A perforation procedure for pulmonary atresia with intact ventricular septum

Abstract

The management of PA-IVS is complex. This is a disease that necessitates a dedicated team and working collaboration between the cardiologists and cardiac surgeons. Cost limitation is essential in developing countries and innovative ideas to reduce costs are essential, especially if comparable success can be expected.

Staged Percutaneous Management of Pulmonary Atresia and Intact Interventricular Septum: Stretching the Limits

“…With advances made in science, technology, and experience, an increasing number of patients who were doomed for univentricular repair are being relocated to the biventricular group with good results. In our 10-year experience with neonates who have pulmonary atresia and intact interventricular septum, [ 15 ] we have established radiofrequency perforation and dilatation of the atretic PV as the gold standard for treatment instead of PDA stenting and the univentricular pathway and/or surgery. Because of pulmonary regurgitation, with or without variable degrees of residual annular stenosis, the RVOT and MPA in addition to RV become dilated in the long term, analogous to what is classically observed following the repair of tetralogy of Fallot with a transannular patch; PPVI is gaining more popularity in these patients with results superior to that of surgical replacement [ 18 , 19 ].…”

“…Since September 2009, we have performed 101 cases of radiofrequency (RF) perforation and dilatation of the PV to manage cases with PA/IVS with Z-scores larger than −3, with a success rate of ∼60% [ 15 ]. Since then, successful cases have been followed up every 6 months to monitor RV growth and the development of the PA and branches, in addition to the degree of pulmonary regurgitation and/or stenosis.…”

Perforation of the atretic pulmonary valve using chronic total occlusion (CTO) wire and coronary microcatheter

“…Percutaneous valve perforation and dilatation is the treatment of choice for PA‐IVS without RV hypoplasia or RV‐dependent coronary perfusion. Various techniques have been developed to perforate the pulmonary valve before dilatation, including radio frequency, laser, or using the hard tip of a coronary guidewire . Since a laser is very expensive and requires preventive measures to protect staff, and the results of perforation with the stiff end of a wire are unpredictable, radio frequency is currently the most preferred method for pulmonary valvotomy.…”

“…Various techniques have been developed to perforate the pulmonary valve before dilatation, including radio frequency, 10 laser, 11 or using the hard tip of a coronary guidewire. 4 Since a laser is very expensive and requires preventive measures to protect staff, and the results of perforation with the stiff end of a wire are unpredictable, radio frequency is currently the most preferred method for pulmonary valvotomy. However, since the emergence of CTO guidewire in the last decade, various publications have demonstrated the efficacy of this wire to perforate the pulmonary valve instead of the costlier and less available radiofrequency.…”

“…Radiofrequency is a well‐established method for perforating the pulmonary valve in case of pulmonary atresia with intact ventricular septum (PA‐IVS) . However, considering the high cost and the low availability of radiofrequency generator and wires, many perforations of the atretic valve are performed with a mechanical procedure using the stiff end of a coronary guidewire, and more recently using chronic total occlusion (CTO) guidewire . Yet, the mismatch between the diameter of the 5‐French Judkins right (JR 5F) usually used for the procedure and the 0.014 inch CTO wire could diminish stability, steering, and pushability of the wire, resulting in failures or right ventricular outflow tract (RVOT) perforation .…”