Quick Fire Cases
Siddharth Trivedi, BSc, BMedSci, MBBS, FRACP, PhD, FCSANZ
Cardiologist
Macquarie University Hospital, Australia
Siddharth Trivedi, BSc, BMedSci, MBBS, FRACP, PhD, FCSANZ
Cardiologist
Macquarie University Hospital, Australia
Esra Gucuk Ipek, MD
Advanced Cardiovascular Imaging Fellow
Brigham and Women's Hospital, Harvard Medical School
Arthur Shiyovich, MD
Advanced Cardiovascular Imaging Fellow
Brigham and Women's Hospital, Harvard Medical School
Jonathan Andrew Aun, DO, FACC, FACP
Chief Clinical Fellow, Noninvasive Cardiovascular Imaging Program
Brigham and Women's Hospital, Harvard Medical School
Rhanderson N. Cardoso, MD
Cardiovascular Fellow
Brigham and Women's Hospital
Sarah Cuddy, MD
Cardiologist
Brigham and Women's Hospital
Sumit Gupta, MD
Radiologist
Brigham and Women's Hospital
Michael Steigner, MD
Director, Vascular CT and MRI
Brigham and Women's Hospital
Bobak Heydari, MD
Professor, Harvard Medical School
Brigham and Women's Hospital
Ayaz Aghayev, MD
Radiologist
Brigham and Women's Hospital
Ron Blankstein, MD
Professor of Medicine
Brigham and Women's Hospital
Raymond Y. Kwong, MD
Professor of Medicine
Cardiovascular Division, Department of Medicine, Brigham and Women's Hospital, Boston, MA, USA
Cardiac magnetic resonance (CMR) imaging demonstrated a similar size mobile pedunculated mass attached to the atrial surface of the tricuspid valve septal leaflet. There was mild tricuspid regurgitation. There was no uptake of contrast on first pass perfusion. The mass was T2 and T1 intense (Figure 2) and demonstrated diffuse hyperintense late gadolinium enhancement (LGE) (Figure 3) with long inversion recovery time. At shorter inversion recovery time, the mass demonstrated heterogeneous LGE uptake with minimal LGE uptake at the core of the mass. These findings suggested a benign mass with low vascularity, with papillary fibroelastoma being the most likely diagnosis.
Learning Points from this Case:
The other differential diagnoses in this case were Libman-Sacks endocarditis related to SLE or other types of endocarditis of the tricuspid valve, myxoma, or thrombus. Given the highly mobile location on the tricuspid valve and heterogenous LGE, thrombus was considered unlikely. Myxomas are one of the most frequently diagnosed benign primary cardiac tumors, and typically appear isointense on T1-weighted imaging and hyperintense on T2-weighted imaging, depending on the mucinous/fluid component. Furthermore, they demonstrate heterogeneous LGE. However, the location of the mass was very atypical for a myxoma. Libman-Sacks was considered less likely as there was no significant valve dysfunction, the antiphospholipid antibodies were negative, and there was no myocarditis. Papillary fibroelastoma is increasingly considered the most common primary cardiac tumour (1). Papillary fibroelastoma may be a source of systemic embolization or stroke due to the migration of thrombus from the tumour surface or tumour embolization. In this patient, serial CMR imaging at four months demonstrated unchanged appearance of the mass. The patient was seen by the cardiac surgery team who deemed surgical risk was too high. Given the remote history of prior PE, recurrence of PE was thought to be the working diagnosis rather than embolism from suspected papillary fibroelastoma. The patient remains well nine months after presentation and continues serial follow-up. This interesting case highlights the utility of CMR in the assessment of mass lesions in patients with SLE.