Cardiovascular Journal of Africa: Vol 32 No 3 (MAY/JUNE 2021)

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CARDIOVASCULAR JOURNAL OF AFRICA • Volume 32, No 3, May/June 2021 AFRICA 171
Basal Takotsubo syndrome induced by
pheochromocytoma rupture
Shanshan Yuan, Tao He, Lijia Yang, Qiang Chu, Weiqing Huang, Hongyan Dai
Abstract
Takotsubo syndrome (TTS), characterised by transient left
ventricular systolic dysfunction, is divided into five types: (1)
apical ballooning, (2) mid-ventricular, (3) basal or inverted,
(4) and focal wall-motion patterns, and (5) other types,
including biventricular type, isolated right ventricular and
global type. The common clinical features of TTS are similar
to acute coronary syndrome, which makes them indistinguish-
able in the early stages. TTS has a wide spectrum of emotional
or physical triggers. Pheochromocytoma has been widely
recognised as a distinct physical trigger of TTS. Alhough
reports of pheochromocytoma causing TTS are not uncom-
mon, spontaneous rupture of pheochromocytoma causing
TTS is extremely rare because of the low incidence of tumour
rupture. Here we report on a case of a 31-year-old man with
adrenal pheochromocytoma rupture developing basal TTS.
Keywords: Takotsubo syndrome, pheochromocytoma, spontane-
ous rupture, cardiogenic shock, heart failure
Submitted 16/2/20, accepted 30/8/20
Published online 5/10/20
Cardiovasc J Afr 2020; 31: 171–174
DOI: 10.5830/CVJA-2020-039
Since its first description in Japan in 1990, Takotsubo
syndrome (TTS), also known as broken-heart syndrome,
stress cardiomyopathy or apical ballooning syndrome, has
emerged as an important form of acute reversible myocardial
injury characterised by transient left ventricular (LV) systolic
dysfunction.
The common clinical features of TTS are cardiac symptoms
(acute chest pain, dyspnoea or syncope), new ECG abnormalities
(ST-segment elevation, ST-segment depression, T-wave inversion
and QTc prolongation) and elevated levels of cardiac biomarkers
(troponin and creatine kinase), which make it indistinguishable
from acute coronary syndrome (ACS) in the early stages.
Recently, it has been shown that TTS has a comparable
in-hospital mortality rate with ST-segment elevation myocardial
infarction (STEMI) and non-STEMI.
Based on the distribution of regional wall-motion
abnormalities, five types of TTS can be differentiated: (1) apical
ballooning (81.7%), (2) mid-ventricular (14.6%), (3) basal or
inverted type (2.2%), (4) and focal wall-motion patterns (1.5%),
and (5) other types, including biventricular type (apical type and
right ventricular involvement), isolated right ventricular and
global type.1,2
TTS has a wide spectrum of emotional or physical triggers,
the former including grief, interpersonal conflict, fear and panic,
anger, anxiety, financial or employment problems and even some
happy emotions. The physical triggers include acute respiratory
failure, pancreatitis, cholecystitis, traumatic injury, sepsis,
malignancy, thyrotoxicosis, and nervous system diseases such as
stroke, head trauma, migraine, intracerebral haemorrhage and
seizure.1,2
Pheochromocytoma, a catecholamine-secreting tumour, has
been widely recognised as a distinct physical trigger of TTS. The
prevalence of TTS in patients with pheochromocytoma may be
up to 3%.3 Although reports of pheochromocytoma causing TTS
are not uncommon, spontaneous rupture of pheochromocytoma
causing TTS is extremely rare because of the low incidence of
tumour rupture.4 Here we report on a case of a 31-year-old man
with adrenal pheochromocytoma rupture developing basal TTS
and pheochromocytoma crisis.
Case report
A 31-year-old man was admitted to hospital due to sudden
severe abdominal pain. Prior to this event, he had experienced
intermittent abdominal pain, headache and palpitations for two
years without any treatment. On arrival at a local hospital, his
blood pressure (BP) was 206/115 mmHg and heart rate was 120
bpm. Plain computed tomography (CT) showed a 3.7-cm mass
of uneven density in the left adrenal gland (Fig. 1A). An ECG
showed ST-segment elevation on the precordial leads V3–V6 and
limb leads I and aVL, and ST-segment depression on limb leads
II, III and aVF. Troponin I (TnI) and creatine kinase isoenzyme
MB (CK-MB) levels were normal at that time.
In less than 12 hours, his abdominal pain progressively
intensified, and BP dropped to 90/60 mmHg. He was transferred
to our hospital. The repeat CT scan revealed the mass had
expanded to 6.5 cm in diameter, with accumulation of fluid
surrounding the left pararenal and parapancreatic space (Fig.
1B).
Haemoglobin and red blood cell counts were in the normal
range. Levels of serum and urine epinephrine (E), serum
and urine norepinephrine (NE), serum metanephrine (MN)
and normetanephrine (NMN), and urine vanillylmandelic acid
(VMN) were found to be markedly elevated (serum E: 767.66
pg/ml, reference range 0–100 pg/ml; serum NE: 2148.52 pg/ml,
Department of Cardiology, Qingdao Municipal Hospital,
Qingdao, Shandong, China
Shanshan Yuan, MD
Tao He
Lijia Yang
Qiang Chu
Hongyan Dai, PhD,
Department of Pathology, Qingdao Municipal Hospital,
Qingdao, Shandong, China
Weiqing Huang