Rheumatol Int (2008) 28:1269–1271
DOI 10.1007/s00296-008-0601-0
C A S E RE P O RT
Right-heart failure after right heart catheterization in a patient
with scleroderma and suspected pulmonary hypertension
Gernot Keyßer · Carola Schwerdt · Christiane Taege
Received: 17 January 2008 / Accepted: 4 May 2008 / Published online: 24 May 2008
© Springer-Verlag 2008
Abstract The case presented here describes the fatal out-
come of a right heart catheterization of a female patient
with scleroderma. At autopsy, a massive Wbrosis of the car-
dial muscle, the atrioventricular, and the sinoatrial node
was described. Patients with scleroderma are prone to car-
diac involvement and have an increased risk of sudden car-
diac death. The discussion of this case reXects on
identiWable risk factors for cardiac complications in sclero-
derma. These are the parallel aVection of the skeletal mus-
culature, the presence of ventricular ectopies and a dilated
right atrium and pericardial eVusions. Physicians should be
aware of the fact that patients with advanced cardiac Wbro-
sis may be at higher risk of complications in relation with
invasive procedures.
Keywords Scleroderma · Pulmonary hypertension ·
Right heart catheterization · Myocardial Wbrosis
Introduction
A 64 years old female patient was admitted to the hospital
due to progressive dyspnea. 7 years earlier she was diag-
nosed with scleroderma and tested positive for anti-Scl70
antibodies. Later, she developed esophageal dysfunction
G. Keyßer (&) · C. Schwerdt · C. Taege
Universitätsklinikum Halle,
Klinik und Poliklinik für Innere Medizin I,
Ernst-Grube-Str. 40, 06097 Halle/S, Germany
e-mail: gernot.keyszer@medizin.uni-halle.de
and a mild basal pulmonary Wbrosis. A slight renal insuY-
ciency led to secondary arterial hypertension. Due to myal-
gias and weakness of proximal muscles she was treated
with low-dose steroids.
On admission, she presented with extensive skin thick-
ening of the trunk and the extremities. Her Wngers were
completely stiVened and showed pitting scars.
The ECG revealed a left anterior fascicular block. The
QT time corrected for the heart rate was normal (423 ms).
A Holter-ECG indicated two brief episodes of suspected
atrial Wbrillation and an increased frequency of supraven-
tricular extrasystoles. An echocardiogram revealed concen-
tric hypertrophy of the left ventricle and a calciWed ring of
the mitral valve, with only marginal mitral insuYciency.
Right ventricle and atrium were hypertrophied and dilated.
There was also a minor pericardial eVusion. The estimated
pressure of the pulmonal artery was 50 mmHg, suggesting
pulmonal arterial hypertension (PAH).
A right heart catheterization (RHC) was performed to
verify the PAH. At catheterization, systolic, and diastolic
pulmonal pressure values were 42 and 18 mmHg, respec-
tively. Pulmonal artery wedge pressure and cardiac output
were normal. When the catheter was withdrawn, the patient
developed a narrow complex tachycardia and a sudden
decrease in blood pressure. Ventricular Wbrillation devel-
oped that initially responded to deWbrillation. Despite
immediate cardiopulmonal resuscitation, the patient devel-
oped wide complex tachycardia and succumbed to elec-
tromechanic dissociation.
At autopsy, no sign of catheter induced iatrogenic dam-
age was found. Instead, the myocardium showed intense
collagen deposition (Fig. 1). The atrioventricular and the
sinoatrial node demonstrated reticular Wbrosis and the oblit-
eration of small arterial vessels (Fig. 2). The walls of pul-
monary arteries were thickened, consistent with PAH.
123
1270
Fig. 1 Elastica-von-Gieson stain of the myocardium of the interven-
tricular septum with intense collagen deposition between myocytes
(£5)
Discussion
Pulmonary hypertension (PAH), deWned as a mean pulmo-
nary artery pressure >25 mmHg at rest or >30 mmHg dur-
ing exercise [1] can be found in 8–18% of scleroderma
patients, with a 3 year survival rate of 56% [1, 2]. Current
diagnostic strategies recommend echocardiography in case
of unexplained dyspnea, followed by RHC, if PAH is sus-
pected sonographically [1]. The fact that our patient under-
went the same diagnostic procedures raises two questions.
Are there identiWable risk factors for cardiac complications
in scleroderma?
The symptomatic aVection of the heart concerns 15% of
scleroderma patients and has a prominent impact on the
mortality [3]. Myocardial Wbrosis is seen on delayed
enhanced MRI in up to 66% of patients [4]. In addition,
Wbrosis of the myocardium and the conduction system,
pericarditis, contraction band necrosis with congestive car-
diomyopathy, coronary artery lesions and, rarely, valvular
disease are observed [5]. The function of the right heart is
often impaired. Of interest, neither right ventricular func-
tion nor the presence of myocardial Wbrosis correlate well
with the occurrence of pulmonary hypertension [4, 6].
Scleroderma patients with skeletal myopathy have myocar-
dial disease more often, with a higher mortality rate due to
cardiac causes [7]. Our patient had clinical signs of myopa-
thy, although CK values were normal.
SigniWcantly, the Wbrotic changes of the heart are associ-
ated with conduction disturbances and arrhythmias [4]. Left
anterior fascicular block, as seen in our patient, is a com-
mon Wnding [8]. Holter monitoring detects supraventricular
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Rheumatol Int (2008) 28:1269–1271
Fig. 2 Elastica-von-Gieson stain of the sinoatrial node with reticular
Wbrosis (red staining) and the obliteration of an arterial branch due to
thickening of the media (£20)
tachycardias in 32% of scleroderma patients [8], similar to
the results of our case. However, the prognostic value of
this Wnding is unknown. Almost 10% of scleroderma
patients with cardiac involvement die suddenly [7]. The
occurrence of ventricular ectopies, which were not seen in
our patient, may predict sudden death [9].
Of interest, echocardiography may provide several
parameters of proven prognostic signiWcance, such as the
presence of pericardial eVusions and an enlarged right
atrium [10]. Both conditions were present in our patient,
underlining her unfavorable prognosis in general.
Nevertheless, the cardiac arrest during RHC was not to
be expected. RHC is usually considered a safe procedure.
Of interest, there are no prospective studies investigating
the complication rate of a diagnostic RHC. However, con-
tinuous monitoring of critically ill patients by pulmonary
artery catheter (PAC) is a procedure comparable to RHC.
There are Wve larger studies exploring a possible excess
mortality due to PAC, with contradictory results. Whereas
one large prospective cohort study stated a signiWcant
increase in mortality in PAC -monitored patients [11], four
randomized studies found neither beneWt nor harm as to this
method [12, 13]. SigniWcantly, non-fatal cardiac arrythmias
due to PAC were reported in up to 17.9% of patients with
PAC [12]. Whether the latter Wndings implicates a higher
risk of PAC or RHC on scleroderma patients in general is a
matter of speculation. Data regarding the safety of both
procedures in scleroderma are lacking.
Could a RHC possibly be replaced by other diagnostic
procedures?
The RHC is the gold standard for the diagnosis of PAH. It
also identiWes patients with good prognosis by means of the
Rheumatol Int (2008) 28:1269–1271
vasoreactivity test [10]. PAH can be detected by echocardi-
ography as well. This depends, however, on the presence of
tricuspid regurgitation. Studies comparing the invasive with
the non-invasive procedure are inconclusive: Echocardiog-
raphy may detect PAH with a sensitivity between 85 and
100%, a speciWcity between 55 and 100% and a positive
predictive value ranging from 52 to 80%, depending on the
study and on the reWnement of the investigation technique
[14, 15]. In a large cohort of scleroderma patients, PAH was
suspected echocardiographically in 33 patients, but RHC
detected PAH in only 18 of these, whereas three had left
ventricular dysfunction and 12 had no PAH [1]. By raising
the cutoV value of the echocardiographic tricuspid gradient
to 40 mmHg, moderate to severe PAH was conWrmed in all
patients who underwent RHC. However, a high echocardio-
graphic threshold may overlook milder forms of PAH [16].
In our patient, the PAH estimated by echocardiography was
50 mmHg. Therefore, the probability to detect PAH by
RHC was high. This raises the question whether a RHC
could be omitted under these circumstances.
The advent of PAH and interstitial lung disease (ILD) is
accompanied by a reduction of the diVusion capacity
(DLCO). However, a robust correlation between DLCO
and PAH was only seen in patients without ILD, since the
latter inXuences DLCO independently of PAH [16]. Our
Patient had both PAH and ILD, making the DLCO an
unsuitable instrument to predict the degree of PAH.
The lesson:
1. Patients with scleroderma are prone to cardiac involve-
ment and have an increased risk of sudden cardiac
death
2. In scleroderma patients with cardiac involvement, the
parallel aVection of the skeletal musculature, the pres-
ence of ventricular ectopies and a dilated right atrium
and pericardial eVusions are signs of poor prognosis
3. Patients with systemic sclerosis should be screened
regularly for PAH. However, physicians should be
aware of the fact that patients with advanced cardiac
Wbrosis may be at higher risk of complications in rela-
tion with invasive procedures
4. Prospective studies to investigate the risks and the
beneWts of RHC in scleroderma patients are desirable.
Further studies should investigate whether a RHC can
be omitted in patients with high echocardiographic
tricuspid gradients to minimize procedure related risks.
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