What is Congenital Heart Disease?
As we are developing from a single egg fertilized by a single sperm, cells divide and begin to develop into the different tissues of our adult bodies. This is a very complex process that is like a kind of perfectly choreographed ballet. A special cluster of cells slowly differentiates into the cardiovascular system. Cells that become the heart must rotate, turn and twist to form a normal heart.
Occasionally this process of normal development does not occur perfectly. This results in abnormal connections in the heart and after birth we call these defects congenital heart disease.
Did You Know that Nearly 1 out of 100 Births Today are Affected by some form of Congenital Heart Disease?
Some kinds of congenital heart disease are at increased risk for developing pulmonary hypertension over their lifetime. The risk of developing pulmonary hypertension exists even if you have had surgery or a procedure to repair the original heart defect. The most common congenital heart problem in childhood that may eventually lead to pulmonary hypertension is a hole between the two bottom pumping chambers of the heart called a ventricular septal defect (VSD). Sometimes this defect may close on its own, other times it may require surgery to be closed. Some patients with small ventricular septal defects may be monitored by specialists in adult congenital heart disease.
The most common congenital heart problem in adulthood that may lead to pulmonary hypertension is a hole between the two top chambers of the heart or an atrial septal defect (ASD). This defect if very small may also be called a patent foramen ovale or a PFO. When the defect is very small it may be something that can be monitored closely by a cardiologist who is familiar with the management of congenital heart disease. In order to treat this defect you may be able to undergo a simple, minimally invasive, catheter-based procedure to have the defect closed, in more complicated cases surgery may be required.
There are different types of atrial septal defects. The most common type is the one that can be closed typically with the previously mentioned catheter-based technique. The other types of atrial septal defect can not be closed with a catheter-based technique and require surgery by an experienced congenital heart surgeon. These types include holes between the two top chambers which are too large or too high or low on the wall to be safely closed with a catheter-based device.
Often, if a procedure or surgery is performed when the patient is an infant or a small child the risks of developing pulmonary hypertension over the patient’s lifetime are not communicated to the patient. The cardiologists and the surgeon rely on the parents and grandparents to remember and tell the child what needs to be done over their lifetime. Many times this message is lost or forgotten as the caregivers age or change and as the child grows into adulthood.
Complex Congenital Heart Disease
There are many more complicated forms of congenital heart disease that are also associated with PAH. These include but are not limited to: Tetralogy of Fallot (TOF), Patent Ductus Arteriosus (PDA), Truncus Arteriosus, and Anomalous Pulmonary Venous Return. We will discuss some of these in dedicated posts.
If you or someone you love had a heart procedure or a heart surgery as a child to have a hole in the heart closed, you should seek out your community’s adult congenital cardiology specialist for an evaluation. This evaluation may include such tests as a walking test to evaluate your ability to exercise, a transthoracic echocardiogram or an ultrasound using soundwaves to take pictures of your heart and a visit with an adult congenital cardiology specialist.
