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.     

Radiofrequency perforation for pulmonary atresia and intact ventricular septum

Surgical or interventional perforation of atretic pulmonary valve as initial palliative procedure has been advocated in patients with pulmonary atresia with an intact ventricular septum. We report two infants with pulmonary atresia and intact ventricular septum, who were treated by radiofrequency perforation and balloon dilatation.     

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.     

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.     

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.     

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.     

Aortic valve atresia: a new classification based on necropsy study of 73 cases.

Certain clinical and morphologic observations are described in 73 necropsy patients with aortic valve atresia. The mean age at death was 5 days; 80 percent died during the first week of life, and 70 percent were boys. Of the 73 patients, 69 (95 percent) had a hypoplastic left ventricle with intact ventricular septum and either an atretic (25 patients) or hypoplastic (44 patients) mitral valve. The other four patients had a well developed left ventricle with one or more defects in the ventricular septum and either an atretic (one patient) or well developed (three patients) mitral valve. Review of previous reports on aortic valve atresia disclosed that a well developed left ventricle or ventricular septal defect in association with absence of the aortic valve was extremely rare. A new classification for aortic valve atresia is presented based on the status of the ventricular septum, which in turn appears to determine the size of the left ventricular cavity. The predilection for male subjects for all types of aortic valve disease, including atresia, is emphasized.     

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.     

Transcatheter treatment of pulmonary outflow tract obstruction: A review

The role of transcatheter methods in the management of pulmonary outflow tract obstruction are discussed in this review. Balloon pulmonary valvuloplasty for relief of isolated pulmonary valve stenosis has been successfully used by many investigators and is the procedure of choice for the management of these lesions. Supravalvar pulmonic stenosis, if discrete, can be relieved by balloon dilatation. Cyanotic children with interatrial right-to-left shunts secondary to severe valvar pulmonary stenosis respond in a manner similar to that observed with isolated pulmonary valve stenosis. In these patients, balloon valvuloplasty is the treatment of choice and may be corrective in most patients. In patients with interventricular right-to-left shunting secondary to pulmonary outflow tract obstruction and in patients with narrowed BT shunts, balloon dilatation may be an effective palliative procedure in a substantial proportion of patients obviating the need for an initial or second palliative shunt. Balloon dilatation is recommended if the patient's size or cardiac anatomy make them unsuitable for safe total surgical correction. In patients with pulmonary atresia, either initial opening of the atretic pulmonary valve by laser or by surgery with subsequent balloon dilatation are potentially beneficial in reducing the total number of surgical procedures that these children are likely to require. However, further clinical trials are needed before their general use.     

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.     

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

BACKGROUND: 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. OBJECTIVE: To determine whether transcatheter perforation of the atretic pulmonary valve is an acceptable management strategy prior to surgical repair. RESULTS: 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. CONCLUSION: 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.     

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.     

Balloon pulmonary valvuloplasty after initial pulmonary valvotomy for pulmonary atresia with intact ventricular septum]

Seven patients with pulmonary atresia and intact ventricular septum have undergone closed pulmonary valvotomy on the early neonatal period (2-6 days) with satisfactory results. Follow-up catheterization showed significant residual pulmonary valve stenosis requiring a second operation. Percutaneous balloon dilatation of the pulmonary valve was successful in 6 cases with the mean right ventricular systolic pressure reduced from 6.8 to 4.9 kPa (50.8 to 37 mmHg) and the mean transvalvular gradient dropped from 5.7 to 2.1 kPa (4.28 to 15.8 mmHg). One case failed and required surgical treatment. The results showed that balloon valvuloplasty could be applied instead of surgery for treatment of selected cases of pulmonary atresia with intact ventricular septum after an initial pulmonary valvotomy.     

Echocardiographic assessment of neonates with pulmonary atresia and intact ventricular septum

In this prospective study, 27 consecutive neonates suspected to be suffering from pulmonary atresia and intact ventricular septum underwent detailed two-dimensional echocardiographic examination before cardiac catheterization. Of the 27 neonates 25 had pulmonary atresia and intact ventricular septum and the remaining 2 had "functional pulmonary atresia" secondary to severe Ebstein's anomaly of the tricuspid valve. In all 25 neonates with pulmonary atresia and intact ventricular septum, the diagnosis and right ventricular morphology based on the tripartite approach were correctly established by echocardiography. The associated Ebstein's anomaly in two babies with pulmonary atresia and intact ventricular septum was also correctly identified by echocardiography. Among the five babies who had a sinusoidal-coronary artery communication, echocardiography demonstrated the fistula in one and provided clues for its diagnosis in two others. In the 25 neonates with pulmonary atresia and intact ventricular septum, the echocardiographic dimensions of their tricuspid anulus, right ventricular infundibulum and main pulmonary artery correlated well with the angiocardiographic measurements (r greater than 0.8). The results of this study suggest that, in the management of neonates with pulmonary atresia and intact ventricular septum, preoperative evaluation by echocardiography is usually sufficient and cardiac catheterization should be reserved for selected cases.     

Ductus arteriosus patency with stenting in critical pulmonary stenosis and pulmonary atresia with intact interventricular septum

We report our experience with stenting of the ductus arteriosus in three neonates. Two patients had pulmonary atresia with an intact interventricular septum and one had critical pulmonary stenosis. Radiofrequency ablation was used to open the atretic pulmonary valve in two patients. In all three patients implanting a stent avoided the need for surgical creation of a Blalock-Taussig shunt. In two patients the procedure was scheduled as elective surgery, and in one it was done as an emergency procedure. Ductus arteriosus stenting is an alternative to palliative cardiac surgery.     

From Atretic to Perforated: A Closer View of Pulmonary Atresia

A 7‐day‐old male infant with membranous pulmonary atresia and intact ventricular septum (PA/IVS) underwent hybrid pulmonary valvotomy. Access was via median sternotomy guided by intra‐operative epicardial ultrasonography. After puncturing the atretic pulmonary valve, a 6 mm Wanda balloon catheter was inflated to dilate the atretic valve. Adequate valve movement with antegrade flow was confirmed using real time epicardial echocardiography. Intra‐operative epicardial ultrasonography is an effective tool for guiding PA/IVS during hybrid procedures. Epicardial echo imaging provides clear images with excellent quality, helps guide the hybrid operation, and prevents the need for the ionizing radiation of fluoroscopy.     

Truncal or aortic valve stenosis in functionally single arterial trunk: A clinical,hemodynamic and pathologic study of six cases

Stenosis of the semilunar valve in the presence of a functionally single arterial trunk is uncommon. Three patients with truncus arteriosus, two with tetralogy of Fallot and pulmonary atresia and one with pulmonary atresia and intact septum were diagnosed as having stenosis of the truncal or aortic valve on the basis of clinical, echocardiographic, hemodynamic and angiocardiographic findings. Echocardiograms consistently showed multiple diastolic closure lines and abnormal semilunar valves in addition to the aortic override in five patients and hypoplastic right ventricle in the patient with pulmonary atresia and intact septum. Peak systolic gradients between the left ventricle and truncus (or aorta) at cardiac catheterization ranged from 20 to 47 mm Hg. Retrograde aortography confirmed a domed and stenotic semilunar valve. Cyanosis was progressive in the three patients with pulmonary atresia. Two patients had arterial anastomosis, and one had a right ventricle-pulmonary arterial graft in addition to aortic valvotomy. One of the three patients with truncus arteriosus underwent complete repair in addition to truncal valvotomy but he died in the postoperative period. The other two patients with truncus arteriosus died of intractable congestive cardiac failure before surgical intervention.It is suggested that the presence of semilunar valve stenosis in these patients adversely affects the prognosis. The myocardium is already jeopardized as a result of hypoxia in pulmonary atresia and left ventricular diastolic overload in patients with truncus arteriosus. The added burden of semilunar valve stenosis may further compromise the functional status of the myocardium.     

Transcatheter management of cyanotic congenital heart defects: a review.

In this review, the role of transcatheter methods in the management of cyanotic congenital heart defects is discussed. In patients with interventricular right-to-left shunting secondary to pulmonary outflow tract obstruction (most commonly tetralogy of Fallot), balloon dilatation may be an effective palliative procedure in a substantial proportion of patients, obviating the need for a palliative shunt. We would recommend this if the patient's size or cardiac anatomy makes that patient an unsuitable candidate for safe total surgical correction. Infundibular myectomy with atherectomy catheter in tetralogy of Fallot patients may become a useful adjunct in the management of these infants. Cyanotic children with interatrial right-to-left shunt secondary to severe valvar pulmonary stenosis respond to balloon pulmonary valvuloplasty in a manner similar to that seen with isolated pulmonary valve stenosis. In these patients, balloon valvuloplasty is the treatment of choice and may be corrective in most cases. In patients with a narrowed Blalock-Taussig shunt, balloon angioplasty may improve pulmonary oligemia and systemic arterial hypoxemia and may obviate the need for a second systemic-to-pulmonary artery shunt. Balloon angioplasty is recommended if the patient's cardiac defect is not amenable to surgical correction at a low risk either because of the size of the patient or because of the complexity of the cyanotic heart defect. In patients with pulmonary valve atresia, initial opening of the atretic pulmonary valve by either laser or surgery with subsequent balloon dilatation is potentially beneficial in reducing the total number of surgical procedures that these children are likely to require. However, further clinical trials are needed prior to their general use.     

Echocardiographic and Doppler study of pulmonary atresia with an intact interventricular septum

Pulmonary atresia with intact ventricular septum represents a congenital cardiac lesion for which the prognosis depends on the patency of the ductus arteriosus. Nine children with pulmonary atresia and intact ventricular septum underwent echocardiographic studies before cardiac catheterization trying to visualize the anatomy of the outflow tract of the right ventricle, the dimension of the ventricular cavity, the characteristics of the tricuspid valve and finally the size and integrity of the interventricular septum. Using Doppler ultrasound it was possible to evaluate the function of the tricuspid valve and the patency of the ductus arteriosus. The results obtained were correlated with the angiographic and anatomic studies. We concluded that noninvasive assessment of this malformation permits an excellent correlation with the angiographic and anatomic studies.