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.     

Determinants of successful balloon valvotomy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum

Objectives. This study reviewed our experience with percutaneous balloon valvotomy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum and defined the anatomic and hemodynamic characteristics of infants in whom this procedure is successful and provides definitive therapy.Background. Unlike children with valvular pulmonary stenosis, the follow-up of infants with critical pulmonary stenosis undergoing percutaneous balloon valvotomy is limited.Methods. Between December 1987 and August 1992, percutaneous balloon valvotomy was attempted in 12 infants with critical pulmonary stenosis (n = 10) or pulmonary atresia with intact ventricular septum (n = 2). Two outcome groups were identified: Group A patients are acyanotic, have mild residual pulmonary stenosis and have not required operation; Group B patients have required operation.Results. Of the 12 infants, 11 had a successful balloon valvotomy procedure. Group A patients (n = 7) have a residual gradient of 22 ± 18.7 mm Hg (mean ± SD) at follow-up of 3.2 years (range 1.2 to 5.0). In Group B (n = 5), operation was required for inability to cross the pulmonary valve (n = 1) or persistent severe hypoxemia for ⪰2 weeks after valvotomy (n = 4). Significant differences (p ≤0.01) between the two groups (Group A vs. Group B) were identified in pulmonary valve annulus (Z value) 8.1 mm (−1.1) versus 5.5 mm (−3.4); tricuspid valve annulus (Z value) 14.0 mm (0.8) versus 8.8 mm (−1.8); right ventricular volume 65 versus 29 ml/m²; and Lewis index 10.9 versus 8.9.Conclusions. Percutaneous balloon valvotomy is effective and likely to provide definitive therapy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum who have a tricuspid valve annulus >11 mm, pulmonary valve annulus ⪰7 mm and right ventricular volume >30 ml/m².     

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-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 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.     

新生儿及婴儿危重型肺动脉狭窄及肺动脉膜性闭锁的外科治疗

目的:总结我科1997年4月至2007年8月对新生儿及婴儿室间隔完整型肺动脉闭锁及危重型肺动脉瓣狭窄的手术治疗经验。方法:手术治疗共23例,年龄6d~11个月。其中室间隔完整型肺动脉膜性闭锁10例,危重型肺动脉瓣狭窄13例。19例经胸正中切口体外循环下心脏不停跳完成手术,4例应用左胸后外侧切口非体外循环方法。除早期1例同期行动脉导管未闭(PDA)结扎及卵圆孔未闭(PFO)缝合外,其余22例均采用保留PDA、单纯切开肺动脉瓣的方法。结果:围手术期死亡2例,分别死于低氧血症及急性肾功能衰竭。术后当日超声心动图测肺动脉跨瓣压差为37~132mmHg(1mmHg=0.133kPa),平均61mmHg。2周后复查示肺动脉跨瓣压差为26~77mmHg,平均43mmHg,较术后早期明显降低(P<0.05)。出院前不吸氧下测动脉血氧饱和度78%~92%,平均85%,较术前明显增高(P<0.05)。随访4个月至10年,平均5.8年。PDA均闭合,肺动脉血流通畅,三尖瓣返流消失或明显减轻。结论:保留动脉导管、单纯肺动脉瓣切开术对于治疗新生儿及婴儿室间隔完整型肺动脉膜性闭锁及危重型肺动脉瓣狭窄是一种安全有效的方法。     

Percutaneous laser valvotomy with balloon dilatation of the pulmonary valve as primary treatment for pulmonary atresia.

A neonate with pulmonary atresia and an intact ventricular septum with a tripartite right ventricle was successfully treated by percutaneous balloon dilatation of the pulmonary valve. This was facilitated by previous laser valvotomy with a hot tip Trimedyne laser wire. There were no major complications. Four weeks later the patient was discharged home on no medication with peripheral oxygen saturations of 70% in air.     

Percutaneous laser valvotomy with balloon dilatation of the pulmonary valve as primary treatment for pulmonary atresia.

A neonate with pulmonary atresia and an intact ventricular septum with a tripartite right ventricle was successfully treated by percutaneous balloon dilatation of the pulmonary valve. This was facilitated by previous laser valvotomy with a hot tip Trimedyne laser wire. There were no major complications. Four weeks later the patient was discharged home on no medication with peripheral oxygen saturations of 70% in air.     

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.     

Evolving management for critical pulmonary stenosis in neonates and young infants

Over the years, management of critical pulmonary stenosis in young infants has evolved from surgical reconstruction of the right ventricular outflow tract and closed pulmonary valvotomy to transcatheter balloon valvoplasty. Our study aimed at evaluating how the changing policy for management had affected the immediate and long term outcomes of babies with this cardiac lesion. Interventions were made in 34 infants at a median age of 8.5 days (2-90 days). Reconstruction of the right ventricular outflow tract reconstruction was performed in 10 patients, closed pulmonary valvotomy in 13, and balloon valvoplasty in 11. Initial procedure-related mortality was 50%, 15% and 0% respectively. Multivariate analysis revealed transannular patching of the right ventricular outflow tract, and male sex, to be significant factors for death. For the 27 survivors, the ratio of right ventricular to systemic systolic pressure decreased from 1.6 +/- 0.3 to 0.3 +/- 0.2 after reconstruction of the outflow tract, 1.8 +/- 0.5 to 0.8 +/- 0.4 after closed valvotomy, and 1.8 +/- 0.6 to 0.9 +/- 0.3 after balloon valvoplasty. The decrease was significantly greater after patch reconstruction (p=0.025) that required no further reinterventions. The overall rate of reintervention for the survivors was 37% (10/27). The freedom from reintervention after closed valvotomy was 82%, 64% and 51% at 1, 5 and 10 years respectively. The figure remained at 78% at both 1 and 5 years (p=0.66) after balloon valvoplasty. The higher reintervention rate for closed valvotomy corresponded to the significantly greater residual gradient across the pulmonary valve noted on follow-up (p=0.01). Reinterventions included balloon dilation (n=6), reconstruction of the outflow tract (n=4), and 1 each of ligation of an arterial duct and systemic-pulmonary arterial shunting. The risk factor for reintervention was a hypoplastic right ventricle. In conclusion, transcatheter balloon valvoplasty appears to be the optimum initial approach in view of its low mortality, efficacy at relieving the obstruction, and low rate of reintervention.     

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.     

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.     

单纯肺动脉瓣疏通术初期治疗危重婴幼儿室间隔完整型肺动脉闭锁

目的:探讨低龄危重婴幼儿室间隔完整型肺动脉闭锁,及危重肺动脉瓣狭窄的初期外科治疗方法。方法:2009年6月至2013年6月,我院共对10例室间隔完整型肺动脉闭锁及2例危重肺动脉瓣狭窄婴幼儿患者,施行经胸正中切口非体外循环下,单纯肺动脉瓣疏通术(改良Brock术)治疗,术中保留动脉导管未闭,三尖瓣反流未做处理。患儿年龄2~9个月,体质量4.3~10kg,平均6.4kg。所有患者均合并动脉导管未闭及房间隔缺损或者卵圆孔未闭,术前患者末梢血氧饱和度62%~73%,平均69%。结果:本组围术期死亡1例(8.3%),死亡原因为术后持续低氧血症,其余11例术后均缺氧状态改善,经皮血氧饱和度上升至84%~93%,平均89%。术后测得肺动脉瓣跨瓣压差为11~28mmHg(1mmHg=0.133kPa),平均15.6mmHg,术后随访11例,PDA均闭合,三尖瓣反流由术前大量转为少量或者消失。结论:作为危重婴幼儿的初期治疗,对于发绀严重,生长发育差,不能耐受体外循环的危重室间隔完整型肺动脉闭锁及危重肺动脉瓣狭窄患儿,Brock术可获得满意的治疗效果。     

Technique of percutaneous laser-assisted valve dilatation for valvar atresia in congenital heart disease.

OBJECTIVE--To investigate the efficacy and safety of transcatheter laser-assisted valve dilatation for atretic valves in children with congenital heart disease. DESIGN--Prospective clinical study. SETTING--Supraregional paediatric cardiology centre. SUBJECTS--Eleven children (aged 1 day-11 years; weight 2.1-35.7 kg) with atresia of pulmonary (10) or tricuspid (one) valve underwent attempted laser-assisted valve dilatation as part of the staged treatment of their cyanotic heart disease. INTERVENTION--After delineating the atretic valve by angiography and/or echocardiography a 0.018 inch "hot tip" laser wire was used to perforate the atretic valve. Subsequently the valve was dilated with conventional balloon dilatation catheters up to the valve annulus diameter. RESULTS--Laser-assisted valve dilatation was successfully accomplished in nine children. In two neonates with pulmonary valve atresia, intact ventricular septum, and coexistent infundibular atresia the procedure resulted in cardiac tamponade: one died immediately and one later at surgery. During a follow up of 1-17 months (mean 11) two infants with pulmonary valve atresia and intact ventricular septum died (one with congestive cardiac failure). The remainder are either well palliated and do not require further procedures (three), or are awaiting further transcatheter or surgical procedures because of associated defects (four). CONCLUSIONS--Laser-assisted valve dilatation is a promising adjunct to surgery in this high risk group of patients. It may avoid surgery in some patients, and may reduce the number of surgical procedures in those requiring staged operations.     

经皮球囊肺动脉瓣成形术治疗婴儿重度肺动脉瓣狭窄及室间隔完整的肺动脉瓣闭锁

目的:总结经皮球囊肺动脉瓣成形术(percutaneous balloon pulmonary valvuloplasty,PBPV)治疗婴儿重度肺动脉瓣狭窄(pulmonary stenosis,PS)及室间隔完整的肺动脉瓣闭锁(pulmonary atresiawith intact ventricular septum,PA/IVS)的经验,评价其疗效及安全性。方法:自2007年1月至2012年10月,采用PBPV治疗婴儿重度PS及PA/IVS患者共33例,男性24例,女性9例,手术年龄3～12(8.9±2.9)个月,体质量5～11.5(9.0±1.6)kg。术前完善心电图、X线片、超声心动图、右心导管检查及右心室造影,采用单球囊扩张完成PBPV。PA/IVS患儿需先行肺动脉瓣射频打孔术。结果:球囊扩张手术技术成功率为96.97%(32/33)。右心室收缩压(right ventricular systolic piessure,RVSP)由术前的95～205(130.8±28.2)mmHg(1 mmHg=0.133kPa)下降至28～135(73.2±27.4)mmHg(t=12.067,P<0.001);肺动脉瓣跨瓣压差由术前的81～180(110.3±26.3)mmHg下降至10～112(47.7±23.8)mmHg(t=12.958,P<0.001)。1例术中出现心脏压塞,转外科急诊手术,术后恢复良好。出院时10例患者复查超声心动图仍显示平均跨肺动脉瓣压差>50 mmHg;随访中值时间26个月,3例残余重度肺动脉瓣狭窄,2例接受二次PBPV后压差降至轻度。本组术后发生轻度以上肺动脉瓣关闭不全(pulmonary insufficiency,PI)22例。结论:随着介入技术的不断提高,经皮肺动脉瓣球囊扩张术,已成为救治婴儿危重先天性肺动脉瓣狭窄及肺动脉瓣闭锁的安全有效的重要方法。     

Outcomes of transcatheter valvotomy in patients with pulmonary atresia and intact ventricular septum

The results of transcatheter valvotomy in pulmonary atresia with intact ventricular septum (PA-IVS) patients are presented with an attempt to identify the predictive factors for pulmonary valvotomy alone as definitive treatment. Between June 1995 and December 1997, 14 PA-IVS neonates with tripartite right ventricle underwent an attempted pulmonary valvotomy. For perforation of the pulmonary valve, a guidewire was used in 4, and a radiofrequency guidewire in 10 patients. Two outcome groups were identified. Group I included those in whom transcatheter treatment achieved a definitive success; group II patients required surgery despite an initial successful valvotomy. The attempt failed in 3 patients, 1 of whom had pericardial effusion. Perforation of the pulmonary valve was achieved in 11 patients: 2 with a guidewire and 9 with a radiofrequency guidewire. A subsequent balloon valvuloplasty was performed in these 11 patients. After valvuloplasty, mean right ventricular pressure decreased from 124 +/- 24 to 60 +/- 15 mm Hg (p <0.01). One died of heart failure and infection 10 days later, despite successful weaning from prostaglandin E1. Group I patients (n = 6) were treated with transcatheter valvotomy alone. Group II patients (n = 4) required right ventricular outflow patch. Significant differences between the 2 groups (group I vs II) were identified in tricuspid valve Z value (0.52 +/- 0.37 vs -1.25 +/- 0.48, p <0.05), pulmonary valve Z value (-3.47 +/- 0.59 vs -5.43 +/- 0.94, p <0.05), and ratio of right-to-left ventricular area on the apical 4-chamber view (0.73 +/- 0.06 vs 0.49 +/- 0.03, p <0.05). There were no significant differences in hemodynamic characteristics between the 2 groups. After a follow-up period ranging from 7 to 35 months (mean 18 +/- 10.3), the most recent echocardiograms in the 10 patients showed a mean pressure gradient across the pulmonary valve of 17 +/- 15 mm Hg. All 10 patients had an oxygen saturation of >92%. Transcatheter valvotomy using a radiofrequency guidewire is a safe and effective treatment in selected patients with PA-IVS. Transcatheter valvotomy can be a definitive treatment in PA-IVS patients with a tricuspid valve Z value > or = -0.1, pulmonary valve Z value > or = -4.1 and ratio of right-to-left ventricular area > or = 0.65.     

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.     

Surgical treatment of pulmonary atresia with intact ventricular septum.

Fifty-nine children with pulmonary atresia and intact ventricular septum underwent various forms of surgical treatment at the Hospital for Sick Children, Toronto, during 1950 to mid 1975. Twenty-three patients had pulmonary valvotomy, 15 direct, 2 indirect, and 6 both direct valvotomy and infundibulectomy. All died, 19 early and 4 late. Of 13 patients who received a systemic-pulmonary artery shunt, 4 combined with surgical atrial septectomy, there are only 2 long-term survivors both of whom were children who had had a Waterston anastomosis. Recently we have been treating infants with small right ventricles with balloon atrial septostomy at cardiac catherterization followed by a Potts anastomosis and pulmonary valvotomy. If the Potts anastomosis appears satisfactory the persistent ductus arteriosus is ligated. This scheme was used in 23 infants, with 4 early deaths and 2 late deaths. Of 17 survivors, further shunts were required in 4 children. One child has had a formal repair, with insertion of valves in both tricuspid and pulmonary areas. We believe that this operative combination of Potts anastomosis and pulmonary valvotomy offers the infant with pulmonary atresia and a small right ventricle a relatively low initial mortality and the possibility of right ventricular enlargement and subsequent repair.     

Longevity of Neonatal Ductal Stenting for Congenital Heart Diseases with Duct‐dependent Pulmonary Circulation

Introduction. Ductal stent (DS) in duct‐dependent pulmonary circulation is less morbid than neonatal Blalock–Taussig shunt. However, there is concern if DS provides an adequately long palliation before definitive repair. Methods. This is a retrospective review of clinical follow‐up of all consecutive infants after successful DS performed by a single operator. They were divided into three anatomic groups. Group A neonates had balloon valvotomy for critical pulmonary stenosis or pulmonary atresia with intact ventricular septum, who needed DS patency until the right ventricle was adequate to provide antegrade pulmonary flows. Group B patients with tetralogy of Fallot and pulmonary atresia suited for later biventricular repair needed ductal patency until conduit surgery was completed. Group C patients with functionally univentricular hearts needed DS patency until bidirectional Glenn shunt completion. Results. Among 22 infants, four Group A patients followed for 26–54 months after balloon pulmonary valvotomy had adequate oxygen saturation and needed only short‐term DS patency. In six out of nine Group B patients, corrective biventricular repair using conduits was performed after 5–14 months at a body weight of 5–7.5 kg. Bidirectional Glenn shunt and confluence repair were performed in seven of nine Group C patients weighing 6–8.5 kg after 8–15 months. The hilar pulmonary artery growth in B and C groups was adequate for surgical repair. No patient needed stent redilatations or additional shunts on follow‐up for hypoxia. Four patients had sudden death. Conclusions. The short‐term patency of DS was adequate after balloon valvotomy for critical pulmonary stenosis or pulmonary atresia with intact ventricular septum. Duration of palliation by DS was also sufficient in univentricular hearts to allow adequate somatic growth before Glenn surgery. In patients with biventricular anatomy treated by DS, conduit repair had to be performed at a relatively early age. Interstage mortality was 18%.     

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.