OBJECTIVE: A group of patients with pulmonary atresia and intact ventricular septum (PAIVS) have to undergo single ventricle repair. In these patients, the presence of the non-functioning right ventricle (RV) may lead to the aggravation of RV to coronary connections and left ventricular (LV) dysfunction. To prevent these deleterious effects, the RV was excluded surgically. METHODS: Between December 2000 and February 2006, 10 patients with PAIVS underwent RV exclusion in conjunction with cavo-pulmonary anastomosis (n=6) or systemic-to-pulmonary artery shunt (n=4). Median age at surgery was 5 months (range, 0.2-13.8). Median z-value of the tricuspid valve was -4.0 (range, -6.5 to -1.3). None had RV dependent coronary circulation. The tricuspid valve was closed directly or using a patch. Thrombotic materials were inserted into the RV cavity in eight patients. RESULTS: There was no mortality. Follow-up was completed in all patients for up to 79.1 months (median, 30.3). Seven patients underwent a Fontan procedure and the other three are waiting. Eight patients showed completely obliterated RV after exclusion. One of the two patients who had residual RV cavity underwent re-exclusion after a Fontan operation. Postoperative echocardiography revealed that LV end-diastolic dimension increased with borderline significance (p=0.050), whereas LV end-systolic dimension showed no significant changes. During follow-up, LV showed no evidence of regional ischemia or global dysfunction, and fractional shortening significantly increased compared with the pre-exclusion value (p=0.017). CONCLUSIONS: This study demonstrates that RV exclusion may be performed safely in a selected group of patients with PAIVS. This procedure may have beneficial effects on LV systolic function, by preventing possible ischemia, and on LV diastolic function, by obliterating the non-functioning cavity and improving the geometry of the ventricular septum.
OBJECTIVE: A group of patients with pulmonary atresia and intact ventricular septum (PAIVS) have to undergo single ventricle repair. In these patients, the presence of the non-functioning right ventricle (RV) may lead to the aggravation of RV to coronary connections and left ventricular (LV) dysfunction. To prevent these deleterious effects, the RV was excluded surgically. METHODS: Between December 2000 and February 2006, 10 patients with PAIVS underwent RV exclusion in conjunction with cavo-pulmonary anastomosis (n=6) or systemic-to-pulmonary artery shunt (n=4). Median age at surgery was 5 months (range, 0.2-13.8). Median z-value of the tricuspid valve was -4.0 (range, -6.5 to -1.3). None had RV dependent coronary circulation. The tricuspid valve was closed directly or using a patch. Thrombotic materials were inserted into the RV cavity in eight patients. RESULTS: There was no mortality. Follow-up was completed in all patients for up to 79.1 months (median, 30.3). Seven patients underwent a Fontan procedure and the other three are waiting. Eight patients showed completely obliterated RV after exclusion. One of the two patients who had residual RV cavity underwent re-exclusion after a Fontan operation. Postoperative echocardiography revealed that LV end-diastolic dimension increased with borderline significance (p=0.050), whereas LV end-systolic dimension showed no significant changes. During follow-up, LV showed no evidence of regional ischemia or global dysfunction, and fractional shortening significantly increased compared with the pre-exclusion value (p=0.017). CONCLUSIONS: This study demonstrates that RV exclusion may be performed safely in a selected group of patients with PAIVS. This procedure may have beneficial effects on LV systolic function, by preventing possible ischemia, and on LV diastolic function, by obliterating the non-functioning cavity and improving the geometry of the ventricular septum.