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Exclusion of the non-functioning right ventricle in children with pulmonary atresia and intact ventricular septum

^a Department of Thoracic and Cardiovascular Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea
^b Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea

Received 14 September 2007; received in revised form 20 November 2007; accepted 23 November 2007.

^_* Corresponding author. Address: Department of Thoracic and Cardiovascular Surgery, Samsung Medical Center, lrwon-dong 50, Gangnam-gu, 135-710 Seoul, Republic of Korea. Tel.: +82 2 3410 3484; fax: +82 2 3410 0089. (Email: tg.jun{at}samsung.com).

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.

Key Words: Pulmonary atresia/surgery • Heart bypass, right • Treatment outcome • Congenital heart disease, cyanotic • Infant, newborn