Pediatric Cardiac Surgery - Understanding Congenital Heart Defects

Congenital heart defects are a malformation in one or more structures of the heart or blood vessels that occurs before a child is born, during the development of the fetus. This developmental defect can affect approximately 8 out of every 1000 children and stems from a variety of causes. While some expectant mothers will fault their own actions when this occurs, fearing the worst for their children, modern technology has brought us to a point where traditional treatments using medication and pediatric cardiac surgery are able to correct most heart defects.

Pediatric Heart Surgery - The Underlying Cause of Congenital Heart Defects

In the majority of patients, the cause for their congenital heart defect is not directly or readily known. Through research however, pediatric specialists have discovered a number of factors that can contribute to or are associated with an increased chance of a child developing a congenital heart defect. This includes:

* Genetic abnormalities or abnormalities in the chromosomes (e.g. - Down syndrome)

* Alcohol or recreational drug use/abuse during pregnancy

* Taking certain prescription medications during pregnancy

* Viral infections during the first trimester of pregnancy, such as rubella

In cases where there is a family history of heart defects, a child has double the chance (16 in 1000) of being born with a defect that may require corrective pediatric cardiac surgery or some other form of treatment.

Defining Congenital Heart Defects

There are several defects that are detected and treated early on in infancy.

Heart Valve Defects - Any one or more of the valves in the heart may malfunction through narrowing or stenosis. Also, complete closure of a valve that impeded/prevents blood flow can occur. Other heart defects include leaky valves that don't close. This reduces the pressure, forces the heart to work harder and allows blood to leak backwards as the chambers of the heart compress.

Patent Ductus Arteriosus - This defect allows blood to bypass the lungs as it circulates, forcing unoxygenated blood back through the body. This starves the body of oxygen and as a result the heart must work harder to oxygenate the tissues of the body.

Transposition of Greater Vessels - Blood from the left and the right side of the heart intermix because the arterial connections in the heart are incorrect.

Aortic Coarctation - This is a pinched Aorta. The narrowing of the Aorta can increase pressure and reduce circulation through the body, creating a variety of symptoms including a failure to thrive. It may also be present with no symptoms.

Pediatric Cardiac Surgery - Diagnosing Congenital Heart Disease

It's important to note that while congenital defects typically develop early on, they can be diagnosed before birth, after birth, throughout childhood later in life when the patient is an adult. For some adults, they live with a heart defect and present with no symptoms or issues. Depending on the patient, the assumed defect, the age of child and other factors, a number of tests can be ordered to check for and confirm a diagnosis. This includes:

* Echocardiogram

* Cardiac Catheterization

* Chest X-ray

* Electrocardiogram

* MRI

Pediatric Heart Surgery & Other Treatments for Congenital Heart Defects

It's typical for congenital heart defects in children to require pediatric cardiac surgery or some kind of interventional procedure in order to repair the defect. To ensure the heart is able to recover and grow normally after birth, children are often treated with medication to help improve the function of the heart after surgical repairs are complete.

Grote Pitman Campbell

Hole In Heart - Congenital Heart Defect

If you have a hole in heart then you could be in big trouble. This is what is called a congenital heart defect and occurs between the chambers of the heart in the tissues that separate the chambers called the septum.

Congenital heart defects, or septal defects, are fairly common, according to the AHA, or American Heart Association. The AHA estimates that approximately 1.3 million people have this type of heart defect. These defects can have quite serious side effects.

The first and foremost symptom that may be noticed is an arrhythmia. An arrhythmia is when the heart is unable to beat a nice constant rhythm. The heart beat is erratic and speeds up or slows down at irregular intervals. The heart can even skip a beat or two. If the heart muscle stretches out due to blood pumping between the chambers then the heart will not ever be the same and cannot recover. You may be able to tell you have an arrhythmia just by feeling your heart beat. If it seems irregular, talk to your doctor about the causes.

Another problem that may arise with a hole in heart is pulmonary hypertension. Blood is pumped from the right lower ventricle of the heart to the lungs to get oxygen. Then the blood goes back to the heart where it is supposed to be pumped through to the rest of the body.

If there is a septal defect the blood really does not know exactly where to go and the heart has to work so much harder to get the blood from the wrong chamber to the correct one. 

When bad blood continually mixes with good blood then the arterial walls start to change and they get thicker increasing the pressure of the blood flowing through them. This can be a precursor to congestive heart failure, a very serious condition.

Because the blood is not flowing normally, then the chance for blood clots forming in the heart and traveling to other parts of the body is enormous. If the blood pools in the right side of the heart and there is a septal defect the clot can travel through the defect to the left side of the heart and get pumped out to any number of bodily structures, including the brain. This is called a stroke, also, very serious.

When the heart has a hole in it, it has to work a lot harder than a normal heart. The energy drain on the rest of the body is huge and there is not much left for the body to have for normal growth and development. This is called failure to thrive. Children with this condition just do not seem to ever have enough energy to play like the other normal kids.

Because the heart has to work harder it gets taxed. Well, these are some taxes that it may not be able to pay for an extended period of time. This is called heart failure. If too many arterial changes have occurred because a hole in heart caused pulmonary hypertension, the heart will eventually fail.

"This article is not to be considered medical advice of any kind and is only for informational and entertainment purposes only. As always you, the reader, should consult with your personal physician or another Licensed Health Professional."

Tomazu Scurcia

Congenital Heart Defect

Congenital heart defect is a defect in the composition of the heart and vessels of a newborn. The majority heart defects either block blood flow in the heart and vessels near it or cause blood to flow through the heart in an abnormal pattern, although other defects affecting heart rhythm such as long QT syndrome can also occur. Heart defects are among the most common birth defects and are the leading cause of birth defect-related deaths.

Congenital Heart Defects - Symptoms

Congenital heart defects because a wide range of symptoms. Your baby may have only mild symptoms and tire easily, for example. They may have serious symptoms, like severe difficulty breathing. Or your baby may not have any symptoms that you observe at birth but may develop them later as he or she grows.

Common symptoms of a congenital heart defect include:

o Difficulty breathing. This often is noticed when your baby is active, such as during feeding and crying. 

o Poor weight gain. When most of a baby's energy is spent pumping blood to the body, little is left for eating and growing. Your baby may tire when eating and may take longer than expected to finish feeding. 

o Sudden weight gain or puffiness and swelling of the skin, seen most often around the eyes and in the hands and feet and may be most noticeable when your baby first wakes up. The weight gain or puffiness can be caused by fluid retention that is related to poor blood circulation. 

o Sweating, especially on the head. You may notice that your baby has damp hair and cool, moist skin. 

o Fatigue and fussiness. Your baby may be too tired to play and may sleep most of the time. 

o Less wet diapers than expected. Following the primary week, the majority of the newborn damp at least six diapers in a twenty four hour time. You might also view the baby's urine is gloomy and pungent.

How are they treated?

Some defects get better on its own and may not need treatment. Your baby's treatment will depend on the kind of defect.

Medicines may be used to help the heart work better. Medicines may also treat symptoms until the defect is repaired.

Some defects can be fixed by using a catheter, which does not require opening up the chest. A physician threads a slim tube known as catheter throughout a blood vessel, characteristically one in the groin. The physician threads the catheter into the heart, where the physician uses this to secure holes and open conical blood vessels and valves.

If a baby has a large or complex defect, the baby may need one or more open-heart surgeries. The surgery may be done right away, done over several steps, and deferred until the infant is stronger. Occasionally surgical procedure is delayed if the baby is premature or until the baby is strong enough to handle the surgery. In some cases, the child may need different types of surgery over time as he or she grows.

Complications of congenital heart defects

The majority children who have correct congenital heart defects guide normal lives. Nevertheless, complications at times develop.

Heart failure is a major complication of congenital heart defects. Heart failure may develop many years after the defect is diagnosed. It can cause a variety of symptoms, like severe difficulty breathing and irregular heartbeats. For more information about signs and symptoms related to heart failure, see the topic Heart Failure.

Amanda C Jones

Symptoms of Patent Ductus Arteriosus - Ways to Know If Your Baby Has PDA

Patent Ductus Arterosus or PDA is a birth defect that is associated with the improper flow of blood from higher to lower pressure aorta. Since all babies are almost the same upon birth, the symptoms of PDA will not be noticeable until the baby is first week or a couple of weeks old and to some a few months old, but how do you know when a baby is suffering from a disease when babies show the same symptoms of pain and suffering? To know whether or not your baby is suffering from PDA, you have to know the symptoms of patent ductus Arteriosus.

The symptoms of PDA vary depending on the size of the baby's ductus arteriosus. Some babies have normally small ductus arteriosus and these babies appear to be normal and healthy. Symptoms for this case will not be diagnosed unless the heart beat and heart sound is evaluated by a doctor and for babies with relatively larger ductus arteriosus, signs and symptoms may be different.

Even though the signs and symptoms of PDA are exhibited differently depending on the size of the baby's patent ductus arteriosus, there are some common signs named to help parents and doctors detect whether or not the baby is suffering from PDA. The first common sign is the heart murmur. Healthy babies have very silent heart murmur most especially when they are sleeping. It is almost impossible to hear the murmur unless you listen to it by intentionally placing your ears to the heart area of the baby. However, when a baby is suffering from PDA, the heart murmur is louder which is very much observable and noticeable upon examinations. Another symptom visible upon X-Ray is the congestion of the lungs. When the X-Ray result shows that your baby's lungs are congested, another examination should be done to ensure the diagnosis of the birth defect. Heart enlargement may also be seen during the test and this is also of the signs of PDA.

There are also symptoms of PDA which are noticeable even without the examinations and tests. Fast breathing, poor growth, resistance to feeding or when the baby gets tired while feeding and poor weight will give parents or just the mother alone a hint of the birth defect present in the baby.

Although some cases of Patent Ductus Arteriosus do not require treatment because it recovers by itself as the baby grows, there are cases when ductus arteriosus doesn't. So when you see that your baby exhibits some of the symptoms of patent ductus Arteriosus, take him to the doctor immediately. Tell your doctor the signs you see and request the doctor to perform clinical trials and examinations. It is always taught that prevention is better than cure and knowing the signs and symptoms of a certain disease is always the first step to prevention.

Rare Heart Diseases That Affect Children From Birth

Congenital Heart Defect is a condition, which includes a defect in the walls, valves, arteries, and veins of the heart in a newborn child. This imperfection in heart structure can possibly decrease the blood circulation or make it flow in the wrong direction or get completely blocked. This is a rare disease and approximately 9 out of 1000 kids are born every year, with poorly structured heart.

Types of well-known Congenital Heart Defect

• Coarctation of aorta

Aorta is the main and largest blood vessel that transports the blood from the heart to various parts of the body. In Coarctation of aorta, a section of aorta remains constricted or narrow, at birth. It becomes difficult for the heart to pump blood out through the aorta. This heart issue is found in people with a particular genetic disorder like Turner syndrome.

Symptoms

The curved part of aorta points upwards to the head and straight part points downwards. Coarctation of aorta can happen anywhere within the vessel. However, its tendency to occur at the top or aortic arc (C-shaped curve) is more. This makes it hard for blood to go out, so the blood pressure, before the narrowed segment is high, and after the constricted part, is low.

• Headaches from HBP (high blood pressure) 

• Camps in legs 

• Cold legs or feet 

• Abnormal heart sounds 

• Poor weight gain in babies because difficulty in feeding 

• Fainting or dizziness

Symptoms occur only, when the narrowing is severe in the aorta.

Treatment

The narrowed section is repaired, before the child's age turns 10. It can be repaired through balloon angioplasty or open-heart surgery.

• Hypoplastic Left Heart Syndrome (HLHS)

'Hypoplastic' means 'underdeveloped'. In HLHS the heart's left side that includes the left ventricle, aortic valve, mitral valve and aorta do not get developed in the womb. A healthy heart has two ventricles. Right ventricle pumps blood towards the lungs to get oxygenated, whereas the left ventricle pumps the oxygenated blood to the other parts of the body.

In HLHS, the left ventricle is very small to pump blood effectively. Usually, there is a hole in the wall that divides the right ventricle and left ventricle.

Symptoms

In the womb the fetus receives oxygen from the mother's circulation via placenta. In a couple of days, after birth the HLHS symptoms can be seen. This occurs because the placenta is disconnected and the newborn has to depend on its own heart for oxygenated blood circulation.

• Rapid heart beat 

• Rapid breathing 

• Cool, clammy and pale skin 

• Blue color lips and face 

• Weak pulses in arms and legs (because of poor circulation)

Treatment

Infant with HLHS, at birth will be given an IV (intravenous) to enhance blood flow. A series of heart surgeries are performed to reroute the blood in the right ventricle. The right side will perform the functions of both ventricles. Even if possible, a heart transplant surgery can be performed to provide the infant with a well-functioning heart.

• Patent Ductus Arteriosus

In the womb, ductus arteriosus allows the blood to evade the lungs of the baby. After birth as soon as the baby starts to breath, the responsibility of ductus arteriosus gets concluded and it shuts down automatically, in a few days. In certain cases, ductus arteriosus remains open and can cause abnormal blood circulation. This condition is called Patent Ductus Arteriosus. The cause of PDA is still unknown.

Symptoms

• Shortness of breath or rapid breathing 

• Sweating with playing or crying 

• Fast heart rate 

• Tire out easily 

• Poor eating & poor development

Treatment

PDA treatment focuses on closing ductus arteriosus to prevent complications. Closing of PDA is done either through medications, catheter, or surgery.

• Tetralogy of Fallot

A group of 4 heart defects at birth is called Tetralogy of Fallot. The flow of blood gets deteriorated, due to these defects causing a reduction in oxygen level.

The four defects are - 

1. Ventricular septal defect - There is a hole in the muscle wall between right and left side of the heart. 

2. Pulmonary valve stenosis - It is the narrowing of large blood vessel entrance. 

3. Over-riding aorta - Basically, aorta function starts from the left ventricle but in Tetralogy condition it begins from the center of the heart, just over the hole between two sides. 

4. Right ventricular hypertrophy - Due to the upper three heart defects the right side has to overwork, so the heart muscle on that side gets enlarged.

Symptoms

• Bluish tint on the lips, fingernails and skin 

• The tint darkens during activities like crying 

• Abnormal heart murmur 

• While feeding the baby gets tired easily, which hinders the growth 

• Fingertips have abnormal rounded shape

Treatment

Open heart surgery is applied to repair the defects of Tetralogy of Fallot. The pediatric cardiologists continue to check the child regularly, even after surgery. Sometimes, the child may need additional surgery, as they grow up because sudden complications may develop.

• Transposition of great arteries (TGA)

Basically, in normal condition the blood from heart goes to lungs to get oxygenated through pulmonary artery. After it comes back the aorta pumps the oxygenated blood through the body. In TGA, the blood comes to the heart but is pumped out directly without going towards the lungs for getting oxygenated.

Symptoms

• The baby has bluish colored skin 

• Rapid heart rate and breathing

Treatment

The baby receives intravenous medication, shortly after birth. If a hole is present in the hearts mid-wall then a hole will be made surgically. This allows the oxygen to mix with blood that is pumped out. Balloon atrial septostomy technique is used. Although this is a temporary treatment but the baby will require open-heart surgery called arterial switch operation within 4 weeks after birth, to correct the defect.

Aneurysm

Aneurysm is the swelling in the walls of blood vessels especially arteries. The swelling and widening of blood vessels causes them to weaken over time and they can rupture. The most commonly observed causes of aneurysm are congenital defects, uncontrolled high blood pressure and atherosclerosis (growing of fatty deposits in arteries). Aneurysm is usually classified by shape, size and location of the bulge. It is most common in arteries at the base of the brain referred as brain or cerebral aneurysm, and in aortic arteries known as abdominal aortic aneurysm. Although aneurysm can occur at any age it is most commonly observed in adults than children. Women are also found slightly more susceptible than men in this regard.

Aortic Aneurysm:

Aorta is the main artery that supplies blood from heart to the rest of the body. Aneurysm can develop anywhere in the aorta but they are most commonly found in aorta with in the abdominal region below the kidneys. The majority of aneurysms which actually start in the abdominal path of aorta eventually extend up to iliac arteries. It may occur in the chest near the heart as well (thoracic aortic aneurysm). The aortic aneurysm usually does not show any symptoms. Mild to moderate symptoms of aneurysm can sometimes be seen though. Pain in the effected area is a common symptom. One of the main causes of aortic aneurysm is smoking. The smokers over 60 years of age seem to have atherosclerosis. It has to be stopped immediately as it increases the risk of aneurysm's widening.

Treatment:

To treat the aortic aneurysm a procedure known as stenting is performed. After locating the position of the bulge, an X-ray guided polyester tube (stent-graft) is passed through a small cut in upper thigh leading to aorta. Now the blood instead of flowing through the damaged artery flow through the stent-graft and eliminates the chance of rupture.

Brain Aneurysm:

Brain Aneurysm usually originates from the base of the brain where the larger arteries combine. This area is known as circle of willis. The causes of brain aneurysm are generally genetic disorders which includes connective tissue disorders, polycystic kidney disease or some circulatory disorder. Patient having brain aneurysm may be found having the symptoms such as excruciating headache, problem with vision, thinking and decrease in concentration level, difficulty in speech and perception, pain in neck or eye and extreme tiredness.

Treatment:

Surgical clipping and coil embolization are methods usually applied for the treatment of brain aneurysm. Patient has to go through some test to get the right diagnosis after which the doctor will determine the method of treatment that is best suited to repair the blood vessel.

Congenital Heart Disease and Physical Therapy

Congenital Heart Disease

Congenital heart diseases refers to the structural (anatomical) or physiological defects in the normal functioning of the heart as a result of birth defects that may be diagnosed soon after birth or may take years to produce full blown cardiac insufficiency. Valvular heart defects form the most common variety of congenital cardiac defect that is associated with high morbidity and mortality in adult years if no management options are employed. This is because the metabolic demands of the body increase with growth and development that poses more pressure on the heart leading to cardiac failure or circulatory deficits. With overall prevalence of 26.6%, it was suggested that only 12.1% cases can be detected by clinical evaluation. Among the most prevalent congenital cardiac defects, ventricular septal abnormalities comprise 17.3% of all congenital anomalies followed by atrial septal defects (6.0%) and other less common cardiac diseases. The mortality is highest with cyanotic heart diseases. 

According to the research statistics reported by Julien I.E Hoffman, over 1 million patients were born with congenital heart defects (during 1940 to 2002). Considering the quality of medical services and surgical/ medical advancements, Hoffman suggested that the total number of survivors with mild heart disease (who may reach well into adulthood) is 750,000 with mild heart disease, 400,000 with moderate heart disease and 180,000 with severe disease (with treatment). Without any management or treatment the survival rate may fall to 400,000 with mild disease, 220,000 with moderate disease, and 30,000 with severe heart disease, suggesting very high mortality. 

Congenital heart diseases are also associated with stunted growth and development in children marked by poor weight gain, failure to thrive and frequent hospitalizations while growing up. In addition, these children also develop frequent episodes of shortness of breath, rapid heart rate (also known as tachycardia) and attacks of fatigue associated with decreased exercise endurance.

Physical therapy 

Physical therapy and mild exercises are helpful in the growth and development of children born with congenital heart disease. It is extremely important not to initiate exercise therapies in these children without seeking the guidance from registered physical therapists who work in coordination with the pediatric cardiologist to deliver best exercise regimens in order to optimize health without overloading the heart. Generally, children and adults can perform moderate static exercises of mild intensity without any complications; however, healthcare providers strongly restrict weight lifting in pediatric aged children and even in adults born with cardiac defects. Caution should be maintained to avoid lifting weight of more than 25 pounds in children and more than 50 pounds in adults. Physical therapist and pediatric cardiologist must assess every child individually and advice customized exercises and treatments according to the severity of illness and overall physical health. Treadmill test, bicycling and echocardiography are mainly used as assessment tools as the risk of sudden death increases if vigorous activity is attempted in children born with aortic stenosis, cyanotic heart diseases and coarctation of the aorta. 

Hardcore or traditional gym exercises increase cardiac output that may overload the heart and may increase the risk of complications or sudden cardiac death. On the contrary, exercises performed under the guidance of physical therapists serve multiple benefits. Exercise or physical activities are needed in order to build stamina and maintain exercise endurance especially in school going children who engage in physical activities with peers. Physical therapy improves the pace of mental and physical development that allows children to develop healthy social relationships with peers, muscle and motor coordination and mental concordance. Physical therapy and periodic assessments are also needed in order to know the physical capacity of child and to track worsening of cardiac defect with age (in order to avoid accidents or unwanted incidents at schools) by restricting excessive physical activity. In some children, healthcare providers delay surgery until the child crosses some developmental milestones; however, it is very important that until then child stays in best possible physical shape to lessen the risk of surgical complications. 

According to the scientific peer-reviewed journal- American Family Physician there are 5 stages of physical activity recommendations of Physical Activity in Children with CHD, ranging from no restriction to extreme limitation of physical activity (wheel chair bound). 

Without any physical therapy, the progression into the severe disability is fairly high. It is the duty of parents to promote healthy physical activity but make sure to prevent contact sports or vigorous activities that may affect cardiac functioning.