key: cord-0835031-i1xzgq7i
authors: Diamond, Jamie E.; Mi, Michael Y.; VanderLaan, Paul A.; Chu, Louis; Gelfand, Eli V.
title: An Unusual Cause of Functional Mitral Stenosis: A Left Atrial Intimal Sarcoma
date: 2021-04-28
journal: JACC Case Rep
DOI: 10.1016/j.jaccas.2021.02.043
sha: 9975325f7871607452712101f8fdfac79b29c01c
doc_id: 835031
cord_uid: i1xzgq7i

Primary cardiac tumors are rare, with an incidence of <0.1% in postmortem series; sarcomas comprise 75% of these. Cardiac sarcomas may be life-threatening at the time of presentation. We describe a left atrial intimal sarcoma presenting with constitutional symptoms, obstructive shock, and systemic emboli, and treated with proton beam therapy. (Level of Difficulty: Intermediate.)

pnea with minimal exertion.

On physical examination, the patient was tachycardic to 104 beats/min, blood pressure was 111/ 73 mm Hg, oxygen saturation was 94% on 2 l/min oxygen by nasal cannula, and temperature was 36.9 C. Cardiac examination was notable for regular tachycardia and no audible murmurs. Diffusely decreased breath sounds and rales in the bilateral lower lung fields were noted. Laboratory studies showed elevated inflammatory markers, including a white blood cell count of 13.7 Â 10 3 /ml with 81%

To highlight the triad of symptoms common to intracardiac cavitary masses including constitutional, embolic, and obstructive phenomena. To describe the rare clinical entity of a cardiac left atrial intimal sarcoma. To review an alternate cause of mitral stenosis physiology. To emphasize novel treatment modalities as emerging therapeutics for intracardiac intimal sarcomas including proton beam therapy and therapies targeting MDM2 cytogenetics. 

Before presentation she had nonalcoholic fatty liver disease, obesity, and osteoarthritis of the knees. She was a former smoker with a 2 pack-year smoking history.

The differential diagnosis for this patient's left atrial mass included primary cardiac tumor (cardiac myxoma, papillary fibroelastoma, or malignant sarcoma), metastatic tumor, organizing thrombus, vegetation, bronchogenic cyst, or hydatid cyst. (8).

The patient had an uncomplicated postoperative recovery. Proton beam therapy was initiated with a plan for 37 fractions totaling 6,600 centiGray. Adjuvant chemotherapy was deferred.

Cardiac intimal sarcoma is a rare, aggressive disease entity with poor prognosis. Presentation varies but may include constitutional, embolic, and obstructive phenomena. Surgical excision with tumor-free margins is the mainstay of treatment but is often not feasible. Emerging treatments including proton therapy may decrease recurrence and improve survival. 

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KEY WORDS acute heart failure, cancer, echocardiography, imaging, intimal sarcoma APPENDIX For supplemental videos, please see the online version of this paper.