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Transcatheter Perforation of Atretic Pulmonary Valve in Pulmonary Atresia

Updated: May 7

with Ventricular Septal Defect

P. Syamasundar Rao, MD


Transcatheter perforation of atretic pulmonary valve membrane by blunt end of regular[1-7] or coronary[8-10] guide wires, laser wires,[11-13] or radiofrequency catheters/wires[3,4,13-22] followed by balloon dilatation has been used as an alternative to surgery in the management of pulmonary atresia with intact ventricular septum, with reasonably good results. However, such an approach to treat pulmonary atresia with Ventricular Septal Defect (VSD) has rarely been used.[12,23-25] This difference may in part be related to anatomic differences between the two types of pulmonary atresia. The purpose of this communication is to present the details of retrograde perforation of atretic pulmonary valve membrane along with anterograde balloon pulmonary valvuloplasty in a patient with pulmonary atresia with VSD (severe Tetralogy of Fallot) and to discuss the role of these procedures in the management of such cases.

Case Report

A three-week-old female infant presented with severe cyanosis (in 1995) and, with cardiac catheterization and selective cine-angiography, was found to have a large VSD and pulmonary valve atresia and underwent urgent central aortopulmonary shunt. The infant improved clinically, and was discharged home shortly after surgery, and followed periodically in the outpatient clinic.

Because of increasing cyanosis and polycythemia, she was referred back to the author for catheter evaluation at the age of 22 months. Right and left heart catheterization (Table 1) with selective cine-angiography confirmed the diagnosis of a large malaligned VSD with bidirectional shunt, pulmonary valve atresia and patent aortopulmonary shunt. There was moderate arterial desaturation (77%) with a pulmonary to systemic flow ratio (Qp:Qs) of 1.1 and normal pulmonary vascular resistance (Table 1).

FIGURE 1a Selected cine-angiographic frame of main pulmonary artery (PA) in posteroanterior view prior to pulmonary valve perforation demonstrating blindly ending PA with opacification of the branch PAs (not labeled). The catheter (C1) was introduced into the PA from the aorta via the central aortopulmonary shunt. C2, catheter in the right atrium.

FIGURE 1b Selected cine-angiographic frame of right ventricle (RV) in postero-anterior view following pulmonary valve perforation demonstrating forward flow from the RV into the PA. Components of the guide wire (GW) rail are marked. Ao, aorta; C, catheter in the RV.

The shunt was cannulated from the aorta with #4-F Glidecath (Meditech, Inc Watertown, MA) with the help of Benston guide-wire (Cook, Bloomington, IN) and a pulmonary artery (PA) cineangiogram was performed to delineate the PA anatomy (Figure 1a). Simultaneous injection of the contrast material into the main PA and right ventricular outflow tract was also performed to visualize the atretic membranous pulmonary valve (Figure 2). Brief attempts to position a catheter below the pulmonary valve anterogradely and to perforate the valve were unsuccessful. Therefore, a retrograde approach was entertained. The #4-F Glidecath catheter that was positioned in the PA via the central shunt was connected to a Toughy-adapter to facilitate injection of the contrast material over the wire. A 0.021 inch straight guide wire was positioned at the tip of the catheter and after assuring that the catheter was in the middle of the pulmonary valve by a test injection through the Toughy-adapter, firm, but gentle pressure was applied, thus allowing the guide wire to traverse through the atretic pulmonary valve. Over this guide wire, the catheter was advanced into the right ventricle and then the guide wire and the catheter together were advanced into the right atrium. At this juncture the guide wire was exchanged with an exchange length 0.025 inch "J" tipped extra stiff Amplatz guide wire (Cordis Corporation, Miami, FL). The "J" component of the guide wire in the right atrium was snared with a 15mm "gooseneck" snare (Microvena, Vadnais Heights, MN) that was introduced through the femoral vein. The tip of the wire was slowly drawn out through the femoral vein while slowly advancing it from the femoral artery entry.

Thus, a loop was formed from the femoral artery through descending and ascending aorta, aortopulmonary shunt, main pulmonary artery, perforated pulmonary valve, right ventricle, right atrium, inferior vena cava, and femoral vein. The size of the pulmonary valve annulus measured 6 to 7 mm.

To read the full article, please go to the November 2018 Issue of CCT.