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Pulmonary Atresia with Intact Interventricular Septum

Background: Pulmonary atresia-intact interventricular septum (PA-IVS) is a rare congenital heart disease. To improve cardiac circulation, the goal now is to carefully select patients to achieve a biventricular repair via Transcatheter Radiofrequency- Assisted Pulmonary Valvotomy and Balloon Valvuloplasty (TRFAPV-BV) and to avoid early open heart surgery. Success rates of >80% have been reported. Due to its rarity, no single institution can provide a consistent interventional guideline. There is much to be learned from a multicentered approach to collecting longitudinal experience to a challenging clinical case.

Objective: We report our procedural and short-term outcomes in our patients who have undergone TRFAPV-BV with an emphasis on possibly identifying the predictors for survival and the need for additional transcatheter Right Ventricular Outflow Tract (RVOT) reintervention.

Methodology: This is a retrospective, descriptive, cohort study of all patients with PA-IVS who underwent TRFAPV-BV from Decemeber 2013 to April 2016. The hospital medical records of each patient was reviewed. Transthoracic  two dimensional echocardiogram reports and clips, and cardiac catheterization reports pre and post-TRFAPV-BV were reviewed. In addition, each patient’s clinical course through medical records were examined until their most recent clinical follow-up.



Judah D Gozar , Jose Jonas D, Del Rosario, Dexter Eugene, and D Cheng 

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