Simple ventricular septal defects are the most common form of congenital heart disease. A ventricular septal defect is a hole in the wall between the right and left ventricles (ventricular septum). A VSD can potentially cause a shunting of blood from the left ventricle to the right ventricle or from the right ventricle to the left ventricle. The position and size of the VSD determine the physiology and, in turn, dictate the timing of intervention.
The ventricular septum is made up of two components, the truncal portion and the conoseptal portion. These two sections fit together like two pieces of a puzzle. The position of the VSD among these two portions of the septum determines the type of VSD.
Five Types of VSD
A conoventricular VSD results when there is a space between where the two portions of the ventricular septum meet. This type of VSD is not typically associated with other forms of congenital heart disease. The size of a conoventricular VSD is the predominate indicator of physiology. A small conoventricular VSD may close on its own as the child grows. A larger VSD may cause greater strain on the heart and need to be repaired surgically. Surgical repair entails patch closure of the VSD using a synthetic material.
Muscular VSDs are the most common type of VSD and are not usually associated with other forms of congenital heart disease. A muscular VSD is a hole located in the truncal portion of the ventricular septum. Again, size is the predominate indicator of physiology. This type of VSD has the highest chance of spontaneous closure and therefore requires less surgery.
A conoseptal VSD is a hole located in the conoseptal portion of the ventricular septum. This type of VSD has almost no chance for spontaneous closure and often requires surgical repair.
An Atrioventricular Canal Type VSD is a hole located in the upper portion of the ventricular septum. Often, this type of VSD is associated with a large ASD as well as malformed atrioventricular valves in a complex congenital heart disease known as Complete Common Atrioventricular Canal Defect (see below).
An Atrioventricular Canal Type VSD allows oxygen-rich blood from the left ventricle to pass into the right ventricle resulting in increased blood flow to the lungs. This type of VSD has no chance of spontaneous closure. Surgical repair is required and involves patch closure of the VSD using a synthetic material
Malalignment of the conoseptal portion of the ventricular septum results in a malalignment VSD. This type of VSD causes one of the most common forms of congenital heart disease known as Tetralogy of Fallot. Because the two portions of the ventricular septum have failed to align properly, the anatomy of other structures in the heart are affected. Namely, there is less space for the growth of the pulmonary valve and artery resulting in pulmonary stenosis. In addition, the aorta is not aligned properly resulting in an overriding aorta (i.e. the aorta lies directly over the VSD). Finally, the right ventricle typically works at the lower, pulmonary pressure. Due to the presence of the VSD, the right and left ventricles are pumping at the same pressure. A secondary condition, known as right ventricular hypertrophy (enlargement of the right ventricle), is a result of the right ventricle working at systemic pressure.
The resistance of blood flow through the stenotic pulmonary valve results in deoxygenated blood flowing from the right ventricle through the VSD directly into the left ventricle. This deoxygenated blood is then pumped from the left ventricle out to the body causing the baby to appear cyanotic or blue. Corrective surgery involves patch closure of the VSD and enlargement of the narrow area of the pulmonary artery and right ventricle.
What is Normal Cardiac Anatomy?
When your child has a congenital heart defect, there's usually something wrong with the structure of his or her heart's structure.
Heart with Normal Cardiac Anatomy
When your child has a congenital heart defect, there's usually something wrong with the structure of his or her heart's structure.
The heart is composed of four chambers. The two upper chambers, known as atria, collect blood as it flows back to the heart. The two lower chambers, known as ventricles, pump blood with each heartbeat to the two main arteries (the pulmonary artery and the aorta). The septum is the wall that divides the heart into right and left sides. The atrial septum separates the right and left atria; likewise, the ventricular septum separates the two ventricles.
There are four valves that control the flow of blood through the heart. These flap-like structures allow blood to flow in only one direction. The tricuspid and mitral valves, also known as the atrioventricular valves, separate the upper and lower chambers of the heart. The aortic and pulmonary valves, also known as the arterial valves, separate the ventricles from the main arteries. Oxygen-depleted blood returns from the body and drains into the right atrium via the superior and inferior vena cavas. The blood in the right atrium then passes through the tricuspid valve and enters the right ventricle.
Next, the blood passes through the pulmonary valve, enters the pulmonary artery, and travels to the lungs where it is replenished with oxygen. The oxygen-rich blood returns to the heart via the pulmonary veins, draining into the left atrium. The blood in the left atrium passes through the bicuspid, or mitral, valve and enters the left ventricle.
Finally, the oxygen-rich blood flows through the aortic valve into the aorta and out to the rest of the body.
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Ventricular Septal Defect
VSDs are the most common congenital heart defect, and in most cases they're diagnosed and treated successfully with few or no complications.
What Is a Ventricular Septal Defect?
To understand this defect, it first helps to review some basics about the way a healthy heart typically works.
The heart has four chambers: The two lower pumping chambers of the heart are called the ventricles, and the two upper filling chambers are the atria.
In a healthy heart, blood that returns from the body to the right-sided filling chamber (right atrium) is low in oxygen. This blood passes to the right-sided pumping chamber (right ventricle), and then travels to the lungs to receive oxygen. The blood that has been enriched with oxygen returns to the left atrium, and then to the left ventricle. It's then pumped out to the body through the aorta, a large blood vessel that carries the blood to the smaller blood vessels in the body.
The right and left-sided pumping chambers (ventricles) are separated by shared wall, called the ventricular septum.
Kids with a VSD have an opening in this wall. As a result, when the heart beats, some of the blood in the left ventricle (which has been enriched by oxygen from the lungs) is able to flow through the hole in the septum into the right ventricle. In the right ventricle, this oxygen-rich blood mixes with the oxygen-poor blood and goes back to the lungs. The blood flowing through the hole creates an extra noise, which is known as a heart murmur. The heart murmur can be heard when a doctor listens to the heart beat with a stethoscope.
VSDs can be located in different places on the ventricular septum, and they can be different sizes. The symptoms and medical treatment of the VSD will depend on those factors. In some rare cases, VSDs are part of more complex types of congenital heart disease.
What Causes a VSD?
Ventricular septal defects occur during fetal heart development and are present at birth. During the first weeks after conception, the heart develops from a large tube, dividing into sections that will eventually become the walls and chambers. If a problem occurs during this process, it can create a hole in the ventricular septum.
In some cases, the tendency to develop a VSD may be due to genetic syndromes that cause extra or missing pieces of chromosomes. The vast majority of these defects, though, have no clear cause.
Signs and Symptoms
VSDs are usually found in the first few weeks of life by a doctor during a routine checkup. The doctor will be able to detect a heart murmur, which is due to the sound of blood as it passes between the left and right ventricles. The murmur associated with a VSD has certain features that allow a doctor to distinguish it from heart murmurs due to other causes.
The size of the hole and its location within the heart will determine whether a VSD causes any symptoms. Small VSDs will not typically cause any symptoms, and may ultimately close on their own. Older kids or teens who have small VSDs that persist usually don't experience any symptoms other than the heart murmur that doctors hear. They might need to see a doctor regularly to check on the heart defect and make sure it isn't causing any problems.
Moderate and large VSDs that haven't been treated in childhood may cause noticeable symptoms. Babies may have faster breathing and get tired out during attempts to feed. They may start sweating or crying with feeding, and may gain weight at a slower rate.
These signs generally indicate that the VSD will not close by itself, and cardiac surgery may be needed. This usually is done within the first 3 months of life to prevent other complications. A cardiologist can prescribe medication to lessen symptoms before surgery.
People with a VSD are at greater risk in their lifetime of developing endocarditis, an infection of the inner surface of the heart. This occurs when bacteria in the bloodstream infect the lining of the heart. Bacteria are always in our mouths, and small amounts are introduced into the bloodstream when we chew and brush our teeth. The best way to protect the heart from endocarditis is to to reduce oral bacteria by brushing and flossing daily, and visiting the dentist regularly. In general, it is not recommended that patients with simple VSDs take antibiotics before dental visits, except for the first 6 months after surgery.
Diagnosing a VSD
If your child is discovered to have a heart murmur, a doctor may refer you to a pediatric cardiologist, a doctor who specializes in diagnosing and treating childhood heart conditions.
In addition to doing a physical exam, the cardiologist take your child's medical history. If a VSD is suspected, the cardiologist may order one or more of these tests:
- a chest X-ray, which produces a picture of the heart and surrounding organs
- an electrocardiogram (EKG), which records the electrical activity of the heart
- an echocardiogram (echo), which uses sound waves to produce a picture of the heart and to visualize blood flow through the heart chambers. This is often the primary tool used to diagnose a VSD.
- a cardiac catheterization, which provides information about the heart structures as well as blood pressure and blood oxygen levels within the heart chambers. This test is usually performed for VSD only when additional information is needed that other tests cannot provide.
Treating a VSD
Once an VSD is diagnosed, treatment will depend on the child's age and the size, location, and severity of the defect. A child with a small defect that causes no symptoms may simply need to visit a cardiologist regularly to make sure that there are no other problems.
In most kids, a small defect will close on its own without surgery. Some might not close but do not get any larger. Kids whose VSD is small and has not closed generally won't have to restrict their physical activities.
For kids with medium to large VSDs, surgery may be necessary. In most cases, this takes place within the first few weeks to months of life. In this procedure, the surgeon makes an incision in the chest wall and a heart-lung machine will maintain circulation while the surgeon closes the hole. The surgeon can stitch the hole closed directly or, more commonly, sew a patch of manmade surgical material over it. Eventually, the tissue of the heart heals over the patch or stitches, and by 6 months after the surgery, the hole will be completely covered with tissue.
Certain types of VSDs may be closed by cardiac catheterization. A thin, flexible tube (a catheter) is inserted into a blood vessel in the leg that leads to the heart. A cardiologist guides the tube into the heart to make measurements of blood flow, pressure, and oxygen levels in the heart chambers. A special implant, shaped into two disks formed of flexible wire mesh, is positioned into the hole in the septum. The device is designed to flatten against the septum on both sides to close and permanently seal the VSD.
After healing from surgery or catheterization, kids with VSDs are considered cured and should have no further symptoms or problems.
Caring for a Child With a VSD
Some kids with VSDs may take heart medication prior to surgery to help lessen the symptoms from the defect. Those who have surgery for larger VSDs usually leave the hospital within 4 to 5 days after surgery if there are no problems.
In most cases, kids who have had VSD surgery recover quickly and without problems. But doctors will closely monitor the child for signs or symptoms of any problems.
Your child may undergo another echocardiogram to make sure that the heart defect has closed completely. If your child is having trouble breathing, call your doctor or go to the emergency department immediately.
Other symptoms that may indicate a problem include:
- a bluish tinge or color (cyanosis) to the skin around the mouth or on the lips and tongue
- poor appetite or difficulty feeding
- failure to gain weight or weight loss
- listlessness or decreased activity level
- prolonged or unexplained fever
- increasing pain, tenderness, or pus oozing from the incision
Call your doctor if you notice any of these signs in your child after closure of the VSD.
Any time a child is diagnosed with a heart condition, it can be scary. But the good news is that your pediatric cardiologist will be very familiar with this condition and how to best manage it. Most kids who've had a VSD corrected have a normal life expectancy and go on to live healthy, active lives.
Reviewed by: Steven B. Ritz, MD
Date reviewed: May 2013