The Transcatheter Management of the Neonate and Infant with Pulmonary Atresia and Intact Ventricular Septum

Abstract

The optimal management of the neonate and infant with pulmonary atresia (PA) and intact ventricular septum (IVS) remains controversial. The ultimate aim of any treatment algorithm is to achieve a four-chambered, biventricular, completely separated circulation. In 1991, transcatheter perfor ation of the atretic membrane followed by successful balloon valvuloplasv was reported using a laser-assisted guidewire in the United Kingdom and the stiffeizd of a guidewire in the United States. The following year, a radiofrequency (RF) guidewire was successfully used, while stenting of the ductus arteriosus to maintain adequate pulmonary blood was also reported. Most recently, a steerable 5 Fr RF catheter was used to "burn '' the atretic membrane. From a series of 15 publications, a total of 69 neonates underwent attempted pulmonary valve perforation: I7 laser guidewire, 28 RF guidewire, 25 stiff end guidewire, and I steerable RF catheter. Successful perforation by technique was: 82.4% laser, 88.5% RF. 68% stiff end, and 100% steerable RF. The accumulative success rate was 79.7%, mortality 4.3%. major complication 18%, and need for additional pulmonary blood 48%. Follow-up thus far has been encouraging. The technique of transcatheter pegoration of the atretic pulmonary valve membrane is demanding and not without risk. but in experienced hands can be successful nearly 90% of the time using the RF guidewire. RF energy is commonly used for other cardiac problems and has inherent cost and availability advantages over laser energy. Since nearly 50% of the neonates still require additional pulmonary bloodflow, the interventionalist must be capable and ready to stent the PDA, or send to surgery. The neonate with right ventricular (RV) dominant coronary circulation remains a high risk group. However, careful cardiac catheterization with temporary transcatheter RV decompression may lead to a better understanding of this complex physiology. (J Interven Cardiol 1998;ll 363-387) General Background: Pulmonary Atresia and Intact Ventricular SeptumRobert Freedom reminds us that babies born with pulmonary atresia (PA) and intact ventricular septum (IVS) present a major challenge to the pediatric cardiology and cardiothoracic team.' While we have made significant advances in the treatment of newborns with transposition of the great arteries, even those with hypoplastic left heart syndrome, the outlook for the neonate with underdeveloped right-sided cardiac structures associated with an atretic pulmonary valve remains p~o r .~,~ The designation of "pulmonary atresia with intact ventricular septum" focuses on the outlet of the heart, and thus there has been an inherent suggestion of "simplicity" to this disorder. However, as we all know, nothing could be further from the truth!' This disorder is characterized by a striking heterogenicity of the right ventricle, its inlet, and its functionalIn addition, a number of these patients have hemodynamically important ventriculocoronary connections between the right ventricle and subepicardial coronary...

Perforating the atretic pulmonary valve with CTO hardware: Technical aspects

“…Previously, reports show that the results of this technique have been inferior to those obtained with radiofrequency, with an initial success rate of 68%, mortality of 4%, and the necessity of additional pulmonary irrigation in 48% . Although refinements in equipment and expertise in CTO may account for the improved outcomes observed in our patients, patient selection remains the single most important determinant of success.…”

Radiofrequency perforation in the treatment of congenital heart disease

“…There are few studies of the histological effects of RF perforation on cardiac tissue [16–18]. A recent study from our laboratory addressed the feasibility of RF energy to achieve atrial septal perforation, with an assessment of the biophysical profile of perforation and the gross appearance of perforated tissue in association with a balloon septoplasty.…”

“…Equipment and techniques have evolved considerably since laser‐based or stiff‐wire approaches to valve perforation were originally described [17–24]. Reviewed below is the general approach applied in the setting of pulmonary atresia with intact ventricular septum in the neonate for perforation of valve atresia.…”

“…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].…”

“…Freedom from the requirement of an aortopulmonary shunt after surgical valvotomy is in the range of 32% to 48% at 1 month [54, 55], and following RF valve perforation and balloon dilation in up to 42% to 66% (Table II) [17, 55, 56]. The incidence of shunt placement directly relates to the threshold for an acceptable systemic saturation while right ventricular compliance improves.…”