Unveiling a Mass Compressing the Right Side of the Heart: A Diagnostic Challenge. (QF_FR_074)

Friday, January 31, 2025

8:10 AM – 8:20 AM East Coast USA Time

Location: Blue Room PreFunction - Kiosk 1

Presenting Author(s)

SR

Samir Rehman, N/A

Advanced Imaging Fellow
National Institute of Cardiovascular Diseases, Pakistan

Primary Author(s)

SR

Samir Rehman, N/A

Advanced Imaging Fellow
National Institute of Cardiovascular Diseases, Pakistan

Co-Author(s)

WH

Wajid Hussain, N/A

senior registrar
national institute of cardiovascular diseases, Pakistan
SB

Sabha Bhatti, MD

Professor
NICVD

Description of Clinical Presentation: We present a case of a 17-year-old male referred to us from Afghanistan. He had complaints of left sternal pain for 4 months, breathlessness on minimal exertion (New York Heart Association functional class II-III) for 3 weeks and significant weight loss with no other cardiac symptoms. He was investigated in Afghanistan and found to have large pericardial effusion with cardiac tamponade on transthoracic echocardiogram (TTE), which was drained. On arrival to our hospital, he looked anxious and tachypneic due to sternal pain. His physical examination revealed that his blood pressure was 80/50 mmHg, his pulse was 108 beats per min, and his respiratory rate was 34 breaths per minute

Diagnostic Techniques and Their Most Important Findings:

Echocardiography indicated a large pericardial effusion with signs of tamponade, as well as an echogenic mass in the right atrium (RA) and right ventricle (RV). After pericardiocentesis and stabilization, CMR was performed for tissue characterization, which revealed a large (86 mm X 63 mm) multilobular mass arising from the anterior mediastinum next to the aorta, extending down to the pericardium and abutting the RA and part of the RV. Black blood images revealed a large pericardial effusion, more towards the anterior side.

T1 and T2-weighted imaging showed hyperintense signal throughout the mass. First-pass perfusion sequences demonstrated a filling defect in the right atrium while late gadolinium-enhanced (LGE) images showed patchy heterogeneous enhancement throughout the mass. Based on the aforesaid results, the mass was suspected to be an angiosarcoma. He underwent surgery to remove the mass, but unfortunately died during the procedure.

Learning Points from this Case: Primary cardiac tumors have an autopsy incidence less than 0.5% and 75% of them are benign. Cardiac angiosarcoma is the most common primary malignant tumor of the heart. It is an endothelial cell tumor of lymphatic or vascular origin with aggressive progression and high metastatic capability. Primary cardiac angiosarcomas most often affect the right atrium and present with non-specific symptoms which makes the diagnosis of cardiac angiosarcoma challenging. Furthermore, severe manifestations can occur, including right heart failure, myocardial rupture, and cardiac tamponade.The use of noninvasive imaging modalities like cardiac MR for a comprehensive analysis of tumor features can improve the early detection of cardiac neoplasms and may provide a specific classification before histopathological analysis. Surgical resection is the mainstay of treatment where possible. However, upfront chemoradiotherapy may be an alternative treatment option for unresectable tumors.