Single ventricle defects

Single ventricle defects

Overview

Many forms of congenital heart disease present with only one functional pumping chamber (ventricle), a rare condition. Unless treated, a single ventricle defect is usually fatal to the child within its first days or months.

These forms of congenital heart disease are the most serious, complex types and include:

  • Tricuspid atresia
  • Hypoplastic left heart syndrome (HLHS)
  • Pulmonary atresia with intact ventricular septum
  • Mitral atresia (often associated with HLHS)
  • Unbalanced atrioventricular canal defects
  • Double-outlet right ventricle.

Virtually all single ventricle defects require at least one surgery.  

Causes

The problem may be that the ventricle is small, undeveloped or missing a valve.

Symptoms

The baby often seems normal at birth but within a few days may appear ashen, have difficulty feeding and have rapid and difficult breathing.

Diagnosis

  • Exercise stress testing
  • Advanced imaging with cardiac MRI
  • Occasionally, cardiac catheterization

Treatment

For each type of single ventricle defect, a different staged surgical palliation is performed. The goal is to separate the blue (oxygen-poor) blood returning from the body to the heart and allow it to flow directly to the lungs for oxygenation. Often, two operations are required to accomplish this task. Lifelong surveillance of the single systemic ventricle is required for assessment of the success of the procedure.

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