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Double-inlet ventricle presenting in infancy. II. Results of palliative operations

R C Franklin1, D J Spiegelhalter, R H Anderson

  • 1Thoracic Unit, Hospital For Sick Children, London, England.

The Journal of Thoracic and Cardiovascular Surgery
|May 1, 1991
PubMed

Insights

Palliative surgery for double-inlet ventricle in infants had a negative immediate survival impact but improved medium-term outcomes for survivors. Systemic-pulmonary artery shunts showed the most benefit, aiding clinical decisions in managing this complex congenital heart defect.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Surgery
  • Neonatal Intensive Care

Background:

  • Double-inlet ventricle is a complex congenital heart defect requiring early intervention.
  • Palliative surgical strategies are often employed to improve survival and quality of life in affected infants.
  • The long-term impact of various palliative procedures on survival remains a critical area of investigation.

Purpose of the Study:

  • To evaluate the influence of palliative surgery on survival rates in infants diagnosed with double-inlet ventricle.
  • To compare the outcomes of different palliative surgical approaches, including systemic-pulmonary arterial shunts, pulmonary trunk banding, and aortic arch obstruction repair.
  • To analyze the immediate and medium-term effects of palliative interventions on patient survival.

Main Methods:

  • Retrospective analysis of 191 infants under one year of age with double-inlet ventricle, treated between 1973 and 1988.
  • Data collection included palliative operations, specific surgical procedures, and survival follow-up (median 8.5 years).
  • Statistical analysis compared survival rates between different surgical groups and non-operative management, calculating relative risks.

Main Results:

  • Overall palliative surgery had a deleterious effect on immediate survival (RR 6.6, p<0.001) but improved medium-term outcomes (RR 0.68, p<0.05) in survivors.
  • Systemic-pulmonary arterial shunts demonstrated the most significant benefit, with reduced early (RR 2.52) and improved later (RR 0.43) mortality.
  • Pulmonary trunk banding, with or without aortic arch repair, did not significantly alter medium-term risk (RR 1.13 and 0.91).

Conclusions:

  • Palliative surgery for double-inlet ventricle in infants presents a complex risk-benefit profile, with immediate survival challenges but potential for improved medium-term outcomes.
  • Systemic-pulmonary artery shunts appear to be the most effective palliative strategy for improving survival in this population.
  • These findings underscore the importance of judicious surgical decision-making and patient selection in the management of infants with double-inlet ventricle.

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