Relief Of Pulmonary Valve Stenosis Research Articles

Analysis of congenital heart defects in 87 consecutive patients with Brachmann‐de Lange syndrome

Congenital heart defects (CHDs) have been estimated to occur in approximately 20% of patients with Brachmann-de Lange syndrome (BDLS, also known as Cornelia de Lange syndrome, OMIM 122470). We report on the results of a prospective echocardiographic evaluation of a cohort of 87 Italian BDLS patients with longitudinal follow-up from 5 to 12 years. A cardiac anomaly was identified in 29/87 (33.3%) including 28 (32.2%) patients with a structural CHD, and an additional patient (1.2%) with isolated non-obstructive hypertrophic cardiomyopathy (HCM). Of the 28 patients with a CHD, 12 (42.9%) had an isolated obstructive CHD, 10 of which were pulmonary stenosis (36%), 8 (28.6%) had an isolated left to right shunt, and the remainder showed a combination of structural anomalies. Overall incidence of pulmonary stenosis was 39% (11/28). Isolated late-onset mitral or tricuspid valve dysplasia, albeit hemodynamically insignificant, was detected at follow-up examination in 4 (14.3%) patients older than 10 years, previously known to be normal. In contrast to previous studies, only two patients required surgery, one for closure of a large perimembranous ventricular septal defect (VSD) and associated ASD closure (1), and another for VSD closure and relief of pulmonary valve stenosis (1). The remainder are receiving medical follow-up. We believe that the overall frequency (33.3%) and evidence of 4 late onset dysplastic valves anomalies justifies both echocardiographic assessment in all BDLS patients at the first diagnostic assessment, and later on during medical follow-up.

Dissecting Aneurysm of the Main Pulmonary Artery

Since the first introduction of pulmonary balloon valvuloplasty by Kan in 1982, the procedure has been used for relief of pulmonary valve stenosis. It is normally recommended that the procedure should be performed for peak-to-peak gradients in excess of 50 mm Hg,1 and the balloon/annulus ratio should be 1.2 to 1.25 for effective and safe results.2 Complications of the procedure are minimal and rare. We present the case of dissecting aneurysm of the main pulmonary artery, a dangerous complication of pulmonary balloon valvuloplasty performed in a child, which was successfully treated with surgical management. A 4-year-old boy was admitted to our hospital in December, 2007, whose history included 2 years of 4/6 systolic murmur heard over the pulmonary artery. The transthoracic echocardiogram showed severe pulmonary valve stenosis, and the Doppler-measured peak instantaneous gradient was 121 mm Hg, which was the indication for pulmonary balloon valvuloplasty. A 6F Mansfield balloon catheter was used (18/30 mm, balloon-to-annulus ratio 1.15). The catheterization-measured peak-to-peak gradient was 125 mm Hg. Manual inflation was performed until the balloon indentation disappeared, and 2 additional inflations were performed. After the inflations, the transvalvular pressure gradient was reduced to 28 mm Hg. The patient was observed in an …

Percutaneous balloon pulmonary valvuloplasty: State of the art

Since the first description of balloon pulmonary valvuloplasty in 1982 by Kan, the procedure has been extensively utilized by several groups of workers for relief of pulmonary valve stenosis. It is generally recommended that the procedure be performed for peak-to-peak gradients in excess of 50 mmHg. The technique involves positioning one or more balloon catheters across the stenotic valve, usually over an extra-stiff guide wire and inflating the balloons with diluted contrast material, thus producing valvotomy. The procedural details are described in this paper. The currently recommended balloon/annulus ratio is 1.2 to 1.25. Immediate reduction of gradient, increase in jet width and free motion of the pulmonary valve leaflets with less doming have been observed following balloon dilatation. Improvement of right ventricular function, tricuspid insufficiency and right-to-left shunt has also occurred. Complication can occur, but are rare and minimal. At mid-term follow-up, both catheterization measured peak-to-peak gradients and Doppler-measured peak instantaneous gradients remain improved for the group as a whole. However, restenosis, defined as gradient >or=50 mmHg, has been observed in nearly 10% of children. Predictors of restenosis include balloon/annulus ratio <1.2 and immediate post-valvuloplasty gradient >or=30 mmHg. Small pulmonary valve annulus, earlier study year and post-surgical complex pulmonary stenosis have also been identified as factors predictive of restenosis. Redilatation with balloons that are larger than those used at the time of initial balloon valvuloplasty produces excellent results and redilatation is the procedure of choice in the management of restenosis after previous balloon pulmonary valvuloplasty. Long-term follow-up results are scanty, but the limited data reveal minimal additional restenosis, event-free rates in mid-80s and mid-70s at 10 and 15 years respectively and significant increase in prevalence of pulmonary insufficiency. Balloon pulmonary valvuloplasty is equally successful in neonates as well as in adult subjects. In conclusion, balloon pulmonary valvuloplasty is the treatment of choice for relief of pulmonary valve stenosis. Use of balloons 1.2 to 1.25 times larger than pulmonary valve annulus may produce optimal results. Life-long follow-up to identify the significance of residual pulmonary insufficiency is indicated.

Comparative Long-Term results of surgery versus balloon valvuloplasty for pulmonary valve stenosis in infants and children

Background We compared the long-term results of surgical valvotomy (S) versus balloon valvuloplasty (BV) for pulmonary valve stenosis in infants and children. Methods Results after surgical pulmonary valvotomy (with concomitant ASD/VSD closure) (n = 62, age 2.9 ± 3.5 years) and balloon valvuloplasty (n = 108, age 3.6 ± 3.9 years) were analyzed. Transvalvular mean pressure gradient decrease, freedom from reintervention for restenosis, pulmonary valve insufficiency, and tricuspid valve insufficiency were considered. Results Mean pressure gradient decreased significantly more in the surgical group (from 64.8 ± 30.8 mm Hg to 12.8 ± 9.8 mm Hg at a mean follow-up of 9.8 years) than after BV (decreasing from 66.2 ± 21.4 mm Hg to 21.5 ± 15.9 mm Hg after a mean of 5.4 years; p < 0.001). Moderate pulmonary valve insufficiency occurred in 44% after surgery, and in 11% after BV ( p < 0.001). Tricuspid valve insufficiency occurred in 2% after surgery, and in 5% after BV. Restenosis occurred in 3 surgical patients (5.6%), 2 patients required reoperation, and 1 patient required a balloon valvotomy. Restenosis developed in 13 BV patients (14.1%): 6 patients were redilated and 7 patients required surgery. Surgical valvotomy led to significantly less reinterventions than balloon valvuloplasty ( p < 0.04). Conclusions Surgical relief of pulmonary valve stenosis produces lower long-term gradients and results in longer freedom from reintervention. Balloon valvuloplasty may remain, despite these results, the preferred therapy for isolated pulmonary valve stenosis, because it is less invasive, less expensive, and requires a shorter hospital stay. Surgery should remain the exclusive form of therapy in the presence of concomitant intracardiac defects, which need to be addressed.

Balloon Pulmonary Valvuloplasty

Since the initial description of balloon pulmonary valvuloplasty in 1982 by Kan and her associates, the procedure has been extensively used by several groups of workers for relief of pulmonary valve stenosis. It is generally recommended that the procedure be performed for peak‐to‐peak gradients in excess of 50 mmHg. Severe and critical obstructions, irrespective of age and pulmonary valve dysplasia, are amenable to balloon dilatation. The technique involves positioning one or more balloon catheters across the stenotic valve, usually over an extra‐stiff guidewire and inflating the balloons, thus producing valvotomy. The recommended balloon:annulus ratio is 1.2 to 1.4. Immediate reduction of gradient, increase in jet width, and free motion of the pulmonary valve leaflets with less doming have been observed following balloon dilatation. Improvement of right ventricular function, tricuspid insufficiency, and right‐to‐left shunt has also occurred. At intermediate‐term follow‐up, both catheterization measured peak‐to‐peak gradients and Doppler‐measured peak instantaneous gradients remain improved for the group as a whole. However, restenosis, defined as gradient ≥ 50 mmHg, has been observed in nearly 10% of children. Predictors of restenosis include balloon:annulus ratio ≥ 1.2 and immediate postvalvuloplasty gradient ≥ 30 mmHg. Small pulmonary valve annulus, earlier study year, and postsurgical complex pulmonary stenosis have also been identified as factors predictive of restenosis. Redilatation with balloons that are larger than those used at the time of initial balloon valvuloplasty produces excellent results, and redilatation is the procedure of choice in the management of restenosis after previous balloon pulmonary valvuloplasty. Regression of right ventricular hypertrophy on ECG following balloon dilatation has been well documented; ECG is a useful adjunct in the evaluation of results of balloon dilatation. However, ECG evidence for hemodynamic improvement does not become apparent until 6 months after balloon dilatation. Doppler gradient is generally reflective of residual obstruction and is a useful and reliable noninvasive monitoring tool. Infundibular gradients occur in nearly 30% of patients: the older the age and higher the severity of obstruction, the higher the prevalence of infundibular reaction. When residual infundibular gradient is ≥ 50 mmHg, beta blockade therapy is generally recommended. The infundibular obstruction regresses to a great degree at follow‐up with the rare patient requiring surgical intervention. While there is some controversy with regard to use of balloon dilatation in patients with dysplastic pulmonary valves, based on our own observations and those of others, the results of balloon valvuloplasty with dysplastic pulmonary valves are comparable to those without. Dysplastic valves did not seem to be responsible for recurrence, and balloons large enough to produce balloon:annulus ratio of 1.4:1.5 may be needed to produce a satisfactory result. Comparison with surgical valvotomy has limitations, but generally there is greater reduction of gradient following surgery, but the degree and frequency of pulmonary insufficiency may be higher following surgery than after balloon therapy. Long‐term follow‐up results are scanty, but the data from our cohort reveal minimal additional restenosis, event‐free rates of 88% and 84% at 5 and 10 years, respectively, and significant increase in the prevalence of pulmonary insufficiency. But, no surgical intervention was warranted for pulmonary insufficiency in any patient following balloon dilatation. In conclusion, balloon pulmonary valvuloplasty is the treatment of choice for relief of pulmonary valve stenosis. Use of balloons 1.2‐1.4 times larger than pulmonary valve annulus may produce optimal results. Ten‐ to 20‐year follow‐up to identify the significance of residual pulmonary insufficiency is indicated.

Balloon pulmonary valvuloplasty for infants with severe tetralogy of Fallot.

Balloon pulmonary valvuloplasty was performed in 3 infants with severe tetralogy of Fallot at days 24, 54 and 86 because of progressive hypoxemia. In two patients, the balloon catheter (4 cm long, 5-8 mm diameter) could not pass through the pulmonary valve. This necessitated a smaller balloon and required a two-step procedure. Initially, a coronary artery balloon (2 cm long, 3.5 mm diameter) was used. Following balloon valvuloplasty, arterial oxygen saturation increased from 63 to 83% in case 1, from 69 to 85% in case 2 and 63 to 86% in case 3. Immediate postvalvuloplasty right ventricular cineangiography revealed that the maximal opening diameter of the pulmonary valve leaflets increased from 1-2 mm to 3-4 mm in cases 1 and 3, and from 2-3 mm to 4-5 mm in case 2. No significant complications occurred. Echocardiographic follow-up data showed that the diameter of the right ventricular outflow tract and pulmonary arteries increased with age. The present results show that the pulmonary valvuloplasty is an effective procedure for relief of pulmonary valve stenosis in tetralogy of Fallot and to improve oxygenation and growth of the pulmonary arteries and right ventricular outflow tract without the need of an immediate aortopulmonary shunt.

Hemodynamic evaluation and morphological changes of right ventricular out-flow tract and pulmonary valve after balloon pulmonary valvuloplasty.

Balloon pulmonary valvuloplasty (BPV) was attempted in 38 cases of congenital pulmonary valve stenosis. It was effective and was done without complication in 36 cases, however it was not effective in two cases of pulmonary valve dysplasia. The balloon used was 20-50% larger in diameter than the pulmonary valve annulus. In the seven cases in which the transvalvular pressure gradient was above 100 mmHg on cardiac catheterization, right ventriculography demonstrated that the functional obstruction of the right ventricular outflow tract increased immediately after BPV, however it subsequently improved at one year after the procedure. At between one and three months after BPV, two-dimensional echocardiography demonstrated that the morphological obstruction to the right ventricular outflow tract had diminished. In two cases, localized right ventricular septal hypertrophy with severe pulmonary valve stenosis was observed by two-dimensional echocardiography and right ventriculography, and persisted at one year. In all cases, two-dimensional echocardiography and angiography demonstrated doming pulmonary valves with valve stenosis, which was diminished by BPV. The pulmonary valve morphology was observed by two-dimensional echocardiography in three cases following BPV. In one case, partial relief of pulmonary valve obstruction was seen to be due to commissural splitting and in the other two, to tearing of cusp tissue. Inspection of the pulmonary valve at operation was made in one case who underwent elective surgery for repair of an atrial septal defect which was associated with pulmonary valve stenosis. It demonstrated partial relief of pulmonary valve stenosis by tearing of cusp tissue. In conclusion, BPV is as effective a treatment for congenital pulmonary valve stenosis as open valvulotomy. In our follow-up study, it has shown no apparent complications. The transient obstruction in the right ventricular outflow tract immediately after BPV improved within one month and improved further after three months and one year. However, in the more long-standing cases the localized right ventricular septal hypertrophy persisted and these cases will require, a long term follow-up.

Long-term results of balloon pulmonary valvuloplasty of valvar pulmonic stenosis

The purpose of this paper is to document long-term results of percutaneous balloon pulmonary valvuloplasty. Forty-one patients, aged 7 days to 20 years, underwent pulmonary valvuloplasty over a 3 1 2 -year period ending in April, 1987. Balloon valvuloplasty resulted in immediate reduction in the pulmonary valvar pressure gradient from 92 ± 45 to 30 ± 22 mm Hg ( p < 0.001). Follow-up (3 to 34 months) clinical, ECG, and echo Doppler data were available in 35 patients. Follow-up (6 to 34 months) cardiac catheterization data were available in 29 of the 35 patients. Short ejection systolic murmurs were heard in all 35, but an early diastolic decrescendo murmur was heard in only 12 patients. Based on the catheterization and Doppler data, the patients were divided into two groups: group I (30 patients) with excellent results and group II (five patients) with poor results (gradients > 50 mm Hg). In group I ECG right ventricular hypertrophy regressed. The echocardiographic right ventricular end-diastolic dimension (21 ± 6 vs 15.9 ± 4.6 mm) decreased ( p < 0.001) while the left ventricular dimension increased ( p < 0.02). Peak Doppler flow velocity in the main pulmonary artery fell from 4.0 ± 0.8 m/sec to 2.3 ± 0.5 m/sec ( p < 0.001). Doppler evidence for pulmonary insufficiency was present in 21 patients. Catheterization-determined pulmonary valvar gradients (24 patients) also fell from 95.6 ± 50.3 mm Hg to 18.3 ± 12.5 mm Hg ( p < 0.001). By contrast, in group II ECG, echocardiographic, and Doppler data and pulmonary valvar gradient at catheterization did not change significantly ( p > 0.1) but improved ( p < 0.01) following repeat balloon valvuloplasty. When the effects of balloon size on the results of valvuloplasty were scrutinized, balloons 1.2 to 1.4 times the size of the pulmonary valve anulus appear to produce sustained relief of pulmonary valve stenosis. These long-term results lead us to recommend balloon pulmonary valvuloplasty as a therapeutic procedure of choice for management of moderate to severe pulmonary valve stenosis.

Percutaneous balloon valvuloplasty for pulmonary valve stenosis in infants and children.

Pulmonary valve stenosis was relieved by balloon dilatation during cardiac catheterisation on 27 occasions in 23 infants and children aged 7 days to 12 years, median 31 months (three aged less than 2 weeks). Pulmonary valve diameter was estimated by cross sectional echocardiography to assist in the choice of balloon size. Before dilatation the right ventricular systolic pressure ranged from 41 to 190 (median 92) mm Hg and was suprasystemic in 10 patients. There were significant reductions in the ratio of right ventricular to systemic systolic pressure and pulmonary systolic pressure gradients immediately after balloon dilatation. Twelve patients underwent recatheterisation (11 at six months and one at one week after balloon dilatation), which showed further improvement with significant reductions in right ventricular pressure or pulmonary valve gradient or both, particularly in the patients with the least satisfactory initial results. This improvement was attributed to resolution of the obstruction at infundibular level. Repeat pulmonary valve dilatation was successfully performed in four patients who had poor results after initial dilatation. Balloon pulmonary valvotomy appears to provide good short term and medium term relief of pulmonary valve stenosis and may obviate the need for surgery in many cases. An apparently poor immediate haemodynamic result does not preclude a good longer term result.

Normothermic caval inflow occlusion: Application to operations for congenital heart disease

The technique of normothermic caval inflow occlusion was introduced primarily for relief of pulmonary valve stenosis and continues to be used for this purpose with excellent results. However, the technique may also be applied to a number of other lesions such as aortic valve stenosis in neonates and infants, for placement of outflow patches or valve excision for pulmonary atresia with intact ventricular septum, for atrial septectomy in children with restrictive atrial septum, and for other conditions wherein a period of up to 2 minutes of intracardiac exposure is sufficient. This report examines the early and long-term results in 140 children who underwent normothermic caval inflow occlusion at The Children's Hospital, Boston, Massachusetts, over the past 11 years. Ninety-four children underwent pulmonary valvotomy (early mortality 0%), 21 had aortic valvotomy (19%), 10 with pulmonary atresia and intact ventricular septum underwent various procedures (50%), 11 had atrial septectomy (9%), and there were four miscellaneous procedures (50%). Apart from avoiding many of the potential complications of cardiopulmonary bypass, inflow occlusion has provided as good or better short-term and long-term results as those obtained with cardiopulmonary bypass, particularly in infants and neonates, and also has proved more cost effective.