Congenital heart disease (CHD) means you are born with a heart condition. It could be a malformation of the central big vessels or a problem with one or more areas of the heart or other blood vessels. Congenital heart disease includes all kinds of defects, from very simple to extremely complex ones.
Patients with simple defects or perfectly balanced defects that are more complex may survive into adulthood without having a procedure to correct the problem. Once symptoms begin, it is possible that the problem is still simple and curable. However, it is also possible that the condition has caused complications and other health problems. In these cases, treatment can be difficult and complicated,
Because of advances in congenital heart surgery, most newborn with CHD now survive into adulthood. Many patients develop new problems and complications that may or may not be related to their CHD and previous surgeries. For example, the first effective surgery available to children born with transposition of the great arteries (TGA) was an atrial switch operation (Mustard or Senning). The blood was redirected at the top chambers of the heart so the right ventricle (built to pump blood at low pressure through the lung circulation) became the main pumping chamber instead of the left ventricle. Patients who had this procedure eventually have problems with the right ventricle because it isn’t meant to do handle systemic blood pressure, and their tricuspid valve may also become leaky (see below under Congenitally Corrected Transposition). Today, babies born with TGA have an arterial switch operation, which moves the aorta and coronary arteries to the left ventricle. The procedure is done shortly after birth, and outcomes are excellent with much fewer problems later in life. However, the arterial switch procedure became the standard treatment fairly recently (1991), so the long-term outcomes are still being studied. The same is true for many other updated treatments. There are risks and benefits, some unknown but many still unknown, with every procedure.
We do know that there are a growing number of patients born with CHD who, regardless of whether they have had treatment, develop new problems as adults and need surgery. Treatment of these patients is a sub-specialty in both cardiology and cardiac surgery. It is important for all patients with CHD patients to choose experts in CHD for their care.
Surgeons and cardiologists who specialize in treatment of adults with CHD care for patients who have:
Conditions first diagnosed as an adult
- Atrial Septal Defect (ASD)
- Bicuspid aortic valve
- Coarctation of the Aorta
- Congenitally Corrected Transposition of the Great Arteries (CCTGA)
- Coronary fistula
- Patent Ductus Arteriosis (PDA)
- Patent Foramen Ovale (PFO)
- Pulmonary Stenosis
- Tetralogy of Fallot
- Ventricular Septal Defect (VSD)
CHD repaired in childhood, but in adulthood:
- It is found the treatment was not completely successful and surgery is needed to correct the
- They have secondary problems related to the original heart defect
- Complications occur and require another repair or surgery
- Treatment is needed for a new, unrelated heart condition, such as coronary artery disease
Conditions Diagnosed as an Adult
Many times, the condition doesn’t cause symptoms early in life. Instead, the body adapts and balances problems with circulation, so there are no symptoms of the disease. But, over time, the problem gets worse and can cause symptoms and even lead to other conditions.
Examples of CHD that are often found in adulthood are:
This is a hole in the top part of the septum (wall that separates the right and left sides of the heart). The top two sections of the heart are called the atria. An ASD is
in the section of the septum that divides the right and left atria. Over time, an ASD can cause the right side of the heart to stretch (dilate). The condition can also lead to a leaky mitral valve (mitral regurgitation), a leaky tricuspid valve (tricuspid regurgitation) and an irregular heartbeat called atrial fibrillation.
Patent foramen ovale (PFO). A foramen ovale is a hole in the top part of the septum (wall that separates the right and left sides of the heart). The top two sections of the heart are called the atria. The foramen ovale is in place during the fetal stage. It works as a one-way valve that allows blood to bypass the lungs and lets the left ventricle develop and work normally. A foramen ovale normally closes after birth. A PFO is a foramen ovale that does not close completely. A PFO is a common condition that affects up to 25% of people. The condition has been linked to embolic stroke, paradoxical embolism, and migraine and is related to decompression sickness. But, complications are very rare. Patients with a PFO who do not have symptoms do not need to have the PFO closed. If you have a PFO and have had a stroke, transient ischemic attack (TIA), or deep vein thrombosis (DVT), your doctor may talk to you about a procedure to close the PFO. Closure can usually be done with a catheter-based procedure (device closure) or surgery. A PFO is often closed during surgery for another heart condition without increasing the risk to the patient.
Patent ductus arteriosus (PDA). Ductus Arteriosus is part of blood circulation between the pulmonary artery and aorta in the fetal stage. It allows the right
ventricle to develop and work normally. While the ductus arteriosus is open, blood flows from the right ventricle to the pulmonary artery through the duct to the aorta (bypassing the lungs). At birth, a baby’s first breath exposes the duct to oxygen-rich blood, and the duct starts to close. If the duct does not fully close, it is called a patent (open) ductus arteriosus (PDA). The blood flows backwards from the aorta into the pulmonary artery, which causes the lungs to get too much blood. If the PDA is very small, treatment may not be needed. But, surgery may be needed to close the defect. The operation is simple and safe. It is performed from the left side of the chest. If a large PDA is not closed, it can cause pulmonary hypertension (high blood pressure that affects the lungs and right side of the heart) and can lead to Eisenmenger syndrome (irreversible severe pulmonary hypertension), and a lung or heart-lung transplant may be needed.
- Cleveland Clinic offers a successful transplant program.
This is an abnormal connection between a coronary artery and one of the 4 chambers of the heart. The coronary arteries are the blood vessels that send blood to the heart. A small coronary fistula may not cause any symptoms, but it may grow over time and be very large when it is found. A large coronary fistula can keep the heart muscle from getting enough blood. This can cause shortness of breath. When a fistula becomes large and causes symptoms, surgery is needed to close it and
create better blood flow.
The aorta is the largest artery in the body. It branches off of the heart and carries oxygen-rich blood to the rest of your body. The aortic valve opens to let blood flow from the heart into the aorta. A normal aortic valve has 3 leaflets. Leaflets are the flaps inside the valve that keep blood from flowing backwards. If you have a bicuspid aortic valve, it means you only have 2 leaflets. Many patients with bicuspid aortic valves are diagnosed when they are 20-40 years old. At this age, the condition causes the aortic valve to leak. Patients who are diagnosed after age 40 usually have a narrowed valve (aortic stenosis). Aortic valve defects can cause the part of the aorta closest to the heart (aortic root) to become larger than normal (dilate). If this happens, your doctor may keep a close eye on the condition and you may need surgery. Other types of CHD that involve the aortic valve are unicuspid aortic valve (one leaflet) and quadricuspid aortic valve (4 leaflets). These problems are less common than bicuspid aortic valves.
Ventricular septal defect (VSD). The bottom two sections of the heart are called the ventricles. A VSD is a hole in the septum, the muscular wall that divides the right and left ventricles. If you have a small VSD, you likely won’t have any symptoms. But, over time, as the opening gets larger, you may have trouble breathing because the condition causes more blood than normal to pump into the lungs. This increases the pressure in the lung’s arteries and can cause permanent damage. A patient with a VSD may develop Eisenmenger’s syndrome (irreversible severe pulmonary hypertension). If this happens, the patient develops right heart failure and, at this stage, a heart and/or lung transplant is needed.
- Cleveland Clinic offers a successful transplant program.
Congenitally Corrected Transposition of the Great Arteries (CCTGA)
Congenitally Corrected Transposition of the Great Arteries (CCTGA). This is a rare condition in which the heart’s bottom, main pumping chambers (ventricles) do the opposite work of normal ventricles ― instead of the right ventricle pumping blood to the lungs, it pumps blood to the entire body. The left ventricle, which is supposed to pump blood to the entire body, instead pumps blood to the lungs. The arteries naturally adjust to the defect. Sometimes, the right ventricle is strong enough to handle the extra work, and the condition does not cause symptoms. But, because it is weaker than the left ventricle, it might wear out, which leads to heart failure. The systemic valve between the left atrium and the systemic right ventricle is the tricuspid valve. It is not able to handle this level of pressure. So, with time, these patients often develop a severe tricuspid valve leak. Patients with CCTGA often also have problems with their heart rhythm (such as heart block).
Conditions Repaired in Childhood
Congenital heart disease was repaired in childhood, but the treatment was not completely successful and surgery is needed to correct the problem. This includes patients with:
- Coarctation or re-coarctation of the aorta. The aorta is pinched in or narrowed, like a kink in a hose. These patients have higher blood pressure in the upper body
- Residual atrial septal defect (ASD). This is an ASD that stays open after treatment to close it with a device or surgery.
- Residual Ventricular Septal Defect (VSD). This is a VSD that stays open after surgery to close it.
- Patients with primum ASDs or complete atrio-ventricular (AV) canal defects often have defects in the mitral valve (cleft anterior leaflet) or tricuspid valve that cause these valves to leak. Repeat surgery is needed to correct the problems.
Congenital heart disease was repaired in childhood, but there are now complications or other related conditions; or the repair needs to be redone. Common examples are:
- Patients who had surgery early in life for pulmonary stenosis, and since then, the valve has split open. As the patient grows, the valve becomes more and more leaky and may need to be replaced.
- Tetralogy of Fallot is a common congenital heart defect that has 4 problems (tetralogy means four) ― pulmonary stenosis (narrowing of the pulmonary valve and main pulmonary artery), VSD, right ventricular hypertrophy (thick muscle in the right ventricle that reduces the amount of blood that is squeezed out), and an over-riding aorta (the aorta is between the ventricles instead of being connected to the left ventricle). Patients who had surgery to correct tetralogy of Fallot often have very leaky or missing pulmonic valves. With time, this can cause the right ventricle to become larger than normal and overworked, which creates the need for pulmonic valve replacement.
- Patients who had a biologic valve or conduit implanted between the right ventricle and the pulmonary artery early in life. Eventually, the patient will outgrow the valve and it needs to be replaced.
- Single Ventricle - Fontan Circulation: Many newborns with CHD have only one chamber of the heart that works properly, and their early treatment has involved procedures done in stages to eventually create normal, synchronized blood flow driven by the one ventricle they have – this treatment is often referred to as single ventricle staged palliation. When completed the systemic and the pulmonary circulations are in series, the blood driven through the lungs by the venous pressure. The chronically elevated venous pressure often leads to organ congestion resulting in other and new medical problems. Patients may require surgery for the late complications of Fontan – or even a heart transplant. All patients in this single ventricle group need close follow-up care for the rest of their lives.
- Patients who have a secondary condition that is related to the original defect. This is most common in patients who do not have an ASD repaired right away.
Secondary but conditions related to the original defect, most commonly due to late repair of an ASD
- Patients who have atrial fibrillation because the atria (top chambers of the heart) become enlarged and stretched-out from extra blood volume.
- Patients who have tricuspid regurgitation because the right side of the heart (atrium and ventricles) are enlarged and stretched-out from extra blood volume.
- Patients who have mitral regurgitation because the left atrium is enlarged and stretched-out from extra blood volume.
The patient had CHD repaired in childhood but needs treatment for a new, unrelated heart condition, such as coronary artery disease.