Transcatheter Management of Neonates With Pulmonary Atresia With Intact Ventricular Septum: A Single Center Experience From Turkey

Abstract

Pulmonary atresia with intact ventricular septum (PAIVS) is characterized by a broad spectrum of heterogeneous morphologies. Perforation of the atretic valve, balloon dilatation, and stenting of the patent ductus arteriosus are the percutaneous techniques that are used with increasing frequency in our clinic as well. They have some advantages over surgery, including short hospital stay and short intensive care unit stay. The main goal of the primary interventional approach is to avoid surgery. However, a group of patients with PAIVS still need surgery due to poor right ventricular growth. Therefore, the final achievement of the initial percutaneous treatment strategies is still debatable. In this article, we present the early- and mid-term results of the percutaneous approach utilized at our clinic in order to investigate the final effects of interventional therapy according to initial morphology. Between May 2010 and May 2012, 15 neonates diagnosed with PAIVS underwent transcatheter intervention. Detailed echocardiographic examination focused on right ventricle size, and tricuspid valve morphology and coronary sinusoids were performed in all the patients before the intervention. Nine of the patients were boys and six were girls. The mean age was 11.40 ± 12.87 days and mean weight was 3.34 ± 0.46 kg. Only one procedure-related mortality occurred. The mean follow-up period was 10.05 ± 3.42 months (1-26 months). The mean duration of intensive care was 7.19 ± 5.14 days. The mean follow-up time was 10.05 ± 3.42 months. After this period, survival rate was 66% (10/15). Two of the patients achieved biventricular physiology after pulmonary valve perforation. Two patients still have univentricular physiology. Six patients have been followed as a one and half ventricle repair candidate. Five out of 15 patients had stent patency during 6 months of follow-up, while restenosis developed in one patient (1/5, 20%), who had undergone the Glenn operation at 5 months of age. Transcatheter management for PAIVS is a feasible, safe, and effective primary palliative treatment in newborns. Shunt surgery may be considered in cases where cyanosis occurs despite transcatheter intervention. Right ventricular size determines the type of intervention. The early outcomes can be comparable with surgical palliation. However, a group of PAIVS, particularly with severe right ventricular hypoplasia, needs surgery even after a successful primary percutaneous intervention.

Anesthesia for high‐risk procedures in the catheterization laboratory

“…The first attempt at transcatheter pulmonary valvotomy in PA/IVS was described by Qureshi in 1990, with a success rate at short‐term follow‐up in four of the five cases. Almost 30 years later, this procedure still carries a high risk …”

“…Almost 30 years later, this procedure still carries a high risk. [56][57][58][59] Mortality rate based on the 2012 Kids' Inpatient database is estimated at 6.69%. 60 Predictors of mortality for children previously diagnosed with PA/IVS were age <12 months, nonelective admission, and the use of ECMO.…”

Transcatheter perforation of atretic pulmonary valve by the stiff end of a coronary wire in neonates with pulmonary atresia with intact ventricular septum: A solution in developing countries

“…Heterogeneity of this pathology classifies the management strategy into two major tracks: two ventricular circulation (or at least one and half ventricle repair) or single ventricle repair [7], [8], [9], [10]. If the track is univentricular repair, the interventional role is patent ductus arteriosus (PDA) stenting as an alternative to surgical Blalock–Taussig (BT) shunt, which can be considered with balloon atrial septostomy if needed [1].…”

“…Conventionally, this was achieved by surgical valvotomy in addition to a modified BT shunt [12]. The accepted interventional alternative is the use of radiofrequency-assisted perforation of the atretic valve and subsequent balloon dilation [9], [12], [13]. However, radiofrequency perforation is expensive and is not available in every center.…”

Ductal Stent Implantation in Tetralogy of Fallot with Aortic Arch Abnormality

“…7 Ductal stenting via the retrograde femoral approach is technically feasible in cases of tricuspid atresia, pulmo nary atresia with intact ventricular septum, and critical pulmonary stenosis with PDA arising from the proxi mal descending aorta. 7,9,19 However, gaining access to the ampulla is much more difficult if the PDA arises proximally to the aortic arch ("vertical" ductus). In such cases, the catheter can be advanced antegradely, after introduction via femoral vein access.…”