Congenital Heart Disease Conditions

An atrial septal defect (ASD) is a congenital condition where a hole has formed in the wall of tissue that separates the right and left atria (upper chambers of the heart). ASDs cause oxygen-rich blood to mix with oxygen-poor blood, which allows oxygen-poor blood to be delivered to vital organs. Large ASDs can eventually lead to pulmonary hypertension or heart failure, and surgery is typically required to repair the defect.

Types of ASDs:

• Ostium secundum is the most common ASD, and it occurs when a portion of the atrial septum fails to develop normally. This defect can most often be closed using a minimally invasive transcatheter procedure.
• Ostium primum is is a deficiency in part of the atrioventricular canal and is associated with a split (cleft) in the mitral valve.
• Sinus venosus is classified as a hole between the superior vena cava (main vein that is returning blood from the upper body) and the right atrium (right upper chamber of the heart).
• Coronary sinus is the rarest ASD. This is characterized by a hole the coronary sinus, a major vein in the heart.

Atrioventricular canal defect results in a hole in the center of the heart, which impacts all four valves and effectively separates the right and left sides of the heart. With atrioventricular canal defect, oxygen-rich blood is mixed with oxygen-poor blood and is re-circulated through the body, making it hard to regulate blood flow to critical organs.

A healthy aortic valve will have three flaps to seal the valve and regulate blood flow. People who are born with the congenital condition bicuspid valve disease only have two aortic valve leaflets. Without the third leaflet, the valve will not close properly and blood can leak backwards into the heart and reduces blood flow to the rest of the body. Patients with bicuspid aortic valve disease typically require a valve repair or replacement surgery to prevent further health problems.

Though coarctation of the aorta (CoA) is typically developed at birth, many patients will not know they have it until adulthood. With CoA, the aorta is pinched and narrowed, making it hard for blood to flow easily out of the heart to the rest of the body. Because CoA requires the heart to work harder to force blood out through the aorta, these patients run the risk of developing high blood pressure, heart disease and aneurysms. Coarctation of the aorta can be treated through cardiac catheterization.

When a patient is born with mitral valve defects, they are at risk for developing valve disease, including mitral stenosis and mitral regurgitation because the valve function is compromised.

 

Types of mitral valve disease

• Mitral stenosis occurs when the valve flaps stiffen or fuse together, making the valve opening smaller and narrower, ultimately decreasing blood flow.
• Mitral regurgitation, also know as mitral insufficiency, occurs when the leaflets, or cusps, of the mitral valve do not close properly, allowing blood to flow backwards through the heart.

Mitral valve anomalies prevent the heart from effectively pumping oxygenated blood out off the heart and into the rest of the body. Normal treatments include a mitral repair or mitral replacement.

The double-outlet right ventricle defect (DORV) is a condition in which the aorta is connected to the right ventricle rather than the left ventricle. With DORV, the oxygen-poor blood from the right ventricle is being circulated through the aorta and pulmonary artery to the lungs. It is common for DORV patients to also have associated disorders or anomalies, such as narrowed arteries, ventricular septal defect and other conditions.

In a healthy heart, the tricuspid valve serves as the control center guiding blood from the right upper chamber to the right lower chamber. For patients born with Ebstein’s anomaly, the tricuspid does not have the necessary flaps to open and close the valve properly. This allows blood to leak backwards in the right upper chamber, which decreases the amount of blood that is being pumped to the lungs. Repairing or replacement the valve can restore proper blood flow to and from the heart.

Hypoplastic left heart syndrome (HLHS) is a birth defect that occurs when the left side of the heart if underdeveloped, with can lead to:

• Left ventricle is too small
• Mitral valve is too small
• Aortic valve is too small
• Ascending aorta is underdeveloped
• Hole between the left and right atrium

With this defects, the aorta and mitral valves do not function properly, making it difficult to pump oxygen-rich blood to the rest of the body because the heart is forced to use the right side to regular both incoming and outgoing blood flow.

Marfan syndrome is a genetic disorder that affects the connective tissue in the body, causing abnormalities in the cardiovascular system, as well as other healthcare problems in the skeletal (bones, tendons, ligaments) and ocular (eyes) systems. For patients with Marfan syndrome, it is common to experience a weakening of the aorta, the largest artery in the body, which can lead to aortic disease. This condition can be diagnosed through clinical evaluation and genetic testing, and while there is no cure for Marfan syndrome, we offer treatment options designed to help manage symptoms and improve their quality of life.

The ductus arteriosus is an opening in the heart that typically closed soon after birth. Patients with patent ductus arteriosus (PDA) have a persistent hole between the aorta and pulmonary arteries – two crucial vessels pumping blood from the heart.

 

Those with a small patent ductus arteriosus should not require any treatment, but if a larger patent ductus arteriosus remains untreated, patients will have blood flow insufficiency and risk further heart damage.

The tetralogy of fallot is a combination of four separate problems that can occur in the heart, impacting blood flows from the heart to the body:

• A hole in the lower chambers
• The aorta obstructs the hole
• The muscle around the lower chamber thickens
• Blockage from the heart to the lungs

Tetralogy of fallot prevents the normal passage of blood from the left side of the heart to the right side. Because of the hole(s) and blockage(s) in the heart, oxygen-poor blood is mixed with oxygen-rich blood in both the right and left chambers, which depletes the supply of oxygen-rich blood needed for the lungs and other vital organs.

Stenosis is characterized by a narrowing of the valve due to plaque build-up (atherosclerosis). When the valve thickens or narrows, the opening becomes smaller and blood cannot pass through the valve as easily. With stenosis, the heart works hard to compensate for the constricted opening and blood flow is reduced to other chambers of the heart and/or to vital organs. In severe cases, heart valve surgery or interventional procedures may be necessary to prevent serious health complications. Patients can be diagnosed with:

• Mitral valve stenosis
• Aortic valve stenosis
• Tricuspid valve stenosis
• Pulmonary valve stenosis

A ventricular septal defect (VSD) is an opening in the intraventricular septum, the wall between the left and right ventricles (lower chambers of the heart). VSDs can be congenital or develop later in life after experiencing a heart attack. The hole allows blood from the left ventricle to flow back into the right ventricle and can lead to low blood pressure, low oxygen content and even heart failure.

 

Types of VSD

• Perimembranous is an opening in the upper section of the septum and is most commonly repaired through open-heart surgery.
• Muscular is the most common type of VSD. Muscular VSD can close spontaneously without intervention or and can be treated using an interventional transcatheter procedure.
• Conal Septal is the rarest VSD and can be found below the pulmonic valve.
• Infarct-Related VSD occurs when a portion of the heart is being deprived of blood, such as during a heart attack. When the heart experiencing prolonged deprivation, heart tissue may begin to die and develop a hole in what previously normally functioning heart tissue.