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Congenital Heart Disease

If you have visited our How the Heart Works section, you now think of a normal healthy heart as a house with 4 rooms, 4 doors, 4 hallways and a good-flowing house plan.

Now that we are thinking of the heart as a house, we will explore what can go wrong with the house. A child could be born with several defects (abnormalities) involving the various parts of the heart house. For simplicity the following abnormalities could be encountered:

Problems with the Walls

1. Atrial Wall

Problem: Atrial Septal Defect

The wall between the right atrium and left atrium is called Atrial Septum. Please note that the word "septum" in Greek means "wall."

A hole between the two atriums called an Atrial Septal Defect (ASD). When the blood coming back from the lungs to the Left Atrium crosses the ASD it mixes with the blue blood coming back from the body in the Right Atrium

Fix: Patch Operation

Doctors fix an Atrial Septal Defect (ASD) by patching the hole between the two atriums.

 

 

2. Ventricular Wall

Problem: Ventricular Septal Defect

The wall between the front two chambers (between the two ventricles) is called Ventricular Septum.

A hole between the two ventricles is called a Ventricular Septal Defect (VSD). When the pink blood in the Left Ventricle crosses the VSD it mixes with the blue blood in the Right Ventricle.

Fix: Patch Operation

Doctors fix a Ventricular Septal Defect (VSD) by patching the hole between the two ventricles.

 

 

Read more: Problems with the Walls

Problems with the Chambers

1. Right Ventricle

Problem: Hypoplastic Right Ventricle

If the right ventricle is small and weak, this condition is called Hypoplastic Right Heart or Hypoplastic Right Ventricle. It happened because the tricuspid valve guiding the blood from the right atrium to the right ventricle did not open in the very early embryonic weeks of the baby's life, and so no blood came through to make the right ventricle grow.

2. Left Ventricle

Problem: Hypoplastic Left Heart Syndrome

If the left ventricle is small and weak, this condition is called Hypoplastic Left Ventricle or Hypoplastic Left Heart Syndrome. It happened because the mitral valve guiding the blood from the left atrium to the left ventricle did not open in the very early embryonic weeks of the baby's life, and so no blood came through to make the left ventricle grow.

 

Read more: Problems with the Chambers

Problems with the Hallways

The hallways leading away from the heart:

1. Aorta

Problem: Coarctation of the Aorta

If this hallway is narrowed as it makes the bend to go down to the body it is called Coarctation of the Aorta.

2. Pulmonary Artery

Problem 1: Branch Pulmonary Artery Stenosis

Problem 2: Transposition of Great Arteries
Fix:  Prostaglandin, Atrial Septostomy, or Switch Operation

If the pulmonary artery is narrowed as it branches to either the right or left lung this condition is called Branch Pulmonary Artery Stenosis.

When these 2 main hallways leading away from the heart are swapped or transposed, it is called Transposition of Great Arteries. 

Read more: Problems with the Hallways

Problems with the Plumbing

When the main coronary artery originates from the Pulmonary Artery instead of the Aorta, this defect is called Anomalous Origin of the Coronary Artery from the Pulmonary Artery.

Anomalous Origin of the Coronary Artery from the Pulmonary Artery

This defect happens when the main coronary artery originates from the Pulmonary Artery instead of the Aorta. The pulmonary artery carries less oxygen in its blood, so now the muscle of the heart which is working very hard instead of getting fresh oxygenated blood it is getting blood with less oxygen.

The muscle of the heart does not like that and starts to become weaker and weaker, the muscle walls of the heart becomes thinner and thinner, and this predisposes to a condition called Cardiomyopathy

Read more: Problems with the Plumbing

Problems Involving More Than One Part of the Heart

1. Tetralogy of Fallot

This involves a hole between the ventricles, a narrow pulmonary hallway, an overriding aortic hallway, and enlargement and thickening of the right ventricular wall. 

 

2. Truncus Arteriosus

Part of the wall between the front large heart chamber is missing. An overriding aorta, which means the door from the left ventricle (aortic valve) originates from both front chambers. Click here to see the operation to fix this defect.

 

3. Common Atrioventricular Canal

This defect involves a hole between the two atriums called a Atrial Septal Defect (ASD) and a hole between the two ventricles called a Ventricular Septal Defect (VSD). There is a common opening between all the chambers of the heart. As a result, the wall that was separating the Mitral Valve and the Tricuspid Valve which usually looks like a cross is missing.

 

4. Ductus Arteriosus

Ductus Arteriosus is a passageway which normally is present in every baby before birth. It is a communication between the two major arteries coming out of the heart. The Ductus usually closes within the first few days after birth. Patent Ductus Arteriosus is a condition that exists when the Ductus does not close. Since the pressure in the Aorta is much higher than in the Pulmonary Artery lots of blood will be going to the Pulmonary Artery and it will be larger and the lungs will be getting much more blood than they really need. 

  • Clip Operation
  • Coil Operation

 

 

 

 

 

Problems with the Doors

1. Tricuspid Valve

The Tricuspid Valve is located between the right atrium and right ventricle. If this door is not developed this condition is called Atresia (Tricuspid Valve Atresia). There is a 3-step procedure to fix this defect:

Stage 1: Shunt Operation

Stage 2: Glenn Operation

Stage 3: Fontan Operation

Since the door did not develop in the baby before birth there was no blood coming from the right atrium to the right ventricle so the right ventricle became underdeveloped (hypoplastic right ventricle).

If the tricuspid door is displaced downward from it's usual location, this will result in the right atrium becoming excessively large and the right ventricle small and underdeveloped. This condition is called Ebstein Anomaly.

 

2. Mitral Valve

The mitral valve is the door between the left atrium and the left ventricle. If the mitral valve does not develop during the very early gestation weeks, the blood in the left atrium will not flow to the left ventricle, and therefore the left ventricle will be small and underdeveloped (Hypoplastic Left Heart Syndrome). There is a 3-step procedure to fix this defect:

Stage 1: Norwood Operation

Stage 2: Glenn Operation

Stage 3: Fontan Operation

 

3. Pulmonic Valve

The pulmonic valve is the door located between the right ventricle and the main pulmonary artery. If this door is a little bit small (depending on how small it is) we call this condition stenosis (Pulmonic Valve Stenosis). If it does not develop at all we call this condition atresia (Pulmonic Valve Atresia).

 

4. Aortic Valve

The aortic valve is the door located between the left ventricle and the aorta. If the valve is small this condition is called stenosis (Aortic Valve Stenosis). If this valve does not develop at all this condition is called atresia (Aortic Valve Atresia).

 

 

 

Read more: Problems with the Doors

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Children's Heart Institute

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