Developmental Pathology

Question 1

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

Answer 1

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

Question 2

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

Answer 2

Late Cyanosis (Blue kids)

Question 3

What kind of shunt developed a early cyanosis?

Answer 3

Right-to-Left shunt (eaRLy cyanosis).

Left-to-Right shunt (Later cyanosis)

Question 4

What is the most common congenital cardiac defect ?

Answer 4

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

Question 5

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

Answer 5

About 40% close spontaneously within the first six months.

Question 6

When is the ductus Arteriosus supposed to close?

Answer 6

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

Question 7

Which is the characteristics murmur in the patent ductus arteriosus?

Answer 7

Continuous machinery-like heart murmur.

Question 8

What is the treatment of the PDA ?

Answer 8

Treatment Involved Indomethacin which block prostaglandins synthesize.

Question 9

In which situation is helpfull keep the PDA open?

Answer 9

Transposition of the great vessels.

Question 10

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

Answer 10

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

Question 11

Which are the characteristics of the Eisenmenger syndrome?

Answer 11

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

Question 12

Why we used Indomethacin in the PDA?

Answer 12

Because we try to prevent Eisenmenger syndrome.

Question 13

What is the definition of Coarctation of the aorta?

Answer 13

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

Question 14

Which congenital heart disease is associated with Turner syndrome?

Answer 14

Coarctation of the Aorta.

Question 15

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

Answer 15

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

Question 16

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

Answer 16

Coarctation of the Aorta.

Question 17

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

Answer 17

Turner syndrome.

Question 18

Which congenital cardiac defect is associated with Down syndrome?

Answer 18

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

Question 19

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

Answer 19

22q11 (DiGeorge) syndrome.

Question 20

Which are the defects associated with congenital rubella?

Answer 20

Septal defects, PDA, Pulmonary artery stenosis.

Question 21

What is the sequence of the Eisenmenger syndrome?

Answer 21

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

Question 22

type of shunt the patients develop cyanosis early?

Answer 22

Right-to-Left.

Question 23

Which are the 5 causes of right Cyanotic Heart Disease?

Answer 23

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

Question 24

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

Answer 24

Persistent Truncus arteriosus.

Question 25

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

Answer 25

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.

Question 26

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

Answer 26

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

Question 27

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

Answer 27

IDDM (Infants of Diabetic Mother).

Question 28

Which are the complications that suffer the IDDM?

Answer 28

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

Question 29

What is the Tricuspid Atresia?

Answer 29

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

Question 30

What is the Ebstein anomaly?

Answer 30

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

Question 31

Which are the characteristics of Ebstein Anomaly?

Answer 31

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.

Question 32

Which are the parts that conform the Tetralogy of Fallot?

Answer 32

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

Question 33

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

Answer 33

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

Question 34

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

Answer 34

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

Question 35

What is the most common congenital cardiac anomaly?

Answer 35

Ventricular Septum Defect.

Question 36

What are the components of the tetralogy of Fallot?

Answer 36

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

Question 37

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

Answer 37

Pulmonary trunk attaches to the LV. | Aorta attaches to the RV.

Question 38

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?

Answer 38

Coarctation of the Aorta. Adult form.

Question 39

Describe blood flow a PDA.

Answer 39

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

Question 40

What heart defect is associated with chromosome 22q11 deletion?

Answer 40

Truncus arteriosus and tetralogy of Fallot.

Question 41

What heart defect is associated with Down syndrome ?

Answer 41

Endocardial cushion defect.

Question 42

What heart defect is associated with congenital rubella?

Answer 42

PDA and pulmonary artery stenosis.

Question 43

What heart defect is associated with Turner syndrome?

Answer 43

Coarctation of the aorta and bicuspid aortic valve.

Question 44

What heart defect is associated with Marfan syndrome?

Answer 44

Aortic insufficiency.

Question 45

RFF: Continuous machinery-like heart murmur.

Answer 45

PDA

Question 46

RFF: Boot-shaped heart

Answer 46

Tetralogy of Fallot.

Question 47

RFF: Rib notching.

Answer 47

Coarctation of the aorta.

Question 48

RFF: Most common congenital cardiac anomaly.

Answer 48

VSD.

Question 49

RFF: Most common congenital cause of Early cyanosis?

Answer 49

Tetralogy of Fallot.

Question 50

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

Answer 50

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

Question 51

Which are the two most common congenital cardiac defects ?

Answer 51

1. VSD | 2. ASD

Question 52

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

Answer 52

Tetralogy of Fallot.

Question 53

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

Answer 53

Tetralogy of Fallot.

Question 54

Name the congenital heart defect associated with lower body cyanosis?

Answer 54

Preductal coarctation.

Question 55

Defect causing upper extremity HTA and diminished lower extremity pulses.

Answer 55

Pstductal coarctation (stenosis distal to the ductus arteriosus)

Question 56

Name the congenital heart defect associated with Turner syndrome?

Answer 56

Coarctation of the aorta.

Question 57

Name the congenital heart defect associated with Down syndrome?

Answer 57

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

Question 58

Name the congenital heart defect associated with rubella.

Answer 58

PDA

Question 59

Name the congenital heart defect associated with 22q11 syndrome

Answer 59

Truncus arteriosus, tetralogy of Fallot.

Question 60

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

Answer 60

Transposition of the great vessels.

Question 61

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

Answer 61

Transposition of the great vessels

Question 62

Valvular defect associated with increased risk of infective endocarditis and calcification

Answer 62

Bicuspid aortic valve.

Question 63

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

Answer 63

Right-to-left.

Question 64

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

Answer 64

Infective Endocarditis

Question 65

What drug is used to induce closure of a PDA?

Answer 65

Indomethacin.

Question 66

What drug is used to prevent closure of a PDA?

Answer 66

Prostaglandins.

Question 67

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

Answer 67

Trisomy 21.