DISCUSSION
The cardiovascular system can be affected during systemic inflammatory
and rheumatologic disorders.2, 6 Valvular disease,
conduction disturbances, myocardial and pericardial involvement, and
also vasculitis can be detected. Similarly, intracardiac masses
associated with a systemic inflammatory disease such as Wegener’s
granulomatosis, sarcoidosis, rheumatoid arthritis, and Behçet’s disease
are reported.2, 6 ICT may develop in the presence of a
known systemic disorder or might be the isolated and first clinical
presentation.2 APS syndrome or Hughes’ syndrome is a
rare prothrombotic autoimmune disease characterized by recurrent
vascular thrombosis events (arterial, venous, or even microvascular
circulation), fetal loss, and persistent (at least 12 weeks apart)
elevated levels of antiphospholipid antibodies, including the lupus
anticoagulant (LA), and /or moderate-to-high levels of the isotypes of
immunoglobulin M (IgM) or immunoglobulin G (IgG) anticardiolipin (aCL)
antibodies or the isotypes of IgG or IgM anti-β2GPI, leading to a
hypercoagulable state and related symptoms.(1-4) The diagnosis is
confirmed in the presence of at least 1 clinical and 1 laboratory
criteria. The annual incidence of APS was estimated at around 5 new
cases per 100 000 people.9 This syndrome may be
developed as a primary condition or a secondary disorder related to an
underlying disease, most commonly systemic lupus erythematosus.
Mavrogeni et al5 described cardiac involvement as a
common complication found in 30% of patients following APS. Also, this
syndrome, itself, was considered a risk factor for cardiovascular
disease.10 Cardiac complications are categorized into
2 main subgroups of valvular heart disease (more
common)5, 11 and non-valvular involvement (less
frequent), including ICT, myocardial disease, LV and RV dysfunction, and
pulmonary hypertension.5 Cardiac thrombosis is an
uncommon complication(4-7) and is responsible for
1.9% of all cases of ICT.15 Albeit ICT is a
life-threatening complication, appropriate treatment may decrease its
morbidity and mortality rates. Ye Z-X et al16described a 22-year-old woman with an RA thrombus, deep vein
thrombosis/pulmonary thromboembolism, and positive aCL antibodies.
Voigtlaender et al17 described a 67-year-old woman
with an RA thrombus, thrombocytopenia, and positive LA antibodies. Zahid
et al18 reported a case of an RA thrombus and
pulmonary thromboembolism, as the first presentations of APS, in a
26-year-old man confirmed by positive LA/aCL antibodies.
In 2011, Permanyer et al19 described a 43-year-old
woman with a cardiac mass at the injunction of RA to IVC (dilated IVC),
which was finally was diagnosed as APS syndrome due to strongly positive
LA/aCL antibodies. In a literature review conducted by Waisayarat et
al9 on 9 patients with ICT and APS, most of the
patients were women aged between 30 and 40 years old. The heart chamber
with the most involvement was RA, and LA and LV were less commonly
involved. LA antibodies, followed by aCL antibodies, were positive in
most cases, and anti-β2GPI was positive in only 2 cases.
In another literature review, Dhibar et al20 showed
that fever of unknown origin was a rare presentation in primary APS.
Their analysis on 28 cases presenting with ICT showed a relatively
female predominance with more common right heart involvement, not least
RA, and to a lesser extent, LA or LV involvement. Only 2 cases had a
simultaneous involvement of LV/LA and right heart. Primary APS was more
prevalent, and in most cases LA/aCL antibodies were positive. Positive
anti-β2GPI was reported in only 3 patients, all of whom had an RA mass
and positive LA and/or aCL antibodies. In contrast to these case
reports, our patient, who was a man presenting with extensive ICT and
simultaneous RA/RV/LV/and IVC masses, had anti-β2GPI as the only
positive antibody in the laboratory assessment. Such discrepancies with
prior cases prompted us to report this case as a relatively strange
example. It is probable that in the follow-up of our patient, other
groups of positive antibodies may be detected.