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BMJ 2010; 340 doi: (Published 02 June 2010) Cite this as: BMJ 2010;340:c2873

A shunt improves survival for babies with underdeveloped left heart

Babies born with the hypoplastic left heart syndrome, an anomaly that affects one in 5000 live newborns, have an incompletely developed mitral valve, left ventricle, aortic valve, and ascending aorta. They survive in the womb because the foramen ovale and a dilated ductus arteriosus allow the right heart to take over the function of running the systemic circulation. However, once born, these babies die of right heart failure within days, unless surgery is performed.

The surgical approach to correcting the anomaly comprises three operations. The first operation, the Norwood procedure, is performed immediately after birth and is associated with the highest mortality. The next two operations are performed before 6 months and 4 years of age, respectively.

A randomised trial of 549 infants treated in 15 North American centres compared two types of shunts used as part of the Norwood procedure: the classic—modified Blalock-Taussig shunt—and a newer—right ventricle-pulmonary artery shunt. Transplant-free survival to 12 months after randomisation was better with the newer shunt (74% (202/274) v 64% (175/275)). After that, no differences were seen in the primary outcome between the groups. Further unplanned surgical interventions and complications, which were mostly respiratory, …

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