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Atrial Myxoma

Clinical Subject Page

Atrial Myxoma

Also called

Cardiac Myxoma

ICD-10

D15.1

Specialty

Cardiology

Onset

Insidious (progressive)

Reviewed

June 2026

On This Page

Overview

Cardiac myxoma is the most common primary tumor of the heart. It is usually benign and arises from primary connective tissue. Around 75% occur in the left atrium, usually attached to the interatrial septum. Right atrial myxomas are less common, while ventricular myxomas are rare. Symptoms mainly result from: Obstruction of intracardiac blood flow. Embolization of tumor fragments.

Etiology & Risk Factors

  • Exact cause is unknown.
  • Most cases are sporadic.
  • About 10% are hereditary:
    • Autosomal dominant inheritance
    • Associated with Carney syndrome

Pathophysiology

Gross pathology

  • Usually pedunculated (attached by a stalk).
  • Gelatinous in consistency.

Microscopic pathology

  • Scattered mesenchymal cells in a mucoid (gelatinous) matrix.
  • Surrounded by glycosaminoglycans.
  • Tumor cells produce VEGF.

Mechanism of symptoms

  • Obstruction of the mitral valve opening causes symptoms similar to mitral stenosis.
  • Embolization may cause stroke or infarction in other organs.
  • IL-6 production by the tumor leads to constitutional symptoms.

Clinical Presentation

Constitutional symptoms

  • Weight loss
  • Fever
  • Pallor
  • Digital clubbing

Symptoms due to obstruction

  • Dyspnea on exertion
  • Orthopnea
  • Paroxysmal nocturnal dyspnea
  • Palpitations
  • Dizziness
  • Recurrent syncope

Physical examination

  • Low-pitched mid-diastolic rumbling murmur at the apex
  • Characteristic “tumor plop” (early diastolic sound)
  • Murmur and heart sounds may change with body position.
  • Mitral regurgitation may occur due to valve damage.

Symptoms due to embolization

  • CNS: TIA, stroke, seizures
  • Abdomen: Visceral infarction or hemorrhage
  • Lungs: Pulmonary embolization

History Taking

Important points include:

  • Progressive exertional dyspnea
  • Episodes of syncope
  • Palpitations
  • Orthopnea or PND
  • Constitutional symptoms (fever, weight loss)
  • History suggestive of embolic events (stroke, TIA, limb or abdominal ischemia)
  • Family history of Carney syndrome or hereditary cardiac myxoma

Investigations

  • Echocardiography is the diagnostic procedure of choice.
  • Diagnosis may be difficult clinically because symptoms are often nonspecific.

Diagnosis

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Diagnosis is based primarily on:

  • Clinical suspicion
  • Echocardiographic visualization of the intracardiac mass

Management

Definitive treatment

  • Immediate surgical resection (curative treatment)

Additional management

  • Treat associated conditions if present:
    • Arrhythmias
    • Heart failure
    • Embolic complications

Complications

  • Systemic embolization
  • Cerebral infarction (stroke)
  • Pulmonary embolization
  • Valvular damage causing mitral regurgitation
  • Sudden death
  • Tumor recurrence after incomplete excision or from another tumor focus

Prognosis

  • Generally favorable after surgical resection.
  • Recurrence can occur, especially after incomplete tumor removal.

Key Points / Clinical Pearls

  • Most common primary benign cardiac tumor.
  • About 75% arise in the left atrium, usually from the interatrial septum.
  • Classic auscultatory finding: “tumor plop.”
  • Symptoms are due to obstruction, embolization, and IL-6-mediated constitutional effects.
  • Echocardiography is the investigation of choice.
  • Immediate surgical excision is the definitive treatment because of the risk of embolization and sudden death.