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Pulmonary Artery Development Over Time in Severe Ebstein Anomaly.

Hiromitsu Shirozu1, Yoshihiko Kodama2,3, Ayako Kuraoka1

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Summary

Severe Ebstein anomaly (EA) patients undergoing the Starnes procedure show smaller central pulmonary arteries (PA) compared to those with pulmonary atresia with intact ventricular septum (PAIVS). Careful monitoring of PA size is crucial for severe EA patients post-Fontan procedure.

Keywords:
Ebstein anomalyPulmonary artery developmentStarnes procedure

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Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease Surgery
  • Vascular Biology

Background:

  • The Starnes procedure has improved outcomes for severe Ebstein anomaly (EA).
  • Understanding time-dependent changes in central pulmonary artery (PA) size after surgical interventions for severe EA remains limited.
  • Pulmonary atresia with intact ventricular septum (PAIVS) serves as a relevant comparison group for surgical outcomes.

Purpose of the Study:

  • To investigate and compare the growth and size of the central pulmonary artery (PA) in patients with severe Ebstein anomaly (EA) versus pulmonary atresia with intact ventricular septum (PAIVS) following neonatal surgical interventions.
  • To assess differences in ventricular volume and their correlation with PA size in these patient groups post-Fontan procedure.

Main Methods:

  • Retrospective chart review of patients who underwent the Starnes procedure for severe EA or PAIVS at Fukuoka Children's Hospital.
  • Inclusion criteria: neonatal surgical intervention, follow-up data available.
  • Data collected: central PA size at various surgical stages, ventricular volume, survival rates.

Main Results:

  • No significant difference in survival was observed between the severe EA (n=14) and PAIVS (n=36) groups.
  • The central PA size was consistently smaller in the severe EA group at all assessed surgical stages (post-shunt, post-Glenn, post-Fontan).
  • Significantly larger ventricular volumes were noted in the severe EA group after the Fontan procedure, indicating poor PA growth.

Conclusions:

  • Patients with severe Ebstein anomaly exhibit impaired central pulmonary artery growth following neonatal surgical management, including the Starnes procedure and subsequent Fontan circulation.
  • Close monitoring of central PA size is recommended for severe EA patients both pre- and post-Fontan procedure.
  • Further research is needed to elucidate the long-term prognosis and optimal management strategies for pulmonary artery development in severe EA.