Transcatheter perforation of the right ventricular outflow tract as initial therapy for pulmonary valve atresia and intact ventricular septum in the newborn

The clinical impact of transcatheter perforation and dilatation of the right ventricular outflow tract in neonates with pulmonary atresia and intact ventricular septum was reviewed. Between April 1992 and December 1994, 8 neonates underwent transcatheter perforation of the right ventricular outflow tract. Radiofrequency energy was employed in 6 patients and wire perforation in 2 patients. Mean patient age at intervention was 1.9 ± 0.6 days and weight 3.4 ± 0.5 kg. Median tricuspid valve annulus was 10.9 mm (range: 4.0–13.0 mm) and Z-value −0.85 (range: −4.5–1.0). The mean right ventricular systolic pressure fell from 117 ± 16 to 55 ± 15 mm Hg (P < 0.0001), and the right ventricular to aortic pressure ratio decreased from 1.81 ± 0.33 to 0.82 ± 0.28 (P < 0.0001). The arterial duct was patent in all. No acute complications occurred. Aortopulmonary shunts were performed in 7 patients at a median 6 days (range: 3–23 days) following catheterization. One patient developed sepsis and died after surgical resection of infected tissue, while a second patient died of a blocked aortopulmonary shunt 17 months following discharge. Median follow-up for the 6 surviving patients was 8 months (range: 4–32 months). One patient has achieved and a second is awaiting biventricular repair. Transcatheter perforation appears to be a promising form of therapy in selected patients with pulmonary atresia, and potentially facilitates algorithms leading to a biventricular repair. Cathet. Cardiovasc. Diagn. 40:408–413, 1997. © 1997 Wiley-Liss, Inc.     

Laser valvotomy with balloon valvoplasty for pulmonary atresia with intact ventricular septum: five years' experience.

OBJECTIVE: To assess immediate and medium term results of transcatheter laser valvotomy with balloon valvoplasty in selected infants with pulmonary atresia and intact ventricular septum. DESIGN: Prospective study. SETTING: Tertiary cardiac unit. PATIENTS: All infants with pulmonary atresia and intact septum with no more than minor tricuspid valve hypoplasia referred between November 1990 and June 1995. Laser valvotomy was attempted in nine infants of median age 4-5 days and median weight 3.6 kg. INTERVENTION: The pulmonary valve was perforated using a 0.018 inch fibreoptic guidewire attached to a NdYag laser and introduced through a catheter positioned beneath the valve. After perforation the valve was dilated with progressively larger balloons. MAIN OUTCOME MEASURES: Successful laser valvotomy and balloon dilatation, complications, pulse oximetry, right ventricular outflow velocities, and need for surgical treatment. RESULTS: Valvotomy was successful in all but one case, the failure being due to laser breakdown. After perforation the valve was dilated to 6-8 mm diameter. Prostaglandin E was withdrawn immediately in six of the eight duct dependent infants, and 28 and 49 days later in two. No patient required an aortopulmonary shunt. Two patients had repeat valvoplasty at 20 days and three months of age, respectively; one required infundibular resection and closure of the atrial septum at age four and one is awaiting similar treatment. CONCLUSIONS: Laser valvotomy with balloon valvoplasty is safe and effective treatment for selected patients with pulmonary atresia and intact ventricular septum and should be considered as first line treatment in place of surgical valvotomy.     

Transcatheter retrograde radio-frequency perforation of the pulmonic valve in pulmonary atresia with intact ventricular septum,using a 2 French catheter

A neonate with pulmonary atresia with intact ventricular septum (PA/IVS) underwent successful retrograde transcatheter perforation of the pulmonary valve, using a 2 Fr radio-frequency catheter with subsequent anterograde balloon dilation of the valve. Due to persistent hypoxemia, the neonate underwent placement of a 4-mm modified Blalock-Taussig shunt. Transcatheter retrograde transductal perforation of the pulmonic valve in PA/IVS is feasible and may be a potential alternative to anterograde perforation. Cathet. Cardiovasc. Diagn. 45:151–154, 1998. © 1998 Wiley-Liss, Inc.     

Stenting the patent arterial duct to increase pulmonary blood flow

BACKGROUND: Use of surgically created aoropulmonary shunt is well-established for improving pulmonary blood flow in infants with critical reduction in pulmonary blood flow. Recently, stenting the patent ductus arteriosus has emerged as an alternative in selected infants with congenital heart disease and reduced pulmonary blood flow. METHODS AND RESULTS: We reviewed records of consecutive infants undergoing stenting of patent ductus arteriosus between August 2003 and October 2005 at our institution. Two of 12 patients underwent patent ductus arteriosus stenting to facilitate preparation of left ventricle for transposition with intact septum. We report the case selection, technique, immediate and short-term follow-up outcome in the remaining 10 patients [median age: 16 days (range 4-290 days): weight 2.7 kg (range 2-6 kg)] with reduced pulmonary blood flow who underwent stenting of patent ductus arteriosus as an alternative to conventional surgical aortopulmonary shunts. Five of the 6 newborns were prostaglandin-dependent and 4 had previously undergone guidewire perforation of the pulmonary valve (n=2) or balloon dilation (n=2). Successful stent implantation was accomplished in all with no major patient-related complication (median fluoroscopy time: 18.6 min; range: 7.7-72 min). The intensive care unit and hospital stays were prolonged in 3 patients because of sepsis (n=2) and pulmonary over-circulation with sepsis (n=1). On follow-up (median 5.5 months; range 1-19 months) all implanted stents were patent. One patient underwent re-dilation of the implanted stent for declining saturations. CONCLUSIONS: The immediate and short-term follow-up results of stenting of the patent arterial duct, as an alternative to the surgical aortopulmonary shunt in carefully selected newborns and infants is encouraging.     

Radiofrequency pulmonary valvotomy using a new 2-French catheter

We report the usefulness of a new 2-French (F) electrode catheter for perforating the atretic pulmonary valve in patients with pulmonary atresia and intact ventricular septum, using radiofrequency energy. The new 2-F electrode catheter was used in three patients. The first patient, weighing 2.1 kg, with pulmonary atresia and an intact ventricular septum, underwent transcatheter valvotomy at the age of 18 days. Due to massive left ventricular volume overload, the patient required surgical ligation of the ductus arteriosus, but she was discharged uneventfully after that. The second patient, weighing 2.2 kg, had Ebstein's anomaly with pulmonary atresia and an intact ventricular septum. She underwent transcatheter valvotomy at the age of 30 days. Although she was weaned from prostaglandin E₁ infusion, she died suddenly (presumed septicemic). A postmortem examination showed a split pulmonary valve. The third patient, weighing 2.3 kg, with pulmonary atresia and an intact ventricular septum, underwent transcatheter valvotomy at the age of 17 days. Prostaglandin E₁ infusion was discontinued on the 3rd day after the transcatheter valvotomy and she was discharged uneventfully. Cathet. Cardiovasc. Diagn. 45:37–42, 1998. © 1998 Wiley-Liss, Inc.     

Pulmonary atresia with intact ventricular septum

Opinion statement The prognosis for patients with pulmonary atresia with intact ventricular septum is poor with and without conventional surgical treatment. Because of this reason, a comprehensive program of medical, transcatheter, and surgical treatment is necessary to improve the long-term outlook of these infants. Algorithms of management plans should be developed based on the presence of right ventricular-dependent coronary circulation as well as size and morphology of the right ventricle. In a tripartite or bipartite right ventricle, transcatheter radiofrequency perforation is preferable. Alternatively, surgical valvotomy may be performed. Augmentation of pulmonary blood flow by prolonged infusion of prostaglandin E₁, stenting the ductus, or a surgical modified Blalock-Taussig shunt may be necessary in some of these patients. In patients with a unipartite or very small right ventricle or a right ventricular-dependent coronary circulation (Tricuspid valve Z score < -2.5), augmentation of pulmonary flow along with atrial septostomy should be undertaken. Follow-up studies to determine the feasibility of biventricular repair should be undertaken and, if feasible, surgical or transcatheter methods may be used to achieve the goals. If not suitable for biventricular repair, one-ventricle (Fontan) or one and one-half ventricular repair should be considered. Comprehensive and well-planned treatment algorithms may help improve survival rate.     

Perforation of the atretic pulmonary valve using chronic total occlusion (CTO) wire and coronary microcatheter

Background and objective: Chronic total occlusion (CTO) guidewire have been recently reported as an alternative to radiofrequency for perforating atretic pulmonary valve. Since procedure failures or perforation of the right ventricle still occurred with CTO, we tried to enhance the stability, steering, and pushability of the wire using a microcatheter in order to improve the safety and efficacy of the procedure.
Methods: We performed pulmonary valve perforation with CTO guidewire and microcatheter in five consecutive newborns with pulmonary atresia with intact ventricular septum (PA‐IVS) under fluoroscopic and echocardiographic control.
Results: The valve was easily perforated at the first attempt for all patients. After perforation, the microcatheter positioned in the main pulmonary artery allowed the exchange of the CTO guidewire for a more flexible wire, avoiding lesion and facilitating manipulation in the distal pulmonary branch arteries. The pulmonary valve was then dilated with balloons of increasing size as usually performed. We did not experience any procedural or early complications. Blalock‐Taussig shunt was performed in 2 children because of a persistent cyanosis, 4 and 10 days after perforation.
Conclusion: The combined use of a CTO guide and a microcatheter appears to be a safe and reliable technique for perforating the pulmonary valve of newborns with PA‐IVS. Further procedures with this approach are needed to confirm this first experience.     

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

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 perforation of the atretic membrane followed by successful balloon valvuloplasty was reported using a laser-assisted guidewire in the United Kingdom and the stiff end 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: 17 laser guidewire, 28 RF guidewire, 25 stiff end guidewire, and 1 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 perforation 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 blood flow, 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.     

Transarterial pulmonary venous wedge arteriography in pulmonary atresia,ventricular septal defect,and intact atrial septum

Transarterial pulmonary venous wedge arteriography was performed in four cases of pulmonary atresia (PA), ventricular septal defect (VSD), and intact atrial septum. In two cases, only the left pulmonary artery was visualized by selective injection to the patent ductus arteriosus (PDA) or Blalock-Taussig anastomosis (B-T shunt), and the right pulmonary artery was opacified by transarterial retrograde pulmonary arteriography. In two other cases, with nonfunctioning B-T shunt, the pulmonary artery was not opacified by either aortography nor by injection into the B-T shunt. Transarterial pulmonary vein wedge arteriography visualized a confluent pulmonary artery. Transarterial pulmonary vein wedge arteriography was a useful and safe procedure for opacifying the pulmonary artery that could not be visualized antegradely in VSD, PA, and intact atrial septum.     

Laser-assisted tricuspid valve balloon dilation for acquired tricuspid valve atresia.

Acquired pulmonary valve atresia is a well-recognized but uncommon complication of surgical systemic-to-pulmonary artery shunts in patients with tetralogy of Fallot. Acquired atresia of the tricuspid valve, however, has not been reported previously. This complication developed in a 3-year-old girl, with pulmonary atresia and an intact ventricular septum, after a Blalock-Taussig shunt and right ventricular outflow tract reconstruction. Percutaneous transcatheter laser-assisted balloon dilation re-established antegrade flow across the tricuspid valve.     

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.     

Percutaneous pulmonary valvulotomy using radiofrequency in pulmonary atresia with intact interventricular septum

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.     

Use of Palmaz stents to palliate pulmonary atresia with ventricular septal defect and stenotic aortopulmonary collaterals

A 29-year-old woman with pulmonary atresia and ventricular septal defect underwent palliative stenting of a stenotic aortopulmonary collateral. Follow-up demonstrated patent stents, with improved oxygenation and exercise capacity. This approach offers an attractive alternative to surgical shunt placement in select patients. Cathet. Cardiovasc. Diagn. 40:387–389, 1997. © 1997 Wiley-Liss, Inc.     

Guided transcatheter valvulotomy in pulmonary atresia with intact ventricular septum

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.     

Radiofrequency-assisted balloon dilatation in patients with pulmonary valve atresia and an intact ventricular septum.

OBJECTIVE--To investigate the efficacy and safety of transcatheter radiofrequency-assisted valve dilatation for infants with pulmonary valve atresia and intact ventricular septum as an alternative to the use of laser heated wires. DESIGN--Prospective clinical study. SETTING--Three paediatric cardiology centres. PATIENTS--Four children (aged 5-101 days, weight 2.8 kg) with pulmonary valve atresia and intact ventricular septum underwent percutaneous radiofrequency-assisted valve dilatation. METHODS--After delineating the atretic valve by angiography, 0.020 inch or 0.018 inch radiofrequency wires were used to perforate the atretic valve. The valve was then dilated with conventional balloon dilatation catheters up to the valve annulus diameter. RESULTS--In all four cases the radiofrequency wire perforated the atretic pulmonary valve and balloon dilatation was successful. In one patient the radiofrequency wire also passed through the anterior wall of the pulmonary artery causing tamponade which required surgical repair shortly afterwards. This patient died from sepsis six days later. One patient died three weeks after the procedure from septicaemia and a paradoxical coronary embolus. Two patients were discharged after 4 and 14 days respectively. CONCLUSIONS--Radiofrequency-assisted valve dilatation is a promising alternative to the recently developed laser wire technique. The major advantages are a reduction in cost and improved safety for the staff performing the procedure.     

Angiojet catheter-based thrombectomy in a neonate with postoperative pulmonary embolism.

A 2-day-old male with pulmonary atresia and an intact ventricular septum underwent central aortopulmonary shunt placement and right ventricular outflow tract reconstruction. Postoperatively, the patient had difficulty weaning from mechanical ventilation and was found to have a large thrombus occluding the entire right lower pulmonary artery. The patient underwent successful percutaneous catheter-based thrombectomy in the early postoperative period with the Possis AngioJet catheter. The patient had immediate improvement in gas exchange and was able to be weaned from the ventilator shortly thereafter. This represents the first published use of mechanical thrombectomy in a postoperative neonate.     

Pulmonary atresia, "intact ventricular septum", and aortopulmonary collateral arteries.

In muscular pulmonary atresia, major aortopulmonary collateral arteries are characteristic of pulmonary atresia with ventricular septal defect and are rarely seen in pulmonary atresia with intact ventricular septum. Two unusual cases of muscular pulmonary atresia are reported, one with an intact septum and one with a perimembranous ventricular septal defect, closed in utero by aneurysmal tricuspid tissue. In both cases the pulmonary blood supply came entirely from aortopulmonary collaterals. In case 1 a collateral artery connected the left subclavian artery and hypoplastic pulmonary arteries, and several aortopulmonary collaterals arose from the descending aorta, without overlap between these two circulations. In case 2 the pulmonary trunk and arterial duct were absent and the pulmonary blood supply came entirely from collateral arteries. The right ventricle was of normal size and tripartite with a closed perimembranous ventricular septal defect, discovered only at postmortem examination. These observations suggest right ventricular outflow tract obstruction early in fetal development, with involution of the pulmonary trunk and sixth arch derivatives, and persistence of primitive aortopulmonary connections. The morphology in case 1 is at odds with the theoretical division of pulmonary atresia with intact septum and pulmonary atresia with ventricular septal defect into two separate pathological entities that occur at different stages in fetal development.     

Pulmonary atresia with an intact septum: indications for the neonatal surgical treatment guided by 2-dimensional and Doppler echocardiography

Twelve neonates with pulmonary atresia and intact ventricular septum underwent surgical treatment based on two-dimensional and Doppler echocardiography. Ten patients with adequate morphology of the right ventricular outflow tract portion shown by means of two-dimensional echocardiography underwent pulmonary valvulotomy and systemic-to-pulmonary shunt. Two neonates with atresia of the right ventricular infundibulum on two-dimensional echocardiography underwent systemic-to-pulmonary artery shunt without valvulotomy. In all patients the qualitative and quantitative echocardiographic diagnosis was confirmed at surgery and/or with the subsequent angiocardiographic study. Two-dimensional and Doppler echocardiography is a precise diagnostic method for planning surgical treatment in neonates with pulmonary atresia and intact ventricular septum.     

Pulmonary atresia with intact ventricular septum associated with severe aortic stenosis

Pulmonary atresia with intact ventricular septum is the complete obstruction of the right ventricular outflow tract due to pulmonary valve atresia in the absence of ventricular septal defect. Pulmonary flow is dependent on the ductus arteriosus. Other morphological anomalies are also present. Aortic stenosis in association with pulmonary atresia with intact ventricular septum is extremely infrequent, and very few cases have been described.We report a 19-year-old primiparous woman in whom the fetal echocardiogram showed pulmonary atresia with intact ventricular septum. The neonate had low oxygen saturation and a systolic murmur originating in the aorta. An echocardiogram showed pulmonary atresia with intact ventricular septum and a bicuspid, thickened, stenotic aortic valve. Low cardiac output ensued and could not be controlled, and the infant died.     

Complete blalock–taussig shunt obstruction in < 24 hours post‐operative period in a neonate treated emergently using transcatheter angioplasty and low dose local recombinant TPA

A 12‐day‐old infant with pulmonary atresia, intact ventricular septum, and pulmonary blood flow through a ductus arteriosus developed complete shunt obstruction within 12 hr of creation of a modified Blalock–Taussig shunt. Low dose recombinant tissue plasminogen activator was administered locally as two 0.03 mg/kg bolus injections and was followed by balloon angioplasty. This resulted in complete recanalization of the shunt without any hemorrhagic complications. © 2013 Wiley Periodicals, Inc.