7.5 Cyanotic Conditions

Question 1

Tetralogy of the Fallot

Answer 1

Tetra means four
- Babies are born with 4 different defects

These include..
- Ventricular Septal Defect (VSD)
- Pulmonary Stenosis
- Hypertrophy of right ventricle
- Overriding Aorta

Characterized by a lot of Cyanosis
- Cyanosis at birth and progressively worsening cyanosis within the first year of life.
- Cyanosis when crying, eating

Symptoms
- Significant Murmur
- Activity Intolerance
- Squatters (When they try to get themselves more oxygen, they squat as a compensatory mechanism to shunt more blood flow to vital organs)
- Clubbing

TET SPELL - Turning cyanotic because they are exerting themselves (crying, feeding)

Treatment
- Surgical repair

Question 2

Transposition of the Great Vessels

Answer 2

• Pulmonary artery and aorta are switched.
• This causes no communication between systemic and pulmonary circulation unless their is a PDA (Pulmonary Ductus Arteriosus) to keep some sort of connection between the oxygenated system and deoxygenated system.
• PDA is left open on purpose

S/S
- Cyanosis at birth
- Poor feeding
- Decreased growth
- Cardiomegaly (enlarged heart) due to heart not functioning properly

TREATMENT
- IV Prostaglandins to KEEP PDA open then surgery to switch ventricles back to their appropriate sides.

Question 3

Truncus Arteriosus

Answer 3

• Blood from both ventricles mix within a common artery.
• Blood ejected from the heart flows to lower pressure (pulmonary artery) so pulmonary blood flow is increased and systemic blood flow is reduced.

S/S
- Classic HF Symptoms
- Cyanosis
- Poor Growth
- Activity Intolerance

Question 4

Hypoplastic Left Heart Syndrome (HLHS)

Answer 4

• Underdeveloped left side of heart which results in hypoplastic left ventricle and aortic atresia
• The left ventricle is the great driver in pushing blood to periphery so because this is underdeveloped blood cannot get out to peripheries.
• Blood from left atrium -> Patent foramen ovale -> Right Atrium -> Right Ventricle -> Pulmonary Artery

S/S
- Mild Cyanosis and HF until PDA closes
- Progressive Cyanosis once PDA closes, decreased cardiac output, which leads to cardiac collapse
- Fatal in the 1st month of life without intervention

Treatment
- Prostaglandins to keep PDA open