Congenital Heart Disease

Question 1

What is congenital heart disease?

Answer 1

A structural abnormality of the heart or the intra-thoracic great vessels that is present at birth and is actually or potentially of great importance

Question 2

What is the prevalence of CHD?

Answer 2

8-9 per 1000 births, 10-20% of still births/miscarriages

Question 3

What is the presentation of CHD in newborns?

Answer 3

Cyanosis
Congestive cardiac failure
Cardiac murmur

Question 4

What associations does CHD have in newborns?

Answer 4

Chromosomal defects (Down’s syndrome)
Syndromes
Extra cardiac defects
Teratogens

Question 5

What causes cyanotic heart disease?

Answer 5

Abnormal connections- transposition of great arteries
Right to left shunts- tetralogy of fallot, pulmonary atresia
Abnormal mixing- univentricular hearts

Question 6

What is the incidence of univentricular heart in patients with CHD?

Answer 6

1%

Question 7

What are the associations of univentricular heart with CHD?

Answer 7

Pulmonary stenosis/atresia
Subaortic stenosis
Coarctation of aorta

Question 8

What are the signs of heart failure in infancy?

Answer 8

Tachypnoea (breathlessness)- R/R > 60 per min
Tachycardia- HR>160 bpm
Hepatomegaly (enlargement of the liver)

Question 9

What are the causes of heart failure in infancy?

Answer 9

Left to right shunts

Obstructive lesions of left heart

Question 10

What is atrial septal defect?

Answer 10

A birth defect in the heart in which there is a hole in the septum that divides the atria of the heart

Question 11

What is the incidence of ASD?

Answer 11

7-10%

Question 12

What associations are there with ASD?

Answer 12

Mitral regurgitation (primum type)
Partial anomalous pulmonary venous drainage (SV type)

Question 13

What is coarctation of the aorta?

Answer 13

Birth defect in which a part of the aorta is narrower than usual

Question 14

What is the incidence of coarctation of the aorta?

Answer 14

5%

Question 15

What are the associations of coarctation of aorta?

Answer 15

Bicuspid valve
Ventricular septal defect
Subaortic stenosis

Question 16

What is aortic stenosis?

Answer 16

Narrowing of the area underneath the aortic valve that causes some degree of obstruction or blockage of blood flow through the heart

Question 17

What is the incidence of aortic stenosis?

Answer 17

2%

Question 18

What are the associations of aortic stenosis?

Answer 18

Mitral valve abnormalities
EFE (endocardia fibroelastosis)
Coarctation of aorta
Left ventricular hypoplasia

Question 19

What are the two possible diagnoses of patients with asymptomatic murmurs in childhood?

Answer 19

Innocent cardiac murmur

Asymptomatic congenital heart disease

Question 20

What causes asymptomatic congenital heart disease?

Answer 20

Small ventricular septal defect
Atrial septal defect 
Pulmonary valve stenosis
Aortic valve stenosis 
Coarctation of aorta

Question 21

What are the characteristics of an innocent murmur?

Answer 21

No symptoms 
Normal heart sounds 
No added sounds 
No thrill 
Normal ECG 
Normal chest X-ray

Question 22

What is the definition of an innocent heart murmur?

Answer 22

Any murmur produced by a normal cardiovascular system under normal resting conditions

Question 23

What prosthetic materials are used in cardiac surgery?

Answer 23

Dacron- patch closure of VSD
Gortex tubes- shunts, conduits
Prosthetic valves- aortic mitral
Pacemaker leads

Question 24

What is infective endocarditis?

Answer 24

Infection of the inner lining of the heart muscle (endocardium) caused by bacteria, fungi or germs that enter through the bloodstream
It is rare in childhood but remains a potential cause of serious morbidity and mortality
Most people effected usually have underlying structural heart disease

Question 25

What organisms cause infective endocarditis?

Answer 25

In most series streptococci (viridans more than enterococci) are the most common causative organism with staphylococci (staphylococcus aureus and staphylococcus epidermis) being the second largest group

Question 26

What lesions are most commonly complicated by infective endocarditis?

Answer 26

Ventricular septal defect Aortic stenosis Tetralogy of fallot Patent ductus arteriosus

Question 27

How is infective endocarditis diagnosed?

Answer 27

Isolation of the infecting organism from blood culture is crucially important in making the diagnosis and guiding appropriate antimicrobial therapy Two or three blood cultures over a 24 hour period Candida infection should also be considered in those with negative blood cultures Echocardiography

Question 28

What are some of the clinical features of infective endocarditis?

Answer 28

``` Fever Heart murmur (new or changing murmur) General malaise Heart failure Splenomegaly Microscopic haematuria Petechiae Osler nodes Splinter haemorrhages ```

Question 29

What is the treatment of infective endocarditis?

Answer 29

Treatment is required for a minimum of 4 weeks and usually continued for 6 weeks For streptococcus viridans the combination of penicillin and gentamicin given intravenously is effective For streptococcus faecalis the combination of amoxicillin and gentamicin is used Flucloxacillin and gentamicin is the first choice therapy for staphylococcal infection Candida infection is difficult to treat and may require a combination of ampotericin and fluocytosine, it may prove impossible to eradicate

Question 30

According to the NICE guidelines, what conditions put patients at risk of infective endocarditis?

Answer 30

``` Acquired valvular heart disease with stenosis/regurgitation Valve replacement Hypertrophic cardiomyopathy Structural congenital heart disease Previous infective endocarditis carditis ```

Question 31

When not to offer antibiotic prophylaxis with IE?

Answer 31

Dental procedures Non-dental procedures- upper and lower GIT, genitourinary tract, upper and lower respiratory tract Do no offer chlorhexidine mouthwash as prophylaxis against IE to people at risk undergoing dental procedures

Question 32

What important information should be given to patients with IE before undergoing dental procedures?

Answer 32

Benefits and risks of antibiotic prophylaxis with explanation to why its not longer routinely recommended The importance of maintaining good oral health Symptoms that may indicate IE The risks of undergoing invasive procedures, including non-medical procedures