European Journal of Echocardiography (2008) 9, 426–427doi:10.1093/ejechocard/jen022
Cardiac complications in Whipple’s disease
Department of Cardiology, Klinik Koesching, University Heidelberg, Krankenhausstr. 19, D-85092 Koesching, Germany
Received 2 November 2007; accepted after revision 23 December 2007; online publish-ahead-of-print 30 March 2008
Whipple’s disease or intestinal lipodystrophy is an infection induced by Tropheryma whipplei. It is rare
with an estimated incidence of 0.4 per million. Symptoms are arthropathy, weight loss, and diarrhoea,
but other organs notably the central nervous system may be affected. We demonstrate a case of cardiac
complications in Whipple’s disease. The patient presented with endocardial inﬁltrations on TEE exam-inations and heart failure and improved after antibiotic treatment.
Although the ultimate proof a biopsy was not feasible, an
echocardiographic follow-up after 6 months of antibiotic
A 54-year-old woman presented with weight loss of about
treatment demonstrated a normalization of left ventricular
20 kg and intermittent fever. She was diagnosed for derma-
function. Therefore, the reaction on antibiotic therapy sup-
tomyositis 10 years ago and treated with steroids. Since
ports our view and suggests a cardiac complication of Whip-
arthropathy worsened, the therapy was escalated with
MTX, Azathioprin, and Chloroquin without clinical improve-ments of her symptoms.
Transthoracic echocardiography demonstrated myocardial
hypertrophy in the absence of arterial hypertention,impaired systolic function, and sclerosis of the aortic valve
Whipple’s disease is rare, and no valid estimate of the inci-
and mitral anulus (see Supplementary material online,
dence is available. Whipple’s disease occurs mainly in
Clip 1). Systolic and diastolic myocardial velocities by tissue-
middle-aged individuals (mean age at diagnosis about 50
Doppler imaging were dramatically reduced (Figure 1).
years) and in about eight times more men than women. A
Transesophageal echocardiography (TEE) examinations, per-
genetic susceptibility is suggested by the ﬁnding that
formed to exclude endocarditis, revealed endocardial and
26% of patients (three to four times more than expected)
intramyocardial echogenic ‘inﬁltrations’, especially in the
free wall of the right atrium (see Supplementary material
Macrophages from infected patients show decreased
online, Clip 2, Figure 2). Small-bowel biopsy characterized
intracellular degradation and a decrease in phagocytosis.
Thus, a subtle defect of cellular immunity seems to
periodic-acid Schiff (PAS), which represent the causative
involve activation and interaction of macrophages and T
bacteria of Whipple’s disease. The patient refused further
cells and allow invasion of the bacillus from the gastrointes-
cardiac investigations, including heart catheterization or
myocardial biopsy to conﬁrm a myocardial infection with
Whipple’s disease has traditionally been regarded as a
gastrointestinal disease with weight loss, abdominal pain,
Prospective studies on the choice or duration of antibiotic
and diarrhoea, but in many cases, the disease begins insi-
treatment are ongoing. Our patient was treated with a
diously with arthropathy. Clinical presentation can vary to
2-week course of intravenous meropenem followed by oral
a great extent owing to differential organ involvement,
and no major organ is excluded from infections by
orally twice daily) given for 1 year. In addition, the patient
T. whipplei. A central nervous system manifestation can
received standard therapy for heart failure including beta-
ﬁrst become apparent as a memory disorder, personality
Cardiac involvement is common and has been reported to
be an important clinical sign. It might present as cardiacmurmurs, insufﬁciency of the aortic or mitral valve necessi-
E-mail address: [email protected]
tating replacement, or with the clinical picture of
Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2008.
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Cardiac complications in Whipple’s disease
Differential diagnosis of these intracardiac abnormalitieswould include congestive heart failure, ischaemic cardio-myopathy, hypertrophy due to hypertension or aortic steno-sis, viral myocarditis, nutritive or toxic cardiomyopathy, orFabry disease. However, the reaction on antibiotic therapysupports our view of a cardiac complication of Whipple’sdisease in this case.
When the disease is suspected, duodenal-biopsy speci-
mens should be obtained. Histological analysis reveals gran-ular foamy macrophages stained purple with PAS. Duodenalsamples are inﬁltrated by macrophages.
Untreated Whipple’s disease can be fatal. However, anti-
biotic therapy leads to rapid improvement in clinical status.
In the past, various antibiotic regimens were used up to 12months on an empirical basis, but no prospective studies
Tissue-Doppler imaging of lateral annulus demonstrate
are available on the choice or duration of antibiotic treat-
signiﬁcantly reduced systolic (s ¼ 8 cm/s) and diastolic velocities
ment. If the patients have a good clinical response, they
(e ¼ 4 cm/s) indicating severe systolic and diastolic heart failure.
can simply be followed up with duodenal biopsies 6 and 12months after diagnosis. Antibiotic treatment can generallythen be stopped if no PAS-positive material is identiﬁed.
Supplementary material associated with this article can befound in the online version.
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