European Journal of Echocardiography Advance Access published online on June 13, 2008
European Journal of Echocardiography, doi:10.1093/ejechocard/jen180
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2008. For permissions please email: journals.permissions@oxfordjournals.org
Fatal pheochromocytoma crisis precipitated by dobutamine stress echocardiography
Prabhdeep S. Sethi1,*,
William Hiser1,
Hasan Gaffar2,
Leng Jiang1,
Ashequl Islam1,
Nitin Bhatnagar1 and
Mara Slawsky1
1 Department of Cardiology, Baystate Medical Center, 759 Chestnut Street, Springfield, MA 01199, USA
2 Department of Pathology, Baystate Medical Center, 759 Chestnut Street, Springfield, MA 01199, USA
Received 7 April 2008; accepted after revision 18 May 2008.
* Corresponding author. Tel: +1 4137948722; fax: +1 4137940198. E-mail address: mdheart{at}gmail.com
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Abstract
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The safety of dobutamine stress echocardiography (DSE) has been
demonstrated in multiple studies with a major complication rate
of <1%. Specifically, ventricular tachycardia during DSE
has a reported incidence of 0.3%, and has been bound to be of
no prognostic significance in patients without obstructive coronary
artery disease. We report a unique case of fatal pheochromocytoma
crisis precipitated by DSE in a patient with heretofore unknown
adrenal disease. We are once again reminded that no diagnostic
modality is absolutely without risk; however, minimal they might
be.
Keywords: Dobutamine echocardiography; Stress echocardiography; Stress testing; Pheochromocytoma crisis; Ventricular tachycardia
A 56-year-old woman with a history of asthma, hypertension,
diabetes mellitus, and hypercholesterolaemia was seen in the
Cardiology clinic with neck and left arm discomfort. She had
recently been hospitalized for chest and epigastric pain along
with headache and nausea. She had been diagnosed with a viral
syndrome, although she had a mildly elevated troponin. Her electrocardiogram
in clinic demonstrated normal sinus rhythm with non-specific
ST-T wave abnormalities. Because of her cardiovascular risk
factors and her recent hospitalization, a dobutamine stress
echocardiogram was ordered.
Resting echocardiographic images revealed normal global and segmental left ventricular function. Peak heart rate was 156 bpm with a blood pressure of 210/110 with 20 µg/kg/min of dobutamine and 0.5 mg of atropine. The patient reported severe substernal chest pain, headache, and dyspnoea, and peak echocardiographic images revealed severe global hypokinesis with akinesis of the mid-distal septal and apical wall (see Supplementary material online, Videos 1 and 2). She then went into a wide complex tachycardia which reverted to sinus tachycardia with 100 mg of lidocaine intravenously. Twelve-lead ECG revealed anterior lead ST elevations. Emergent cardiac catheterization was performed and demonstrated no obstructive coronary artery disease. An intra-aortic balloon pump was placed and she was intubated for respiratory failure and admitted to the coronary care unit. She continued to experience labile hypertension alternating with hypotension requiring inotropic support along with continued pulmonary oedema. A repeat echocardiogram revealed continued evidence of global severe hypokinesis. Cardiac surgery was emergently consulted and the patient was taken to the operating theatre for placement of a left ventricular assist device. Despite aggressive support, the patient died 5 h later of respiratory failure and shock. An autopsy was performed.
The heart weighed 320 g and demonstrated mild concentric left ventricular hypertrophy. The coronary arteries were patent with mild focal atherosclerosis. Histological sections demonstrated contraction band necrosis and myocytolysis, consistent with acute myocardial injury secondary to ischaemia and/or catecholamine stimulation (Figure 1). The left adrenal gland weighed 65 g and was surrounded by haemorrhage. Sectioning demonstrated an encapsulated, haemorrhagic tumour mass with a friable consistency, measuring 6.0 x 5.0 x 2.5 cm (Figure 2). The tumour was situated within the adrenal medulla and compressed the surrounding adrenal cortex. Histological sections showed a nested proliferation of polyhedral cells with amphophilic cytoplasm. By immunohistochemistry, the neoplastic cells were strongly positive for neuroendocrine markers synaptophysin (Figure 3) and chromogranin, but did not stain with inhibin. The pathological diagnosis was pheochromocytoma.
Pheochromocytomas are rare tumours of chromaffin cells with
a prevalence in hypertensive patients of 0.1–0.6%.
1 This
case highlights the non-specific nature of clinical presentation
which may delay accurate diagnosis for years. A retrospective
analysis by Baguet found the classic triad of headache, sweating,
and palpitations to be present in <25% of cases. Alteration
in blood pressure occurred in
50% of cases.
2 Our patient developed
the most feared consequence of occult or known pheochromocytoma,
the so-called pheochromocytoma crisis. This is the first report
of a crisis precipitated by dobutamine stress echocardiography
(DSE), a procedure routinely performed for the detection of
coronary artery disease. Multiple studies have demonstrated
the safety of DSE with a major complication rate of <1%.
3–5 In a survey of stress laboratories around the world, Varga
et al.6 report the occurrence of five deaths among 35 103 patients
undergoing dobutamine stress testing. Katritsis
et al.7 have
reported 0.3% incidence of sustained monomorphic ventricular
tachycardia during DSE. They also found that VT had no prognostic
significance in the absence of angiographic coronary artery
disease. In our patient, the escalating dobutamine dose likely
precipitated haemorrhage into the tumour. The subsequent abrupt
release of catecholamines caused direct cardiotoxicity and ultimately
cardiogenic shock. The catecholamine excess of pheochromocytoma
leads to multi-organ toxicity. Cardiac effects are mediated
via massive calcium influx. There is global myocardial depression
due to cell necrosis and there have been several reports of
severe pheochromocytoma-related cardiomyopathy.
8,9 The crisis
can be precipitate by certain contrast media, movements of the
abdominal viscera, and a variety of drugs.
10–12 This tragic
case once again reminds us the importance of investigating a
patient's constellation of symptoms rather than focusing in
on one such as chest pain in this case.
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Supplementary data
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Supplementary data are available at
European Journal of Echocardiography online.
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References
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