Right ventricle-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome.
OBJECTIVE
Pulmonary overcirculation through a systemic-pulmonary shunt has been one of the major causes of early death after the Norwood procedure. To avoid this lethal complication, we constructed a right ventricle-pulmonary shunt in first-stage palliation of hypoplastic left heart syndrome.


METHODS
Between February 1998 and February 2002, 19 consecutive infants, aged 6 to 57 days (median, 9 days) and weighing 1.6 to 3.9 kg (median, 3.0 kg), underwent a modified Norwood operation with the right ventricle-pulmonary artery shunt. The procedure included aortic reconstruction by direct anastomosis of the proximal main pulmonary artery and a nonvalved polytetrafluoroethylene shunt between a small right ventriculotomy and a distal stump of the main pulmonary artery. The size of the shunt used was 4 mm in 5 patients and 5 mm in 14.


RESULTS
All patients were managed without any particular manipulation to control pulmonary vascular resistance. There were 17 survivors (89%), including 3 patients weighing less than 2 kg. Two late deaths occurred due to obstruction of the right ventricle-pulmonary artery shunt. Thirteen patients underwent a stage II Glenn procedure after a mean interval of 6 months, with 2 hospital deaths. To date, a stage III Fontan procedure has been completed in 4 patients. Overall survival was 62% (13/19). Right ventricular fractional shortening at the last follow-up (3-48 months after stage I) ranged from 26% to 43% (n = 13, mean, 33%).


CONCLUSIONS
Without delicate postoperative management to control pulmonary vascular resistance, the modified Norwood procedure using the right ventricle-pulmonary shunt provides a stable systemic circulation as well as adequate pulmonary blood flow. This novel operation may be particularly beneficial to low-birth-weight infants with hypoplastic left heart syndrome.
Sano
Shunji
S
Department of Cardiovascular Surgery, Okayama Graduate School of Medicine and Dentistry, 2-5-1 Shikata-cho, Okayama-City 700-8558, Japan. s_sano@cc.okayama-u.ac.jp
Ishino
Kozo
K
Kawada
Masaaki
M
Arai
Sadahiko
S
Kasahara
Shingo
S
Asai
Tomohiro
T
Masuda
Zen-ichi
Z
Takeuchi
Mamoru
M
Ohtsuki
Shin-ichi
S
D001794:Blood Pressure / Q000502:physiology; D003971:Diastole / Q000502:physiology; D015150:Echocardiography, Doppler; D005260:Female; D018729:Fontan Procedure; D006352:Heart Ventricles / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000601:surgery; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D007223:Infant; D007229:Infant Welfare; D007231:Infant, Newborn; D007234:Infant, Premature; D007564:Japan; D008297:Male; D010100:Oxygen / Q000097:blood; D010166:Palliative Care; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery*; D012720:Severity of Illness Index; D016019:Survival Analysis; D013997:Time Factors; D016896:Treatment Outcome; D014262:Tricuspid Valve Insufficiency / Q000175:diagnosis / Q000601:surgery
D010100:Oxygen