Congenital Heart Defects

Adopt Special Needs: Special Needs - Congenital Heart DefectsA normal heart has valves, arteries and chambers that carry the blood to the body. When all chambers and valves work correctly, the blood is pumped through the heart, to the lungs for oxygen, back the heart and out to the body with oxygen. When valves, chambers, arteries and veins are malformed, this circulation pattern can be impaired.

Congenital heart defects are structural problems with the heart that are present at birth. They result when a mishap occurs during heart development soon after conception and often before the mother is aware that she is pregnant. Defects range in severity from simple problems, such as "holes" between chambers of the heart, to very severe malformations, such as complete absence of one or more chambers or valves.

Below is a list of the most common congenital heart defects seen in children waiting for forever families. We provide the following information for reference purposes only and cannot attest to the accuracy of the information. We highly recommend speaking with an experienced physician for further details on each condition.

Common Congenital Heart Defects

Atrial Septal Defect

Adopt Special Needs: Special Needs - Congenital Heart Defects - ASD


Other common names: 
ASD

An Atrial Septal Defect (ASD) is a hole in the wall between the two upper chambers of the heart. An ASD allows freshly oxygenated blood to flow from the left upper chamber of the heart (left atrium) into the right upper chamber of the heart (right atrium). Oxygenated blood is then allowed to travel to the lungs along with deoxygenated blood. If not treated, the right side of the heart eventually enlarges and weakens. Over time, there will be less oxygen in the blood that goes to the body. In some cases, the blood pressure in your lungs increases as well, leading to pulmonary hypertension. Many babies born with smaller atrial septal defects don't have outward signs or symptoms. ASD's are usually discovered when a child experiences a shortness of breath, fatigue, swelling of the legs, or a bluish skin color. An examination by a doctor may also help detect a heart murmur or skipped beats. 

Causes:
Congenital heart defects are generally caused by an error in heart development in utero. The exact cause is unknown, although could be attributed to genetic or environmental factors. Rubella or drug and alcohol use during pregnancy increase the risks that a baby will be born with CHD. 

Possible complications:
Small ASDs typically present no additional concerns. If left untreated, larger ASDs can cause right-sided heart failure, rhythm abnormalities, a shortened life expectancy and an increased risk of stroke. Less common, but possible complications also include pulmonary hypertension (increased blood pressure in the lungs) or Eisenmenger Syndrome (permanent lung damage from pulmonary hypertension). 

Treatment:
Small ASDs typically require no treatment and will close naturally as the child ages.  Larger ASDs require surgery. Surgery involves patching the abnormal opening. This can be done through two methods:

  • Cardiac catheterization. A thin tube (catheter) is inserted into a blood vessel in the groin and guided to the heart. Through the catheter, a mesh patch or plug is put into place to close the hole. The heart tissue grows around the mesh, permanently sealing the hole.
  • Open-heart surgery. This type of surgery is done under general anesthesia and requires the use of a heart-lung machine. Through an incision in the chest, surgeons use patches or stitches to close the hole.

Prognosis:
In infants small ASDs (less than 5 mm) will often close on their own or cause no problems. Larger ASDs (8 to 10 mm) most often do not close and may need a procedure. There are several factors that would determine the prognosis following surgery including the size of the defect, the amount of extra blood flowing through the opening, and how long the child has gone without surgery. Some children with an ASD may have other congenital heart conditions, such as a leaky valve or a hole in another area of the heart. 

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Complete Transposition of the Great Arteries

Adopt Special Needs: Special Needs - Congenital Heart Defects - TGA


Other common names:
TGA, Transposition of the Great Vessels

Transposition of the Great Arteries is a very rare congenital heart defect in which the two main arteries leaving the heart are transposed - or reversed.This changes the way the blood circulates through the body. The blood from the heart that circulates through the body does not have enough oxygen. Children with TGA will often have a bluish tint to their skin (cyanosis), clubbing of the fingers and toes (rounded nails), difficulty feeding, and shortness of breath. These symptoms are typically significant enough to be noticed without an intensive medical exam. Doctors will perform a series of exams to determine the proper treatment plan. 

Causes:
Although some factors, such as rubella or other viral illnesses during pregnancy, maternal age over 40, or maternal diabetes, may increase the risk of this condition, in most cases the cause is unknown.

Possible complications:
With TGA, a baby's tissues will receive too little oxygen. Unless there's some mixing of oxygen-rich blood and oxygen-poor blood within the baby's body, he or she won't be able to survive. Heart failure — a condition in which the heart can't pump enough blood to meet the body's needs — may develop over time because the right ventricle is pumping under higher pressure than usual. This added stress may make the muscle of the right ventricle stiff or weak. The lack of oxygenated blood also causes damage to the lungs, making breathing difficult. Even after surgery, complications such as narrowing of the coronary arteries, arrythmias, weak heart muscles and leaky valves can cause issues. 

Treatment:
Typically a baby would receive medication to keep a blood vessel called the the ductus arteriosus open, allowing some mixing of the two blood circulations. Treatment is recommended within the first week of life and surgery within the first month, however, many children adopted internationally will not have early intervention, which puts them at greater risk for additional complications. There are two surgical options used to correct TGA:

  • Arterial switch operation. This surgery is used most often use to correct TGA. During this operation, the pulmonary artery and the aorta are moved to their normal positions: The pulmonary artery is connected to the right ventricle, and the aorta is connected to the left ventricle. The coronary arteries also are reattached to the aorta.

  • Atrial switch operation. In this surgery, the surgeon makes a tunnel (baffle) between the heart's two upper chambers (atria). This diverts the oxygen-poor blood to the left ventricle and the pulmonary artery and the oxygen-rich blood to the right ventricle and the aorta. With this procedure, the right ventricle must pump blood to the entire body, instead of just to the lungs as it would do in a normal heart. Possible complications of the atrial switch operation include irregular heartbeats, baffle obstructions or leaks, and heart failure due to problems with right ventricle function. Children adopted internationally are more likely to have had this procedure. 

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Double Outlet Right Ventricle

Adopt Special Needs: Special Needs - Congenital Heart Defects - DORV


Other common names:
DORV, Taussig-Bing anomaly; DORV with doubly-committed VSD; DORV with noncommitted VSD; DORV with subaortic VSD

Normally the aorta arises from the left ventricle (the chamber of the heart that usually pumps blood to the body). The pulmonary artery normally arises from the right ventricle. With Double Outlet Right Ventricle, both arteries arise from the right ventricle. This is a problem because the right ventricle carries oxygen-poor blood, which the aorta then carries throughout the body. DORV always includes a ventricular septal defect (VSD). Pulmonary valve stenosis, or transposition of the great arteries may also be part of the defect. Children with DORV may have shortness of breath, fatigue, swelling of the legs, a bluish skin color, or excessive sweating. 

Causes:
Congenital heart defects are generally caused by an error in heart development in utero. The exact cause is unknown, although could be attributed to genetic or environmental factors. Rubella or drug and alcohol use during pregnancy increase the risks that a baby will be born with CHD. 

Possible complications:
Complications from DORV may include Congestive Heart Failure, pulmonary hypertension, and irreversible damage to the lungs. 

Treatment:
DORV requires open-heart surgery. The type of surgery depends on the individual child's condition. A patch may be used to create a tunnel between the right and left ventricles. This allows the oxygenated blood to be moved to the correct ventricle. In more extensive procedures, surgeons may detach and reattach the aorta to the correct ventricle. In more serious cases, either ventricle may be too small or malformed to ever function correctly. Surgeons will reconfigure the heart and circulatory system so that the heart functions with one ventricle (pumping chamber), instead of two. This is called a Fontan procedure and and often requires three open heart surgeries. Many children adopted internationally will not have early intervention, which puts them at greater risk for additional complications.

Prognosis:
The prognosis depends on the severity of the defects as well as the presence of other defects in the heart. Most children do not require continuous intervention and lead healthy lives.

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Endocardial Cushion Defect

Adopt Special Needs: Special Needs - Congenital Heart Defects - ECD


Other common names: 
ECD, Atrioventricular (AV) canal defect, Atrioventricular septal defect, AVSD

The endocardial cushions eventually develop into the wall (septum) that separates the four chambers of the heart. They also form the mitral and tricuspid valves, the valves that separate the atria (top collecting chambers) from the ventricles (bottom pumping chambers). With Endocardial Cushion Defect, these walls do not form properly. There are two types of ECD:

  • Complete ECD: A complete ECD involves an atrial septal defect (ASD) and a ventricular septal defect (VSD) along with only one large heart valve (common AV valve) instead of two distinct valves (mitral and tricuspid).
  • Partial (or incomplete) ECD: Only an ASD and VSD are present. There are two distinct valves, but one of them (the mitral valve) is often abnormal with an opening ("cleft") in it, often letting blood leak back through the valve.

A child with ECD may experience fatigue, cyanosis (bluish tint of the skin), lack of appetite, rapid breathing or heartbeat, sweating, swollen legs or trouble breathing. 

Causes:
Congenital heart defects are generally caused by an error in heart development in utero. The exact cause is unknown, although could be attributed to genetic or environmental factors. Rubella or drug and alcohol use during pregnancy increase the risks that a baby will be born with CHD. 

Possible complications:
ECD is strongly associated with Down syndrome. ECD may also be associated with other congenital heart defects such as: Double outlet right ventricle, Single ventricle, Transposition of the great vessels or Tetralogy of Fallot. 

Treatment:
Surgery is needed to close the holes between the heart chambers and to separate the common valve into distinct tricuspid and mitral valves. Babies with Down syndrome tend to develop lung disease earlier, and therefore early surgery is very important for these babies. Surgery would typically be done in the first year of life before irreversible lung damage occurs; however many children who are adopted internationally may not have had surgery this young. 

Prognosis:
The prognosis for a child with ECD depends on the severity of the ECD, the child's overall health, and whether lung disease has already developed. Many children with early intervention live normal, active lives after the ECD is corrected. Long-term complications, especially for those children with delayed treatment, include congestive heart failure, Eisenmenger, pulmonary hypertension, and irreversible damage to the lungs. 

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Patent Foramen Ovale

Adopt Special Needs: Special Needs - Congenital Heart Defects - PFO


Other common names: 
PFO

During development, a small flap-like opening (foramen ovale) is usually present between the right and left upper chambers of the heart. It normally closes naturally during infancy. When the foramen ovale doesn't close, it's called a Patent Foramen Ovale. It is estimated that 1 in 5 adults has a PFO; however, most people with the condition never know they have it because it usually has no symptoms. A PFO is often discovered during tests for other problems.

Causes:
Congenital heart defects are generally caused by an error in heart development in utero. The exact cause is unknown, although could be attributed to genetic or environmental factors. Rubella or drug and alcohol use during pregnancy increase the risks that a baby will be born with CHD. 

Possible complications:
A PFO usually does not cause any issues. Some studies have found the disorder is more common in individuals with certain conditions, such as unexplained stroke and migraine with aura. In most cases, there are other reasons for these conditions, and it's just a coincidence the person also has a patent foramen ovale. The possible link between patent foramen ovale and stroke or migraine is controversial and research studies are ongoing.

Treatment:
Treatment is usually not necessary as PFOs do not typically present any issues. Surgery is typically only used to correct a PFO if a child is having heart surgery for other defects. 

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Pulmonary Atresia

Adopt Special Needs: Special Needs - Congenital Heart Defects - PA


Other common names:
PA, Pulmonary atresia - intact ventricular septum, PA/IVS

Pulmonary Atresia means the pulmonary valve hasn't formed correctly or is closed. The Pulmonary Valve allows blood to flow from the lower right chamber (right ventricle) of the heart to the lungs. Children with PA may also have an underdeveloped tricuspid valve. They may also have an underdeveloped right ventricle and abnormal blood vessels feeding the heart. Pulmonary atresia may occur with or without a ventricular septal defect (VSD). Symptoms of PA are similar to other heart defects: bluish skin, shortness of breath, trouble eating, and fatigue. 

Causes:
Congenital heart defects are generally caused by an error in heart development in utero. The exact cause is unknown, although could be attributed to genetic or environmental factors. Rubella or drug and alcohol use during pregnancy increase the risks that a baby will be born with CHD. 

Possible complications:
Children with PA are at greater risk for delayed growth and development, seizures, stroke, heart failure and death.

Treatment:
Treatment would typically include medication to help blood move through the lungs. Surgery may also be necessary. There are several surgical options: a thin, flexible tube (heart catheterization) to repair the problem, open heart surgery to repair or replace the valve or to place a tube between the right ventricle and the pulmonary arteries, reconstructing the heart as a single ventricle, or, in extreme cases, a heart transplant. Many children adopted internationally will not have early intervention, which puts them at greater risk for additional complications. 

Prognosis:
The prognosis for PA is generally very good. However, how well a child does depends on the severity of the defect and whether or not they have additional heart defects. 

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Tetralogy of Fallot

Adopt Special Needs: Special Needs - Congenital Heart Defects - TOF
Other common names:
TOF, Tet

Tetralogy of Fallot affects the blood flow in the heart and is made up of four different heart defects:

  • VSD
  • Pulmonary stenosis: A narrowing of the pulmonary valve and main pulmonary artery. 
  • The aortic valve, which opens to the aorta, is enlarged and seems to open from both ventricles. In this defect, the aortic valve sits directly on top of the ventricular septal defect.
  • The muscular wall of the lower right chamber of the heart (right ventricle) is thicker than normal. 

These defects cause oxygen-poor blood to be pumped through the body. Children with TOF experience the same symptoms as children with most heart defects: cyanosis (bluing of the skin), fatigue, trouble eating or gaining weight, and clubbed fingers. 

Causes:
Congenital heart defects are generally caused by an error in heart development in utero. The exact cause is unknown, although could be attributed to genetic or environmental factors. Rubella or drug and alcohol use during pregnancy increase the risks that a baby will be born with CHD. 

Possible complications:
Children with TOF are at greater risk of endocarditis (infection in the heart), arrhythmia, dizziness, fainting, seizures and delayed growth and development. 

Adopt Special Needs: Special Needs - Congenital Heart Defects - TOFTreatment:
Children with TOF will need to have open heart surgery. Ideally, a child would have a intracardiac repair within the first year of life. The surgeon will patch the VSD and repair the narrow pulmonary valve and artery. If a child is not ready for an intracardiac repair (premature or underdeveloped artery), surgeons will do a temporary repair called a bypass or shunt between the aorta and pulmonary artery. This helps increase blood flow to the lungs so the baby can grow stronger for intracardiac repair. Many children adopted internationally will either have received no surgery at all or had a shunt procedure.

Prognosis:
Children who have had an intracardiac repair, generally do very well. However, they will still need to be monitored for complications. Possible complications include a leaky pulmonary valve or restricted blood flow. Children with these issues may require an additional surgery or a valve replacement. Prognosis depends significantly on the severity of the defects and how long the child went without corrective surgery. 

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