Pulmonary Valve Perforation Research Articles

Outcomes of transcatheter approach for initial treatment of pulmonary atresia with intact ventricular septum

To review the short and medium term outcome of transcatheter pulmonary valve perforation (PVP) in patients with pulmonary atresia-intact ventricular septum and non-right ventricular dependant coronary circulation (PA/IVS non-RVDCC). PVP in patients with PA/IVS non-RVDCC has become more common in the past two decades. However, data on outcomes with this strategy are mixed. Data were reviewed retrospectively for all patients with PA/IVS non-RVDCC treated from 1996- 2010 at our institution. Patients who had severe neonatal Ebstein malformation, or initial interventional management at another institution were excluded. PVP was attempted in 30 of 50 patients (60%); 26 (87%) of these had a successful procedure. Twenty-four patients (48%) had surgery without PVP. There were no deaths in the cohort. Complications of PVP included 5 (17%) myocardial perforations. Of those with successful PVP, 10 (38%) did not have surgery (PVP-NS) and 16 (62%) had surgery (PVP-S) prior to discharge. Tricuspid valve (TV) Z-score was larger in the PVP-NS than in PVP-S patients, with median TV diameter Z-scores of +0.7 (-0.9, 1.7) and -1.1 (-2.8, 2), respectively (P = 0.01). Time from PVP to either hospital discharge (PVP-NS group) or surgery (PVP-S group) was significantly different between groups: 15 (7, 22) and 8 days (0, 46), respectively (P = 0.01). There were no differences in the number of trials or lowest arterial PaO2 off prostaglandins between groups. All patients in the PVP-NS group had a biventricular circulation at a median follow-up of 4.3 years. The results of a collaborative approach to treating neonates with PA/IVS non-RVDCC are excellent. Smaller TV size is associated with greater likelihood of surgery prior to discharge, and may serve as a surrogate for early RV inadequacy.

Pulmonary atresia with intact septum: the use of Conquest Pro coronary guidewire for perforation of atretic valve and subsequent interventions

To determine the feasibility and safety of the Conquest Pro wire as an alternative to radiofrequency wire for perforation of atretic pulmonary valve and subsequent balloon dilatation and patent ductus arteriosus stenting in patients with pulmonary atresia with intact ventricular septum. Radiofrequency valvotomy and balloon dilatation has become the standard of care for pulmonary atresia with intact ventricular septum in many institutions today. We report eight consecutive patients in whom we used the Conquest Pro coronary guidewire, a stiff wire normally reserved for revascularisation of coronary lesions with chronic total occlusion, for perforation of atretic pulmonary valve and subsequent balloon dilatation, and stenting of the patent ductus arteriosus. Perforation of atretic pulmonary valve was successful in seven out of eight cases. Radiofrequency valvotomy was employed after failure of perforation by the Conquest Pro wire in one case where the right ventricular outflow tract was broad based and tapered towards the pulmonary valve, and was heavily trabeculated. Failure of the Conquest Pro wire to perforate the pulmonary valve plate was mainly attributed by the failure to engage the wire at the correct position. The Conquest Pro wire for perforation and subsequent interventions in the more straightforward cases of pulmonary atresia with intact ventricular septum is effective and safe, simplifying the entire procedure. However, the radiofrequency generator and wires remain essential tools in the paediatric interventional catheter laboratory.

Use of a telescopic system for transcatheter radiofrequency perforation and balloon valvotomy in infants with pulmonary atresia and intact ventricular septum

Pulmonary atresia and intact ventricular septum is a complex congenital heart disease with great morphological variability. Approximately two-thirds of patients may be suitable for transcatheter pulmonary valvotomy. We reviewed our experience in the use of two different percutaneous approaches to evaluate the impact on fluoroscopy time and morbidity of a new technique to perform transcatheter radiofrequency perforation and valvotomy in newborns with pulmonary atresia and intact ventricular septum. In all, 31 patients underwent radiofrequency perforation of the pulmonary valve. The first 14 infants were treated using a 5 French Judkins right coronary catheter, which was manoeuvred directly underneath the atretic pulmonary valve (Group A). The others were treated using a telescopic system consisting of Northstar Lumax Flex and White Lumax Guiding Catheters (Cook; Group B). In both groups, after radiofrequency perforation of the pulmonary valve, a 0.014-inch superfloppy guidewire was advanced into the descending aorta and balloon dilations were performed. Required fluoroscopy time was significantly lower in Group B (48.5 ± 28.1 versus 24.9 ± 14.4 minutes, respectively; p < 0.01). A higher incidence of unfavourable events including the need for early surgery was found in Group A. In our experience, telescopic catheter proved to be a valid option able to decrease the fluoroscopy time of percutaneous radiofrequency perforation of pulmonary valve and consequently patients’ exposure to procedure-related risks.

Decreased Incidence of Right-Ventricular Outflow Tract Complications Using a Retrograde Snare Technique for Radiofrequency Pulmonary Valve Perforation

Perforation of the atretic pulmonary valve with balloon dilation in infants with pulmonary atresia with intact ventricular septum (PA-IVS) is standard initial therapy for right-ventricular (RV) decompression. This procedure often results in adequate pulmonary blood flow, thus eliminating the need for neonatal surgery. Nonetheless, the incidence of RV outflow-tract complications and mortality for this intervention is significant. We report our experience with retrograde snare-guided radiofrequency (RF) perforation in an attempt to improve accuracy and decrease procedural complications. Medical records were reviewed for the period between March 2007 and May 2010 for all patients with PA-IVS who presented to the catheterization laboratory for attempted RF perforation in infancy. Specific details reviewed included demographics, preprocedural echocardiographic (echo) data, procedural technique and complications, pre- and post-RV pressures and pulmonary valve gradients, need for surgical intervention in the neonatal period, and short- to medium-term follow-up. Eleven neonates with PA-IVS underwent RF perforation using a retrograde snare-guided technique during the study period. The pulmonary valve was successfully perforated and the wire snared in all 11 patients. Six of 11 atretic valves were crossed on the first attempt with low energy (5 W × 2 s). No episodes of tamponade or RV/PA perforation occurred as confirmed by echocardiogram performed immediately after the procedure. There was no ductal spasm with retrograde catheter manipulations. Sequential dilation of the perforated valve was not necessary. RV pressures decreased from 169 % systemic before dilation to 93 % after dilation (p < 0.001) with a residual pulmonary valve gradient of 16 ± 6 mm Hg. Eight of 11 patients (73 %) were discharged without surgery at an average 16 days after the intervention. Three patients required Blalock-Taussig shunts to augment pulmonary blood flow. Femoral artery thrombus occurred in 4 of 11 patients treated with anticoagulation, and 2 patients had atrial arrhythmias during the procedure. No other complications occurred. No preprocedural echo parameters predicted requirement for surgical shunt before hospital discharge. Retrograde snare-guided pulmonary valve perforation in infants with PA-IVS is safe and may decrease the incidence of significant procedural complications.

AS-198: Pulmonary Atresia with Intact Septum: Use of Conquest Pro Coronary Guidewire for Perforation of Atretic Valve and Subsequent Interventions

Radiofrequency valvotomy (RFV) and balloon dilatation has become the standard of care for pulmonary atresia with intact ventricular septum (PAIVS) in many institutions today. The objective of the study was to determine the feasibility and safety of the Conquest Pro wire as an alternative to Radiofrequency (RF) wire for perforation of atretic pulmonary valve and subsequent balloon dilatation ± patent ductus arteriosus stenting in patients with PAIVS.

Invited Commentary

Invited Commentary

Multiple transcatheter interventions in the same session in congenital cardiopathies

Background:To investigate the methods of percutaneous transcatheter interventions for combined congenital heart disease and to evaluate its efficacy in children.Materials and Methods:Thirty cases (ages 3 days-13.5 years, body weight 3-35 kg) that underwent two multiple transcatheter interventions for combined or solitary congenital heart disease were retrospectively analyzed and presented.Results:A total of 61 transcatheter interventions were performed in 30 patients as combined procedures. In 29 patients, two different procedures were combined in the same session, in remaining one patient, combination of three interventions were performed in the same catheter session. Interventions performed in combined procedures were as follows: Coarctation balloon angioplasty in 12 cases, pulmonary balloon valvuloplasty in 10, PDA coil embolization in 10, aortic balloon valvuloplasty in 8, VSD closure in 5, ASD closure in 4, ductal stent implantation in 4, palliative pulmonary balloon valvuloplasty in 3, recanalization and angioplasty of the systemic-pulmonary shunt in 2, balloon atrial septostomy in one, aortic coarctation stent implantation in one, coil embolization of a pulmonary lobar sequestration in one and pulmonary valve perforation plus pulmonary balloon valvuloplasty in one were performed as first or second procedure. There was no mortality or major morbidity in relation to combined procedures.Conclusion:Multiple transcatheter interventions in the same session are feasible, safe and effective with satisfactory good results. Second intervention may be performed as complementary procedure or independently to the first intervention.

Midterm Results for Collaborative Treatment of Pulmonary Atresia With Intact Ventricular Septum

We report a single-institution experience using a collaborative surgical and catheter-based approach to the initial treatment of pulmonary atresia with intact ventricular septum. A retrospective review was conducted of all neonates admitted with pulmonary atresia with intact ventricular septum from 1996 to March 2007. We identified 24 patients with a mean age at first intervention of 4.5 days with mean follow-up of 6.05 years (range, 1.9 to 12.7 years). Initial palliation was determined by right ventricular size, morphology, and presence or absence of right ventricular-dependent coronary circulation. Initial catheter-based pulmonary valve perforation and valvuloplasty was performed in 41.7% (10 of 24 patients; group A), and 58.3% (14 of 24 patients) had an initial systemic-to-pulmonary artery shunt (group B). Tricuspid valve size was significantly smaller in group B (median z-score, -0.52 group A versus -2.40 group B; p < 0.001). Placement of a shunt after valvuloplasty in group A was required in 70.0% (7 of 10 patients). There was no mortality in group A, and 70.0% (7 of 10 patients) are in a two-ventricle pathway and 30.0% (3 of 10 patients) are in a 1.5-ventricle pathway. Group B had mortality of 14.3% (2 of 14 patients), both within 5 days of surgery. All group B patients remain in a single-ventricle pathway. Overall survival is 91.7% (22 of 24 patients). An individualized approach to this complex lesion has good results. If the right ventricle can be safely decompressed and appears usable, the need for a shunt after valvuloplasty does not preclude two-ventricle (or 1.5-ventricle) repair. Anatomy mandating a shunt as initial palliation has substantial early mortality.

Novel catheter positioning technique for atretic pulmonary valve perforation

Percutaneous pulmonary valve perforation in selected neonates with pulmonary valve atresia and intact ventricular septum has been well established as a therapeutic option. Pulmonary valve perforation is associated with significant risks including perforation of the right ventricle. Optimal catheter positioning is crucial to the success of the procedure. This report describes a novel technique for accurate positioning of a guiding catheter during perforation of an atretic pulmonary valve.

Radiofrequency perforation of the right ventricular outflow tract as a palliative strategy for pulmonary atresia with ventricular septal defect

Radiofrequency perforation (RF) of the right ventricular outflow tract (RVOT), while an effective management strategy in children with an intact ventricular septum, has not been fully detailed in those presenting with a ventricular septal defect. To determine whether transcatheter perforation of the atretic pulmonary valve is an acceptable management strategy prior to surgical repair. Valve perforation was attempted in eight children seen between May 2000 and March 2006, five being infants between 1 and 9 days of age. In five children, this was the first of two procedures, the second a planned surgical correction. The RF was successful in six children with one child requiring additional stenting of the RVOT. Of these children, three attained a biventricular repair within the next year without additional palliative surgical procedures. Of the remaining three patients, one is awaiting surgical correction, one did not require further surgery, and one had this procedure as the only planned palliation. The two children in whom RF was not possible were referred for surgical augmentation of pulmonary blood flow. A treatment strategy that includes pulmonary valve perforation as initial palliation to increase pulmonary blood flow may be effective. Additional experience to better define those children who would benefit from this treatment algorithm is required.

Changes in tissue Doppler characteristics in a patient with pulmonary atresia and intact ventricular septum

Tissue Doppler measurements of the right and left lateral ventricular walls were made before and after perforation of the pulmonary valve using radiofrequency energy in a patient with pulmonary atresia and intact ventricular septum. The ratio of peak tissue velocity during rapid ventricular filling to atrial contraction increased for both atrioventricular valves after perforation of the pulmonary valve, and the patient was able to be weaned off prostaglandins without further intervention. Such measurements made using tissue Doppler may aid in the management of patients with pulmonary atresia and intact ventricular septum by predicting improvements in right ventricular relaxation.

Catheterization in neonates with pulmonary atresia with intact ventricular septum

Cardiac catheterization is infrequently required in babies with pulmonary atresia with intact ventricular septum. The main indications are to decide on the best form of treatment, the options being determined by the right ventricular as well as infundibular morphology and the presence of right-ventricle dependent coronary circulation. In most cases, an interventional approach is appropriate. This can consist of radiofrequency or laser perforation of the atretic pulmonary valve, combined with balloon dilation. In some patients, additional stenting of the arterial duct may be needed.

Treatment of Infants with Pulmonary Atresia and Intact Ventricular Septum: Long-term Follow-up

Cardiology| July 01 2003 Treatment of Infants with Pulmonary Atresia and Intact Ventricular Septum: Long-term Follow-up AAP Grand Rounds (2003) 10 (1): 5–6. https://doi.org/10.1542/gr.10-1-5-a Views Icon Views Article contents Figures & tables Video Audio Supplementary Data Peer Review Share Icon Share Facebook Twitter LinkedIn MailTo Tools Icon Tools Get Permissions Cite Icon Cite Search Site Citation Treatment of Infants with Pulmonary Atresia and Intact Ventricular Septum: Long-term Follow-up. AAP Grand Rounds July 2003; 10 (1): 5–6. https://doi.org/10.1542/gr.10-1-5-a Download citation file: Ris (Zotero) Reference Manager EasyBib Bookends Mendeley Papers EndNote RefWorks BibTex toolbar search toolbar search search input Search input auto suggest filter your search All PublicationsAll JournalsAAP Grand RoundsPediatricsHospital PediatricsPediatrics In ReviewNeoReviewsAAP NewsAll AAP Sites Search Advanced Search Topics: interventricular septum, pulmonary atresia, pulmonary valve Source: Agnoletti G, Pichaud JF, Bonhoeffer P, et al. Perforation Agnoletti G, Pichaud JF, Bonhoeffer P, et al. Perforation of the atretic pulmonary valve. Long-term follow-up. J Am Coll Cardiol. 2003;41:1399–1403. In many centers, catheter perforation of the atretic pulmonary valve followed by balloon pulmonary valvuloplasty has become the initial palliative approach for babies born with pulmonary atresia and intact ventricular septum (PAIVS). This report represents the accumulated experience of a group in Paris, France with 39 selected neonates with PAIVS over 11 years (1991–2001). They excluded cases with complicating features known to affect the short- and long-term course of PAIVS that were independent of the quality of relief of valve obstruction. Thirty-nine neonates in this very favorable subset of infants born with PAIVS underwent attempted catheter perforation of the pulmonary valve, and 33 procedures were deemed acutely successful (85%). There were 2 periprocedural deaths (5%), both attributed to catheter perforation of the right ventricle. Four others required urgent surgical attention after the catheter-mediated approach failed. Periprocedural complications were frequent, and included 3 cases each of atrial flutter and necrotizing enterocolitis. Mean total follow-up was 5.5 years among the 33 acute successes. Seventeen (52%) required surgical revision in the neonatal period. Three others had elective surgery later, yielding an actuarial freedom from operation of 35% at 400 days of postnatal life. Multiple operations were required in 9 patients, and further surgery was planned for several others. Although many patients had hemodynamic abnormalities at long-term follow-up, including tricuspid and pulmonary valve regurgitation, right-to-left atrial shunts, and partial cavopulmonary circulation, all surviving patients except 1 were reported to be in New York Heart Association functional class I. The authors conclude that the mortality and morbidity of this procedure is “non-negligible,” and that it offers hope of a satisfactory outcome for a subset of neonates with PAIVS, provided that the selection criteria for patients undergoing the procedure are sufficiently strict. In the neonate, PAIVS presents 2 basic problems. First, the only blood reaching the pulmonary artery arrives through the ductus arteriosus, which is soon to close. Second, considerable cyanosis results from right-to-left atrial shunting due to ineffective forward flow through the right heart. Treatment of PAIVS must, therefore, have 2 objectives. It must supply enough pulmonary blood flow so that the ductus arteriosus is no longer required, and it must promote sufficient right heart flow so that the right-to-left intracardiac shunting resolves. One is struck by how difficult it has been in the past to meet these objectives without multiple interventions and considerable mortality and morbidity.1,2 In the same way, we might initially be tempted to be too critical of the current report in which rates of acute mortality, acute failure, periprocedural complication, and necessity for further neonatal surgery are dismayingly high. We must, it seems, continually remind ourselves that PAIVS is a spectrum of diffuse, hypoplastic right heart disease that involves, with greater or lesser severity, the tricuspid annulus and valve; the right ventricular inlet,... You do not currently have access to this content.

Perforation of the atretic pulmonary valve: long-term follow-up

Perforation of the atretic pulmonary valve: long-term follow-up

Perforation of the atretic pulmonary valve: long-term follow-up

ObjectivesWe evaluated the long-term results of perforation of the pulmonary valve in patients with pulmonary atresia with an intact ventricular septum (PA-IVS). BackgroundInterventional perforation of the pulmonary valve is considered the elective first stage treatment for PA-IVS, particularly in patients with a tripartite right ventricle (RV) and normal coronary circulation. However, the long-term results of this procedure are lacking. MethodsBetween January 1991 and December 2001, 39 newborns with a favorable form of PA-IVS underwent attempted perforation of the pulmonary valve. We evaluated the early and long-term outcomes. ResultsMedian tricuspid and pulmonary zvalues were −1.2 and −2.4, respectively. Perforation was successful in 33 patients. Among them, 17 needed neonatal surgery, 13 did not need any surgery, and 3 had elective surgery after the first month of life. There were two procedure-related deaths, seven nonfatal procedural complications, and four postsurgical deaths. Compared with patients needing neonatal surgery, those having no or elective surgery had a higher incidence of a tripartite RV and a higher median tricuspid zvalue (92% vs. 53%, p = 0.04 and −1.7 vs. −0.5, p = 0.03). At a median follow-up of 5.5 years (range 0.5 to 11.5), survival was 85% and freedom from surgery was 35%. Five patients, four of whom had neonatal surgery, underwent a partial cavo-pulmonary connection. ConclusionsOur results show that this technique, although burdened by non-negligible mortality and morbidity, is effective in selected patients with a normal-sized RV. Preselection of patients allows interventional or surgical biventricular correction in the majority of cases.

Valvulotomía mecánica percutánea dirigida en la atresia pulmonar con septo íntegro

Transcatheter valvulotomy in pulmonary atresia with an intact ventricular septum can be used as a first step to create biventricular circulation and to stimulate further development of the hypoplastic right ventricle. We describe our experience in a case of a neonate with this congenital cardiac defect who underwent successful transcatheter perforation of the atretic pulmonary valve. This report highlights the utility of a special technique based on the use of a gooseneck snare positioned just above the atretic valve to guide the advance of a coronary guidewire. Other therapeutic alternatives are considered.

Initial and late results after catheter intervention for neonatal critical pulmonary valve stenosis and atresia with intact ventricular septum: A technique in continual evolution

Critical pulmonary valve stenosis or atresia with intact ventricular septum is a rare congenital cardiac defect that can be technically difficult to alleviate in the catheterization laboratory. Over the past 10 years, several techniques and modifications with variable results have been advocated to facilitate the valvuloplasty procedure. This report describes a single operator's experience using various techniques in 28 neonates with critical pulmonary stenosis or atresia who were considered candidates for transcatheter intervention. The first two patients underwent a gradational balloon valvuloplasty approach that resulted in prolonged fluoroscopy exposure. Thereafter, a "snare assisted" umbilical artery approach was developed which facilitated the valvuloplasty procedure and resulted in significantly fewer balloons used and shorter fluoroscopy times. Early in our experience, stiff guidewire perforation of atretic pulmonary valves was used, whereas in our last two patients, a simplified perforation technique with a new 0.9-mm excimer laser catheter was used. Late echocardiographic and clinical follow-up evaluation in 27 patients demonstrates persistent gradient relief, resolution of tricuspid valve insufficiency, and elimination of right to left shunting at the atrial level. Balloon valvuloplasty is the treatment of choice for critical pulmonary valve stenosis or atresia with intact ventricular septum. When necessary, the use of umbilical artery "snare assistance" facilitates the valvuloplasty technique and shortens procedure time while laser perforation is currently preferable for perforation of the atretic pulmonary valve.

Pulmonary atresia with intact ventricular septum (PA/IVS): diagnostic and interventional cardiac catheterization in the neonate

Treatment paradigms for neonates with pulmonary atresia and intact ventricular septum require accurate knowledge of the tricuspid valve size and structure, degree of right ventricular hypoplasia and coronary artery anatomy. Complete hemodynamic and angiographic studies are essential even when the diagnosis is established by echocardiography. Associated coronary artery stenosis and interruption can only be demonstrated by coronary angiography. Transcatheter perforation of the atretic pulmonary valve followed by balloon dilatation can establish right ventricular–pulmonary artery circulation followed by growth of the right ventricle in selected cases.

Valvulotomía pulmonar percutánea mediante radiofrecuencia en la atresia pulmonar con septo interventricular íntegro

Early pulmonary valvulotomy in patients with pulmonary atresia and intact interventricular septum allows the development and growth of the right ventricle and two-ventricle circulation. Percutaneous valvulotomy today is a valid alternative procedure to surgical valvulotomy. With the use of the radiofrequency 5F currently available for the treatment of arrythmias atretic pulmonary valve perforation and consecutive balloon dilation may be safely and effectively performed. We describe a case of perforation of a pulmonary valve by radiofrequency in a 15-day-old neonate with pulmonary valve atresia and intact ventricular septum.

Early Postinterventional Stent Stenosis of a Ductus Stent Implanted for Palliation in a Newborn with Pulmonary Atresia with Intact Ventricular Septum: Successful Management with Bailout Stenting

A newborn with pulmonary atresia with intact ventricular septum (PA-IVS) was treated with radiofrequency current perforation of the atretic pulmonary valve. As the right ventricle was hypoplastic (z-value of the tricuspid valve: —4) the arterial duct was stented with a Gianturco-Roubin GR II stent. Early postinterventionally, the patient became cyunotic and compromised blood flow across the stented ductus arteriosus despite adequate stent position was detected echographically. The newborn was treated successfully with the implantation of a Palmaz stent (Johnson & Johnson Interventional Systems, Warren, NJ, USA) into the obstructed Gianturco-Roubin GR II stent. The Gianturco-Roubin GR II stents might be associated with the risk of early stent stenosis after implantation in actively contracting tissues like the ductus arteriosus. In patients with early stent stenosis after ductal stenting, bailout implantation of a subsequent stent can be performed. Transcatheter procedures can be effective means for therapy of PA-IVS. (J Interven Cardiol 2000;13:39–44)