Congenital heart defects treatment

Question 1

What is the recommended treatment for perimembranous VSD according to NICE?

Answer 1

Transcatheter endovascular closure

Question 2

In which condition is balloon atrial septostomy indicated?

Answer 2

Transposition of the great arteries (TGA) to improve oxygenation

Question 3

What is the purpose of pulmonary artery banding in congenital heart defects?

Answer 3

To reduce pulmonary overcirculation as a preparatory step before complete surgical repair

Question 4

What is HLHS? And what fetal intervention is considered for critical aortic stenosis leading to HLHS?

Answer 4

HLHS (Hypoplastic Left Heart Syndrome) is a rare, severe congenital heart defect in which the left side of the heart is underdeveloped, making it unable to effectively pump blood to the bod

intervention for critical aortic stenosis: Percutaneous balloon valvuloplasty

Question 5

🫀 Acyanotic Congenital Heart Defects

Answer 5

Ventricular Septal Defect (VSD)

Atrial Septal Defect (ASD)

Patent Ductus Arteriosus (PDA)

Coarctation of aorta

Question 6

🩵 Cyanotic Congenital Heart Defects

Answer 6

Transposition of the Great Vessels (TGV) aka TGA (blue baby)

Tetralogy of Fallot (TOF)

Persistent Truncus Arteriosus

Question 7

Persistent Truncus Arteriosus treatment (NICE)

Answer 7

Prostaglandin E1 to keep the ductus arteriosus open before surgery; then surgery either:

• Norwood procedure (neonatal)
• Glenn shunt (~4–6 months)
• Fontan procedure (~2–4 years)

Question 8

Transposition of the Great Vessels (TGV) treatment (NICE)

Answer 8

prostaglandin E1 to keep ductus arteriosus open pre-surgery

Balloon atrial septostomy is used to improve oxygenation by enhancing atrial mixing (aka atrial switch)

n.b. this is often a temporizing measure before definitive surgical correction

Question 9

Tetralogy of Fallot (TOF) treatment (NICE)

Answer 9

sometimes to buy time they give Prostaglandin E1 to maintain ductus arteriosus pre-surgery

Pulmonary artery banding (PAB) can be employed as a palliative procedure to reduce pulmonary overcirculation in preparation for complete repair (open heart surgery)

Clinical Note: Adjustable PAB allows for tailored management of pulmonary blood flow

Question 10

Ventricular Septal Defect (VSD) treatment (NICE)

Answer 10

may not need treatment if minor VSD

If need then; pre-surgery diuretics, additional feeding

Surgery; Transcatheter endovascular closure is recommended for suitable cases, especially perimembranous VSDs

Question 11

Atrial Septal Defect (ASD) treatment (NICE)

Answer 11

Endovascular atrial septostomy may be indicated in certain cases, such as in the context of cyanotic congenital heart disease to improve oxygenation

Procedure aims to enhance systemic oxygenation by allowing interatrial mixing.​

Question 12

Small PDAs do not increase risk of heart failure, but what do they increase the risk of?

Answer 12

bacterial endocarditis

Question 13

Patent Ductus Arteriosus (PDA)

Answer 13

Indomethacin (NSAID) injection closes PDA= works by causing the PDA to constrict, and this closes the blood vessel

Indomethacin is a non‐selective cyclooxygenase inhibitor, which prevents the enzymatic process leading to the production of prostaglandins. Inhibition of prostaglandin production results in arteriolar vasoconstriction, which aids ductal closure

for severe PDA: transcatheter device closure is a common practice

Question 14

Which drugs open and which drugs close ductus arteriosus?

Answer 14

PGE1= opens ductus aretriosus

PGE₁ is used in:
- Cyanotic congenital heart defects (e.g. Transposition of the Great Arteries, Hypoplastic Left Heart Syndrome) to maintain ductal patency and systemic or pulmonary blood flow until surgery.

NSAIDs= closes ductus arteriosus

NSAIDs (Indomethacin/Ibuprofen) are used in:
- Preterm infants with PDA to promote closure of a persistently open ductus arteriosus.

Question 15

Diagnosis of ASD; what type of murmur can be heard?

Answer 15

• systolic murmur best heard at left upper sternal border (left 2nd ICS)
• fixed S2 splitting

Question 16

Diagnosis of VSD; what type of murmur can be heard and where?

Answer 16

• pansystolic murmur best heard at left sternal edge 2-4th ICS (loud 2nd heart sound if pulmonary hypertension present)
• echocardiography
• In large VSD, CXR= cardiomegaly, prominent pulmonary markings
• ECG; ventricular hypertrophy

Question 17

Diagnosis of Patent Ductus Arteriosus

Answer 17

• continuous murmur with machinery-like character
• best heard left upper sternal border/ infraclavicular region

Question 18

Patent Ductus Arteriosus is acyanotic but it can become cyanotic, how? What is the name for this syndrome/ complication?

Answer 18

• if pulmonary pressure gets too high= reverses shunt to RIGHT to LEFT= EISENMENGER’S SYNDROME= cyanosis

R-L shunt is distal to aortic arch= lower body cyanosis

Question 19

Diagnosis of tetralogy of fallot murmur?

Answer 19

features= outlet VSD, pulmonary stenosis, right ventricular hypertrophy, overriding aorta

• systolic murmur heard at left upper sternal edge
• axilla radiation

N.B. boot shape on CXR

Question 20

Transposition of the Great Vessels (TGV) is not compatible with life and they develop cyanosis in first hours of life. Diagnosis?

Answer 20

• CXR egg on string
• echocardiography confirms diagnosis

Question 21

Coarctation of aorta treatment NICE

Answer 21

Balloon angioplasty of aortic coarctation with or without stenting, for treating coarctation or recoarctation of the aorta in both adults and children. It’s a minimally invasive procedure that involves inserting a catheter into a large blood vessel, usually in the groin

Question 22

What is Eisenmenger syndrome?

Answer 22

Eisenmenger syndrome is a late complication of an uncorrected left-to-right cardiac shunt (e.g., VSD, ASD, PDA) that leads to pulmonary hypertension, eventually reversing the shunt to a right-to-left direction, causing cyanosis.