Recurrent Left Ventricular Myxoma in Carney Complex: A Multimodality Imaging Case

Presenting Author(s)

• MS

  María Clara Scattini, MD

  Cardiologist
  Hospital Italiano de Buenos Aires
  Ciudad Autonoma de Buenos Aires, Ciudad Autonoma de Buenos Aires, Argentina

Primary Author(s)

• RG

  Roveda Guido, MD

  Resident of Cardiology
  Hospital Italiano de Buenos Aires, Argentina

Co-Author(s)

• RG

  Roveda Guido, MD

  Resident of Cardiology
  Hospital Italiano de Buenos Aires, Argentina

Co-Author(s)

• CF

  Cantora Florencia belen, MD

  Resident of Cardiology
  Hospital Italiano de Buenos Aires, Argentina
• SD

  Santiago Decotto, MD

  Faculty
  Hospital Italiano de Buenos Aires
  Buenos Aires, Buenos Aires, Argentina
• 

  Sebastian Tonso, MD

  Medical associated
  Hospital Italiano de Buenos Aires
  Buenos Aires, Ciudad Autonoma de Buenos Aires, Argentina
• 

  Santiago del castillo, MD

  Cardiologist
  Hospital Italiano de Buenos Aires
  Buenos Aires, Ciudad Autonoma de Buenos Aires, Argentina
• PD

  Perez de Arenaza Diego, MD

  Cardiologist
  Hospital Italiano de Buenos Aires
  Capital federal, Buenos Aires, Argentina
• BR

  Blanco Rocio, MD

  Cardiologist
  Hospital Italiano de Buenos Aires
  Capital federal, Buenos Aires, Argentina
• AA

  Anibal Arias

  Head of the Coronary Care Unit
  Hospital Italiano de Buenos Aires, Argentina

Description of Clinical Presentation: A 19-year-old female with a history of atrial myxoma resection at age 16 and recurrence at age 18 presented after her third cerebrovascular accident, with dysarthria, dysphagia, and left facial-brachial paresis. She had mild hirsutism and vitiligo. Family history was remarkable for multiple relatives with cardiac myxomas and sudden death, suggesting a hereditary syndrome. Physical examination revealed hemodynamic stability but persistent neurological deficits.

Diagnostic Techniques and Their Most Important Findings: Neuroimaging demonstrated acute ischemic foci, fusiform and saccular dilatations of the basilar and posterior cerebral arteries consistent with post-embolic aneurysms, and multiple calcified cavernomas. Electrocardiogram showed sinus rhythm with repolarization abnormalities. Transthoracic echocardiography identified a large, mobile left ventricular mass nearly obstructing the cavity (2.8 × 4.8 cm).
Cardiac magnetic resonance (CMR) confirmed a heterogeneous, lobulated mass measuring up to 5.1 cm, hypointense on cine, hyperintense on T1/T2 and T2-STIR, with no perfusion and heterogeneous late gadolinium enhancement, consistent with myxoma. Mild left ventricular systolic dysfunction (EF 54%) and focal transmural fibrosis suggested embolic infarction. The constellation of recurrent tumors, extracardiac findings, and striking family history raised suspicion of Carney complex, prompting genetic testing.
After neurological stabilization, the patient underwent surgery. Intraoperative findings revealed more than 20 myxomas implanted in the posterior papillary muscle and lateral LV wall, all excised successfully. Histopathology confirmed the diagnosis. Postoperative recovery was favorable.

Learning Points from this Case: Cardiac myxomas are rare, with < 4% localized in the left ventricle. Recurrence after resection occurs in 10–15%, but multifocal disease in a young patient with family history should raise suspicion of Carney complex, an autosomal dominant disorder linked to PRKAR1A mutations.
CMR was essential in this case for accurate mass characterization, evaluation of myocardial involvement, and surgical planning.
Multimodality imaging also delineated embolic sources and cerebrovascular complications, influencing timing of intervention. Early recognition of Carney complex is critical, as recurrent and aggressive myxomas are the leading cause of mortality in affected patients.

Tissue characterization
AMI inferolateral medial segment, embolic origin
Surgical specimen