Introduction and objectives:

Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare but serious cyanotic congenital heart disease. Depending on the patient's anatomy, different therapeutic strategies surgical or trancatheter can be planned.

The objective of this study was to describe the results of transcatheter pulmonary valve perforation in patients with PA/IVS in a single tertiary center, and compare transjugular to transfemoral approach. The need for additional source of pulmonary flow (ductal stenting or systemic-to-pulmonary artery fistula) at follow-up was reviewed to identify possible risk factors associated with this reintervention.

Methods:

patients with PA/IVS referred for transcatheter pulmonary valve perforation as first-line therapy from February 2004 through May 2022 were included. Technical procedural details, total procedural and fluoroscopy times, and demographic and echocardiographic data were studied.

Results:

A total of 22 patients were included. Procedure was successful in 20 cases (91%). The rate of complications was 2/22 (9%). No deaths were reported. The transjugular and transfemoral approaches were equally safe and effective. The total median procedural (n = 20) and fluoroscopy times (n = 16), however, were shorter in the transjugular compared to the transfemoral approach (85 min vs 156 min, and 31 min vs 62 min, respectively), which reached statistical significance. At follow-up, 8/20 (40%) patients needed additional flow (4 ductal stenting, 4 systemic-to-pulmonary artery shunts). No significant risk factors regarding this reintervention were reported.

Conclusions:

Transcatheter mechanical pulmonary valve perforation may be feasible in expert hands and properly selected patients being an attractive alternative to surgery. In our own experience, transjugular approach seems to simplify the procedure, and reduces procedural and fluoroscopy times.

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