Long term survival of hypoplastic left heart syndrome infants: Meta-analysis comparing outcomes from the modified Blalock–Taussig shunt and the right ventricle to pulmonary artery shunt

Jacob Y. Cao, Kevin Phan, Julian Ayer, David S. Celermajer, David S. Winlaw*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

17 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)107-116
Number of pages10
JournalInternational Journal of Cardiology
Volume254
DOIs
StatePublished - Mar 1 2018

Keywords

  • Hypoplastic left heart syndrome
  • Modified Blalock–Taussig shunt
  • Right ventricle to pulmonary artery shunt
  • Stage 1 palliation

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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