Should we always plan a Fontan completion after a Kawashima procedure?

Area of Science:

• Congenital Heart Surgery
• Pediatric Cardiology
• Vascular Malformations

Background:

• The Kawashima procedure, or azygos continuation of the inferior vena cava, is a palliative surgical approach for complex single-ventricle physiology.
• Pulmonary arteriovenous malformations (PAVMs) are a known complication following certain Fontan-type operations.

Purpose of the Study:

• To investigate the long-term incidence of pulmonary arteriovenous malformations (PAVMs) after the Kawashima procedure.
• To evaluate the need for subsequent interventions, such as Fontan completion, in these patients.

Main Methods:

• Retrospective analysis of 21 patients who underwent the Kawashima procedure between 1990 and 2006.
• Detailed review of patient anatomy, surgical history, and long-term clinical outcomes, including development of PAVMs.

Main Results:

• Thirteen of 21 patients (62%) developed PAVMs at a median of 4 years post-Kawashima.
• Freedom from PAVMs was 47% at 5 years.
• 16 patients underwent subsequent Fontan completion, primarily for cyanosis related to PAVMs.

Conclusions:

• The majority of patients with a Kawashima procedure are likely to develop PAVMs.
• Elective, non-fenestrated Fontan completion should be considered in the management of these patients to address PAVMs.
• Ensuring hepatic blood flow to both lungs may be crucial in preventing PAVM development.