Congenital heart disease Flashcards
What are Acyanotic conditions?
Left - to - right shunt
examples:
Ventricular septal defects
Atrial septal defects
Patent ductus arteriosus
Coarctation of the aorta
What is the most common congenital heart defect?
ventricular septal defects
What is VSD?
Hole in septum between two ventricles
What causes VSDs?
Chromosomal abnormalities:
Down syndrome
Edwards syndrome
Patau syndrome
Symptoms of VSD?
Failure to thrive
Features of heart failure (hepatomegaly, tachypnoea, tachycardia, pallor)
Pansystolic murmur at lower left sternal edge
How to investigate for VSD?
Echocardiogram
CXR may also show signs of heart failure
How to manage VSD non pharmacologically?
Spontaneous closure is common 50% - self resolves
How to manage VSD medically?
Digoxin
Diuretics + ACEi for heart failure
How to manage SVD surgically?
Do it early if you will - to avoid eisenmenger’s syndrome
eisenmenger’s - permanent lung damage from PHTN and high blood flow
Complications of having VSD?
Aortic regurgitation
Infective endocarditis
Eisenmenger’s syndrome
Pulmonary hypertension
Murmur in VSD
Pansystolic
What is Eisenmenger’s syndrome
occurs when congenital heart defects are untreated. The left-to-right shunt is reversed due to pulmonary hypertension. The reversal generally occurs in teenagers and causes cyanosis, clubbing and right-ventricular failure. This is a serious complication and can need heart–lung transplant or palliation. It is irreversible and can lead to severe cardiac failure.
What is ASD?
Hole in septum between two atria
defect in the fossa ovalis in the centre of the atrial septum
Murmur heard in ASD?
Ejection systolic murmur at left sternal edge + fixed splitting of S2
How to manage ASD?
Cardiac catherisation + insertion of occlusive device at 3 years old
What is patent ductus arterious
Connection between pulmonary artery and descending aorta
High risk babies for PDA?
Patent ductus arteriosus
**premature babies **
low birth weight
high altitude
**maternal rubella
hyaline membrane disease **
Symptoms of PDA?
Wide PP
Large volume bounding collapsing pulse
Machinery murmur
PDA murmur?
Left subclavicular continuous machinery murmur
How to manage PDA?
Indomethacin or Ibuprofen:
the prostaglanding synthesis inhibition closes the connection
What is coarctation of the aorta?
Congenital narrowing of descending aorta
Coarctation of the aorta associations? (common diseases/ risks)
Turner syndrome!
Biscupid aortic valve
berry aneurysms
neurofibromatosis
Features of coarctation of the aorta?
Heart failure in infancy
Hypertension in adults
Radiofemoral delay
Mid-systolic murmur
absent or weak femoral pulses
Murmur in coartation of the aorta?
Mid systolic murmur
What are cyanotic heart conditions?
Right to left shunt (TTT)
What are examples of cyanotic congenital heart disorders?
Tetralogy of fallot
Transposition of the great arteries
Tricuspid atresia
What is the most common cause of cyanotic congenital heart disease?
Tetralogy of Fallot
When does Tetralogy of Fallot typically present?
In the first few weeks to months of life
What are the four coexisting pathologies in Tetralogy of Fallot? (PROV)
✅ Pulmonary stenosis (causes ejection systolic murmur & determines cyanosis severity)
✅ Right ventricular hypertrophy
✅ Overriding aorta
✅ Ventricular septal defect (VSD)
What type of murmur is heard in Tetralogy of Fallot, and where?
Ejection systolic murmur at the upper left sternal border due to pulmonary stenosis
What are the characteristic symptoms of Tetralogy of Fallot?
Hypercyanotic ‘tet’ spells while crying or feeding
What are the risk factors for Tetralogy of Fallot?
Rubella infection, maternal age >40, alcohol consumption during pregnancy, maternal diabetes
What is the first-line investigation for Tetralogy of Fallot?
Echocardiogram
What is the characteristic CXR finding in Tetralogy of Fallot?
Boot-shaped’ heart due to right ventricular thickening
How is Tetralogy of Fallot managed initially?
Maintain PDA with prostaglandin E1 (alprostadil)
What is the definitive treatment for Tetralogy of Fallot?
Corrective surgery at 6 months (BT shunt initially if needed)
How are severe ‘tet spells’ managed?
Last-line treatment: vasoconstrictors (e.g., phenylephrine)
What is the underlying defect in Transposition of the Great Arteries?
Failure of the aorticopulmonary septum to spiral during septation
What is the anatomical defect seen in TGA on echocardiogram?
Parallel aorta and pulmonary trunk (aorta attached to RV, pulmonary artery to LV)
When does TGA typically present?
Within the first few hours after birth
Which maternal condition increases the risk of TGA?
Maternal diabetes
What are the key clinical signs of TGA?
Cyanosis, tachypnoea, loud single S2 (NO murmurs), prominent right ventricular impulse
What is the characteristic finding on a CXR for TGA?
‘Egg-on-side’ appearance
How is TGA managed initially?
Maintain ductus arteriosus with prostaglandin E1 infusion
What is the emergency procedure to improve blood mixing in TGA?
Balloon atrial septostomy
What is the definitive treatment for TGA?
Surgical correction (arterial switch operation)
When does Tricuspid Atresia typically present?
In the first few minutes of life with cynaosis and breathlessness
What is the underlying defect in Tricuspid Atresia?
Absence of the tricuspid valve, resulting in a non-functional right ventricle
Why does Tricuspid Atresia cause cyanosis?
No blood flow to the right ventricle leads to common mixing of blood in the left atrium
What ECG finding is associated with Tricuspid Atresia?
Left axis deviation
How is Tricuspid Atresia managed?
Surgical intervention
Atrioventricular Septal Defect (AVSD)
Down’s syndrome
When does AVSD typically present with cyanosis?
At 2-3 weeks of life
Does AVSD have a murmur?
No, it presents with cyanosis but no murmur
How is AVSD usually diagnosed?
outine echocardiogram in babies with Down’s syndrome
What are the key features of an innocent murmur?
Soft, Systolic, Short, Single, Symptomless, Sternal Edge, Standing/Sitting (varies with position)
What are two common types of innocent murmurs?
✅ Venous hum → continuous blowing noise below the clavicles (turbulent blood flow in great veins)
✅ Still’s murmur → low-pitched murmur at lower left sternal edge
What is the first-line investigation for suspected congenital heart disease?
Echocardiogram
How do you determine if neonatal cyanosis is due to a cardiac cause?
Nitrogen washout test (Hyperoxia Test): Give 100% O₂ for 10 minutes, then measure ABG. If pO₂ < 15 kPa → cyanotic congenital heart disease is likely
What is the first step in managing neonatal cyanosis?
A-E assessment and oxygen administration
If cyanosis is due to congenital heart disease, what is the next step?
Prostaglandin E1 (alprostadil) infusion to maintain ductal patency
