ABSTRACT
In Japan, more than 9,000 patients with congenital heart disease reach adulthood every year with improved medical treatment, and perioperative encounter with patients requiring the second intra-cardiac operation in adulthood is more often. Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease, and long-term prognosis is good. Therefore, we have more chances to care the postoperarive adult patients with TOF. This is a retrospective study in 6 patients with TOF who underwent the second intra-cardiac operation in adulthood from 2008 to 2012. Among the six patients, six had pulmonary valve replacement; four had tricuspid valve replacements or valvuloplastys; four had ventricular septal defect closures; two had right ventricular outflow tract obstruction releases; one had aortic valve replacement; and three had cryoablations. Right ventricular end-diastolic volume was 194.1 +/- 83.5 ml x m(-2) (mean +/- SD), and right ventricular ejection fraction was 38.2 +/- 4.8%. The factors which influence the anesthetic management were as follows: right cardiac failure by abnormality of the pulmonary valve; residual disease and deuteropathy of two or more combination; the risks associated with the second open chest surgery such as long operative time and severe bleeding. Perioperative management of an adult with congenital heart disease requires full understanding of its pathophysiology such as congenital heart disease, adult-specific complications, and the second open chest surgery.
