Paediatrics Cardiology

Question 1

What congenital heart defect is Down’s syndrome associated with?

Answer 1

Ventricular Septal Defect

very - v- resiliant

Question 2

What congenital heart defect are patients with Turner’s Syndrome associated with?

Answer 2

Coarction of the aorta (descending aorta usually effected)

Question 3

What type of aneurism are children with Kawasaki at risk of?

Answer 3

coronary artery aneurism

Question 4

‘cyanosis’ in the newborn is a buzzword for?

Answer 4

congenital heart defect

Question 5

How do you differentiate between 1st and 2nd heart sound?

Answer 5

feel the pulse at the same time

Question 6

What is the ‘foramen oval’ its purpose and when does it close?

Answer 6

Theforamen ovaleis a hole in the wall between the left and right atria of every human fetus. It allows blood to bypass the fetal lungs, which cannot work until air is inhaled after birth

-therefore foramen ovale is present in babies not been born yet!

Question 7

What is the name of the condition if the ‘foramen ovale’ fails to close at birth?

Answer 7

Atrial Septal Defect

(*as foramen ovale between right and left atria)

Question 8

What is ductus arteriosus?

Answer 8

This is a shunt between the pulmonary artery and aorta allowing for it to bypass the foetal lungs. This is held open due to the rise in Prostoglandin E2.

Shunt closed when baby is born and prostaglandin levels drop

Question 9

What is the name of the shunt which bypasses the foetal lungs and connects the pulmonary artery to the aorta?

Answer 9

ductus arteriosus- between the pulmonary arteries and aorta

Question 10

What is the role of E2 prostaglandin in Patent Ductus Arteriosus?

Answer 10

-high levels during foetal life, levels drop when baby is born allowing for shunt to close

ductus arteriosus- shunt between pulmonary arteries and aorta

Question 11

“machine like murmur” is associated with?

Answer 11

patent ductus arteriosus

make PAint in machine

Question 12

What congenital heart condition has normal S1 and abnormal S2

Answer 12

patent ductus arteriosus

Question 13

What is the management of Patent Ductus Arteriosus?
b) what is the purpose of this medication

Answer 13

1st line- indomethacin, NSAIDs or paracetamol

-blocks the E2 prostaglandin synthesis and makes the shunt close

Question 14

What is the mechanism of atrial septal defects?

Answer 14

This is when there is a defect between the right and left atria in the heart. Therefore allowing for blood to flow between them.

Question 15

‘crescendo-decrescendo murmur loudest at the upper left sternal border’ is associated with what heart defect?

Answer 15

Atrial Septal Defect- crescendo- top- atria

Question 16

What is the diagnostic investigation for most Congenital Cardiac Defects in paediatrics?

(e.g ASD, Patent Ductus arteries and Tetralogy of Fallot etc)

Answer 16

Diagnostic Investigation:

1st line- echocardiogram

Question 17

What is Ventricular Septal Defect?

Answer 17

This is a congenital hole in the septum between the left and right ventricles. Blood flows from the left to the right ventricle because the pressure is higher in the left.

(left > right shunt formed

Question 18

What are the x2 heart murmurs which can cause a ‘pan-systolic murmur?’

Answer 18

Mitral regurgitation and tricuspid regurgitation

Question 19

What congenital condition is a ‘pan systolic murmur associated with’

Answer 19

pan- cook -venison

Ventricular Septal Defect

Question 20

What genetic condition is associated with coarction of the aorta?

Answer 20

Turner’s syndrome

Question 21

What is correction of the aorta?
b) does it effect ascending or descending aorta

Answer 21

nition:

This refers to a congenital narrowing of the descending aorta.

Question 22

What are the X4 conditions associated with the Tetralogy of Fallot?

(POVR)

Answer 22

X4 Congenital Conditions:

• Pulmonary Stenosis
• Over-Riding Aorta
• Ventricular Septal Defect
• Right ventricular hypertrophy

Question 23

What side-side of the heart does blood flow

Answer 23

left > right as left side is stronger

Question 24

‘boot shaped heart’ on CXR is associated with?

Answer 24

Tetralogy of Fallot

Question 25

What is the management of tetralogy of fallot? b) what is the management of cyanotic episodes?

Answer 25

**Management:** 1st line- E2 prostoglandin infusion (do not want it to shut as we need the blood flow) Cyanotic Episodes: 1st line- beta blockers may help with the infundibular spasm

Question 26

What congenital defect is associated with mother taking these medications during pregnancy? -NSAIDs -lithium -phenytoin -sodium valproate or methotrexate

Answer 26

-NSAIDs (*mainly 1st trimester)-congenital heart defects as blocks prostaglandin synthesis -lithium- Ebstein's anomaly -phenytoin- cleft palate -sodium valporate- neural tube defects (e.g spina bifida)

Question 27

What is Ebstein's Anomaly, and what valve is it associated with?

Answer 27

This is a congenital heart condition where the tricuspid valve is set lower in the right side of the heart.

Question 28

What is Transposition of the Great Arteries?

Answer 28

This is a form of cyanotic congenital heart disease. This is where the pulmonary trunk and aorta in the heart swap positions.

Question 29

What is the acute and definitive management of Transposition of the Great Arteries? (*MLA question)

Answer 29

**Management:** 1st line- E2 prostoglandin to keep the ductus arteriosus open and maintain blood supply **Definitive Mangement:** Arterial Switch Operation- Aorta and pulmonary trunk are switched to correct their positions

Question 30

What is the definitive management for Transposition of the Great Vessels?

Answer 30

**Definitive Mangement:** Arterial Switch Operation- Aorta and pulmonary trunk are switched to correct their positions

Question 31

Ejection systolic at the left sternal edge is most associated with? (*common MLA)

Answer 31

Atrial septal defect (ASD) (a CLOSEST to E- need to know)

Question 32

What is a Tet Spell? b) What congenital cardiac condition is it associated with? c) what are the causes

Answer 32

Tet spells are intermittent cyanotic episodes caused by a temporary worsening of the right-to-left shunt. causes- distress, squatting or physical exertion due to extra CO2 retention Management:

Question 33

What is the management of Tet Spells? b) these are associate with Tetralogy of fallot exacerbations due to excess VO2 retention during exercise or distress

Answer 33

Oxygen IV morphine (decreases respiratory drive and pulmonary vascular resistance) IV fluids (increases circulating volume) IV beta blockers (e.g., propranolol) Phenylephrine infusion (increases systemic vascular resistance)

Question 34

high flow O2 makes no difference in what congenital heart condition

Answer 34

transposition of great arteries-pulmonary arteries and aorta switched so circuit is fucked

Question 35

What main feature in tetralogy of fallot controls the extent of cyanosis

Answer 35

pulmonary stenosis