test 6 double inlet left ventricle (DILV)

Question 1

Double-Inlet Left Ventricle (DILV) Anatomy

Answer 1

■ Both atrioventricular valves enter into the LV
■ LV is connected to a hypoplastic RV by a VSD
■ RV can give rise to both great vessels or the aorta alone
– Pulmonary stenosis is very common
■ Transposition of the great vessels is common

Question 2

Double-Inlet Left Ventricle (DILV) symptoms

Answer 2

– Cyanosis
– Failure to gain weight
– Difficulty breathing
– Pale skin
– Sweating
– Tachycardia
– Pulmonary edema
– CHF

Question 3

DILV Pathophysiology

Answer 3

■ Complete intracardiac mixing of systemic and pulmonary blood
■ Severity of cyanosis is dependent upon the presence of pulmonary artery stenosis
– Little/no stenosis = pulmonary overcirculation
– Pulmonary stenosis = more balanced circulation, but evident cyanosis

Question 4

Surgical Repair of DILV

Answer 4

■ PA banding can be used early on to allow the child to grow
■ Conversion to single ventricle via Damus-Kaye-Stansel procedure
– Staged palliation thereafter
– Ultimately leads to Fontan

Question 5

Damus-Kaye-Stansel Procedure (DKS)

Answer 5

• Aorta and pulmonary artery are sutured together above their valves
• Modified BT shunt provides pulmonary bood flow
■ Provides unobstructed systemic outflow
■ Physiology is relatively fragile post op
■ With a BT shunt, coronary perfusion is dependent on retrograde aortic perfusion (diastolic blood pressure)
– Can lead to ”diastolic steal” phenomenon
– Often remain in hospital until next staged procedure (Fontan)

Question 6

DKS Palliation

Answer 6

■ DKS with BT shunt
■ Glenn
■ Fontan completion

Question 7

CPB Considerations

Answer 7

■ Incision: Median sternotomy
■ Cannulation:
■ Arterial: Innominate artery or Aorta
■ Venous: single atrial
■ Hypothermia: Moderate to Deep
■ Cardioplegia: Antegrade, direct ostial, retrograde