Tetralogy of Fallot is a relatively common congenital heart disease. Many patients were diagnosed in infancy as “blue babies.”
Most patients with Tetralogy of Fallot have undergone surgery in infancy or childhood.
All patients with Tetralogy of Fallot should be evaluated at a dedicated center for the care of adults with congenital heart disease such as the University of Michigan Adult Congenital Heart Program.
Patients require regular follow-up with a cardiologist.
Many patients require repeat surgeries later in life.
Concerning symptoms for patients with Tetralogy of Fallot can include shortness of breath with activity, irregular heart beats, loss of consciousness (syncope), and abdominal or leg swelling.
Transposition of the Great Arteries
In Transposition of the Great Arteries (TGA), the main pumping chamber of the heart, the left ventricle, gives rise to the pulmonary artery which supplies the lungs. The right ventricle supplies blood to the body. Babies with Transposition of the Great arteries can be very cyanotic (blue).
Almost all patients with TGA will have undergone some sort of surgery in infancy.
Younger patients (mid-20’s and younger) will generally have undergone the arterial switch repair.
The U-M was the first hospital in the state of Michigan to perform the arterial switch surgery.
Older patients (mid 20’s and older) will have undergone atrial switch surgeries, known as the Mustard or Senning procedures.
All patients with TGA require evaluation in a dedicated adult congenital heart program such as the U-M Adult Congenital Heat Program.
Patients with TGA requiring pacemakers or other procedures should have them performed at an experienced adult congenital heart center.
Common problems with patients following the Mustard and Senning procedures include heart failure and problems with abnormal heart rhythms.
The Fontan procedure was developed for patients born with only one pumping chamber (ventricle) of their heart.
Many patients require surgery prior to their Fontan procedure, which often occurs in early childhood.
All patients after a Fontan require evaluation in a dedicated adult congenital heart program such as the U-M Adult Congenital Heat Program.
Patients should have close follow-up for development of abnormal heart rhythms, or worsening heart function.
Women with the Fontan procedure should undergo extensive evaluation and consultation with an adult congenital heart disease specialist prior to consideration of pregnancy.
Symptoms of swelling and chronic diarrhea can be signs of a serious medical problem in Fontan patients and should be evaluated.
Patients with the Fontan procedure generally require some type of blood thinner because of the increased risk for blood clots.
Adults with atrial septal defect may not be diagnosed until there 30’s, 40’s, or 50’s.
A heart murmur or the development of abnormal heart rhythms can be the first sign that an atrial septal defect is present.
Other symptoms of atrial septal defect can include worsening shortness of breath with activity.
Not all atrial septal defects require intervention. Very small defects which do not make the heart enlarge can often just be watched without intervention.
Many atrial septal defects can be closed safely without surgery. The U-M has a long experience with percutaneous closure of atrial septal defect and was involved with many of the initial studies of safety of closure defects.
Larger atrial septal defects, or those in unusual anatomic locations (known as sinus venosus septal defects or primum atrial septal defects) require surgical closure.
Most adults with large ventricular septal defects will have undergone surgical repair in their youth.
Patients with repaired or spontaneously closed ventricular septal defects require occasional follow-up in an experienced adult congenital center to evaluate for specific late complications which can occur.
Many small ventricular septal defects do not require any time of intervention, although patients with such defects still require life-long follow-up.
Patients with large ventricular septal defects who have not undergone surgical repair will develop a significant complication known as Eisenmenger Syndrome.
Patients with Eisenmenger syndrome are extremely high risk with anesthesia for surgery and at the time of pregnancy.
Patients with Eisenmenger syndrome should be evaluated at an center experienced in congenital heart disease.
Some patients with pulmonary stenosis require surgery or a catheterization procedure in childhood or infancy, while others may need procedures later in life.
Many patients who underwent surgery in childhood for pulmonary stenosis will require repeat procedures later in life for either worsening narrowing of the valve (stenosis), or development of leakage(insufficiency) of the valve.
Pulmonary stenosis can often be treated at the time of cardiac catheterization without the need for open heart surgery.
