241. Cyanotic Defects Flashcards
What does CCHD stand for?
Cyanotic congenital heart disease
How many cases of CCHD occur per million live births?
1300 per million live births
What is hypoxemia?
PaO2 value below the normal 80–100 mm Hg
What causes cyanosis?
At least 5 g of desaturated hemoglobin
Name two physiologic adaptations to chronic cyanosis.
- Postnatal fetal hemoglobin production
- Right shift of the Hb-O2 dissociation curve
What are three side effects of cyanosis?
- Increased blood viscosity
- Coagulopathy
- Urate metabolism
What is hypoplastic left heart syndrome (HLHS)?
A CCHD where systemic venous blood enters the right heart and lungs
What are the two necessary structures for survival in HLHS?
- Atrial septal defect (ASD)
- Patent ductus arteriosus (PDA)
What is the purpose of the Norwood operation?
Aortic arch reconstruction
What is superior cavopulmonary anastomosis (Glenn)?
Anastomosis of the superior vena cava to the pulmonary artery
What does the Fontan operation involve?
Anastomosis of the inferior vena cava to the pulmonary artery
What is d-transposition of the great arteries (d-TGA)?
A CCHD where the great arteries arise from opposite ventricles
What is reverse differential cyanosis?
Higher oxygen saturation in the lower body than the upper body in d-TGA
What is the most common CCHD?
Tetralogy of Fallot (TOF)
Name the four classic lesions of Tetralogy of Fallot.
- Pulmonic stenosis
- Ventricular septal defect (VSD)
- Overriding aorta
- Right ventricular hypertrophy
