CASE PRESENTATION
A previously healthy 38-year-old man was referred to our emergency
department with a diagnosis of a cardiac mass according to 2D
echocardiography. The patient complained of intermittent fever, loss of
appetite, and unintentional weight loss (≈ 8 kg) within the preceding 3
months. His history was negative for similar prior episodes and symptoms
related to the gastrointestinal tract or the pulmonary and urinary
systems. Additionally, he had no complaints of headache and other
neurologic symptoms, myalgia, arthralgia, arthritis, or even
mucocutaneous manifestations. Still, he had a history of recurrent
painful oral aphthosis without a history of genital ulcers. The physical
examination revealed a low-grade fever (38.5°C oral), a blood pressure
of 120/80 mm Hg, and normal sinus rhythm. The cardiopulmonary
examination was unremarkable. The lower limbs were symmetric in size and
pulse without edema. The rheumatologic and neurologic examinations
showed no positive findings.
Blood culture and complete workup were done for the evaluation of
malignancy and hypercoagulable state. Despite the remote possibility of
endocarditis, empirical antibiotics were administered. The complete
blood count showed thrombocytopenia (platelet count =104 /L) and anemia
(hemoglobin = 11.3 g/dL) without leukocytosis. The activated partial
thromboplastin time was within the normal limit. The results of
comprehensive thrombophilia and rheumatologic workup revealed only a
mild elevation in the homocysteine level with weakly positive
anti-β2glycoprotein (anti-β2GPI). The pathergy test to evaluate Behçet’s
disease was also negative.
Given the patient’s hypercoagulopathy state in the current COVID-19
pandemic, a polymerase chain reaction test was performed, which was
negative. The most important lab data are summarized in Table 1.
Parallel to the laboratory evaluation, an imaging study was conducted.
Comprehensive 2D transthoracic echocardiography, followed by
transesophageal echocardiography, visualized multiple intracardiac
masses (Table 2): a large lobulated intracavity mass (maximum size = 6 ×
3 cm) in the right ventricle (RV) with extension to RV apex, a large
homogenous fixed right atrial (RA) mass (4 × 3 cm) in the superior part
of RA roof, and a small round left ventricular (LV) apical mass (6 × 9
mm). In addition, a plethoric inferior vena cava (IVC) with a smoky
pattern and a large lobulated thrombus (≈ 6 cm) with extension to RA-IVC
junction was noticeable (Fig. 1). In the first step, deep venous
thrombosis and pulmonary embolism were ruled out. Multi-slice axial
chest computed tomography (CT) was normal, except for the presence of
masses in the chambers, which was compatible with the echocardiographic
findings. Comprehensive abdominopelvic sonography revealed normal
findings, with the exception of moderate ascites.
The initial clinical and paraclinical assessments ruled out infectious
endocarditis and secondary tumors. Further, given the rarity of multiple
myxomas and the patient’s insignificant response to parenteral
anticoagulation, the most probable diagnosis was inflammatory
thrombosis, APS, or Behçet’s disease. Notwithstanding that our findings
could not fulfill the diagnostic criteria of any one of them,
interestingly, ICT, especially right heart involvement, could be the
first presentation of systemic inflammatory diseases, and in particular
Behçet’s disease.
For the establishment of the diagnosis, cardiac magnetic resonance
imaging was performed using a 1.5T Siemens Avanto machine (Fig. 2). Cine
sequences were obtained using balanced steady-state free precession
(bSSFP), perfusion sequences, and delayed enhancement (turbo field echo
with inversion pulse and recovery, 10 minutes after the injection of 0.2
mmol/kg of the gadolinium contrast medium). Filling defects were
detected in the apex of RV (3 × 2.1 cm), LV (0.6 × 0.56 cm), and RA (4 ×
3 cm). Large elongated filling defects were also depicted in the hepatic
portion of IVC that extended toward RA. Additionally, RV apex was
obliterated by the intracardiac mass. Based on different sequences and
tissue characterizations, the findings of the cardiac magnetic resonance
imaging were compatible with intracardiac thrombosis. Moreover,
subendocardial late gadolinium enhancement was observed in the
biventricular apical portions in the affected segments, suggestive of
endomyocardial fibrosis.
With a diagnosis of APS and under the supervision of a rheumatologist,
the patient received anti-inflammatory treatment with corticosteroid
pulse therapy at a dose of 500 mg for 3 days, intravenous
cyclophosphamide monthly, and ASA 80 mg daily.
The patient’s clinical status was improved, and his fever was resolved.
Transthoracic echocardiography demonstrated a relative reduction in the
size of the RA/RV mass, with the complete elimination of the LV apical
thrombus. In addition, during the course of treatment, abdominopelvic CT
scanning illustrated abdominal arterial/venous thrombosis with pelvic
extension. The late occurrence of these common features of APS was a
strong confirmation of the diagnosis of this syndrome. The extensive
thrombosis necessitated high-dose medication. Follow-up echocardiography
showed a significant reduction in the size of the RV/RA mass, with a
remarkable improvement in LV/RV function. Additionally, the IVC mass was
also eliminated.
Up to the time of writing this paper, the close monitoring of the
patient revealed no new findings.