Section 2: Congenital Cardiac Conditions

Question 1

Prenatal risk factors for cardiac abnormalities

Answer 1

1. Maternal Health Conditions: Conditions like diabetes, phenylketonuria, and viral infections (e.g., rubella) during pregnancy can increase the risk of congenital heart defects
2. Medications: Certain medications taken during pregnancy, such as angiotensin-converting enzyme (ACE) inhibitors and retinoic acids, can raise the risk
3. Substance Exposure: Smoking, alcohol, and drug use during pregnancy can negatively impact fetal heart development

Question 1

Health Checks for Down Syndrome

Answer 1

1. Early Screening: All infants with Down syndrome should undergo an echo-cardiogram and evaluation by a paediatric cardiologist within the first few months of life.
2. Regular Monitoring: Regular cardiovascular check-ups are essential, including annual auscultation of the heart and periodic echocardiograms, especially if new symptoms arise.

3.Specialist Care: Lifelong care by specialists in congenital heart disease is recommended to manage and monitor any ongoing or new cardiac issues

Question 2

Treatment Pathway for Down Syndrome with a Ventricular Septal Defect (VSD)

Answer 2

• Some VSDs can be closed by inserting a thin, flexible sheath (tube/catheter) via the femoral artery/femoral vein in the groin area. This is not as invasive and does not require open heart surgery.
• Approx a 3-day hospital stay
• Unfortunately, some are too big or in the wrong position to be able to close via cardiac catheter.

Question 2

List and explain the baseline observations for a patient with Down Syndrome and a Ventricular Septal Defect (VSD)

Answer 2

Baseline Observations:

1. Oxygen Saturations – Generally VSD is an acyanotic Congenital Heart Defect, this means no signs of cyanosis (bluish skin tone particularly to the lips, bridge nose, nail beds) but can cause cyanotic heart disease if left untreated. Mixing of oxygenated and deoxygenated blood in the right ventricle can create higher lung pressure which in turn lowers O2 saturations.
2. Temperature – Monitoring for signs of infection is crucial, as infections can increase surgical risks.
   It is essential for the patient to be in optimal health before undergoing surgery.
3. Blood Pressure – A good baseline but also to ensure that there is no signs of hypertension which there could be if the heart and lungs have had to work harder.
4. Heart rate – baseline, ensuring that pulse is strong and regular. Tachycardia (fast heart rate) may indicate heart failure or increased cardiac workload.
   Bradycardia (slow heart rate) could be concerning if it signals conduction system abnormalities.

Question 3

Define congenital heart disease

Answer 3

Congenital heart disease is a heart condition or defect that develops in the womb before a baby is born

Question 4

Congenital Heart Defects are broadly classified into which two categories

Answer 4

1. ACHD: Acyanotic congenital heart disease
   Ventricular septal defect
   Atrial septal defect
   Patent ductus arteriosus
   Coarctation of the aorta
   Aortic/pulmonary stenosis
2. CCHD: Cyanotic congenital heart disease
   Tetralogy of Fallot
   Truncus arteriosus
   Transposition of the great arteries
   Tricuspid atresia
   Total anomalous pulmonary venous return

Question 5

What is Ventricular septal defect (VSD)?

Answer 5

A hole in the septum in between the ventricles. Blood from the left ventricle (high in oxygen from the lungs) can flow into the right ventricle.

Blood from the right ventricle (low in oxygen) can flow into the left ventricle

Question 6

What can Ventricular septal defect lead to?

Answer 6

• heart failure
• irregular heart rhythm
• Eisenmenger syndrome
• leaky heart valve
• stroke
• pulmonary hypertension

Question 7

What is Eisenmenger syndrome?

Answer 7

Eisenmenger syndrome is a condition where a long-standing heart defect that causes left-to-right blood flow (like VSD) leads to:
-pulmonary hypertension
-eventually reversing the flow to right-to-left, causing cyanosis

Question 8

What are the two common types of VSD?

Answer 8

• Muscular VSD, in the lower part of the wall between the heart chambers (ventricles).
• Peri-membranous VSD, near the heart valves

Question 9

What are the symptoms of VSD?

Answer 9

-Small VSDs might not cause noticeable symptoms and can close on their own.

Larger VSDs can cause:
-rapid breathing
- difficulty feeding
- poor weight gain
- heart murmur

Question 10

Treatment for VSD?

Answer 10

• Small VSDs can close by themselves and do not need treatment
• Larger VSDs will need to be closed:
  transcatheter closure
  open heart surgery

Question 11

What is atrial septal defect? And what does it lead to?

Answer 11

Atrial Septal Defect is a hole in the septum in between the upper chambers of the heart.

Typically, blood moves from oxygenated left atrium to the less oxygenated right atrium.

This can lead to:
- Increased pulmonary blood flow
- Right atrium and ventricle hypertrophy
- Pulmonary hypertension

Question 12

Clinical presentation of septal defect.

Answer 12

• Machinery Murmur
• Shortness of breath especially when exercise
• Tiredness with activity
• Arrhythmia
• Palpitations
• Frequent respiratory infections

Question 13

Nursing care considerations for children and young people with VSD and ASD

Answer 13

1. Cardiac Function: Regularly monitor heart rate, rhythm, and signs of heart failure, such as fatigue, poor feeding, or respiratory distress.
2. Growth and Development: Track weight and height to ensure proper growth, as these children may experience delays due to increased energy expenditure.
3. Oxygenation: Assess oxygen saturation levels and watch for cyanosis or signs of hypoxia
4. High-Calorie Diet: Provide nutrient-dense meals to meet their increased energy needs.
5. Feeding Assistance: Offer smaller, more frequent feedings to reduce fatigue during meals.
6. Prophylactic Antibiotics: Administer as prescribed to prevent infective endocarditis.

Question 14

What is Milrinone?

Answer 14

• Milrinone is an inodilator.
• It reduces pulmonary vascular resistance and improves cardiac contractility,
• improving cardiac output
• often used after cardiac surgery and in heart failure.

Question 15

What is the mechanism of action of Milrinone?

Answer 15

• Inhibits the degradation of cyclic adenosine monophosphate
• Works to increase heart contractability and decrease pulmonary vascular resistance
• Increases the strength of the heart muscle and widens the blood vessels
• It improves cardiac contractility (inotropy) and cardiac relaxation (lusitropy) and induces vasodilation.
• It has the overall effect of increased cardiac output and enhanced cardiac mechanical efficiency

Question 16

What is Milrinone used for?

Answer 16

• Medication administered to patients with acute heart failure, pulmonary hypertension and or chronic heart failure.
• Indicated for cardiac support.
• Improves cardiac contractility and relaxation.
• It acts by decreasing vascular resistance and assisting the heart with contraction.
• Only to be put via a central line on a continuous IV.

Question 17

Indication of Milrinone?

Answer 17

• Short-term treatment of severe congestive heart failure unresponsive to conventional maintenance therapy (not immediately after myocardial infarction) Acute heart failure, including low output states following heart surgery
• Low cardiac output following cardiac surgery
• Congestive heart failure

Question 18

Side effects of Milrinone?

Answer 18

• Arrhythmia (increased risk in patients with pre-existing arrhythmias)
• Headache
• Hypotension
• Dizziness
• Tremor
• Chest pain
• Renal failure

Question 19

Define heart failure and consider the four factors that can cause this condition.

(4 marks)  

Answer 19

Heart failure is a complex condition in which the heart is not able to pump sufficient blood in the systemic and pulmonary circulation to meet the body’s metabolic demand. It is caused by: 

• Volume overload (caused by left to right shunt) 
• Impaired contractility (cardiomyopathy)
• Pressure overload
• High cardiac output demand (sepsis) 

Question 20

Milrinone infusion is often used after cardiac surgery to treat and prevent a low cardiac output state (LCOS).

What are the safety considerations that a nurse needs to take while administering Milrinone? (4 marks) 

Answer 20

• Milrinone may cause arrhythmias, so continuous ECG monitoring is necessary using 3 or 5 leads.
• Milrinone can also cause hypotension, so hourly BP should be recorded. If the patient is in ITU, use an arterial line.
• Milrinone can cause extravasation, so hourly site checks performed using VIP score, especially if a PVC is used.
• Milrinone can also cause thrombocytopenia (low level of platelets in the blood) so regular monitoring of patient’s blood results, reporting abnormal platelets levels.

Question 21

Briefly explain three steps that demonstrate how to plan for the transition from paediatric to adult services for a child with congenital heart disease. (3 marks) 

Answer 21

The transition from a child to an adult service should be planned when the child is 14 years old to be ready when the official transition happens when the child is 16yrs.

The Multi Disciplinary Team (MDT) needs to be involved in this process so that each member of the team can look at the individualised child’s needs. This includes also special educational needs if the child presents with learning disabilities.

The Childrens Team needs to cooperate with the adult Team which will take care of the child after the transition. The children’s team is in charge of “creating a bridge” between services.

Question 22

What kind of nursing observations should you chart whilst looking after a child with a chest drain? (4 marks) 

Answer 22

After cardiac surgery, chest drain volumes should be monitored frequently: every 15 minutes in the first hour and then hourly, to detect signs of blockage. This prevents cardiac tamponade, or bleeding.

A sudden decrease or sudden increase in drainage needs to be escalated.

Keep the chest drain bottle below the level of the patient’s chest, to prevent drained fluid being reintroduced. If the chest drain bottle needs to be lifted above the patient’s chest the tubing must be clamped.

The chest drain site needs regular inspection to prevent accidental or purposeful dislodgment.

Question 23

Nursing management for VSD/ASD

Answer 23

Surgical Repair: Catheter closure (for small VSDs), open-heart surgery for larger defects Medications: Milrinone: Inotrope to improve heart contractility and reduce pulmonary resistance Diuretics: Help with fluid overload (e.g. Furosemide) ACE inhibitors: Reduce afterload

Question 24

Key Nursing Considerations (Children/Infants) congenital heart defects

Answer 24

Feeding difficulties → small, frequent feeds Growth monitoring → weight gain, BMI, charts O2 monitoring → especially in cyanotic defects Family education → explain surgeries, prognosis Chest drain care post-op: Monitor drainage colour, volume Check site: clean, not leaking/dislodged Ensure drain below chest level

Question 25

❓Q1. (6 Marks) Describe the signs and symptoms of a Ventricular Septal Defect (VSD) in an infant and explain why they occur.

Answer 25

Infants with a VSD may present with breathlessness, poor feeding, failure to thrive, and a heart murmur. The defect allows blood to flow from the higher-pressure left ventricle to the right ventricle, increasing pulmonary blood flow. This places extra workload on the lungs and heart, leading to pulmonary congestion and symptoms of heart failure. The murmur results from turbulent blood flow across the septal defect.

Question 26

❓Q2. (8 Marks) Explain the role of the nurse in supporting a child undergoing surgery for a congenital heart defect and the care required post-operatively.

Answer 26

Pre-operatively, the nurse must educate the child and family about the surgical procedure, expected outcomes, and what the child may look like post-op (e.g. chest drain, IV lines). Emotional support is essential, particularly in paediatric settings. The nurse should ensure all baseline observations are recorded, and that the child is fit for surgery (e.g. no signs of infection). Post-operatively, nursing care includes close monitoring of vital signs, oxygen saturation, fluid balance, and signs of infection. Specific attention must be given to chest drains: checking drainage volume, colour, and ensuring the site is clean and secure. Pain management, wound care, and early identification of complications like arrhythmias or hypotension are also critical. The family should be supported with information and involvement in care to promote confidence and emotional reassurance.

Question 27

List two congenital heart defects and state whether they are cyanotic or acyanotic.

Answer 27

Ventricular Septal Defect (VSD) – Acyanotic Tetralogy of Fallot – Cyanotic

Question 28

Describe how a congenital heart defect such as Tetralogy of Fallot can affect an infant’s growth and development.

Answer 28

Tetralogy of Fallot reduces the amount of oxygenated blood that reaches the body due to right-to-left shunting. This chronic hypoxia can result in poor weight gain, delayed motor development, and fatigue during feeding or activity. The increased workload on the heart and lungs also raises caloric demands. As a result, affected infants often struggle to meet developmental milestones unless their condition is surgically corrected and closely monitored.

Question 29

Explain the nursing responsibilities for monitoring a child recovering from cardiac surgery with a chest drain in situ.

Answer 29

Nursing care post-cardiac surgery includes close monitoring of vital signs, oxygen saturation, and signs of respiratory distress. Specific to the chest drain, the nurse must assess the volume and colour of the drainage, ensuring it remains appropriate (e.g., initial blood-tinged fluid that lightens over time). The drain must stay below chest level to prevent backflow, and the site must be checked for leakage, dislodgement, or infection, noting that it is stitched in place. Pain management is essential, as discomfort may reduce deep breathing, increasing the risk of atelectasis. The nurse should document all observations, encourage age-appropriate activity as tolerated, and involve the family in care to reduce anxiety.

Question 30

What are the signs and symptoms of heart failure in a child, and how do they differ from adults?

Answer 30

In children, heart failure symptoms include breathlessness, poor feeding, failure to gain weight, excessive sweating (especially during feeding), fatigue, and irritability. Oedema may present around the eyes or legs. Unlike adults who often present with chest pain or ankle swelling, children may show signs through faltering growth and feeding difficulties. They may also have a rapid heart rate and increased work of breathing (nasal flaring, grunting). These signs reflect the child’s reduced ability to compensate for poor cardiac output.