Insights in Pediatric Cardiology

Procedural and Short Term Outcomes of Transcatheter Radiofrequency: Assisted Pulmonary Valvotomy and Balloon Valvuloplasty of Patients with 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.

Materials and Methods: This is a retrospective, descriptive, cohort study of all patients with PA-IVS who underwent TRFAPV-BV from December 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.

Results: There are 29 pediatric patients diagnosed with PAIVS between December 2013 to April 2016. Out of these 29 patients with PA-IVS, 9 pediatric patients had a tripartite right ventricle underwent TRFAPV-BV, in whom 8 were described as successful, 89% (8/9). This study observed that the following parameters had a favorable outcome; Tricuspid Valve (TV) annulus z-score >-2.5, tripartite right ventricle, absence of ventricular to coronary connections, tricuspid to mitral valve ratio >0.5 and right-to-left ventricular pressure ratio of >1. Moreover, parameters after TRFAPV-BV of higher residual gradient across pulmonary valve and lower pulmonary valve annulus zscores may be predictive of the need for subsequent RVOT reintervention.

Conclusion: Using these parameters might predict good survival of patients and anticipate the need for a subsequent RVOT reintervention.

Author(s): Judah D Gozar

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