Transposition of the Great Arteries

Overview

Transposition of the great arteries is a rare congenital heart defect found in infants. The defect occurs when the two main arteries that carry blood from the heart are in the wrong place, or transposed. Transposition of the great arteries is also known as dextro-transposition of the great arteries, or d-TGA. Without surgery to repair the defect, a child cannot survive. Even patients who have undergone successful surgeries are at risk for developing additional cardiovascular complications later in life.

There are two types of surgeries to correct d-TGA. One is called an intra-atrial baffle (Mustard or Senning procedure) and the other is called an arterial switch. The latter has been the preferred option since the 1980s, but patients in their mid-40s or older likely had the former surgery, which can cause heart problems later in life.

Symptoms

Adults who had surgery to correct d-TGA are more likely to develop congestive heart failure, systemic ventricular dysfunction, arrhythmias and other complications. The risks for each of those developments increase with age. Adults who had surgery as a child may experience symptoms of complications, including:

  • Abnormal heart rhythm
  • A weakened right ventricle that causes congestive heart failure
  • Blockages in the tunnel between the atria created during surgery to correct the transposition
  • Heart valve leakage
  • Coronary artery problems

Prevention

d-TGA is a congenital condition. There is currently no known way to prevent d-TGA from developing. Adults living with the condition will need lifelong care from a cardiologist who can monitor their health and help reduce the risk for further complications.

Risk Factors

  • Increasing age

Diagnosis

Patients are diagnosed with and undergo treatment for d-TGA as infants. An expert in the care of adults will monitor adults living with the condition for complications, using:

  • Electrocardiograms, also known as ECG or EKG, to measure the heartbeat’s electrical activity
  • Echocardiograms, an ultrasound of the heart’s structures
  • Holter monitors, portable electrocardiograms that record the heart’s activity for 24 to 48 hours
  • Stress testing, which measures the heart’s function during exercise
  • MRIs, an imaging test that uses magnets to create images of the heart’s structure and function
  • Heart catheterization, also known as cardiac catheterization, helps visualize cardiac arteries, muscle and valves, as well as measure the blood flow and pressure within your heart

Treatment

Treatment for adults living with d-TGA depends on the complications experienced. For some patients:

  • Diuretic medications may help regulate fluid accumulation.
  • Electrophysiology studies can help treat heart rhythm issues.
  • Medications can assist the heart’s pumping efficiency and control blood pressure.
  • Pacemaker implantation can help patients with heart rhythm issues.
  • Surgery may be necessary to repair leaking valves, narrowing in the aorta or pulmonary arteries, or to insert a stent in the baffle.

Follow-up Care

As children with d-TGA age, they will need regular cardiological care for the remainder of their lives, specifically from cardiologists who are experts in the care of adults with congenital heart disease.

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