A 35 year old woman with transposition of the great arteries repaired with a Mustard procedure attends your clinic for annual follow-up.

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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.

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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.

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ANATOMY OF THE HEART
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Atrial Septal Defect(ASD)
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Ventricular Septal Defect(VSD)
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Patent Ductus Arteriosus(PDA)
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• 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 lungsThese 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 lungsThese 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 bodyThese 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.

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• 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.

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• 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.

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• 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.

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• 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.
  

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• 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.

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• 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

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• 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).

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• 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.

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• 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).Althoug
  h 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.

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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.