My heart

So, I've been thinking about my heart. I think that it's been about 7 (I was pregnant with Cameron) years since I last saw a cardiologist. I don't like them telling me what to do, or what's wrong with me. I hate all of the x rays, EKG's and echo cardiograms. I prefer to think that everything is just hunky dory. However, I was told 9 years ago that I do have minor leakage on a couple of valves. Who knows what that could lead to. I'm sure I was told at that time, but I can't remember right now. I was told when I was around 20 that I may possibly need a pace maker when I was older. Who wants to hear that when they're 20? See, so if I don't go back, I won't hear things that I don't want to hear. Like the fact that I should be doing regular exercise. Is once or twice every 1 to 2 weeks regular enough? No. Then I guess that lately, I have been a slacker. I've just felt very frustrated by my feet. I'm trying to do the whole mind over matter thing and tell my brain that my feet don't hurt and that I'll be just fine. I'm still being a little whoosy. I give in and convince myself that it will be easier and better if I stop. I know that Ryan is concerned about me and my heart. He doesn't want me dropping dead because then who would cook the dinners that nobody will eat. :0) (I of course, know there's so much more to it than that.) I'm doing the exercises for my feet that Ryan found. I just need to stop being afraid that they might cramp up when I run. So, I guess it's time to stop being lazy and it's time to get back to a cardiologist. So Dad, I suppose I will finally ask for the number of the doctor you have been seeing.

The following is a description of the birth defect I had:

What is total anomalous pulmonary venous return?


When a heart is functioning normally, a system of vessels carries blood to and from the heart. Total anomalous pulmonary venous return (TAPVR), a congenital (present at birth) heart defect, occurs when the vessels that bring oxygen-rich (red) blood back to the heart from the lungs are improperly connected.
Normally, oxygen-poor (blue) blood returns to the right atrium from the body, travels to the right ventricle, then is pumped through the pulmonary artery into the lungs where it receives oxygen. Oxygen-rich (red) blood returns to the left atrium from the lungs through four pulmonary veins. It then passes into the left ventricle, and is pumped through the aorta out to the body.

Total anomalous pulmonary venous return: In TAPVR, the four pulmonary veins are not connected to the left atrium as they should be. Instead, there are several possible places where the pulmonary veins can connect incorrectly. The most common connection is to a blood vessel that brings oxygen-poor (blue) blood back to the right atrium, the superior vena cava.
In TAPVR, oxygen-rich (red) blood that should return to the left atrium, the left ventricle, the aorta and then the body, instead mixes with the oxygen-poor (blue) blood flowing into the right side of the heart. This creates a critical situation, because there is no way for oxygen-rich (red) blood to be delivered to the body.
Other heart defects are often associated with total anomalous pulmonary venous return, and they actually help the heart of an infant with TAPVR to function until treatment, including surgery, is possible. These defects are:

An opening in the atrial or ventricular septum, called an atrial septal defect or a ventricular septal defect, will allow blood from one side to mix with blood from another, creating "purple" blood with an oxygen level somewhere in between that of the oxygen-poor (blue) and the oxygen-rich (red) blood.
Patent ductus arteriosus also will allow mixing of oxygen-poor (blue) and oxygen-rich (red) blood through the connection between the aorta and pulmonary artery. The "purple" blood that results from this mixing is beneficial, providing at least a little oxygen to the body.
Total anomalous pulmonary venous return occurs in one out of every 15,000 live births. It occurs in boys just as often as in girls.

What causes total anomalous pulmonary venous return?

The heart forms during the first eight weeks of fetal development. The problem occurs during this time, allowing the pulmonary veins to be attached incorrectly.

TAPVR can be caused by a number of factors, though most of the time this heart defect occurs sporadically (by chance), with no apparent reason for its development.

Some congenital heart defects may have a genetic link, either occurring due to a defect in a gene, a chromosome abnormality or environmental exposure, causing heart problems to occur more often in certain families.

Why is TAPVR a concern?

Babies with TAPVR cannot supply oxygen-rich (red) blood to the body after birth. Without one of the additional heart defects (mentioned above) that allows mixing of oxygen-poor (blue) and oxygen-rich (red) blood, such as an atrial or ventricular septal defect, infants with TAPVR will have a mixture of oxygen-rich (red) and oxygen-poor (blue) blood circulating through the right heart and back to the lungs -- a situation that is critical.

In all newborns, two fetal "shunts," or connections present in the fetal circulation, remain open for a short while after birth:

The foramen ovale, an opening between the right atrium and the left atrium, will allow some of the blood returning to the right atrium to flow into the left side of the heart, and out to the body.
The ductus arteriosus, connecting the aorta to the pulmonary artery, allows some blood to mix between the two vessels, allowing more partially oxygenated blood to go to the body.
When these two "shunts" close, however, a baby with TAPVR will become very blue (cyanotic) unless other connections are present, such as an atrial or ventricular septal defect. Even with an additional defect present that allows mixing, babies with TAPVR often will not have enough oxygen in the bloodstream to meet the body's demands.

Another problem that occurs with TAPVR is that too much blood flows through the blood vessels in the lungs, since the pulmonary veins add extra blood back into the circulation on the right side of the heart. The lungs become stressed by this extra blood flow, and can eventually become damaged from it.

What are the symptoms of total anomalous pulmonary venous return?

The obvious sign of TAPVR in a newborn is cyanosis (blue coloring of the skin, lips and nailbeds) in the first days of life after the maternal source of oxygen (from the mother's placenta) is removed. The degree of cyanosis is related to the presence of other defects that allow blood to mix.

Babies with large atrial or ventricular septal defects, allowing a mixture of oxygen-rich (red) and oxygen-poor (blue) blood to enter the left heart and go to the body, may have less cyanosis and fewer symptoms at birth. There also may be a delay in symptoms of a few days or weeks after birth in babies who have a large foramen ovale or a ductus arteriosus that remains open for a while.

The following are the other most common symptoms of total anomalous pulmonary venous return. Each child may experience symptoms differently. Symptoms may include:

rapid breathing
labored breathing
rapid heart rate
cool, clammy skin
lethargy
poor feeding
The symptoms of total anomalous pulmonary venous return may resemble other medical conditions or heart problems. Always consult your child's physician for a diagnosis.

How is total anomalous pulmonary venous return diagnosed?

A pediatric cardiologist and/or a neonatologist may be involved in your child's care. A pediatric cardiologist specializes in the diagnosis and medical management of congenital heart defects, as well as heart problems that may develop later in childhood. A neonatologist specializes in illnesses affecting newborns, both premature and full-term.

Cyanosis and rapid breathing are major indications that there is a problem with your newborn. Your child's physician also may have heard a heart murmur during a physical examination. A heart murmur is simply a noise caused by the turbulence of blood flowing through the openings that allow the blood to mix.

Other diagnostic tests are needed to help with the diagnosis, and may include the following:

Chest X-ray — A diagnostic test that uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.
Electrocardiogram (ECG or EKG) — A test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias) and detects heart muscle stress.
Echocardiogram (echo) — A procedure that evaluates the structure and function of the heart by using sound waves, recorded on an electronic sensor, that produce a moving picture of the heart and heart valves.
Cardiac Catheterization — A procedure that gives very detailed information about the structures inside the heart. Under sedation, a small, thin, flexible tube (catheter) is inserted into a blood vessel in the groin and guided to the inside of the heart. Blood pressure and oxygen measurements are taken in the four chambers of the heart, as well as in the pulmonary artery and aorta. Contrast dye is injected to more clearly visualize the structures inside the heart.
What are the treatments for TAPVR?

Specific treatment for TAPVR will be determined by your child's physician based on:

your child's age, overall health and medical history
extent of the disease
your child's tolerance for specific medications, procedures or therapies
how your child's doctor expects the disease may progress
your opinion or preference
Your child may be admitted to the intensive care unit (ICU) or special care nursery once symptoms are noted. Initially, your child may be placed on oxygen or a ventilator to assist his/her breathing. Intravenous medications may be given to help the heart and lungs function more efficiently.

Other important aspects of initial treatment include the following:

Cardiac Catheterization — A procedure that can be used as a diagnostic procedure, as well as an initial treatment procedure for some heart defects. A cardiac catheterization procedure usually will be performed to evaluate the defect(s) and the amount of blood that is mixing.
As part of the cardiac catheterization, a procedure called balloon atrial septostomy may be performed to improve mixing of oxygen-rich (red) and oxygen-poor (blue) blood.
A special catheter with a balloon in the tip is used to create an opening in the atrial septum (wall between the left and right atria).
The catheter is guided through the foramen ovale -- a small opening present in the atrial septum that closes shortly after birth -- and into the left atrium.
The balloon is inflated.
The catheter is quickly pulled back through the hole, into the right atrium, enlarging the hole allowing blood to mix between the atria.
Medication — Intravenous medication called prostaglandin E1 may be given to keep the ductus arteriosus from closing.
Surgical Repair — The surgical repair of anomalous pulmonary veins is carried out through an incision in the middle of the chest. The breastbone is split in the middle and spread apart to expose the heart. A heart-lung machine is used to do the work of the heart while the heart is cooled, stopped, emptied and opened. The abnormal veins are redirected to the left atrium, usually by connecting them directly to the back wall of the atrium. The heart is restarted as the heart-lung machine is withdrawn.
What is the long-term outlook after TAPVR surgical repair?

Many infants who have had TAPVR surgical repair will grow and develop normally. After TAPVR repair, however, your infant will need to be followed periodically by a pediatric cardiologist, who will make assessments to check for any heart-related problems.

As some children grow, the area where the pulmonary arteries were reconnected to the left atrium may become narrowed, preventing blood from moving from the lungs to the left atrium. Treatment may include:

Placement of a wire mesh device called a stent into the narrowed vein(s) to open it, done during a cardiac catheterization procedure.
Surgical enlargement of the narrowed pulmonary vein connection(s).
Consult your child's physician regarding the specific outlook for your child.

What is the latest research on TAPVR?

Complementing the Cardiovascular Program of Children's Hospital Boston is the nation's most intensive clinical and basic research program focused on pediatric heart disease. Children's is a world leader in opening new avenues of "translational research," bringing laboratory advances to the bedside and doctor's office as quickly as possible. All senior medical staff members of the Cardiovascular Program participate in clinical research activities.

Children's Hospital Boston has pioneered interventional catheterization repair of many types of congenital heart defects, including initial repair of TAPVR.