Review
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Cardiol. Apr 26, 2022; 14(4): 206-219
Published online Apr 26, 2022. doi: 10.4330/wjc.v14.i4.206
Cardiac myxomas: A narrative review
A K M Monwarul Islam
A K M Monwarul Islam, Department of Cardiology, National Institute of Cardiovascular Diseases, Dhaka 1207, Bangladesh
Author contributions: Islam AKMM conceptualized the review and wrote and approved the manuscript.
Conflict-of-interest statement: All authors declare having no conflicts of interest.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: A K M Monwarul Islam, MD, FCPS, FACC, FESC, FASE, FACP, FRCP, Associate Professor, Department of Cardiology, National Institute of Cardiovascular Diseases, Sher-e-Bangla Nagar, Dhaka 1207, Bangladesh. drmonwarbd@yahoo.com
Received: March 18, 2021
Peer-review started: March 18, 2021
First decision: July 18, 2021
Revised: August 28, 2022
Accepted: March 27, 2022
Article in press: March 27, 2022
Published online: April 26, 2022
Processing time: 396 Days and 8.7 Hours
Abstract

Cardiac myxomas are common primary neoplasms of the heart. They are biologically benign but “functionally malignant” because of the potential for embolization. They arise most commonly from the left atrium, but no chambers of the heart are immune. They may be sporadic in the majority but also familial as a part of the Carney complex. Two morphological forms exist: polypoid and papillary. Polypoid myxomas often present with obstructive features, while the papillary forms are more prone to embolization. Histogenesis is still controversial; the current view centres around origin from the primitive pluripotent mesenchymal cells. They may be of giant proportion, be calcified or get infected. Clinical presentation typically involves the triad of intracardiac obstruction, embolic events and constitutional symptoms. Precordial examination findings may simulate those of mitral or tricuspid stenosis. The presence of tumour plop and change of the physical findings with changing position may help differentiation between the two. Echocardiography is the investigation of choice. Echogenic polypoid or papillary mobile mass within the atrial cavity remaining attached to the interatrial septum through a stalk are the tell-tale echocardiographic features. Cardiac magnetic resonance and computed tomographic scanning may have incremental diagnostic value. Histopathological examination reveals abundant loose myxoid stroma with scattered round, polygonal or stellate cells with dense irregular nuclei. Genetic testing may detect mutations in the PRKAR1A gene in the familial form of cardiac myxoma, i.e. the Carney complex. Surgical excision is the mainstay of treatment with low operative mortality, excellent postoperative survival and low recurrence rate. The current trend favours minimal-access surgery with or without robotic assistance. Physicians should have appropriate preparedness to make a timely diagnosis and enthusiastic treatment to avoid potentially fatal complications.

Keywords: Myxoma; Cardiac; Neoplasm; Carney Complex; Echocardiography; Embolism

Core Tip: Cardiac myxomas are biologically benign but “functionally malignant”. They can cause life-threatening embolic events. Associated constitutional symptoms may mimic those of inflammatory or connective tissue disorders. Timely diagnosis is of utmost importance because it offers a scope for definitive treatment, i.e. surgical excision. Cardiac myxoma is a relatively rare diagnosis, so physicians should have appropriate preparedness to deal with this entity. This review article has summarised the available information, offered practical tips and highlighted the recent advances.