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.