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Pathology 3001 > Congenital Heart Defects > Flashcards

Congenital Heart Defects Flashcards

1
Q

How common are congenital heart defects?

A

8 out of every 1000 newborns

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2
Q

What CHDs are non-cyanotic?

A
  • Ventricular Septal Defect
  • Atrial Septal Defect
  • Patent Ductus Arteriosus
  • Coarctation of aorta
  • Pulmonary stenosis
  • Aortic stenosis
  • Atrioventricular canal (endocardial cushion defect)
  • Ebstein’s anomaly (mild)
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3
Q

What CHDs are cyanotic?

A
  • Tetralogy of Fallot
  • Total anomalous pulmonary venous return
  • Transposition of the great vessels
  • Tricuspid atresia
  • Truncus arteriosus
  • Hypoplastic left heart
  • Pulmonary atresia
  • Ebstein’s anomaly (severe)
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4
Q

What conditions cause increased pulmonary blood flow?

A
  • ASD
  • VSD
  • PDA
  • Atrioventricular canal
    (All acyanotic)
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5
Q

What conditions cause obstruction to blood flow from ventricles?

A
  • Coarctation of aorta
  • Aortic stenosis
  • Pulmonic stenosis
    (all acyanotic)
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6
Q

What conditions cause a decrease in pulmonary blood flow?

A
  • Tetralogy of Fallot
  • Tricuspid atresia
    (Cyanotic)
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7
Q

What conditions have a mixed blood flow?

A
  • Transposition of the great vessels
  • Total anomalous pulmonary venous return
  • Truncus arteriosus
  • Hypoplastic left heart syndrome
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8
Q

What is the most common Congenital Heart Disease?

A

Ventricular Septal Defect (39%)

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9
Q

What percentage of individuals with down syndrome have a congenital heart Defect?

A

~ 50%

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10
Q

What procedure was initially the only treatment available for tetralogy of fallot (palliative but not curative)?

A

Blalock-Thomas-Taussig procedure

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11
Q

What is the survival rate for tetralogy of fallot after 30 years?

A

90%

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12
Q

What conditions can patients with repaired tetralogy of fallot get?

A
  • RV hypertrophy
  • Ventricular dysfunction
  • Atrial tachyarrhythmias
  • Residual outflow obstruction
  • Pulmonary regurgitation
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13
Q

What is hypoplastic left heart syndrome?

A
  • Left ventricle is underdeveloped and too small
  • Mitral valve is not formed or very small
  • Aortic valve is not formed or too small
  • Ascending portion of aorta is underdeveloped or is too small
  • May appear normal at birth
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14
Q

How is hypoplastic left heart syndrome treated?

A
  • Newborns are given IV prostaglandin E1 to keep the ductus arteriosus open
  • Often a treatment can involve a series of 3 heart surgeries
  • Often Balloon Atrial Septostomy used
  • Less commonly, heart transplantation
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15
Q

What is a balloon atrial septomostomy?

A

A catheter with a balloon on the end is guided through a blood vessel into the heart and is then inflated to widen the foramen ovale, sometimes a stent may be placed to keep it open and to allow blood to mix freely between the atria, therefore allowing more oxygenated blood to reach the body.

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16
Q

What is truncus arteriosus?

A

The pulmonary trunk and aorta arise from a common trunk.

- Blood from both ventricles mixes together as it all exits through the single valve exiting from the heart.

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17
Q

What is involved in the treatment of truncus arteriosus?

A

Sewing a flexible tube with its own valve from the right ventricle to bring blood to the pulmonary arteries.

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18
Q

What percentage of infants with CHDs have ECAs?

A

25% have extracardiac anomalies

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19
Q

What eye anomalies are associated with CHD?

A

Cataracts, glaucoma, strabismus, nystagmus, micropthalmia, and iris dysplasia

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20
Q

What condition during pregnancy can cause CHDs?

A

Poorly controlled blood sugar in woman who have diabetes during pregnancy.

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21
Q

What infection can cause CHDS?

A

Rubella

22
Q

What drugs can cause CHDs?

A

Retinoic acid for acne, chemicals, alcohol

23
Q

What are the main symptoms/signs of heart failure in infants with CHD?

A
  • Cyanosis
  • Poor blood circulation
  • Rapid breathing (tachypnoea)
  • Extra work of breathing
  • Grunting
  • Fatigue (poor feeding)
  • Faltering growth
  • Sweating
  • Murmurs
  • Enlarged liver (hepatomegaly)
24
Q

What is eisenmenger’s syndrome?

A

The result of an untreated CHD where blood reverses to flow from right to left.
Results in:
- Pulmonary hypertension
- Cyanosis

25
Q

How are ASDs treated?

A
  • Half close on their own over time

- Medium and large ASDs can be repaired using a catheter procedure or open-heart surgery

26
Q

How are PDAs treated?

A
  • NSAIDs (within first 10-14 days of life)
  • Catheter closure
  • Surgical ligation
27
Q

What can coarctation of aorta clinically present with?

A
  • Angina pectoris
  • Leg claudication
  • Diminished femoral pulses
  • Differential blood pressure between upper and lower extremities
28
Q

How can coarctation of the aorta be treated?

A
  • Primary surgical repair with excision of the coarctation and end-to-end anastomosis
  • Balloon angioplasty
  • Subclavian flap repair
29
Q

What is Pulmonary atresia?

A

When the pulmonary valve does not form at all meaning blood from the right ventricle cannot reach the pulmonary trunk

30
Q

How is sever pulmonary valve stenosis treated?

A

Catheter procedure

31
Q

How is aortic valve stenosis treated?

A
  • Balloon aortic valvuloplasty

- Surgical repair or replacement (often in patients who have had a failed balloon aortic valvulopasty)

32
Q

What is Tetralogy of Fallot?

A
  • Pulmonary stenosis
  • Right ventricular hypertrophy
  • Overriding aorta
  • Ventricular septal defect
33
Q

What shape is the heart described as having in Tetralogy of Fallot?

A

Boot shaped

34
Q

What do Tet spells involve?

A
  • Sudden episodes of profound cyanosis and hypoxia (may be lethal)
35
Q

What position do infants with Tetralogy of Fallot go into?

A

Knee-chest position

36
Q

What is Blalock-Thomas-Taussig procedure used to treat?

A
  • TOF
37
Q

How is TOF repaired?

A

Open-heart surgery

38
Q

How are infants able to survive with Transposition of the great arteries?

A
  • Shunt between the two sides of the heart
  • Atrial septal defect needs to actually be enlarged to allow adequate mixing of blood to deliver enough nutrients around the body
39
Q

What is ebstein’s anomaly?

A
  • Abnormality of tricuspid valve
  • Deformed valve allows a large amount of blood to flow backwards from the right ventricle to right atrium when RV contracts
40
Q

What other abnormalities may severe ebstein’s anomalies contain?

A
  • ASD (causing right to left shunt, causing cyanosis)

- Can also happen with PFO

41
Q

What is total anomalous pulmonary venous drainage?

A

Pulmonary veins are not connected to left atrium and instead connect to one of the veins from the main circulation resulting in them draining back into the right side of the heart.

42
Q

What CHD is common in babies with Down syndrome

A

Atrioventricular Septal Defect

43
Q

What is a Atrioventricular Septal Defect?

A

Holes between the chamners of the right and left sides

44
Q

What is AVSD also known as?

A

Atrioventricular canal defect or endocardial cushion defect

45
Q

What are the 5 heart questions to ask at 20 weeks?

A
  1. Is the heart rate normal?
  2. Do you look at the arteries as part of your scan
  3. Are the heart and the stomach in the correct position
  4. Do you see 4 chambers
  5. Is the heart function normal
46
Q

What percentage of mothers had a CHD diagnosis while they were pregnant?

A

15%

47
Q

Mothers were more likely to report that they had received a diagnosis during pregnancy if?

A
  • They were over 30 years of age
  • They had tyoe 1 or type 2 diabetes
  • Someone else in their family had a CHD
  • They were carrying twins or multiple babies
  • Their baby had a more complex heart defect or other birth defects in addition to the CHD
48
Q

What are the causes of sudden unexpected collapse in the first week of life?

A
  • Duct dependant CHD
  • Sepsis (particularly Group B Strep)
  • Hypoglycaemia
  • Congenital Adrenal Hyperplasia
  • Inborn errors of metabolism
49
Q

What is eisenmenger’s syndrome?

A
  • Shunt reversal
  • Initially large left to right shunt such as large VSD
  • Pulmonary Arterial Hypertension to the point of the right sided heart pressure exceeding left with shunt reversal and development of cyanosis
  • Aquired Cyanotic Heart Disease
50
Q

What is pulse oximetry?

A

Test used to measure the oxygen saturation of the blood. Measures how well oxygen is being carried to the furthest parts from the heart such as arms and legs.
Unreliable however but can pick up on CHDs and other disorders

Pathology 3001 (15 decks)

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