Abstract
Introduction
Intracardiac masses are always a challenging diagnosis, especially when it involves the right side of the heart. There are multiples etiologies that can be responsible for these masses, namely thrombosis, neoplasm or vegetations. Occasionally, these may be related to an autoimmune process not yet discovered.
Case Report
17-year-old male, with a previous history of genital ulcers, medicated with penicillin with complete resolution of symptoms.
In January 2019, he started an history of recurrent fever, associated with right anterior thoracalgia, weight loss and oral afthosis. He went to the emergency department several times, where he was medicated with antibiotic, with partial symptom relief.
Three months later, he returned to medical attention due to an episode of abundant hemoptysis, followed by hematemesis and cough. At hospital admission, he was hemodynamically stable, tachycardic (100/min) and with occasional episodes of cough. Cardiac and pulmonary auscultation were unremarkable. Thoracic CT revealed the presence of pulmonary thromboembolism (PTE) and a large mass in the right ventricle (RV). It was performed an echocardiogram (echo) that confirmed the presence of a large mass in the RV (50x53mm) from which a projecting hypermobile mass appeared to prolapse into the right atrium.
Taking into account the diagnosis of PTE and the presence of a right ventricular mass, the patient was hospitalized and started anticoagulation. The case was immediately discussed with cardiac surgery, that confirmed that there was no surgical indication. During hospitalization, there were no more episodes of hemoptysis or hematemesis.Consecutive echos were performed, that did not reveal a significant decrease in mass dimensions despite anticoagulation. Viral serologies and autoimmunity panel were all negative. Cardiac RMI was performed raising the suspicion of a possible mass covered with thrombus.
After discussion with rheumatology, and according to clinical signs, the hypothesis of vasculitis was placed, and the patient started treatment with steroids. This treatment had to be suspended after a few days due to an infectious intercurrence. After a course of antibiotic therapy, the patient started therapy with cyclophosphamide with good clinical and echocardiographic response (reduced mass dimensions).
It was admitted Behçet’s disease with cardiac complications, and the patient was referred to the rheumatology consultation.
Conclusion
Behçet’s disease is a multi-system, chronic disorder that behaves like vasculitis.There are some typical clinical manifestations associated with this disease, such as oral and genital afthosis, uveitis, arthritis, skin lesions and nervous system involvement.Presentations with cardiac symptoms are one of the extremely rare manifestations of this disease, posing a challenge for the treating physician.
Abstract 475 Figure. Right ventricular mass
Novel intronicDICER1variation associated with pleuropulmonary blastoma in two siblings
