Congenital Heart defects

Question 1

Define congenital heart disease?

Answer 1

Abnormality of the structure of the heart, present at birth

Question 2

What are the ranges of severity seen in these diseases?

DONT need to memorise all of this

Answer 2

• Mild: asymptomatic, usually resolves itself. Usually more problematic in adult life.
• Moderate: needs specialist intervention
• Severe: die newborn or in infancy

Question 3

How does an unwell baby present?

Answer 3

• Cyanosis
• Cardiac failure
• Shock.
  The sooner the disease presents, the more dangerous it is.

Question 4

What is the management of antenatally diagnosed congenital heart disease?

Answer 4

Depends on disease
May deliver in cardiac surgical centre
May need prostaglandin infusion if duct lesion dependent.

Question 5

What happens in cyanosis? (blue baby)

Answer 5

Causes bluish discolouration

• Deoxygenated blood bypass lungs into systemic circulation
• Mixed oxygenated/deoxygenated blood enters systemic circulation.

Question 6

Differential diagnosis for Cyanosis in newborn?

Answer 6

• Respiratory disease
• PPHN
• Cardiac disease

Question 7

Clincal signs of failure in babies?

Answer 7

• Failure to thrive
• Slow feeding
• Breathlessness
• Hepatomegaly
• crepitations
• Sweatiness

Question 8

What would be the treatment in this case?

Answer 8

1. ABC
2. Prostaglandin if duct problem
3. Taken to cardiac surgical centre

Question 9

Examples of congenital defects where systemic circulation is duct dependent?

Answer 9

1. Hypoplastic left heart- left side severely undeveloped.
2. Critical aortic stenosis
3. Interrupted aortic arch
4. Critical coarctation of aorta (narrowing)

Question 10

Examples of congenital defects where pulmonary circulation is duct dependent?

Answer 10

1. Tricuspid atresia (narrowing)

2. Pulmonary atresia (narrowing)

Question 11

When do newborns present with cardiac failure (in terms of pathology)

Answer 11

Left to right heart shunts.
As pulmonary pressure drops days after birth, more blood flows through the shunt.
Murmur heard.
Effect–> congestive heart failure, increased pulmonary circulation

Question 12

Long term management for congenital heart defects?

Answer 12

• Surgical management
• Developmental problems
• further surgical interventions
• emotional/social issues

Question 13

What happens in a patent ductus arterosus repair?

Answer 13

Catheter inserted in the duct to stop the flow through it.

Question 14

What happens in a VSD (ventricular septal defect)

Answer 14

Patch inserted to close opening in the septum.

Question 15

What happens in HLHS?

Answer 15

Major surgery for hypo plastic left heart.
End result–> RV supplying systemic circulation
Will fail over time.