Developmental Pathology Flashcards

1
Q

Which are the 3 main causes to left-to-Right shunts?

A

1) Atrial septal defect.
2) Ventricular septal defect.
3) Patent ductus arteriosus.

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2
Q

What is one of the characteristics of left-to-right shunts?

A

Late Cyanosis (Blue kids)

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3
Q

What kind of shunt developed a early cyanosis?

A

Right-to-Left shunt (eaRLy cyanosis).

Left-to-Right shunt (Later cyanosis)

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4
Q

What is the most common congenital cardiac defect ?

A

Ventricular septal defect; Asymptomatic at birth, Most self resolve. Left-to-right shunt.

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5
Q

How long is the ranges since the birth to the close of the ventricular septal defect?

A

About 40% close spontaneously within the first six months.

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6
Q

When is the ductus Arteriosus supposed to close?

A

In the first 24 hrs after birth but it may take after 2 weeks

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7
Q

Which is the characteristics murmur in the patent ductus arteriosus?

A

Continuous machinery-like heart murmur.

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8
Q

What is the treatment of the PDA ?

A

Treatment Involved Indomethacin which block prostaglandins synthesize.

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9
Q

In which situation is helpfull keep the PDA open?

A

Transposition of the great vessels.

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10
Q

Which are the effects of the prostaglandins and Indomethacin in the PDA?

A

Prostaglandins: Keep PDA open.
Indomethacin: Close the PDA.

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11
Q

Which are the characteristics of the Eisenmenger syndrome?

A
Uncorrected left-to-right shunt.
Pulmonary hypertension.
Right ventricular hypertrophy.
Increase right ventricular pressure.
Right-to-left shunt systemic circulation.
Shortness of breath
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12
Q

Why we used Indomethacin in the PDA?

A

Because we try to prevent Eisenmenger syndrome.

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13
Q

What is the definition of Coarctation of the aorta?

A

Aortic narrowing near insertion of ductus arteriosus (“Juxtaductal”). In this situation you might have to keep the PDA open temporarily with prostaglandins.

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14
Q

Which congenital heart disease is associated with Turner syndrome?

A

Coarctation of the Aorta.

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15
Q

What is the adult type presentation of the Coarctation of the aorta?

A

Hypertension in upper extremities and weak, delayed pulse in lower extremities. Collateral arteries erode ribs (notched appearance on CXR).

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16
Q

Which is the disease associated with Aortic regurgitation, bicuspid aortic valve and Turner syndrome.

A

Coarctation of the Aorta.

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17
Q

Which disorder is associated with Bicuspid aortic valve, infantile coarctation of the aorta?

A

Turner syndrome.

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18
Q

Which congenital cardiac defect is associated with Down syndrome?

A

Endocardial cushion defect (AV septal defect), VSD, ASD.

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19
Q

Which disorder is associated with Tetralogy of Fallot and Truncus arteriosus?

A

22q11 (DiGeorge) syndrome.

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20
Q

Which are the defects associated with congenital rubella?

A

Septal defects, PDA, Pulmonary artery stenosis.

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21
Q

What is the sequence of the Eisenmenger syndrome?

A

Left-to-right shunt –> Pulmonary hypertension –> Right Ventricular Hypertrophy –> increase Right Ventricular pressure –> Right -to- left shunt.

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22
Q

type of shunt the patients develop cyanosis early?

A

Right-to-Left.

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23
Q

Which are the 5 causes of right Cyanotic Heart Disease?

A
  1. Truncus arteriosus.
  2. Transposition of the great vessels.
  3. Tricuspid atresia.
  4. Tetrology of Fallot.
  5. Total Anomalous Pulmonary Venous Return (TAPVR).
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24
Q

What is the congenital heart disease defined as a fail to divide into pulmonary trunk and aorta due to lack of Aorticopulmonary septum formation?

A

Persistent Truncus arteriosus.

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25
Q

Which are the characteristics of D-Transposition of great vessels?

A

Ao leaves RV and Pulmonary trunk leaves LV resulting in a separation of systemic and pulmonary circulation. Due to a failure of the Aorticopulmonary septum to spiral.

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26
Q

What is the congenital heart disease that need PDA present to be compatible with life?

A

D-Transposition of great vessels, without surgical intervention, most infants die within the first few months of life.

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27
Q

Which are the patients with more likely to suffer from a LGI (Large of Gestational Age)?

A

IDDM (Infants of Diabetic Mother).

28
Q

Which are the complications that suffer the IDDM?

A

Shoulder dystocia, clavicle fracture, brachial plexus lesion (Duchenne palsy).

29
Q

What is the Tricuspid Atresia?

A

Absence of tricuspid valve and hypoplastic RV; requires both ASD and VSD for viability.

30
Q

What is the Ebstein anomaly?

A

Displacement of the septal and posterior, but not the anterior, leaflets into the RV.

31
Q

Which are the characteristics of Ebstein Anomaly?

A

Associated with maternal Lithium use.
80% have a patent Foramen Ovale with a R to L shunt.
Dilated right atrium causing increased risk of SVT and WPW (ventricular preexcitation syndrome).
Physical exam: Widely split S2, tricuspid regurgitation.

32
Q

Which are the parts that conform the Tetralogy of Fallot?

A
  1. Pulmonary valve stenosis.
  2. Right ventricular hypertrophy.
  3. Ventricular Septal Defect.
  4. Overriding Aorta.
33
Q

Which a are the occasions when the tetralogy of Fallot becomes Right-to-Left?

A

When they crying, feeding, increase the activity and they become Cyanotic.

34
Q

What is the classic X-Ray finding in tetralogy of Fallot?

A

The classic boot-shaped heart with prominence of the RV and a concavity in the RV outflow tract.

35
Q

What is the most common congenital cardiac anomaly?

A

Ventricular Septum Defect.

36
Q

What are the components of the tetralogy of Fallot?

A
  1. Pulmonary stenosis.
  2. Right ventricular Hypertrophy.
  3. Ventricular Septal defect.
  4. Overriding aorta.
37
Q

Explain how the great vessels are attached in a transposition of the great vessels.

A

Pulmonary trunk attaches to the LV.

Aorta attaches to the RV.

38
Q

A 45-year-old man presents with a BP of 160/90 on the right arm and 170/92 on the left arm. There are no palpable pulses in the feet/ankle. What problem does this patient most likely have?

A

Coarctation of the Aorta. Adult form.

39
Q

Describe blood flow a PDA.

A
1) In utero:
Right-to-left shunt.
Pulmonary artery --> Aorta.
Bypasses pulmonary circulation. 
2) After birth:
Left-to-right shunt.
Aorta--> Pulmonary artery.
40
Q

What heart defect is associated with chromosome 22q11 deletion?

A

Truncus arteriosus and tetralogy of Fallot.

41
Q

What heart defect is associated with Down syndrome ?

A

Endocardial cushion defect.

42
Q

What heart defect is associated with congenital rubella?

A

PDA and pulmonary artery stenosis.

43
Q

What heart defect is associated with Turner syndrome?

A

Coarctation of the aorta and bicuspid aortic valve.

44
Q

What heart defect is associated with Marfan syndrome?

A

Aortic insufficiency.

45
Q

RFF: Continuous machinery-like heart murmur.

A

PDA

46
Q

RFF: Boot-shaped heart

A

Tetralogy of Fallot.

47
Q

RFF: Rib notching.

A

Coarctation of the aorta.

48
Q

RFF: Most common congenital cardiac anomaly.

A

VSD.

49
Q

RFF: Most common congenital cause of Early cyanosis?

A

Tetralogy of Fallot.

50
Q

Name the congenital heart defect associated with three most common causes of R->L shunting

A
  1. Atrial septal defect (ASD).
  2. VSD.
  3. Patent ductus arteriousus.
51
Q

Which are the two most common congenital cardiac defects ?

A
  1. VSD

2. ASD

52
Q

Name the congenital heart defect associated with pulmonic stenosis, Right ventricular hypertrophy, Overriding Aorta, VSD

A

Tetralogy of Fallot.

53
Q

Name the congenital heart defect associated with boot-shaped cardiac silhouette

A

Tetralogy of Fallot.

54
Q

Name the congenital heart defect associated with lower body cyanosis?

A

Preductal coarctation.

55
Q

Defect causing upper extremity HTA and diminished lower extremity pulses.

A

Pstductal coarctation (stenosis distal to the ductus arteriosus)

56
Q

Name the congenital heart defect associated with Turner syndrome?

A

Coarctation of the aorta.

57
Q

Name the congenital heart defect associated with Down syndrome?

A

ASDs, VSDs, and AV valve abnormalities (due to endocardial cushion abnormalities).

58
Q

Name the congenital heart defect associated with rubella.

A

PDA

59
Q

Name the congenital heart defect associated with 22q11 syndrome

A

Truncus arteriosus, tetralogy of Fallot.

60
Q

Common Cyanotic congenital heart defect in children born to diabetic mothers.

A

Transposition of the great vessels.

61
Q

Aorta arises from right ventricle and pulmonary trunk arises from left ventricle.

A

Transposition of the great vessels

62
Q

Valvular defect associated with increased risk of infective endocarditis and calcification

A

Bicuspid aortic valve.

63
Q

What is the consequence of leaving a left-to-right stunting untreated?

A

Right-to-left.

64
Q

What type of infection are patients with VSD at an increased risk for?

A

Infective Endocarditis

65
Q

What drug is used to induce closure of a PDA?

A

Indomethacin.

66
Q

What drug is used to prevent closure of a PDA?

A

Prostaglandins.

67
Q

What is the most common genetic cause of congenital heart disease?

A

Trisomy 21.