Objective:

To report on a child with pulmonary atresia with intact ventricular septum (PAiVS) and large right ventricle-to-coronary artery fistulae, where right ventricular-dependent coronary circulation (RVDCC) was excluded angiographically, followed by interventional closure of the fistulae and transcatheter perforation of the pulmonary valve to achieve biventricular physiology.

Approach:

• Method: label
• Method: text

Key Findings:

• The patient had multiple large RV-to-coronary fistulae that were successfully closed.
• Transcatheter interventions led to improved biventricular function and reduced right ventricular pressure.
• At 22 months, the patient exhibited normal oxygen saturation and preserved biventricular function.

Interpretation:

The case illustrates the importance of catheter-based interventions in managing complex congenital heart defects, particularly in patients with PAiVS and RV-to-coronary fistulae.

Limitations:

• The initial diagnostic cardiac catheterization did not definitively exclude RVDCC.
• Long-term outcomes beyond 22 months are not reported.

Conclusion:

The successful closure of coronary fistulae and RVOT intervention in this patient demonstrates the potential for achieving biventricular circulation in complex congenital heart disease.