Congenital Heart Disease

Question 1

List the 5 acyanotic heart diseases

Answer 1

Ventricular septal defect (most common)
Atrial septal defect
Patent ductus arteriosus
Coarctation of aorta
Aortic valve stenosis

Question 2

List the 3 cyanotic heart diseases

Answer 2

Tetralogy of fallot (most common)
Transposition of the great arteries
Tricuspid atresia

Question 3

Which cyanotic heart disease tends to present first?

Answer 3

TGA presents at birth
ToF presents at 1-2 months

Question 4

Define congenital heart disease.

Answer 4

Heart disease present at birth caused by a malformation.

Question 5

Explain the aetiology of left-to-right shunts (“Acyanotic”) congenital heart disease.

Answer 5

• Atrial septal defects: Secundum ASD; Partial AVSD
• Atria-ventricular septal defect
• Ventricular septal defects

Blood flows from L to R as pressure is greater on LHS. Thus less oxygenated blood flows around the body. More blood flows through the pulmonary artery which can lead to increased resistance + ultimately pulmonary stenosis.

• PDA: blood flows from aorta into the pulmonary artery as pressure is greater in the aorta. Similar consequences as in ASDs, VSDs, + AVSDs

Question 6

What are the 4 components of ToF?

Answer 6

Pulmonary valve stenosis
RV hypertrophy
VSD
Over-riding aorta

Question 7

What is tricuspid atresia?

Answer 7

Lack of tricuspid valve, blood can’t flow from RA to RV
Blood pushed through ASD to LA, through mitral valve to LV, some through VSD to RV + lungs, some to body (deoxygenated)

Question 8

Explain the aetiology of right-to-left shunts (“Cyanotic”) congenital heart disease.

Answer 8

Blood is flowing from R to L– effectively bypassing the lungs.

Question 9

What is true ToGA? What is the consequence of this?

Answer 9

Pulmonary artery connected to LV + aorta to RV
which means no oxygenation at all= fatal
Thus ToGA must also present with a VSD to be compatible with life.

Question 10

Explain the aetiology of obstructive congenital heart disease.

Answer 10

• Coarctation of aorta
  
  • Arterial duct tissue encircling the aorta at the point of insertion of the duct. When the PDA closes so does the artery.
• Pulmonary stenosis : Fusing of the pulmonary artery valve leaflets.

Question 11

Summarise the epidemiology of congenital heart disease.

Answer 11

1% of live births.
TOF is the most common cyanotic disorder.

Question 12

What are the presenting symptoms for left-to-right congenital heart disease?

Answer 12

None, except for large L-to-R congenital heart disease which presents with heart failure.

Question 13

What are the signs for secundum ASD left-to-right congenital heart disease?

Answer 13

ESM at ULSE
Fixed wide split S2

Question 14

What are the signs for partial AVSD left-to-right congenital heart disease?

Answer 14

ESM at ULSE
Fixed split S2
Pansystolic murmur at apex

Question 15

What are signs of VSD?

Answer 15

Pansystolic/ early systolic murmur at LLSE

Question 16

What are 5 signs of an AVSD?

Answer 16

Hyperactive precordium
Pansystolic murmur
Systolic thrill
Tachypnoea
Hepatomegaly

Question 17

What are 5 signs for PDA left-to-right congenital heart disease?

Answer 17

Continuous “machinery” mumur at ULSE + subclavicular
Bounding pulses
Left subclavicular thrill
Wide pulse pressure
Heaving apex beat

Question 18

What are 5 signs and symptoms of Tetralogy of Fallot right-to-left congenital heart disease?

Answer 18

Loud ESM at 2nd ICS, ULSE
Clubbing of fingers + toes (older)
Hypercyanotic spells
“Boot shaped” heart of CXR
RV Hypertrophy of ECG

Question 19

What are the signs and symptoms of transposition of the great arteries right-to-left congenital heart disease?

Answer 19

Neonatal cyanosis
No murmur
Tachypnoea
Loud single S2
Prominent RV impulse
“Egg on side” on CXR

Question 20

What are 4 signs and symptoms of Eisenmenger’s syndrome right-to-left congenital heart disease?

Answer 20

No murmur
Right heart failure (late)
Cyanosis
Clubbing

Question 21

What are the signs and symptoms of pulmonary stenosis obstructive congenital heart disease?

Answer 21

Asymptomatic
ESM at ULSE, +/- thrill.
Left parasternal heave.
Cyanosis (if a/w septal defect)

Question 22

What are 4 signs and symptoms of duct-dependent coarctation of aorta obstructive congenital heart disease?

Answer 22

Acute circulatory collapse 2 days of age when duct closes.

Severe heart failure signs.

Absent femoral pulses.

Severe metabolic acidosis.

Question 23

What are some appropriate investigations for congenital heart disease?

Answer 23

• Bloods
• ECG: May show axis deviation, ventricular hypertrophy.
• Echocardiography
• CXR
• Nitrogen washout test- To diagnose R-to-L shunts.
  
  • Patient is placed on a ventilator with 100% O2 for 10 mins. If PaO2 remains low after 10 mins = diagnostic of cyanotic heart disease.

Question 24

What is the management plan for acute cyanosis in congenital heart disease? (ToF, TGA, Tricuspid atresia)

Answer 24

• A-E approach
• Start prostaglandin E1 infusion (5 ng/kg per min) to promote mixing blood + keep the ductus arteriosus open.
• Consider balloon atrial septostomy (TGA)
• Consider Blalock-Taussig shunt (ToF + TA)

Question 25

List 5 side effects of prostaglandins, precautions and what should be monitored

Answer 25

SE: apnoea, hypoglycaemia, seizures, hypotension, fever
Intubate
Monitor BMs

Question 26

What is the management plan for Tetralogy of Fallot?

Answer 26

Prostaglandin E1 infusion

Blalock-Taussig shunt: tube between subclavian artery + pulmonary artery

Definitive surgery at 4-6 months to repair the pulmonary valve, closing the VSD + relieving RV outflow tract obstruction.

Question 27

What is the management plan for transposition of the great arteries?

Answer 27

Correct acidosis + hypoglycaemia if present
Prostaglandin E1 infusion
Balloon artery septostomy
Arterial switch procedure in first 2w of life

Question 28

What are potential complications associated with congenital heart disease?

Answer 28

In L-to-R there is an increased risk of Eisenmenger syndrome.

Cyanotic complications.

Death if not identified early.

Shorter life expectancy.

Question 29

What is the prognosis of congenital heart disease?

Answer 29

Dependent on early detection but due to a/w other syndromes life expectancy is usually reduced.

Question 30

Which congenital heart disease presents with ‘tet spells’?

Answer 30

ToF
Paroxysmal hyperpnoea + increased cyanosis that occur spontaneously/ after feeding/ prolonged crying

Question 31

What is balloon artery septostomy?

Answer 31

Breaks the flap valve of the foramen oval + encourages mixing of blood
Bolus heparin given before BAS

Question 32

Name a prostaglandin used in cyanotic heart disease, what is the MOA

Answer 32

Alprostadil
Maintains patency of ductus arteriosus

Question 33

How does Eisenmenger syndrome develop?

Answer 33

Untreated L-R disease
In L-R shunt, increased pulmonary blood flow leads to pulmonary vascular remodelling
Leads to pulmonary vascular resistance
Causes RV hypertrophy
RV pressure >. LV pressure
Shunt reverses
Blood bypasses lungs causing cyanosis