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  • Updated 07.20.2022
  • Released 06.11.2001
  • Expires For CME 07.20.2025

Atrial myxoma

Introduction

Overview

Atrial myxoma, the most common intracardiac tumor in adults, may cause cardiac dysfunction embolic stroke and systemic manifestations that mimic infective, immunologic, and malignant diseases. Several cutaneous syndromes associated 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, its central location often results in significant morbidity and mortality.

• Myxomas have a wide variety of cardiac, embolic, and systemic manifestations, including paraneoplastic syndromes, mediated by numerous 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 successful in patients with acute stroke caused by myxoma.

• Hemorrhagic complications after thrombolysis may be due to recent, undiagnosed infarcts or aneurysms.

• Intra-arterial recanalization is effective in patients with large artery occlusion due to myxoma embolization.

Historical note and terminology

Left atrial myxoma was first described in the 19th century (71) and was diagnosed by angiocardiography in 1952 (73). The first surgical excision occurred 3 years later (29).

The first nonembolic dermatologic manifestation of atrial myxomas was described in 1973 as the nevus, atrial myxoma, myxoid neurofibromata, and ephelides or endocrine hyperactivity (NAME syndrome). Later, it was described as the lentigines, atrial myxoma, and blue nevi (LAMB syndrome), and Carney complex (atrial myxoma associated with multiple neoplasia lentigines) was reported (49). Sneddon syndrome, which commonly presents with a livedo-type rash and diffuse cerebrovascular disease, has also been associated with atrial myxoma (139).

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