiac ward with palpi-
tations and chest pain. She had experienced such symptoms since a few
years, but they had always terminated within 10 min. She was pale, di-
aphoretic but normotensive. ECG showed ST depression, and troponins
were elevated. She was started on standard acute coronary syndrome
treatment and received metoprolol intravenously due to increasing
ventricular arrhythmia on telemetry. Besides premature ventricular
extra-systoles, episodes of ventricular tachycardia (VT) with changing
frontal plane axis (bi-directional VT (BDVT), Fig. 1) were observed. Cor-
onary angiography was normal. The patient developed tachypnea and
desaturation. Blood pressure was now highly elevated, N200 mm Hg.
A computed tomography (CT) showed a surprising finding of a large
tumor at the site of the right adrenal gland. Plasma catecholamine levels
were highly elevated.
A diagnosis of pheochromocytoma was made and in addition to the
β-blocker she was put on an α-blocking agent. She stabilized and
4 weeks later underwent right adrenectomy, with complete symptom
relief. Histopathological examination confirmed the diagnosis.
⁎ Corresponding author at: Department of Clinical Physiology, Växjö Central Hospital,
351 88 Växjö, Sweden.
E-mail address: Thomas.a.lindow@gmail.com (T. Lindow).
Four years later, a 61-year old woman was admitted to the emer-
gency department. She had experienced intermittent palpitations and
had been referred for Holter monitoring, which had revealed short epi-
sodes of VT during walking. She had been started on metoprolol. A myo-
cardial perfusion imaging test was normal. 12-lead Holter monitoring
showed short episodes of BDVT (Fig. 2). Two weeks after being put on
metoprolol, she turned up at the ED with palpitations, vomiting and di-
aphoresis and was admitted to the cardiac ward. Based on the symp-
toms and the finding of BDVT, and with the previous patient in mind,
a CT scan was ordered – this time actively looking for an adrenal gland
tumor (Fig. 3). High catecholamine levels confirmed the diagnosis of
pheochromocytoma.
Bidirectional VT in pheochromocytoma
Pheochromocytoma is a rare, catecholamine-producing tumor aris-
ing from chromaffin cell lines in the adrenal medulla or paraganglia.
Typically, patients present with symptoms associated with increased
levels of catecholamines, such as hypertension, tachycardia, pallor or
headache [1]. In some cases, patients present with severe cardiovascular
complications such as pulmonary edema, malignant arrhythmia or
myocardial infarction [2–4]. The diagnosis is made by a combination
of biochemical markers and imaging evidence of the tumor. Patients
are treated with surgical excision of the tumor. Before surgery, pharma-
cological treatment to control heart rate and blood pressure is essential,
often by combining β- and α-blocking agents [5]. This combination is
important, since sole β-blocking therapy may result in symptom pro-
gression, as in both our patients.
BDVT is a rare arrhythmia most often seen in patients with catechol-
aminergic polymorphic VT (CPVT), elicited by physical exercise, or in

https://doi.org/10.1016/j.jelectrocard.2019.09.023
0022-0736/© 2019 Elsevier Inc. All rights reserved.

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8 T. Lindow et al. / Journal of Electrocardiology 58 (2020) 7–9

Fig. 1. Print-out from telemetry which shows ventricular tachycardia at 180 bpm with changing frontal plane axis, i.e. BDVT.

digitalis toxicity [6,7]. In digitalis toxicity, increased sodium/Ca2+ ex-
change causes Ca2+ overload [8]. In CPVT, a genetic defect in the calcium
channels of the sarcoplasmatic reticulum causes increased diastolic
Ca2+ leakage in response to adrenergic stimulation [6]. In parallel to
the increased sensitivity to adrenergic stimulation in CPVT, the
increased levels of catecholamines in pheochromocytoma leads to over-
stimulation of β-adrenergic receptors in the myocytes, which increases
intracellular Ca2+ concentration. Ca2+ overload may lead to delayed
after-depolarizations (DAD) [9]. The BDVT pattern occurs when a
DAD-triggered beat in one part of the distal conduction system elicits
another, which reciprocally activates the first site. In turn, the first site

Fig. 2. Print-out of the limb leads from the 12-lead Holter monitoring showing a short run
of BDVT. Fig. 3. A CT scan of patient #2 showed a right adrenal gland tumor.

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 T. Lindow et al. / Journal of Electrocardiology 58 (2020) 7–9 9

crisis. Bidirectional ventricular tachycardia can be an important clue in

the diagnosis of pheochromocytoma.

Fig. 4. Intracellular calcium overload results in delayed after-depolarizations which
triggers an action potential and starts the arrhythmia. In this example, a premature beat
in the left anterior fascicle elicits a beat in the posterior fascicle, which in turn elicits a
beat in the anterior fascicle. This “ping-pong” in the conduction system explains the
changing QRS axis in the frontal plane [10].
activates the second site again (“conduction system ping pong”) (Fig. 4)
[10].
Conclusion
Pheochromocytoma with cardiac manifestation is a life-threatening
condition. β-blockade without α-blockade can elicit a hypertensive
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Descargado para Jairo Yepes (pitalitounal@yahoo.com) en Pontifical Xavierian University de ClinicalKey.es por Elsevier en mayo 12, 2020.
Para uso personal exclusivamente. No se permiten otros usos sin autorización. Copyright ©2020. Elsevier Inc. Todos los derechos reservados.