What is a Kawashima–Glenn Procedure?
A Kawashima procedure is a bilateral bidirectional Glenn shunt (superior cavopulmonary anastomosis) performed specifically in patients with single ventricle physiology who have interrupted inferior vena cava (IVC) with azygos continuation to the superior vena cava (SVC). 1
Anatomic Context
The Kawashima operation is essentially a bidirectional Glenn shunt adapted for a specific anatomic variant:
- Standard bidirectional Glenn: End-to-side anastomosis of the divided SVC to the undivided right pulmonary artery, directing SVC blood to both lungs 1
- Kawashima modification: When IVC is interrupted with azygos continuation to the SVC, the procedure creates bilateral cavopulmonary connections because all systemic venous return (including what would normally be IVC flow) comes through the SVC/azygos system 2, 3
Role in Single Ventricle Palliation
This is a Stage II palliative procedure in the typical three-stage single ventricle pathway 4:
- Performed after initial Stage I palliation (e.g., Norwood, BT shunt, PA band)
- Redirects systemic venous return directly to pulmonary circulation
- Reduces volume load on the single ventricle
- Typically performed at 5-9 months of age, though can be done earlier 5, 6
Critical Physiologic Difference
The Kawashima procedure directs substantially greater venous blood volume to the lungs compared to standard Glenn because all systemic venous return (upper and lower body via azygos) flows through the cavopulmonary connection 2. This is why:
- The procedure is often delayed to older age/larger size compared to standard Glenn
- Postoperative management requires careful attention to pulmonary vascular resistance
- Some centers use fenestration (calibrated right-to-left shunt) to decompress the system 7
Major Long-Term Complication: Pulmonary Arteriovenous Malformations (PAVMs)
The most significant issue with Kawashima is the high incidence of PAVMs 3, 8:
- Freedom from PAVMs is only 47% at 5 years after Kawashima 3
- PAVMs develop because hepatic venous blood (containing hepatic factor) does not reach the lungs
- This is the same mechanism seen in classic Glenn procedures 1
- Median time to PAVM development: 4 years (range 2-9 years) 3
Clinical Manifestations of PAVMs:
- Progressive cyanosis
- Decreased exercise capacity
- Systemic arterial desaturation
Subsequent Management: Hepatic Vein Incorporation (HVI)
Most patients (76-80%) ultimately require Fontan completion with hepatic vein incorporation to prevent or treat PAVMs 3, 8:
- Median interval from Kawashima to HVI: 2.6 years 8
- HVI directs hepatic venous blood to both lungs, providing hepatic factor
- PAVMs improve in approximately 75% of patients after HVI 8
- Elective non-fenestrated Fontan completion should be recommended rather than waiting for PAVM development 3
Protective Strategy: Preserving Antegrade Pulmonary Blood Flow
Leaving some antegrade flow from the ventricle to the pulmonary arteries may be protective against PAVM development 3, 8, 6:
- Patients with preserved antegrade flow at 9+ years post-Kawashima did not develop PAVMs 3
- 75% of patients left with antegrade PBF remained without PAVMs 6
- This strategy requires careful balance to avoid volume overload
Contemporary Outcomes
Recent data shows excellent early results 5, 6:
- Zero operative mortality in recent series
- Median hospital stay: 9 days
- Median ICU stay: 3 days
- Long-term survival: 90.5% at 5 years, 82.9% at 10 years 8
- 100% survival at 10 years when performed ≤9 months of age 8
Common Pitfalls
- Delaying the procedure too long: While historically performed at older ages, contemporary data supports earlier intervention (6-9 months) with excellent outcomes 5, 6
- Not planning for eventual HVI: Given the 53% incidence of PAVMs by 5 years, early counseling about eventual Fontan completion is essential 3
- Bilateral SVC with unilateral hepatic flow: This anatomic variant has particularly high PAVM risk, as hepatic flow is excluded from one lung 8