Congenital Heart Disease

Question 1

***Atrial septal defect? Signs? Ix? Mx?

Answer 1

(1.) Higher pressure in LA than RA causes a left-right shunt via fossa ovale (shunt reversal). So, there is an inc flow into the right heart and lungs

(2. ) Signs
- Small ASD will be asymptomatic and can be left alone
- Large ASD = RV volume overload Sx: dyspnoea, SoB, chest infections
- splitting S2 sound

(3. ) Ix
- ECHO = RVH
- CXR = Big pulmonary arteries and heart

(4.) Mx: Surgical or percutaneous

Question 2

Atrio-ventricular septal defects

Answer 2

(1. ) Often trisomy 21 (down syndrome)
(2. ) Hole in centre of heart - involving venticular septum, atrial septum, mitral and tricuspid valves. Can be partial or complete. Instead of two separate valves, there is one big malformed AV valve.

(3. ) Signs and Symptoms
- Complete defect = breathlessness as neonate, poor weight gain, poor feeding, torrential pulmonary blood flow, eisenmenger syndrome,
- Partial defect = can present in late adulthood, presents like a small VSD/ASD, may be left alone if there is no right heart dilation

(4. ) Management
- Complete defect needs repair or PA band
- This is usually in infancy making repair is surgically challenging

Question 3

Patent ductus arteriosus

Answer 3

(1. ) Large = torrential flow form the aorta to the pulmonary arteries in infancy
- Breathlessness, poor feeding, failure to thrive
- More common in prem babies
- Needs to be surgically closed

(2. ) Small = little flow from aorta to PA
- Usually asymptomatic
- Murmur found incidentally - Endocarditis risk

(3. ) Clinical signs
- Continuous ‘machinery’ murmur
- If large, big heart, breathless
- Eisenmenger syndrome (blue and clubbed toes, pink and not-clubbed fingers)

(4. ) Closure by surgical or percutaneous
- Venous approach

Question 4

***Contraction of the Aorta? Signs? Ix? Mx?

Answer 4

(1. ) Narrowing of aorta at ductus arteriosus/ligamentum arteriosum.
(2. ) Due to the location of the narrowing (before subclavian artery), BP is raised in head and neck vessels but reduced distally. So BP is high in upper body but low in lower body.
(3. ) Death may occur due to LV failure.

(4. ) Signs and Sx
- HTN: right arm, head and neck
- ‘bruits’ (turbulent flow)
- Radio-femoral delay
- Murmur

(5. ) Ix = MRI
(6. ) Mx = Surgery

Question 5

Bicuspid aortic valve and aoropathy

Answer 5

Normal aortic valve has 3 cusps, whereas BAV has two

• BAV degenerate quicker than normal valves
• Can become severely stenotic
• Become regurgitant earlier than normal valves

Aoropathy

• BAV is usually associated with ascending aortic dilation
• Protective surgery needed for some (>55mm)
• BAV is occasionally associated with coarctation of the aorta

Question 6

***Pulmonary stenosis

Answer 6

(1. ) Obstruction to blood flow from RV to PA
- Severe = RV failure, collapse, Poor pulmonary blood flow, RVH, tricuspid regurgitation
- Moderate/mild = Well tolerated for many years, RVH

(2. ) Dyspnoea, Fatigue, Oedema, Ascites
(3. ) ECG, ECHO
(4. ) Balloon Valvuloplasty

Question 7

Tetra of Fallot

Answer 7

(1. ) Dislocation of the septum below the pulmonary outflow, this results in the following:
- Pulmonary stenosis
- Overriding aorta
- VSD
- RVH

(2.) This elevated RV pressure and right-to-left shunting of blood

(3. ) Signs
- Cyanosis
- ‘fallot spells’

(4.) Surgery = relief of stenosed valve and closure of VSD

Question 8

***What is Eisenmenger Syndrome?

Answer 8

• Due to prolonged pulmonary HTN from the left-to-right shunt
• Results in RVH and increased RV pressure.
• This eventually exceeds LV pressure resulting in reversal of blood flow
• This in turn results in cyanosis and clubbing, blue
• Eisenmenger’s complex is an indication for a heart-lung transplant

Question 9

Univentricular hearts

Answer 9

(1. ) Hearts with one ventricle
(2. ) Pts are pink, reduced exercise capacity

(3. ) Fontan Procedure
- redirecting blood flow from the lower body to the lungs
- IVC disconnected form the heart

Question 10

***What is VSD? Aetiology? Signs in post-natal screens? Mx? Complications?

Answer 10

(1. ) It is the most common cause of congenital heart disease, they close spontaneously in 50%
(2. ) Often associated with Down’s syndrome

(3. ) This may be detected at 20w, post-natal presentation includes:
- Failure to thrive
- hepatomegaly
- Tachypnoea
- Tachycardia
- pallor
- murmur in smaller VSDs

(4. ) Mx:
- Small VSDs = Asx and close spontaneously, just require monitoring
- Moderate-large VSDs = HF Sx develop -> nutritional support, diuretics, surgical closure

(5. ) Complications
- aortic regurgitation
- Infective endocarditis
- Eisenmenger
- RSHF
- Pulmonary HTN