• Source: Single ventricle

Single ventricle is a rare congenital heart defect, which constitutes just over 1% of congenital cardiovascular diseases. The single functional ventricle could be morphologically right or left with the second ventricle usually hypoplastic and/or insufficiently functional. Therefore, there are several subtypes of the disease, depending on which ventricle is underdeveloped.

Single ventricle with functional left ventricle:
Pulmonal atresia
Tricuspidal atresia
Double inlet left ventricle
Double outlet left ventricle
Single ventricle with functional right ventricle:
Hypoplastic left heart syndrome
Double inlet right ventricle
Double outlet right ventricle
Single ventricle heterotaxy syndrome is also included to this category.




Physiology


In neonates with single ventricle, the systemic and pulmonary blood flow mix with each other in the single functioning ventricle with consequent lower systemic oxygen saturations (75–85%). The single ventricle then provides both the systemic and pulmonary blood flow.




Treatment


Due to the different types of a single ventricle heart disease, treatment should be individualized. Usually it requires open-heart surgery. The goal is to allow the functioning ventricle to supply the systemic circulation and to connect the systemic veins to the pulmonary arteries. The deoxygenated blood from the systemic veins flows directly into the lungs without passing the heart. The oxygenated blood then returns to the heart and enters the systemic circulation. Although surgical intervention depends on the type of single ventricular disease, any combination of three general procedures are utilized to address those defects:

Stage I – Norwood procedure
Stage II – Glenn procedure
Stage III – Fontan procedure




Prognosis


Prenatal diagnosis is associated with higher survival in neonates with single ventricle physiology. Outcome and life expectancy are highly depending on the underlying morpho-functional subtype and individual characteristics. More than half survive two years with the average length of up to 30 to 40 years.




References

Kata Kunci Pencarian:

• Daftar istilah biologi
• Ikan zebra
• Organoid
• Single ventricle
• Lateral ventricles
• Norwood procedure
• Fourth ventricle
• Axolotl
• Fontan procedure
• Hypoplastic left heart syndrome
• Double inlet left ventricle
• Atrium (heart)
• Bidirectional Glenn procedure