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.     

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.     

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. © 2008 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.     

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.     

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.     

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.     

Muscular Infundibular Atresia Is Associated with Coronary Ostial Atresia in Pulmonary Atresia with Intact Ventricular Septum

Objective. To determine whether infundibular morphology is associated with coronary ostial atresia and/or right ventricle dependent coronary circulation in patients with pulmonary atresia with intact ventricular septum. Design. Neonatal echocardiograms of patients with pulmonary atresia with intact ventricular septum were evaluated for tricuspid valve size and infundibular anatomy (muscular pulmonary atresia vs. membranous pulmonary atresia). Right ventricle dependent coronary circulation and coronary ostial atresia were diagnosed angiographically. Medical record review determined patient outcome. Setting. Tertiary‐care pediatric hospital. Results. Of 72 patients, 24 had muscular pulmonary atresia including 23 with right ventricle dependent coronary circulation. Fourteen of these had unilateral or bilateral coronary ostial atresia. Of 46 patients with membranous pulmonary atresia, 5 five had right ventricle dependent coronary circulation and none had coronary ostial atresia. Muscular pulmonary atresia patients had smaller tricuspid valve z‐scores and were less likely to achieve a biventricular repair than those with membranous pulmonary atresia (P < .01). Muscular pulmonary atresia had 82% sensitivity, 98% specificity, and 96% positive predictive value for presence of right ventricle dependent coronary circulation. In the group with membranous pulmonary atresia, there were two deaths, no transplants, and 23 (48%) achieved a biventricular repair. In contrast, of the 24 with muscular pulmonary atresia, there were seven deaths, two transplants, and no biventricular repairs. Conclusions. In this cohort, muscular pulmonary atresia was strongly associated with right ventricle dependent coronary circulation and coronary ostial atresia, and appears to be a useful morphologic marker for poor outcome among pulmonary atresia with intact ventricular septum patients. This information may be useful during prenatal counseling and for presurgical evaluation.     

Severe tricuspid stenosis presenting as tricuspid atresia. Echocargraphic diagnosis and surgical management.

Two cases of pulmonary atresia with intact ventricular septum and severe tricuspid stenosis are described in which the initial angiographic diagnosis was tricuspid atresia and pulmonary atresia. Two dimensional echocardiography showed the features of an imperforate tricuspid valve because in each case contrast echocardiography failed to show anterograde flow from the right atrium to right ventricle. Successful radical surgical repair was achieved in one patient with performing a tricuspid valvotomy and inserting an external valved conduit between the hypoplastic right ventricle and the main pulmonary artery. In the second case, an infant died four weeks after tricuspid valvotomy and right ventricular outflow tract reconstruction.     

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.     

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 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.     

Catheter‐based Therapy for Small Preterm Infants with Pulmonary Atresia/Intact Ventricular Septum

Catheter‐based therapy is the preferred treatment for term infants with pulmonary atresia and intact ventricular septum without right ventricular‐dependent coronaries, membranous atresia with patent infundibulum, and acceptable‐sized tricuspid valve. However, in smaller preterm infants, it is more difficult to determine the adequacy of the tricuspid valve and right ventricle for two‐ventricle repair and there are increased procedural risks. An excellent result in a small preterm infant with pulmonary atresia and intact ventricular septum is described using the following algorithm: determination of the tricuspid/mitral annular ratio, right‐sided catheter‐based intervention, and a combination of transthoracic echocardiography and angiography.     

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.     

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.     

Congenital pulmonary atresia with tricuspid insufficiency: Morphologic study

In an anatomic study of 21 cases of pulmonary atresia with tricuspid insufficiency (pulmonary atresia with intact ventricular septum, type II), the morphologic features of the tricuspid valve and the right ventricle were found to differ greatly from those seen in pulmonary atresia with tricuspid stenosis (pulmonary atresia with intact ventricular septum, type I). Morphologically, pulmonary atresia with tricuspid insufficiency (type II) has a greater resemblance to Ebstein's disease with pulmonary atresia than to type I pulmonary atresia. The anomaly may be more amenable to surgery than pulmonary atresia with tricuspid stenosis because the right ventricle in the former may be converted into a functional chamber by a valvotomy combined with a shunting procedure and atrial septostomy.     

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.     

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.     

Atrial septal motion in obligatory right to left shunt

An adult patient with a hypoplastic right heart with intact ventricular septum, pulmonary atresia, secundum ASD and a PDA was studied by 2-D echocardiography. A striking flap valve motion of the primum portion of the interatrial septum was observed. This is perhaps the first report of its kind in an adult.     

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.