Exclusion of the non-functioning right ventricle in children with pulmonary atresia and intact 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.
Yang
Ji-Hyuk
JH
Department of Thoracic and Cardiovascular Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Gangnam-gu, Seoul, Republic of Korea.
Jun
Tae-Gook
TG
Park
Pyo Won
PW
Sung
Kiick
K
Kim
Wook Sung
WS
Lee
Young Tak
YT
Huh
June
J
Kang
I-Seok
IS
D003326:Coronary Circulation; D018729:Fontan Procedure; D018610:Heart Bypass, Right; D006352:Heart Ventricles / Q000002:abnormalities / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D010166:Palliative Care / Q000379:methods*; D018579:Patient Selection; D018633:Pulmonary Atresia / Q000473:pathology / Q000503:physiopathology / Q000601:surgery*; D012086:Reoperation; D016896:Treatment Outcome; D014261:Tricuspid Valve / Q000601:surgery; D016276:Ventricular Function; D054088:Ventricular Septum / Q000473:pathology