Acyanotic heart diseases

Question 1

Give examples of acyanotic heart diseases

Answer 1

Ventricular septal defect (VSD)
Atrial septal defect (ASD)
Atrio-ventricular septal defect (AVSD)
Patent ductus arteriosus (PDA)

Outflow obstruction:
Pulmonary stenosis
Coarctation of aorta

Question 2

How do acyanotic heart diseases generally present

Answer 2

Normal Skin colour
Exercise intolerance
Fatigue with feeding
Failure to thrive
Recurrent bronchopulmonary infections

Question 3

Describe a ventricular septal defect and what are its risk factors

Answer 3

Most common acyanotic heart disease
Defect in ventricular septum (blood from left to right ventricle)
Leads to RV hypertrophy → pulmonary HTN → LV hypertrophy

Down’s syndrome
Maternal diabetes
Maternal alcohol consumption

Question 4

How do VSDs usually present (symptoms and signs)

Answer 4

Small: Usually asymptomatic until later in life (ES)
Medium-large: FTT, recurrent bronchopulmonary infections, exertional dyspnoea

Pansystolic murmur (intensifies with hand clenching) - louder when the defect is smaller
Mid-diastolic murmur

Question 5

Management fo VSDs

Answer 5

Small: watch and wait (30-50% spontaneously close)

Large: surgery

Question 6

What are ASDs and what are the risk factors (+important complication)

Answer 6

Defect in the atrial septum

Down syndrome
Maternal alcohol consumption

Complication: paradoxical embolus → stroke

Question 7

How do ASDs present (symptoms and signs)

Answer 7

Usually asymptomatic until later life

Ejection systolic murmur
Wide split fixed S2

Question 8

How are ASDs managed

Answer 8

Small: watch and wait (most heal spontaneously)

Large: surgery (patch or plug)

Question 9

Describe patent ductus arteriosus and its risk factors

Answer 9

Failure of the PDA to close after birth (usually closed by day 1)
Left to right shunt → volume overload to pulmonary vessels

Prematurity
TORCH infections (esp. rubella)
Maternal alcohol consumption

Question 10

How does a PDA present (Symptoms and signs)

Answer 10

Small: asymptomatic
Large: non specific symptoms i.e. failure to thrive

Machinery murmur: loud and continuous in the left infraclavicular region
Thrill
Large volume, bounding, collapsing pulse
Wide pulse pressure
Heaving apex beat

Question 11

How are PDAs managed

Answer 11

Symptomatic → echo at 1 week

Indomethacin or ibuprofen infusion
Surgical ligation

Question 12

Describe coarctation of the aorta and its risk factors

Answer 12

Narrowing of the aorta at the distal part of the arch (close to the ductus arteriosus)
Can be preductal or post ductal

Turners syndrome (Bicuspid valve)

Question 13

How does coarctation of the aorta present (symptoms and signs)

Answer 13

Leg pain
Tiredness
Headaches
Dizziness
Epistaxis

Brachio-femoral delay
Raised BP in upper extremities
Reduced BP in lower extremities

Question 14

How do you manage coarctation of the aorta (investigations and management)

Answer 14

CXR: rib notching

1. PGE1 infusion
2. Surgery or balloon angioplasty

Question 15

What are the complications of coarctation of aorta

Answer 15

Secondary HTN
Endocarditis
Berry aneurysm → cerebral haemorrhage

Question 16

Describe atrio-ventricular septal defects and what is a risk factor for ti

Answer 16

Endocardial cushion defect
Complete: ASD + VSD
Partial: Only ASD

Down’s syndrome

Question 17

Describe pulmonary stenosis

Answer 17

Right ventricular outflow obstruction → RVH

Question 18

Describe Eisenmenger syndrome and its pathophysiology

Answer 18

Progression of untreated left-right shunt (acyanotic) which becomes cyanotic/right-left shunt

Prolonged pulmonary HTN due to the LR shunt → remodelling of the pulmonary vessels → compensatory RVH → RV pressure exceeds LV pressure → shunt reversal

Question 19

How does the cyanosis differ between cardiac conditions after Eisenmenger syndrome

Answer 19

ASD/VSD → global cyanosis
PDA → differential cyanosis (lower body only)