Novel surgical approach 'intrapulmonary-artery septation' for Fontan candidates with unilateral pulmonary arterial hypoplasia or pulmonary venous obstruction.

It is difficult to manage patients with single ventricular physiology and unbalanced pulmonary arteries. Since 1998, we started a novel approach [Intrapulmonary-artery Septation (IPAS)] to improve the result for those with severe unbalanced pulmonary arteries consisting of a well-grown pulmonary artery and an inadequately-grown pulmonary artery. The inadequately-grown pulmonary artery includes severe pulmonary arterial hypoplasia and pulmonary venous obstruction. This approach is based on the following concepts: (1) A reliable blood source should be secured to recover the inadequately-grown pulmonary artery; (2) Wasteful volume-load should be prevented for the heart; (3) Long stenosis or non-confluence of pulmonary artery should be avoided. IPAS primarily consists of (A) a Glenn shunt; (B) a systemic-pulmonary artery shunt; and (C) a septation-patch. Both (A) and (B) are adjoined on a well-grown pulmonary artery, and (C) is placed between (A) and (B). PAS brings two separate blood flows of a Glenn shunt to the well-grown side and SPS on the inadequately-grown side. IPAS was performed in 20 patients. Seventeen reached the Fontan operation. Eleven underwent postoperative catheterization and seven had acceptable-balanced pulmonary blood flow distribution. IPAS can pilot more complicated cases having severe unbalanced pulmonary arteries to the Fontan circulation.