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Left Atrial Stenosis Induced Pulmonary Venous Arterialization and Group 2 Pulmonary Hypertension in Rat
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An adult with a sinus venosus atrial septal defect and dilated cardiomyopathy

Luke Oakley1, Sean Foley2, Justin Cox1

  • 1Department of Cardiology, Naval Medical Center, San Diego, California, USA.

BMJ Case Reports
|May 24, 2014
PubMed

Insights

A rare combination of dilated cardiomyopathy and atrial septal defect in an adult was managed with a left ventricular assist device. The atrial septal defect may have initially protected the patient from heart failure symptoms.

Area of Science:

  • Cardiology
  • Adult Congenital Heart Disease

Background:

  • Dilated cardiomyopathy, heart failure, and atrial septal defects are common individually but rarely co-occur.
  • Increased survival of congenital heart disease patients into adulthood leads to more complex adult cardiology cases.

Observation:

  • A 32-year-old man presented with dilated cardiomyopathy and a sinus venosus-type atrial septal defect.
  • He exhibited a significant pulmonary to systemic flow ratio of 5:1.
  • The atrial septal defect potentially acted as a 'pop-off' valve, mitigating pulmonary congestion and heart failure symptoms.

Findings:

  • The patient's condition did not respond to standard medical therapy for dilated cardiomyopathy.
  • A left ventricular assist device was ultimately implanted due to disease progression.
  • The unique pathophysiology of this combined condition was explored.

Implications:

  • This case highlights the evolving landscape of adult congenital heart disease.
  • Understanding the interplay between atrial septal defects and dilated cardiomyopathy is crucial for managing complex cardiac conditions.
  • Further research into such rare combinations can refine treatment strategies.

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