Download A Case of Left Atrial Sarcoma Presenting with Mitral Valve

Southern Medical Association
A Case of Left Atrial Sarcoma Presenting with
Mitral Valve Obstruction and Left Heart Failure
P-7
The following abstract will be presented at the Southern Medical Association Annual Scientific Assembly, November 3-5, 2016, in Chattanooga, TN.
Author and
Co-Authors
Eric Arguelles, MD, Department of Cardiology; Christopher I. Jones, MD, Department of Cardiology;
Carolina de Elia, MD, Department of Internal Medicine; Ellen Thompson, MD, FACC, Professor of
Medicine, Division of Cardiology; Joan C. Edwards School of Medicine - Marshall University,
Huntington, WV.
Upon completion of the presentation, learners should be better prepared to:
Objectives
Abstract
1) Recognize sarcomas as rare yet potential mimickers of left atrial myxomas.
2) Identify tumor obstruction of the mitral valve as possible cause of left heart failure and atrial
fibrillation, especially when associated with systemic symptoms.
Introduction: Primary cardiac tumors are extremely rare with an incidence ranging from
0.0017 to 0.019% [1]. Myxoma, a benign tumor, represent by far almost three quarters of them while the
remaining are malignant, predominantly sarcomas [2], [3]. On the other hand, metastatic tumors in the
heart are roughly 20 - 30 times more frequent than primary tumors [4]. While some cardiac tumors may
present with varied symptoms depending on their location and interference with cardiac function, others
are completely asymptomatic and are found incidentally. Presenting symptoms can either be systemic in
nature, including fever, chills, and fatigue, or specific to the hemodynamic effects. Left atrial tumors, in
particular, are known for causing systemic embolization; mitral obstruction and other associated
hemodynamic and electrical phenomena [5]. Most left atrial tumors are initially presumed to be benign
myxoma. A minority of cases is eventually suspected to be sarcomas intraoperatively when invasion of
adjacent structures is noticed at the time of operation. Due to rarity of these tumors, there is no current
consensus on how to treat these patients while the optimal adjuvant therapy remains to be determined [6].
We present herein an interesting case of left atrial sarcoma initially presumed to be myxomatous in
nature.
Case Report: 74 year-old woman with a distant history of coronary artery disease, myocardial
infarction and percutaneous coronary intervention presented to the emergency department (ED) with
several days of dry cough, malaise, shortness of breath, initially treated as community acquired
pneumonia at a local urgent care center. Upon arrival to the ED she was found to be in atrial fibrillation
with rapid ventricular response, and clinical and radiological picture of heart failure. She was initially
admitted to telemetry for rate control and heart failure management. A transthoracic echocardiogram
(TTE) revealed a preserved left ventricular ejection fraction and a large, spherical left atrial mass
suggestive of a myxoma that seemed to be attached to the anterior leaflet of the mitral valve. The
transmitral Doppler spectrum revealed a reversible restrictive pattern, indicative of decreased left
ventricular diastolic compliance and/or increased left atrial pressure. And there was also evidence of right
ventricular volume and pressure overload (Figure 1 and 2). Next day TEE confirmed prior TTE findings
(Figures 3, 4, 5, 6 and 7). A working diagnosis of left atrial myxoma was entertained and surgical
evaluation was requested. Sudden deterioration of her respiratory status prompted an urgent surgical
intervention demonstrating a large size friable left atrial mass originating from the atrioinferior
pulmonary vein web occupying about 90% of the left atrial space and wedging at the mitral valve.
Further inspection of the tumor revealed invasion of the posterior atrial wall including the inferior
pulmonary veins and left atrial appendage by it. Complete excision was deemed impossible at that
moment and a partial excision with reconstruction of the left atrium was performed.
Final Diagnosis: Pathological exam of the operative specimen yielded a final diagnosis of left
atrial undifferentiated pleomorphic sarcoma.
Outcome: She had a satisfactory post-operative course and after 23 days she was transferred to
a short-term rehabilitation center. She had a post-discharge follow up with oncology and a poor
prognosis was deemed then. She was transferred to ED barely a month later in cardiac arrest after few
minutes of sudden severe dyspnea. She expired ultimately despite all resuscitative efforts.
References:
1. Straus R, Merliss R. Primary tumor of the heart. Arch Pathol. 1945;39:74–78
2. Reardon , Smythe : Cardiac neoplasms. Cardiac surgery in the adult. Edited by: Edmunds LH Jr. 2003,
New York, NY:McGrawHill, 1373-1400. 2
3. Mc Allister HA, Fenoglio JJ: Tumours of cardiovascular system in: Atlas of tumour pathology, series
2. 1978, Washington, DC: Armed Forces Institute of Pathology
4. Silverman NA: Primary cardiac tumours. Ann Surg. 1980, 19: 127-131.
5. R. S. Beroukhim, A. Prakash, E. R. Valsangiacomo Buechel et al., “Characterization of cardiac tumors
in children by cardiovascular magnetic resonance imaging: a multicenter experience,” Journal of the
American College of Cardiology, vol. 58, no. 10, pp. 1044–1054, 2011.
6. Putnam JB, Sweeny MS, Colon R, Lanza LA, Frazier OH, Cooley DA: Primary cardiac sarcoma. Ann
Thorac Surg. 1991, 51: 906-10.
Disclosure
Eric Arguelles, MD – No Relevant Financial Relationships to Disclose
Carolina de Elia, MD – No Relevant Financial Relationships to Disclose
Christopher I. Jones, MD – No Relevant Financial Relationships to Disclose
Ellen Thompson, MD, FACC – No Relevant Financial Relationships to Disclose