Adult congenital heart disease

Question 1

What is congenital heart disease?

Answer 1

any structural heart abnormality that is present from birth

Question 2

Name 4 common congenital heart malformations

Answer 2

atrial septal defect
ventricular septal defect
coarctation of aorta
Tetralogy of Fallot

Question 3

Potential complications in adult congenital heart disease

Answer 3

endocarditis
right ventricular dysfunction
Eisenmenger syndrome
polycythaemia
arrhythmia
pregnancy risks
contraception
genetics
social issues

Question 4

Describe atrial septal defect

Answer 4

(pre-tricuspid shunt)

high pulmonary flow
right heart dilatation
- right atrial dilation
- right ventricle dilation
- pulmonary artery dilation

Question 5

When does atrial septal defect present?

Answer 5

young child - adult (not neonate)

Question 6

How can small atrial septal defects present in young children and adults?

Answer 6

small = incidental murmur in child - paradoxical embolus stroke in adult

Question 7

How can medium atrial septal defects present in young children and adults?

Answer 7

medium = recurrent pneumonia, poor growth and incidental murmur in child - exercise intolerance, recurrent pneumonia and atrial arrhythmia in adult

Question 8

How can large atrial septal defects present in young children and adults?

Answer 8

large = exercise intolerance, fatigue and poor growth in children - atrial arrhythmia, tricuspid regurgitation, heart failure and pulmonary htn in adult

Question 9

CXR signs in atrial septal defect in older child/adult

Answer 9

cardiomegaly
dilation of RA
dilation of RV
prominent main pulmonary artery
increased pulmonary vascular markings

[LA normal in size, aortic arch small/normal]

Question 10

Atrial septal defect signs in older child/adult?

Answer 10

prominent RV impulse
soft, ejection systolic murmur - pulmonary area
widely split, soft S2, fixed in all stages od respiration

Question 11

Atrial septal defect indications for closure

Answer 11

symptomatic
RA and RV enlargement
net left to right shunt Qp;Qs>1.5
no cyanosis at rest or exercise
systolic PA pressure <50% systemic pressure
PVR < 1/3 SVR

Question 12

Describe ventricular septal defect

Answer 12

ventricular level shunt
high pulmonary blood flow
left heart dilatation:
- LA dilatation
- LV dilatation

Question 13

Presentation of young child/adult with small ventricular septal defect

Answer 13

young child = incidental murmur

adult = incidental murmur, endocarditis

Question 14

Presentation of neonate/young child/adult with medium ventricular septal defect

Answer 14

neonate = >4 weeks, incidental murmur, sweatiness with feeds

young child = poor growth, murmur, frequent chest infections

adult = incidental murmur, endocarditis

Question 15

Presentation of neonate/young child/adult with large ventricular septal defect

Answer 15

neonate = >4 weeks, tachypnoea, poor feeding, failure to thrive

young child = frequent chest infections, exercise intolerance, fatigue, poor growth

adult = breathlessness, fatigue, heart failure, pulmonary htn

Question 16

Indications for ventricular septal defect closure

Answer 16

symptomatic
LA and LV enlargement
net L to R shunt Qp:Qs>1.5
no cyanosis at rest or exercise
systolic PA pressure <50% systemic pressure
PVR <1/3SVR

Question 17

What is the main complication from small ventricular septal defect?

Answer 17

infective endocarditis

Question 18

Where would infective endocarditis vegetations occur in a patient with a small ventricular septal defect?

Answer 18

at the tricuspid valve
opposite or around the defect
on the aortic valve

Question 19

How is infective endocarditis prevented in congenital heart disease patients?

Answer 19

good dental hygiene = best protection
avoid tattoos + piercing
prophylactic antibiotics for high risk congenital heart disease only (right to left shunts (cyanosis), valve replacements, previous endocarditis)

Question 20

Describe Eisenmenger syndrome

Answer 20

typically presents in children and young adults who have uncorrected ventricular septal defect

progresses over time as a result of the effects of high blood pressure in the lungs due to congenital defects that cause a left-to-right shunt

Question 21

Eisenmenger syndrome problems/considerations

Answer 21

polycythaemia
iron deficiency
acne
gout
hypertrophic polyarthropathy
high pregnancy risk
paradoxical embolus at IV sites
individual medical management of vasodilator therapy
arrhythmia risks

Question 22

What is coarctation of the aorta?

Answer 22

discrete luminal narrowing anywhere in aorta

Question 23

What is coarctation of aorta associated with?

Answer 23

bicuspid aortic valve
ventricular septal defects

Question 24

What methods can be used to repair coarctation of the aorta?

Answer 24

end to end repair (resection)
left subclavian flap repair (neonate)

Question 25

What are the 4 components of Tetralogy of Fallot?

Answer 25

ventricular septal defect
aortic override
pulmonary valve stenosis
right ventricular hypertrophy

Question 26

What surgeries can be done in the 1st year of life to fix Tetralogy of Fallot?

Answer 26

RVOT enlargement
patch closure of VSD
patch enlargement of the PA if necessary

Question 27

What physiological changes occur in the circulatory system during pregnancy?

Answer 27

increased cardiac output
increased heart rate
increased stroke volume
decreased pulmonary vascular resistance

Question 28

What predictors of maternal risk can be used for adult congenital heart disease patients?

Answer 28

prior cardiac event (TIA, stroke, arrhythmia, heart failure)
NYHA>grade 2 pre-pregnancy
left heart obstruction - mitral or aortic stenosis, CoA
LV EF <40%

Question 29

Which 2 heart defects cause definite high risks to pregnancy?

Answer 29

Eisenmenger
sevrere aortic stenosis