Congential Heart Defects Flashcards

1
Q

What is the aetiology of congenital heart disease ? ( causes )

A

GENETIC CAUSES : eg Down’s syndrome , Marfans syndrome , polygenic. People with down syndrome (50% of them often have heart problems)

2) ENVIRONMENTAL CAUSES: alcohol , drugs
3) MATERNAL INFECTIONS : Rubella , toxoplasmosis

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2
Q

What is the oxygen saturation percentage of blood that enters the left atria from the pulmonary veins ?

A

99%

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3
Q

What is the oxygen saturation percentage of blood that enters right side of the heart?

A

67%

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4
Q

Which side of the heart has a higher pressure ?

A

LEFT - ventricle has a pressure of 80/4 whereas the right ventricle has a pressure of 25/3 mmgh

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5
Q

What is required for by the heart if blood was shunted from left to right ?

A

1) requires a hole

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6
Q

What is required for by the heart , if blood was shunted from the right to the left side of the heart ?

A

1) a hole

2) distal obstruction

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7
Q

What occurs if blood is shunted from the left side to the right ?

A
  • blood from the left heart is returned to the lungs instead of going to the body
  • increased lung blood flow by itself is not damaging , but increased pulmonary artery or pulmonary venous pressure is.
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8
Q

What occurs if blood is shunted from the right side of the heart to the left ?

A
  • deoxygenated blood bypasses the lungs
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9
Q

What are the two things we classify congenital heart diseases into ?

A

1) CYANOTIC

2) ACYANOTIC

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10
Q

What does cyanotic mean ?

A
  • patient often appears b, blue

- this is a very complex disease , often resulting in right to left shunt.

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11
Q

Give examples of cyanotic diseases

A
  • tetralogy of fallot
  • transposition of the great arteries
  • uni ventricular heart
  • total anaomlpus pulmonary venous drainage
  • pulmonary atresia and tricuspid atresia
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12
Q

What does acyanotic mean ?

A
  • patient does not appear blue
  • this is more common congenital heart disease
  • blood being shunted from the left to the right side of the heart in some diseases
  • other compartments of the heart just have to work harder
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13
Q

Give examples of acyanotic examples of congenital heart disease ?

A
  • patient ductus arteriosus
  • aortic stenosis
  • pulmonary stenosis
  • coarctation of the aorta , mitral stenosis
  • atrial septal defect
  • ventricular septal defect
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14
Q

What is the cause of atrial septal defect ?

A

Under development of the septum primum or the septum secundum resulting in a hole in the atrial septum

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15
Q

What is the haemodynamic effect of atrial septal defect?

A
  • BLOOd will now shunt from the left atrium into the right atrium.
  • increased volume of blood in the right ventricle
  • increased volume of blood being pumped around the pulmonary circuit ( into the lungs)
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16
Q

If left untreated , how can atrial septal defect be a big problem ?

A
  • the increased volume of blood being pumped around the pulmonary circuit would cause damage to the vasculature and fibrosis of the arteries in the lungs
  • this would make the arteries less stretchy and increases resistance to blood flow , which results in increased pulmonary hypertension ( although this is quite rare )
  • pulmonary hypertension means that the right side of the heart must work against an increased afterload. Putting strain on the heart , cashing ride sided heart failure.
17
Q

What is the cause of ventricular septal defect ?

A
  • the membranous portion of the septum fails to develop properly.
  • this means that the primary inter-ventricular foramen remains open.
18
Q

What is the haemodynamic effect of ventricular septal defect?

A
  • blood in the left ventricle will flow into the right ventricle.
  • increased volume of blood in the left ventricle
  • increases volume of blood being pumped around the pulmonary circuit
19
Q

If left untreated , what are the effects of ventricular septal defect ?

A
  • due to the increased volume of blood being pumped around the pulmonary circuit
  • this could damage the vasculature and cause fibrosis of the arteries in the lungs. This makes the arteries less stretchy , which increases resistance to blood flow.
  • this could lead to pulmonary hypertension ( as less blood would be pumped away from the pulmonary arteries due to decreased resistance so more blood remains in these arteries).
  • the right ventricle would have to work much harder against the increased afterload- this would cause ride sided heart failure.
20
Q

WHat is atrioventricular septal defect ?

A
  • caused by failure of the endocardial cushions to develop properly
  • this results in a large hole in the middle of the heart between the atria and the venitrcles.
  • they have one common AV valve instead of a mitral and a pulmonary valve.
21
Q

Where is is atrioventricular septal defect common in ?

A

Down syndrome

22
Q

What is aortic /pulmonary stenosis ? And what are the consequences ?

A
  • aortic or pulmonary valve narrows
  • The right / left ventricle would have to work much harder to generate more force to push blood through the stenosed valve.
  • this causes hypertrophy of the left or right ventricle
  • this can lead to heart failure
23
Q

What is coarctation of the aorta ?

A
  • narrowing of part of the aortic lumen in the region of the ligamentum arteriosum ( former ductus arteriosus)
  • the narrowing the aorta increases the afterload of the left ventricle and can lead to left ventricular hypertrophy.
  • coarctation of the aorta usually occurs beyond the blood vessels that branch off to your upper body and BEFORE the blood vessels that lead to your lower body. This can often lead to HIGH blood Pressure in the arms but low blood pressure in your legs and ankles. ( a very weak femoral pulse will be detected )
24
Q

CYANATIC DISEASE : tetralogy of fallot - what are the four defects that present together ?

A

1) pulmonary stenosis : the pulmonary valve is narrowed so less blood can enter the arteries from the right ventricles.
2) overriding aorta : the aorta is larger and situated next to the ventricular septal defect so most of the blood in the heart flows through the aorta. (. Mixed o2. + de02)
3) ventricular septal defect : allows blood from the right ventricle which cannot be pumped through the stenosed pulmonary valve to move into the left ventricle and be pumped around the body via the aorta.( RIGHT TO LEFT SHUNT)
4) hypertrophy of the right ventricle : to generate more force to pump blood through the stenosed pulmonary valve , the ventricles undergo hypertrophy,

25
Q

CYANOTIC : HYPOPLASTIC OF THE LEFT HEART

A
  • mitral or aortic valves are stneosed in utero which means that less blood flows into the left ventricle.
  • this results in the left ventricle being underdeveloped so less blood pumped around the body.

-

26
Q

How would a clinician respond to a a baby born with hypoplastic left heart syndrome ?

A
  • would administer prostaglandins which would keep the ductus arteriosus open.
  • often babies born with hypoplastic left heart syndrome ALSO have atrial septal defect( a hole between the left and right atria). This is required for the survival of the baby.
  • with the ASD, oxygenated blood would shunt from the left side of the heart into the right side of the heart due to pressures in the left atria being higher than the right. This would then be pumped via the pulmonary artery. The patent ductus arteriosus would then allow blood to be shunted from the pulmonary artery / trunk DIRECTLY into the aorta so that it can be delivered around the body.
27
Q

What is univentrocular heart ?

A

The ventricular septum does not for,.So oxygenated and deyxogentaed blood mixes in the ventricle and gets pumped into both the aorta and pulmonary trunk.

28
Q

What is transposition of the great arteries ?

A

Often , the right ventricle is connected to the aorta

  • the left ventricle is connected to the pulmonary trunk/arteries.
  • this is a problem because when the vena cava delivers deoxygenated blood into the right atrium - right ventricle and then aorta - deoxygenated blood would be pumped around the body , whereas in the left side of the heart when oxygenated blood enters the left atrium via pulmonary veins - left ventricle - pulmonary arteries - the oxygenated blood would flow to the lungs. Thus , no oxygenated blood would be flowing around the body.
29
Q

How do clinicians deal with a child born with the trans positioning of the great arteries ?

A
  • neonatal emergency
  • they must give the baby prostaglandins immediately in order for the ductus arteriosus to remain open. Babies are often born with atrial septal defect too.
  • surgery is required to often change the arteries around.coronary arteries must also be changed around too.
30
Q

Why can a fetus survive with transpositioning of the great arteries ? But not after birth

A

Because the ductus arteriosus shunts blood from the pulmonary trunk /arteries into the aorta. Which means that oxygenated blood would enter the aorta which can then be delivered around the body.

  • also , the foramen ovale would shunt deoxygenated blood from the right atrium into the left atrium which would allow the deoxygenated blood to then leave the pulmonary trunk to be oxygenated by the lungs.
31
Q

What is tricuspid atresia ?

A

The tricuspid valve fails to form properly

32
Q

What occurs as a result of tricuspid atresia ?

A

Blood cannot flow from the right atrium into the right ventricle.

  • in these babies , there is an atrial septal and ventricular septal defect which allows blood to be shunted from the right atrium into the left atrium.
  • this means that deoxygenated blood mixes with the oxygenated blood in the left side of the heart as it can only move through the atrial septal defect as the valve is fused. This mixing means that blood being pumped into the aorta and body will have a low PO2 resulting in central cyanosis
33
Q

What is pulmonary atresia ?

A

The pulmonary valve fails to form

34
Q

What are the consequences of pulmonary atresia ?

A
  • children are often born with VSD
  • so blood is shunted from the right ventricle into the left ventricle where it mixes with oxygenated blood.
  • this results in cyanosis.