Congenital Heart Disease

Question 1

Indications and contraindications for repair of an atrial septal defect

Answer 1

Indications:
Symptoms - orthodexia and platypnoea
Evidence of RV failure with Qs/Qp >1.5/1
Presence of paradoxical embolism if <60 years old and no other cause for stroke

Contraindications: severe pulmonary hypertension, Eisenmenger’s syndrome

Question 2

Disorders and associated congenital heart defects:

1. Trisomy 21
2. 22q deletion (DiGeorge)
3. Lithium
4. Rubella

Answer 2

1. Trisomy 21 -> VSD/AVSD
2. 22q deletion -> great artery malformations, Tetralogy of Fallot
3. Lithium - Ebstein anomaly, TV anomaly
4. Rubella - peripheral PA stenosis

2.

Question 3

Congenital heart disease -> examples of shunts, stenoses, cyanotic

Answer 3

Shunts
* ASD
* VSD
* PDA
* AV septal defects
* Anomalous pulmonar veins

Stenotic
* PS
* AS
* Coarctation

Cyanotic
* Tetralogy of Fallot
* Transposition of the Great Arteries
* Truncus Arteriosus
* Univentricular heart

Question 4

Notes on atrial septal defects

Answer 4

Ostium primum
* Lesion involves AV valves -> can get MR, TR, VSD

Ostrium secundum
* 90& ASDs - defect in the part of the septum that does not involve AV alves
* Can often be treated percutaneously as opposed to more inferior/superior lesions or an ostium primum lesion which often needs to be closed surgically

Question 5

Notes on ventricular septal defects

Answer 5

• VSDs close to aortic valve can often lead to AR -> require surgical closure

Question 6

Notes on patent ductus arteriosus

Answer 6

Normal foetal circulation
* Oxygen poor blood -> RV -> pulmonary artery -> ductus arteriosus -> descending aorta to deliver to lower limbs allowing it to go out umbilical artery to get oxygenated in placenta
* Oxygen rich blood from unbilical veins -> liver -> RA -> through foramen ovale -> LA -> LV -> out aorta (most oxygen rich blood going to head)

Pathology
* Should close within 72 hours of birth. If not -> shunt
* If large shunt -> left ventricular failure becomes apparent in first few days of life - closed in first few weeks - drugs or coil

Question 7

Notes on pulmonary valve stenosis

Answer 7

• Can be valvular, sub-valvular, supra-valvular
• Seen in Noonan’s syndrome and William Syndrome
• Treatment different depending on level of lesion - balloon valvuloplasty if valvular level (sometimes also if subvalvular)

Question 8

Notes on coarctation of the aorta

Answer 8

• Associated with bicuspid aortic valve, HTN, arch hypoplasia, cerebral aneursyms (NB headaches), aortic dissection
• At risk of aortic aneursyms after surgical repair
• Congenital narrowing of the aorta usually just distal to the left subclavian artery
• More common in amles
• A/W Turner’s syndrome - all should be screen for coarctation

Question 9

Notes on Tetralgy of Fallot

Answer 9

Features
1. VSD
2. Right ventricular hypertrophy
3. Overriding aorta (moved towards the right) > 50% double outlet RV
4. Pulmonary stenosis

Often repaired in infancy
Options for treatment = initial shunt operaiton to increase size of pulmonary tree with repair after shunt, primary repair or transannular patch

Post repair
Pulmonary regurgitations (all with transannular patch - well tolerated for up to 10 years)
Pulmonary stenosis
Arrhythmias
RV/LV dysfunction
Aortic root dilatation

Question 10

Notes on Transposition of the Great Vessels

Answer 10

D-TGA
* Aorta comes off the RV which is situated to the right side of the body - D for dextro = right

L-GTA
* RV is on the left side of the heart, aorta comes off the right ventricle e.g. congenitally corrected transposition