INTRODUCTION A 35 year old woman with transposition of the great arteries repaired with a Mustard procedure attends your clinic for annual follow-up. Her main concern, she tells you, is that her recent life insurance application was refused because of congenital heart disease. She is disturbed because at yearly clinic visits she has always been reassured that she is ‘doing very well’. She asks you to write to her insurance company on her behalf.

SCOPE OF THE PROBLEM Congenital heart disease (CHD) affects 8/1000 live births. techniques for the diagnosis and management of CHD over the last fifty years, expected survival into adulthood has reached 85%. Adding to this number are an estimated 1600 cases with complex or significant congenital heart lesions and many more with simple lesions entering the adult age group each year. These figures will continue to increase over time, leading to an ever growing adult patient group seeking the financial security of insurance coverage.

ANATOMY OF THE HEART

Atrial Septal Defect(ASD)

Ventricular Septal Defect(VSD)

Patent Ductus Arteriosus(PDA)

What are the different types of congenital heart defects? We can classify congenital heart defects into several categories in order to better understand the problems the baby will experience. They include: problems that cause too much blood to pass through the lungs These defects allow oxygen-rich blood that should be traveling to the body to re-circulate through the lungs, causing increased pressure and stress in the lungs. problems that cause too little blood to pass through the lungs These defects allow blood that has not been to the lungs to pick up oxygen (and, therefore, is oxygen-poor) to travel to the body. The body does not receive enough oxygen with these heart problems, and the baby will be cyanotic, or have a blue coloring. problems that cause too little blood to travel to the body These defects are a result of underdeveloped chambers of the heart or blockages in blood vessels that prevent the proper amount of blood from traveling to the body to meet its needs. Again, in some cases there will be a combination of several heart defects, making for a more complex problem that can fall into several of these categories.

patent ductus arteriosus (PDA) - this defect, which normally occurs during fetal life, short circuits the normal pulmonary vascular system and allows blood to mix between the pulmonary artery and the aorta. Prior to birth, there is an open passageway between the two blood vessels, which closes soon after birth. When it does not close, some blood returns to the lungs. Patent ductus arteriosus is often seen in premature infants.

atrial septal defect (ASD) - in this condition, there is an abnormal opening between the two upper chambers of the heart - the right and left atria - causing an abnormal blood flow through the heart. Some children may have no symptoms and appear healthy. However, if the ASD is large, permitting a large amount of blood to pass through the right side, symptoms will be noted.

ventricular septal defect (VSD) - in this condition, a hole in the ventricular septum (a dividing wall between the two lower chambers of the heart - the right and left ventricles) occurs. Because of this opening, blood from the left ventricle flows back into the right ventricle, due to higher pressure in the left ventricle. This causes an extra volume of blood to be pumped into the lungs by the right ventricle, which can create congestion in the lungs.

atrioventricular canal (AVC or AV canal) - atrioventricular canal is a complex heart problem that involves several abnormalities of structures inside the heart, including atrial septal defect, ventricular septal defect, and improperly formed mitral and/or tricuspid valves.

Some of the problems that cause too little blood to pass through the lungs include the following: tricuspid atresia - in this condition, there is no tricuspid valve, therefore, no blood flows from the right atrium to the right ventricle. Tricuspid atresia defect is characterized by the following: a small right ventricle a large left ventricle diminished pulmonary circulation cyanosis - bluish color of the skin and mucous membranes caused from a lack of oxygen. A surgical shunting procedure is often necessary to increase the blood flow to the lungs.

transposition of the great arteries (TGA) - with this congenital heart defect, the positions of the pulmonary artery and the aorta are reversed, thus: the aorta originates from the right ventricle, so most of the blood returning to the heart from the body is pumped back out without first going to the lungs. the pulmonary artery originates from the left ventricle, so that most of the blood returning from the lungs goes back to the lungs again

tetralogy of Fallot (TOF) - this condition is characterized by the following four defects: an abnormal opening, or ventricular septal defect, that allows blood to pass from the right ventricle to the left ventricle without going through the lungs a narrowing (stenosis) at or just beneath the pulmonary valve that partially blocks the flow of blood from the right side of the heart to the lungs the right ventricle is more muscular than normal the aorta lies directly over the ventricular septal defect Tetralogy of Fallot results in cyanosis (bluish color of the skin and mucous membranes due to lack of oxygen).

truncus arteriosus - the aorta and pulmonary artery start as a single blood vessel, which eventually divides and becomes two separate arteries. Truncus arteriosus occurs when the single great vessel fails to separate completely, leaving a connection between the aorta and pulmonary artery.

aortic stenosis (AS) - in this condition, the aortic valve between the left ventricle and the aorta did not form properly and is narrowed, making it difficult for the heart to pump blood to the body. A normal valve has three leaflets or cusps, but a stenotic valve may have only one cusp (unicuspid) or two cusps (bicuspid). Although aortic stenosis may not cause symptoms, it may worsen over time, and surgery may be needed to correct the blockage - or the valve may need to be replaced with an artificial one.

Conclusion . With newer surgical and catheter techniques, advancing medical therapy and improved risk stratification, overall prognosis and hence insurability will continue to improve for adults with congenital heart disease.