Congenital Heart Disease

Question 1

What is congenital heart disease?

Answer 1

Abnormality of the structure of the heart present at birth

Question 2

Mild congenital heart disease

Answer 2

Asymptomatic, may resolve spontaneously

Question 3

Moderate congenital heart disease

Answer 3

Requires specialist intervention and monitoring in a cardiac centre

Question 4

Severe congenital heart disease

Answer 4

Present severely ill/die in newborn period or early infancy

Question 5

Major congenital heart disease

Answer 5

Requires surgery within first year of life

Question 6

When can congenital heart disease present?

Answer 6

Antenatally, soon after birth, day 1-2 baby check, day 3-7, 4-6 weeks, 6-8 weeks GP check

Question 7

Signs of congenital heart disease soon after birth

Answer 7

Cyanosis

Question 8

Signs of congenital heart disease at day 1-2 baby check

Answer 8

Murmurs, abnormal pulses, cyanosis

Question 9

Signs of congenital heart disease at day 3-7

Answer 9

Sudden circulatory collapse, shock, cyanosis, sudden death

Question 10

Signs of congenital heart disease at 4-6 weeks

Answer 10

Signs of cardia failure - reduced feeding, failure to thrive, breathlessness, sweating

Question 11

Signs of congenital heart disease at 6-8 weeks

Answer 11

Incidental finding of murmurs at other clinical contents

Question 12

Management of antenatally diagnosed congenital heart disease

Answer 12

Depends on disease, expert team available, may decide to deliver in cardiac surgical centre, prostaglandin infusion if duct dependent lesion

Question 13

What does antenatal screening show in relation to heart?

Answer 13

Ultrasound at 18-22 weeks will show 4 chamber heart view and outflow tract view

Question 14

What does the newborn screening exam involve in relation to the heart?

Answer 14

Femoral pulses, heart sounds, presence of murmurs

Question 15

What can a murmur at baby check indicate

Answer 15

Small muscular ventricular septal defect - no haemodynamic consequences and many close spontaneously

Question 16

What can cause cyanosis in a baby?

Answer 16

Any condition causing deoxygenated blood to bypass the lungs and enter the systemic circulation, any condition where mixed oxygenated and deoxygenated blood enters the systemic circulation from the heart

Question 17

Example of congenital heart condition that can cause cyanosis of the newborn

Answer 17

Transpositon of the great arteries

Question 18

Differential diagnosis of cyanosis in a newborn

Answer 18

Cardiac disease, respiratory disease, persistent pulmonary hypertension of the newborn

Question 19

Signs of collapse at time of duct closure

Answer 19

Severe cyanosis or pallor, tachycardia, distress, rapid deterioration to death, prolonged capillary refill time, poor or absent pulses, hepatomegaly, crepitations

Question 20

Treatment for collapse at time of duct closure

Answer 20

ABC, Prostaglandin E2, multisystem supportive treatment

Question 21

Examples of duct dependent conditions - duct dependent systemic circulation

Answer 21

Hypoplastic left heart, critical aortic stenosis, interrupted aortic arch, critical coarctation of the aorta

Question 22

Examples of duct dependent conditions - duct dependent pulmonary circulation

Answer 22

Tricuspid atresia, pulmonary atresia

Question 23

Clinical signs of cardiac failure in babies

Answer 23

Failure to thrive, slow/reduced feeding, breathlessness, sweating, hepatomegaly, crepitations

Question 24

Patent ductus arteriosus repair

Answer 24

Closure by device, catheter procedure, couple of follow up appointments to ensure the flow is stopped and the device is in the correct position

Question 25

Ventricular septal defect repair

Answer 25

Closure by patch, follow up during childhood/adolescence. Rhythm problems or valve problems may occur, but generally expected to go on to live a normal life

Question 26

Hypoplastic left heart surgery

Answer 26

3 stage complex surgery with significant mortality at each stage and between. Ends with RV supplying circulatory system but will fail over time so a transplant will be needed