New percutaneous techniques for perforating the pulmonary valve in pulmonary atresia with intact ventricular septum

Abstract

Even though pulmonary atresia with intact ventricular septum is an infrequent defect, accounting for less than 1% of congenital heart defects, it is the 3 rd most frequent cyanotic heart defect in the neonatal period 1 . Its morphological spectrum is broad with cases ranging from extremely hypoplastic tricuspid valves and right ventricles to ventricular cavities of almost normal dimensions [2][3][4] . Ebstein's anomaly of the tricuspid valve and extreme ventricular dilation have also been reported, and they are associated with a poor prognosis 5 . In addition, communication between the right ventricular cavity and the coronary arteries is a relatively common finding, which is sometimes associated with coronary circulation partially or totally dependent on the right ventricle [6][7][8] . Due to this anatomic heterogeneity, the therapeutic algorithm should be individualized 6 . The final objective is always to attain biventricular correction with total separation between systemic and pulmonary circulations 6 . However, this is sometimes impossible, and correction with a 1½ ventricle or of the univentricular type (Fontan) is necessary [6][7][8][9] . Cardiac transplantation should also be considered for treating cases with severe stenoses or multiple interruptions in the coronary arteries and secondary left ventricular dysfunction 6,8 . The initial therapeutic approach in the neonatal period should, whenever possible (if the coronary circulation pattern allows), open the pulmonary valve to decompress the right ventricle and stimulate its growth 3,[6][7][8] . During the last decade, perforation of the pulmonary valve to establish continuity between the right ventricle and the pulmonary artery with the aid of interventional catheterization became a reality [10][11][12][13][14][15] , even in Brazil 16. We report 2 percutaneous techniques of valve perforation, which were recently introduced into clinical practice, and their advantages and disadvantages are discussed. Case reportWe report the cases of 3 patients referred to our service from other neonatal units for investigation or treatment of cyanotic congenital heart defects. The clinical, echocardiographic, and hemodynamic data, are listed in tables I, II, and III. It is worth noting that patient 2 had a previous diagnosis of critical pulmonary stenosis. All patients had mild to moderate cyanosis under continuous infusion of prostaglandin; on auscultation, the 2 nd cardiac sound was single and low, and was followed by a mild systolic murmur in the dorsum. On chest X-ray, the cardiac silhouette was slightly enlarged, mainly because of the right atrium, and the pulmonary flow was reduced. The electrocardiogram showed sinus rhythm and left ventricular hypertrophy in all patients. QRS axes ranged from 90º to 120º. In regard to the echocardiographic findings, all patients had situs solitus, pulmonary atresia with imperforate pulmonary valve, and intact ventricular septum. The right ventricle was hy-

Paediatric and congenital heart disease in South America: an overview

“…The technique has evolved and neonates with critical PVS or PA and IVS could be managed similarly. The application of radiofrequency energy for valvar perforation in selected cases of PA and IVS has been employed with good results 3940. The same technology has also been used for vessel recanalisation or trans-septal access to the left atrium in infants 4142.…”

Recanalization of a discrete atretic right pulmonary artery segment with a new radiofrequency system

“…In our case, some modifications were required because of the underlying anatomy and limited vascular access. Also, due to some of the advantageous features of the Baylis Medical RF system [4], the procedure could be completed in a relatively straightforward manner. In our case, perforation was not performed in a blind fashion.…”

“…In addition, the low-profile and flexible Nykanen RF perforation catheter did not distort the Judkins catheter course. The amount of energy and time used for vessel perforation was determined arbitrarily, being a little more than that previously used for pulmonary valve perforation [4].…”

“…This patient was an 18-month-old boy with PA and IVS who underwent successful RF perforation of the pulmonary valve according to a previously described technique [4] at the age of 3 days. In the neonatal period, there was a well-developed infundibulum, a tripartite right ventricle, and confluent, good-sized pulmonary arteries.…”

Radiofrequency perforation in the treatment of congenital heart disease

“…In the absence of a right ventricular–dependent coronary circulation, decompression of the right ventricle is a component of a treatment algorithm, which attempts to salvage the right heart as a component of a biventricular or so‐called one‐and‐a‐half ventricular repair [36]. An alternative to primary surgical decompression strategies is the use of percutaneous laser or RF‐assisted perforation of the atretic valve and subsequent balloon dilation [5–9, 17, 19– 26, 28, 29, 37–53].…”