Heart Diseases Part IX - Congenital Heart Disease

Congenital heart diseases affect any part of the heart such as heart muscle, valves, and blood vessels. Congenital heart disease refers to a problem with the heart's structure and function due to abnormal heart development before birth. Every year over 30,000 babies are born with some type of congenital heart defect in US alone.

Congenital heart disease is responsible for more deaths in the first year of life than any other birth defects. Some congenital heart diseases can be treated with medication alone, while others require one or more surgeries. The causes of congenital heart diseases of newborns at birth may be in result from poorly controlled blood sugar levels in women having diabetes during pregnancy, some hereditary factors that play a role in congenital heart disease, excessive intake of alcohol and side affects of some drugs during pregnancy. Congenital heart disease is often divided into two types: cyanotic which is caused by a lack of oxygen and non-cyanotic.

1. Cyanotic 

Cyanosis is a blue coloration of the skin due to a lack of oxygen generated in blood vessels near the skin surface. It occurs when the oxygen level in the arterial blood falls below 85-90%. The below lists are the most common of cyanotic congenital heart diseases:

a)Tetralogy of fallot - Tetralogy of fallot is a condition of several congenital defects that occur when the heart does not develop normally. It is the most common cynaotic heart defect and a common cause of blue baby syndrome.

b)Transportation of the great vessels - Transportation of the great vessels is the most common cyanotic congenital heart disease. Transposition of the great vessels is a congenital heart defect in which the 2 major vessels that carry blood away from the aorta and the pulmonary artery of the heart are switched. Symptoms of transportation of the great vessels include blueness of the skin, shortness of breath and poor feeding.

c)Tricuspid atresia - In tricuspid atresia there is no tricuspid valve so no blood can flow from the right atrium to the right ventricle. Symptoms of tricuspid atresia include blue tinge to the skin and lips, shortness of breath, slow growth and poor feeding.

d)Total anomalous pulmonary venous return - Total anomalous pulmonary venous return (TAPVR) is a rare congenital heart defect that causes cyanosis or blueness. Symptoms of total anomalous pulmonary venous return include poor feeding, poor growth, respiratory infections and blue skin.

e)Truncus arteriosus - Truncus arteriosus is characterized by a large ventricular septal defect over which a large, single great vessel arises. Symptoms of truncus arteriosus include blue coloring of the skin, poor feeding, poor growth and shortness of breath. There are many more types of cyanotic such as ebstein's anomaly, hypoplastic right heart, and hypoplastic left heart. If you need more information please consult with your doctor.

2. Non-cyanotic 

Non-cyanotic heart defects are more common because of higher survival rates. The below lists are the most common of non-cyanotic congenital heart diseases:

a)Ventricular septal defect - Ventricular septal defect is a hole in the wall between the right and left ventricles of the heart causing right and left ventricles to work harder, pumping a greater volume of blood than they normally would in result of failure of the left ventricle. Symptoms of ventricular septal defect include very fast heartbeats, sweating, poor feeding, poor weight gain and pallor.

b)Atrial septal defect - Atrial septal defect is a hole in the wall between the two upper chambers of your heart causing freshly oxygenated blood to flow from the left upper chamber of the heart into the right upper chamber of the heart. Symptoms of atrial septal defect include shortness of breath, fatigue and heart palpitations or skipped beats.

c)Coarctation of aorta - Coarctation of aorta is a narrowing of the aorta between the upper-body artery branches and the branches to the lower body causing your heart to pump harder to force blood through the narrow part of your aorta. Symptoms of coarctation of aorta include pale skin, shortness of breath and heavy sweating.

There are many more types of non-cyanotic such as pulmonic stenosis, patent ductus arteriorus, and atrioventricular cana. These problems may occur alone or together. Most congenital heart diseases occur as an isolated defect and is not associated with other diseases.

( Kyle J Norton )

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