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Cardiology

Urgent Need for Diagnosis of a Rapidly Growing Right Atrial Mass

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.

1 Credit CME

JAMA Cardiology

Published Online: December 30, 2020

JAMA Cardiology Clinical Challenge

Article Information and Disclosures

Sneha Thatipelli, MD¹;

Sasan Raissi, MD²;

Nausheen Akhter, MD³

Author Affiliations

¹Department of Medicine, Northwestern Memorial Hospital, Chicago, Illinois
²Bluhm Cardiovascular Institute, Northwestern Memorial Hospital, Chicago, Illinois
³Feinberg School of Medicine, Division of Cardiology, Department of Medicine, Northwestern University, Chicago

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A middle-aged man with no relevant medical history presented to the emergency department with progressive shortness of breath, a productive cough, fatigue, weight loss, and night sweats for several months. A few days prior to presentation, he had developed palpitations, chest discomfort, and lower-extremity swelling. He denied taking any medications or recent international travel. In the emergency department, he had a normal blood pressure with an irregular heart rate at 104 beats per minute. On examination, he had bilateral basilar crackles and traces of lower extremity edema. An electrocardiogram revealed that he was experiencing atrial flutter. A computed tomographic angiogram showed an acute pulmonary embolism, multiple bilateral pulmonary nodules, mediastinal lymphadenopathy, and a right atrial mass. He then underwent a transthoracic echocardiogram with 2-dimensional speckle-tracking strain, which revealed a 4.5 × 3.6 × 3.1-cm pedunculated right atrial mass attached to the interatrial septum near the fossa ovalis (Figure 1A; Video). Left ventricular function was noted to be reduced and cardiac mechanics by 2-dimensional speckle-tracking strain were markedly abnormal, suggesting a more diffuse myocardial process (Figure 1B).

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Primary cardiac synovial sarcoma

A. Cardiac magnetic resonance imaging

A cardiac magnetic resonance image (cMRI) was the next imaging modality chosen, because it provided more information regarding the new left ventricular dysfunction and markedly abnormal strain. A cMRI provides optimal multiplanar images for tissue and blood flow characterization without radiation. Since a malignant condition is in the differential diagnosis in a patient with a cardiac mass, constitutional symptoms, pulmonary nodules, and mediastinal lymphadenopathy, T1-weighted and T2-weighted images were better able to distinguish different tumor characteristics than a positron emission tomography–computed tomography (PET-CT) scan (choice B).¹^,2 Furthermore, a transesophageal echocardiogram (choice D) would not provide further tissue characterization of the right atrial mass. For example, more vascularized masses are enhanced on T1-weighted imaging, and areas of hemorrhage, edema, and necrosis are enhanced on T2-weighted imaging. Additionally, the use of first-pass perfusion imaging with early and late gadolinium enhancement can identify infiltration, thrombus, and hemorrhage.¹^- 4 This combination of findings can create a virtual fingerprint to differentiate between cardiac masses, such as fibromas, myxomas, and sarcomas, and assist in biopsy guidance. For these reasons, a cMRI was pursued prior to a PET-CT scan.

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Article Information

Corresponding Author: Sneha Thatipelli, MD, Department of Medicine, Northwestern Memorial Hospital, 251 E Huron St, Chicago, IL 60611 (sneha.thatipelli@northwestern.edu).

Published Online: December 30, 2020. doi:10.1001/jamacardio.2020.6107

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient’s representative for granting permission to publish this information.

References

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Wang JG , Li NN . Primary cardiac synovial sarcoma. Ann Thorac Surg. 2013;95(6):2202-2209. doi:10.1016/j.athoracsur.2013.01.030 PubMed Google Scholar Crossref

Varma T , Adegboyega P . Primary cardiac synovial sarcoma. Arch Pathol Lab Med. 2012;136(4):454-458. doi:10.5858/arpa.2011-0008-RS PubMed Google Scholar Crossref

Cheng Y , Sheng W , Zhou X , Wang J . Pericardial synovial sarcoma, a potential for misdiagnosis: clinicopathologic and molecular cytogenetic analysis of three cases with literature review. Am J Clin Pathol. 2012;137(1):142-149. doi:10.1309/AJCP34ZVFLAUTMGL PubMed Google Scholar Crossref

AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
1.00 CME points in the American Board of Surgery’s (ABS) Continuing Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.