TY - JOUR
T1 - Long term survival of hypoplastic left heart syndrome infants
T2 - Meta-analysis comparing outcomes from the modified Blalock–Taussig shunt and the right ventricle to pulmonary artery shunt
AU - Cao, Jacob Y.
AU - Phan, Kevin
AU - Ayer, Julian
AU - Celermajer, David S.
AU - Winlaw, David S.
N1 - Publisher Copyright:
© 2017
PY - 2018/3/1
Y1 - 2018/3/1
N2 - Background: Stage 1 palliation of hypoplastic left heart syndrome (HLHS) involves the Norwood procedure combined with a modified Blalock–Taussig shunt (mBTS) or right ventricle to pulmonary artery shunt (RVPAS). Short-term survival has been described previously, whereas longer-term outcomes remain a subject of debate. This meta-analysis aimed to describe the short and long-term survival outcomes of these two shunts, and explore factors that might influence survival. Methods: Medline, Cochrane Libraries and EMBASE were systematically searched, and 32 studies were included for statistical synthesis, comprising 1348 mBTS and 1258 RVPAS patients. Results: While early in-hospital survival was superior in the RVPAS group (RR = 1.5, p < 0.05, 95% CI: 1.21–1.85), this difference was lost from 2 years post-stage 1 palliation (RR = 0.91, p > 0.05, 95% CI: 0.79–1.04), and maintained unchanged up to 6 years. This shift in survival was also reflected in inter-stage survival, with superior RVPAS outcomes between stage 1 and 2 (RR = 1.62, p < 0.05, 95% CI: 1.39–1.88), and equivalent outcomes between stage 2 and 3. Potential contributors to this included a significantly higher rate of pulmonary artery stenosis in the RVPAS group and an increased requirement for shunt re-intervention in this group prior to stage 2. Conclusions: Despite early advantages, RVPAS and mBTS for palliation of hypoplastic left heart syndrome produced comparable long-term survival. The RVPAS patients experienced more pulmonary artery stenosis and requirement for shunt re-intervention. The impact of shunt type on quality and survival with a Fontan is yet to be assessed.
AB - Background: Stage 1 palliation of hypoplastic left heart syndrome (HLHS) involves the Norwood procedure combined with a modified Blalock–Taussig shunt (mBTS) or right ventricle to pulmonary artery shunt (RVPAS). Short-term survival has been described previously, whereas longer-term outcomes remain a subject of debate. This meta-analysis aimed to describe the short and long-term survival outcomes of these two shunts, and explore factors that might influence survival. Methods: Medline, Cochrane Libraries and EMBASE were systematically searched, and 32 studies were included for statistical synthesis, comprising 1348 mBTS and 1258 RVPAS patients. Results: While early in-hospital survival was superior in the RVPAS group (RR = 1.5, p < 0.05, 95% CI: 1.21–1.85), this difference was lost from 2 years post-stage 1 palliation (RR = 0.91, p > 0.05, 95% CI: 0.79–1.04), and maintained unchanged up to 6 years. This shift in survival was also reflected in inter-stage survival, with superior RVPAS outcomes between stage 1 and 2 (RR = 1.62, p < 0.05, 95% CI: 1.39–1.88), and equivalent outcomes between stage 2 and 3. Potential contributors to this included a significantly higher rate of pulmonary artery stenosis in the RVPAS group and an increased requirement for shunt re-intervention in this group prior to stage 2. Conclusions: Despite early advantages, RVPAS and mBTS for palliation of hypoplastic left heart syndrome produced comparable long-term survival. The RVPAS patients experienced more pulmonary artery stenosis and requirement for shunt re-intervention. The impact of shunt type on quality and survival with a Fontan is yet to be assessed.
KW - Hypoplastic left heart syndrome
KW - Modified Blalock–Taussig shunt
KW - Right ventricle to pulmonary artery shunt
KW - Stage 1 palliation
UR - http://www.scopus.com/inward/record.url?scp=85041640278&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85041640278&partnerID=8YFLogxK
U2 - 10.1016/j.ijcard.2017.10.040
DO - 10.1016/j.ijcard.2017.10.040
M3 - Article
C2 - 29407078
AN - SCOPUS:85041640278
SN - 0167-5273
VL - 254
SP - 107
EP - 116
JO - International Journal of Cardiology
JF - International Journal of Cardiology
ER -