Congenital Cardiovascular Disease

Question 1

Acyanotic congenital CVD

Answer 1

With shunt:

• ventricular septal defect
• atrial septal defect
• patent ductus arteriosus

Without shunt:

• pulmonary stenosis
• coarctation (narrowing) of aorta
• aortic/left heart obstruction

Question 2

Cyanotic congenital CVD

Answer 2

With shunt:

• transposition of great vessels
• Fallot’s teratology

Without shunt:

• hypoplastic left heart
• severe pulmonary stenosis
• pulmonary/tricuspid atresia with or without intact septum

Question 3

Blood flow with acyanotic CVD

Answer 3

• increased pulmonary blood flow

- obstruction to blood flow from ventricles

Question 4

Blood flow with cyanotic CVD

Answer 4

• decreased pulmonary blood flow

- mixed blood flow

Question 5

Causes of congenital CVD

Answer 5

• genetic/chromosomal syndromes which can be passed through families
• retinoic acid for acne
• alcohol
• infections
• poorly controlled blood sugar in diabetics

Question 6

Extracardia anomalies in CHD

Answer 6

• consequence of CHD
• usually in MSK system
• increases mortality in infants with CHD

Question 7

Symptoms/signs of heart failure in infants with CHD

Answer 7

• cyanosis
• rapid breathing (tacypnoea)
• extra work for breathing
• grunting
• tachycardia
• poor circulation
• faltering growth
• fatigue (poor feeding)
• sweating
• enlarged liver
• murmurs

Question 8

Complications of CHD

Answer 8

• faltering growth (failure to thrive)
• paradoxical embolus
• bacterial endocarditis
• pulmonary hypertension
• polycythaemia (high conc of RBC)
• haemoptysis (coughing blood)
• arrythmias

Question 9

Ventricular septal defect

Answer 9

• small ones may cause no problem
• large VSDs allow large amount of blood from left ventricle to right
• left side works harder than normal
• extra flow increases BP in right heart and lungs
• extra workload can result heart failure/poor growth
• if hole doesn’t close can result in scarring of arteries and pulmonary hypertension/shunt damage (Eisenmenger’s Syndrome)

Question 10

Atrial septal defect

Answer 10

• small ones don’t need special treatment and most close themselves as heart grows
• med/large ASDs can be treated with a catheter procedure or open-heart surgery
• if hole doesn’t close can result in scarring of arteries and pulmonary hypertension/shunt damage (Eisenmenger’s Syndrome)

Question 11

Patent ductus arteriosus

Answer 11

• if respiratory distress/impaired systemic oxygen delivery treatment needed
• IV indomethacin/ibuprofen can close PDA if given in first 2 weeks
• can be closed with catheter/surgical ligation

Question 12

Coarctation of aorta

Answer 12

• isolated defect or associated with other lesions such as bicuspid aortic valve/VSD
• usually in thoracic aorta
• left ventricular hypertrophy
• congestive heart failure in infants
• hypertension in older children

Question 13

Clinical presentation of coarctation of aorta

Answer 13

• non-severe stenosis: asymptomatic
• angina pectoris
• leg claudication
• diminished femoral pulses
• differential upper and lower extremity blood pressure

Question 14

Treatment of coarctation of aorta

Answer 14

• primary surgical repair with excision of coarctation and end to end anastomosis
• balloon angioplasty
• subclavian flap repair

Question 15

Valve atresia

Answer 15

valve doesn’t form correctly, lacks hole for blood to pass through

Question 16

Valve stenosis

Answer 16

flaps of valve thicken, stiffen or fuse together

Question 17

Valve regurgitation

Answer 17

valve doesn’t close tightly, blood leaks back through valve

Question 18

Pulmonary valve stenosis

Answer 18

• most common
• heart murmur
• no treatment if mild
• treated with catheter procedure

Question 19

Aortic valve stenosis treatment

Answer 19

• balloon aortic valvuloplasty

- if failure, surgical repair/replacement of aortic valve

Question 20

What is Teratology of Fallot?

Answer 20

• combination of 4 defects
• pulmonary valve stenosis
• large VSD
• overriding aorta: deoxygenated blood can flow into aorta instead of pulmonary trunk
• right ventricular hypertrophy

Question 21

Tet spells

Answer 21

• unrepaired teratolgy
• sudden episodes of cyanosis and hypoxia
• can be triggered by events that decrease O2 sat/systemic vascular resistance/tachycardia/hypovolemia

Question 22

Hypoplastic left heart syndrome

Answer 22

• after ductus arteriosus closes

- blood flows to lungs then to left heart where it is blocked and cannot circulate body

Question 23

Transposition of great arteries

Answer 23

• pulmonary artery is supplied by left ventricle
• aorta supplied by right ventricle
• opposite arrangement
• survival only if shunt between left and right heart and enlarged ASD

Question 24

Truncus arteriosus

Answer 24

• aorta and pulmonary trunk both arise from common trunk

- blood mixes and exits through single valve

Question 25

Ebstein's anomaly

Answer 25

- tricuspid valve allows blood to flow backwards from right ventricle into right atrium during contraction - mild: acyanotic - severe: cyanotic

Question 26

Total anomalous pulmonary venous drainage

Answer 26

- pulmonary veins connected to veins from main circulation instead of left atrium - returns blood to right side of heart - blue babies with signs of heart failure

Question 27

Atrioventricular septal defect

Answer 27

- holes between chambers of left and right sides of heart - malformation of valves between chambers - common in Down Syndrome

Question 28

Eisenmenger's syndrome

Answer 28

- shunt reversal - large left to right shunt - pulmonary arterial hypertension, right sided heart pressure exceeds left side and shunt reverses - cyanosis