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  • Updated 04.06.2020
  • Released 06.11.2001
  • Expires For CME 04.06.2023

Atrial myxoma

Introduction

This article includes discussion of atrial myxoma and cardiac myxoma. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Atrial myxoma is the most common intracardiac tumor in adults. Atrial myxoma may impair the cardiac function and cause stroke by embolization. Additionally, myxoma may mimic infective, immunologic, and malignant diseases. Several cutaneous syndromes have been described that may facilitate timely recognition. Its protean clinical manifestations make the clinical diagnosis difficult. Approximately 60% of all tumors are found by echocardiogram. The definitive treatment is surgical excision.

Key points

• Atrial myxoma is the most common intracardiac tumor. Although benign by histopathologic criteria, its central location may result in a fatal outcome if not treated in a timely manner.

• Myxomas have a wide variety of cardiac, embolic, and systemic manifestations, including paraneoplastic syndromes, mediated by a multitude of humoral factors.

• No guidelines for acute ischemic stroke caused by atrial myxoma embolism treatment have been published.

• Intravenous thrombolysis within a 3-hour window was used successfully in some patients with acute stroke caused by myxoma.

• The risk of hemorrhagic transformation may be higher than in other patients due to prior asymptomatic infarcts or aneurysms.

• Intra-arterial recanalization is effective in patients with large artery occlusion.

Historical note and terminology

Left atrial myxoma was first described over 150 years ago (69). The first antemortem diagnosis, by angiocardiography, was in 1952 (71), and the first surgical excision documented 3 years later (28).

The first documented association between nonembolic dermatologic manifestations and atrial myxomas was made in 1973. The nevus, atrial myxoma, myxoid neurofibromata, and ephelides or endocrine hyperactivity (NAME syndrome); lentigines, atrial myxoma, and blue nevi (LAMB syndrome); and Carney complex (atrial myxoma associated with multiple neoplasia lentigines) were first reported in 1980, 1984, and 1985, respectively (47). Sneddon syndrome, which commonly presents with a livedo-type rash and diffuse cerebrovascular disease, has also been associated with atrial myxoma (137).

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