Congenital Heart Disease

Question 1

What are acyanotic heart defects?

Answer 1

Left to right shunt
VSD
ASD
PDA
Coarctation of aorta
Aortic valve stenosis

Question 2

Most common CHD?

Answer 2

VSD

Question 3

What are cyanotic heart defects? Most common?

Answer 3

Right to left shunt
Tetralogy of fallot TOF
Transposition of the great arteries (TGA)
Tricuspid atresia
Hypoplastic left heart
Truncus arteriosus

Question 4

What are symptoms of congenital heart disease?

Answer 4

Can present hours-days (TGA) to during adulthood (ASD, VSD, coarctation)

Can cause decompensation and heart failure

Right to left shunt causes cyanosis

Increased pulmonary vascular resistance can result in reversal of left-to right flow across a shunt - Eisenmenge’s syndrome

Poor feeding, dyspnoea, tachycardias hepatomegaly, cool peripheries, acidosis, pulmonary venous congestion on CXR

Question 5

What investigation in congenital heart disease

Answer 5

FBC
CR
ECG
ECHO
Cardiac catheter

Question 6

What is management of heart failure in neonate?

Answer 6

Sit upright
Oxygen
Calories via NG feed
Diuretics - furosemide ± spironactone
Duct dependent cyanotic conditions will need alprostadil (prostaglandins PGE1) to maintain patency

Question 7

What are signs of VSD?

Answer 7

Harsh loud pansystolic blowing murmur ± thrill
ECG shows ventricular hypertrophy and strain
CXR shows pulmonary engorgement and cardiomegaly

Question 8

What is most common heart defect in trisomy 21?

Answer 8

AVSD
VSD
ASD
Tetralogy of fallot

Question 9

What are signs ASD?

Answer 9

Widely split fixed S2
Systolic murmur upper left sternal edge

ECG: RVH ± partial RBBB

Question 10

What is persistent vs patent ductus arterisous? symptoms/signs?

Answer 10

Persistent: term babies at > 1 month
Patent in premature babies

Symptoms are rare unless large defect causing CCF and pulmonary HTN

Continuous machine murmur below left clavicle
Thrill
Collapsing pulse
Failure to thrive
Pneumonia

Question 11

How is PDA treated?

Answer 11

Patent treated with oral of IV ibuprofen
Persistent with ibuprofen early or endovascular surgery

Closure avoids spontaneous bacterial endocarditis and circulatory overload

Question 12

What are signs of coarctation of the aorta? symptoms? How is it treated?

Answer 12

HTN
Radio-femoral delay
Reduced femoral pulse volume
Ejection systolic murmur at upper left sternal edge

Stent or surgery by 5 years to avoid pulmonary HRN and end organ damage

Question 13

What are symptoms of TGA?

Answer 13

Cyanosis day 1-2

Question 14

What is management of TGA?

Answer 14

Maintain PDA - prostaglandins
Balloon catheter atrial septal perforation
Surgery with arterial switch in first few days of life

Question 15

What are the features of TOF? Investigations?

Answer 15

Ventricular septal defect (VSD)
Right ventricular hypertrophy
Pulmonary stenosis
Overriding aorta

Cyanosis
Causes a right-to-left shunt
Ejection systolic murmur due to pulmonary stenosis (the VSD doesn’t usually cause a murmur)
A right-sided aortic arch is seen in 25% of patients
CXR shows a ‘boot-shaped’ heart
ECG shows right ventricular hypertrophy

Question 16

What is management of TOF?

Answer 16

Surgical repair is often undertaken in two parts at 6 months

Cyanotic episodes may be helped by beta-blockers to reduce infundibular spasm

Question 17

What is Ebstein’s anomaly? What can this cause?

What can cause this?

Answer 17

Low insertion of the tricuspid valve resulting in a large RA and small RV
Atrialisation of the RV

Associated with tricuspid incompetence (pan-systolic murmur)
WPW syndrome

May be caused by exposure to lithium in utero

Question 18

What is Eisenmenger’s syndrome? Mx?

Answer 18

Reversal of a left-to-right shunt in a congenital heart defect due to pulmonary hypertension.

Uncorrected left-to-right (VSD, ASD, PDA) leads to remodeling of the pulmonary microvasculature, eventually causing obstruction to pulmonary blood and pulmonary hypertension

Heart-lung transplantation is required