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[Balloon valvuloplasty of congenital pulmonary valve stenosis].

Percutaneous balloon valvuloplasty can be considered a therapeutic alternative to surgery for pulmonary valve stenosis. In this analysis, our results will be reported with respect to the various balloon sizes as related to the diameter of the valvular ring as well as differing balloon catheters and the presence of other anomalies in addition to pulmonary valve stenosis. Balloon valvuloplasty was carried out in 23 infants and children ranging in age from one month to eleven years and in two adults at the age of 22 and 50 years, respectively, with a systolic pressure gradient across the pulmonary valve of more than 50 mm Hg or, in the presence of pressure gradients between 40 and 50 mm Hg, in association with a right ventricular pressure of at least 60% of that of the systemic pressure. In all patients echocardiography was carried out prior to the procedure to rule out valve dysplasia and Doppler echocardiographic determination of the pressure gradient was performed before and after dilatation. The diameter of the valvular ring was measured echocardiographically as well as from the right ventriculogram and the size of the balloon chosen accordingly. After diagnostic catheterization, the balloon catheter was advanced to the pulmonary valve with a guidewire. Under fluoroscopy, the balloon was completely inflated then the contrast medium immediately withdrawn, a procedure of seven to 23 seconds with the single-balloon catheter and five to nine seconds with the trefoil catheter. At the first dilatation, the larger portion of the balloon was advanced to the main stem of the pulmonary artery and was placed proximally for subsequent dilatations.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

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↗

Total percutaneous correction of a tetralogy of Fallot variant with dominant pulmonary valve stenosis.

OBJECTIVE: To study the feasibility, efficacy, and safety of total percutaneous correction of a tetralogy of Fallot variant with dominant pulmonary valve stenosis. DESIGN: Percutaneous correction of a variant of tetralogy of Fallot with dominant pulmonary valve stenosis, on the basis that there are transcatheter methods for the correction of malalignment-type ventricular septal defect (VSD) (transcatheter patch) and valvar pulmonary stenosis (balloon valvoplasty). PATIENTS: Two patients with tetralogy of Fallot, 4 and 7 years old, were admitted for percutaneous correction. Their aortic saturations were 72% and 88%. Both had severe right ventricular outflow obstruction with dominant valvar pulmonary stenosis with total gradients of 120 and 70 mm Hg. Large malalignment subaortic VSDs, 14 and 16 mm in diameter, were present. The first patient had a previous percutaneous correction of a small atrial septal defect and an aortopulmonary collateral. INTERVENTIONS: Balloon valvoplasty was first performed, followed by balloon test occlusion of the VSD and double balloon patch occlusion. Forty eight hours after implantation the supporting balloons were extracted, releasing the patches. RESULTS: Both patients became acyanotic with oxygen saturations of 96%. There was mild residual infundibular stenosis with 40 and 30 mm Hg gradients. Both VSDs were effectively occluded with only trivial residual shunts. One patient developed mild haemolysis, which resolved spontaneously in a few days. Both patients were doing well at six and 12 months' follow up visits. CONCLUSIONS: Total percutaneous correction of the tetralogy of Fallot variants with dominant pulmonary valve stenosis is feasible and successful. Larger clinical trials are required to further assess effectiveness and safety.

Catheterization↗

'Isolated' pulmonary valve stenosis as part of more widespread cardiovascular disease.

In 25 patients aged 6 days to 9 years presenting as 'isolated' pulmonary valve stenosis, histology of the myocardium of right and left ventricles, coronary arteries, and ascending aorta has shown abnormality in one or all these areas. Myocardial necrosis, old and recent, unrelated to coronary occlusion was frequent. Myocardial 'dysplasia' involving both ventricles, and resembling hypertrophic cardiomyopathy (HOCM, ASH) was found in 10 and a relation of this to myocardial injury in the fetus is postulated. Varying degrees of coronary occlusion were frequently seen in both right and left coronary arteries. The histology of the ascending aorta was abnormal showing 'higgledy-piggledy' disorder of smooth muscle components in 12 (48%). In a different series of 53 patients who had pulmonary valvotomy for apparent 'isolated' pulmonary valve stenosis there were 14 with clinical evidence of left ventricular abnormality consistent with the pathological changes described, 2 with the same aortic histological changes, and 2 with macroscopical left ventricular hypertrophy. Two of them developed classical hypertrophic cardiomyopathy years later. It is suggested that when pulmonary valve stenosis presents with a thick tricuspid poorly mobile valve, particularly in infants or in patients with evidence of other congenital stigmata, it may be part of a more widespread cardiovascular abnormality. This should be recognized and considered in the evaluation of surgical patients and late survivors who may show unexpected clinical features.

Aortic Diseases↗

Echocardiographic assessment of isolated pulmonary valve stenosis: which outpatient Doppler gradient has the most clinical validity?

Maximum instantaneous Doppler gradient has been the primary variable used to assess severity of pulmonary valve stenosis. However, recent observations suggest that outpatient maximum Doppler gradient often overstates clinical severity, as measured by catheter peak-to-peak gradient (PPG). This study sought to determine which outpatient Doppler gradient was most predictive of subsequently measured PPG. Ninety patients were included. Outpatient mean Doppler gradient showed the best correlation and agreement with the catheter PPG (r = 0.82, bias = -5 mm Hg). Maximum Doppler gradients, even when corrected for proximal velocity, consistently overestimated the PPG (bias = +21-26 mm Hg). Outpatient mean gradient was most predictive of subsequent PPG and should be used to determine whether to intervene for patients with isolated pulmonary valve stenosis. Use of only the maximum Doppler gradient to assess pulmonary valve stenosis will lead to a systematic overstatement of the severity of the stenosis.

Adolescent↗

Single-stage balloon valvuloplasty for critical pulmonary valve stenosis in the neonate.

Balloon valvuloplasty (BV) in neonates with critical pulmonary valve stenosis (CPVS) is limited by technical considerations, mainly the difficulty of traversing the stenotic valve. To simplify the procedure we used a 4F Cobra Type I catheter to cross the pulmonary valve (PV) without the aid of a guidewire, and performed single-stage BV, using low-profile balloons, in 12 neonates with CPVS. Procedure and total fluoroscopy times were 69 +/- 33 min (42-125 min) and 34 +/- 19 min (20-58 min), respectively. Following BV, right ventricular systolic pressure (RVSP) decreased from 102 +/- 17 mm Hg to 56 +/- 15 mm Hg (p < 0.001); and the ratio of RVSP to aortic systolic pressure decreased from 1.39 +/- 0.22 to 0.73 +/- 0.21 (p < 0.001). No significant complications were observed. BV failed in two patients, who subsequently had surgery. At follow-up (a mean of 19 months), Doppler gradient was 19 +/- 12 mm Hg (0.50 mm Hg). BV was repeated in one patient. We conclude that in neonates with CPVS, the use of the 4F Cobra type I catheter facilitates crossing of the PV and allows performance of BV in a single stage; this enhances safety and time-efficiency, and shortens exposure to radiation.

Cardiac Catheterization↗

[Mild pulmonary valve stenosis: the possible spontaneous cure in the natural history of the defect].

OBJECTIVE: To determine the long-term natural history of patients with mild pulmonary valve stenosis. METHODS: Throughout a 24-year evolutive period (1980 to 2004), 83 patients with pulmonary valve stenosis were evaluated, with 29 of them presenting a mild form of the defect. Of these 29, 13 patients had a long-term follow up. The mean age at the first and last follow-up visits was 34 months (1 month to 15 years) and 10.5 years (3 to 24 years), respectively. In addition to evolutive elements, those regarding clinical, electrocardiographic and echocardiographic aspects were also assessed. RESULTS: All patients remained asymptomatic throughout the study. Regarding the pulmonary valve pressure gradient, 5 (38.4%) presented a decrease, 3 (23%) remained unchanged, 4 (30.7%) presented elimination and 1 (7.6%) presented accentuation to a moderate degree of involvement. The mean initial and final gradient was 24.2 mmHg (15-30) and 13.6 mmHg (0-54), respectively. The initial gradients of the 4 patients who achieved spontaneous cure were 30, 19, 30 and 20 mmHg. The systolic murmur and right ventricular overload had a direct correlation to the gradient pressure degree, subdivided at each 10 mmHg. CONCLUSION: The spontaneous cure of the mild pulmonary valve stenosis is possible to achieve, similarly to other acyanogenic anomalies.

Adolescent↗

"Balloon valvulotomy" of congenital pulmonary valve stenosis with tricuspid valve insufficiency.

The rare congenital anomaly of pulmonary valve stenosis and massive tricuspid valve insufficiency with intact ventricular septum is a lethal condition without reported survival after attempted treatment. In a neonate suffering from this syndrome, the pulmonary valve stenosis was relieved by rupturing the fused valve with a balloon catheter introduced transvenously. The desperate condition of the patient quickly improved after this procedure, with subsequent disappearance of the tricuspid valve incompetence. Balloon rupturing of fused valves at angiography may represent a therapeutic alternative in cases in which surgical valvulotomy is associated with a high mortality.

Cardiac Catheterization↗

Pulmonary valve stenosis and hemophilia A. Report of three cases and discussion of a possible genetic linkage.

We treated three unrelated patients with hemophilia A and congenital pulmonary valve stenosis. In two patients, the occurrence of the cardiac malformation was sporadic and in one familial. The coexistence of hemophilia A and pulmonary valve stenosis might suggest a genetic linkage for both disorders. Review of the literature supports the hypothesis that the inheritance of pulmonary valve stenosis is dominant and X-linked.

Adolescent↗

Success determining factors in percutaneous transluminal balloon valvuloplasty of pulmonary valve stenosis.

Twenty-two percutaneous transluminal balloon valvuloplasty procedures were performed on 21 patients with congenital pulmonary valve stenosis. The peak systolic pressure gradient was immediately reduced from 79.1 +/- 7.4 to 22.2 +/- 1.8 mmHg, (P less than 0.0001) and follow-up cardiac catheterization at 5.3 +/- 0.4 months in 19 patients revealed no further significant change in gradient (23.5 +/- 3.2 mmHg). The best results were obtained when balloons larger than the pulmonary annulus were used, i.e. an immediate residual transvalvular gradient of 22.0 +/- 2.2 mmHg with a balloon/annulus ratio greater than 1, as opposed to 44.2 +/- 5.4 with a balloon/annulus ratio = 1 (P less than 0.001). The angiographically determined cusp thickness of the stenotic pulmonary valves was significantly greater than that of the control group of 24 patients without pulmonary valve stenosis (1.21 +/- 0.09 vs 0.59 +/- 0.02 mmHg, P less than 0.00001). The relationship between this parameter and the residual transvalvular gradient at follow-up was found to be significant (r = 0.77, P less than 0.001). It is concluded that balloon size is a determinant factor in achieving good results with percutaneous balloon valvuloplasty although cusp thickness, a factor to which scant regard has hitherto been paid, also plays a significant role in the residual transvalvular gradient measured at follow-up.

Adolescent↗

Triple-balloon pulmonary valvuloplasty: an advantageous technique for percutaneous repair of pulmonary valve stenosis in the large pediatric and adult patients.

This report describes a new valvuloplasty procedure for the treatment of pulmonary valve stenosis (PVS) and large pulmonary valve annulus (PVA) diameters using a triple-balloon valvuloplasty (TBV) technique. We sought to demonstrate the safety and efficacy of this new technique. Percutaneous balloon pulmonary valvuloplasty (BPV) is the preferred treatment for PVS. BPV in older patients with large PVA remains a challenge, even when the double-balloon valvuloplasty technique is performed. The technique was first attempted in a case where a large single balloon was unsuccessful and double-balloon valvuloplasty met with technical difficulties. Three subsequent patients underwent the procedure safely and successfully. An algorithm is presented to determine balloon size for TBV. The median PVA diameter was 23.9 mm. Balloon diameters ranged from 12 to 18 mm. The median peak right ventricular-to-pulmonary artery systolic pressure gradient was 43.5 mm Hg before valvuloplasty and was reduced to 12 mm Hg following intervention. No major complications were encountered. Percutaneous valvuloplasty using TBV can be performed safely and provides excellent clinical results. This technique offers advantages over single-balloon and double-balloon valvuloplasty techniques, especially in treatment of PVS in large pediatric or adult patients.

Adolescent↗

Percutaneous transluminal balloon valvuloplasty for pulmonary valve stenosis: report on six cases.

Percutaneous transluminal baloon valvuloplasty is currently the treatment of choice for most cases of pulmonary valve stenosis. In the first series of cases performed at Kenyatta National Hospital, six patients aged 4 to 24 years with severe pulmonary valve stenosis and no other associated cardiac lesions were selected for the procedure. Immediately following baloon valvuloplasty, the pressure gradients across pulmonary valve measured by both echo-Doppler technique and cardiac catheterisation dropped very significantly (P < 0.001). Catheterisation peak systolic gradients (psg) dropped from 162.5 +/- 23.7 to 56.5 +/- 19.0 while echo-Doppler pressure gradients dropped from 112.0 +/- 11.9 to 42.8 +/- 16.0. No complications occurred during or after the procedure. This initial short-term experience in our set-up confirms the safety and effectiveness of this procedure. Furthermore, this procedure is much cheaper and technically easier to perform than cardiac surgery.

Adolescent↗

[Simultaneous treatment of pulmonary valve stenosis and patent ductus arterious by interventional catheterization].

The association of pulmonary valve stenosis and patent ductus arteriosus is uncommonly found in clinical practice of pediatric cardiology. The authors describe 2 patients with this association that were successfully treated in the same procedure by interventional cardiology: first case, a 14 month old boy was submitted to percutaneous pulmonary valvoplasty with reduction of systolic gradient between pulmonary artery and right ventricle from 71 to 3 mmHg. The 2.0 mm of diameter and conical shape duct was successfully occluded using a 38-5-5 coil; second case, a 20 month old girl was submitted to percutaneous pulmonary valvoplasty with reduction of systolic gradient between pulmonary artery and right ventricle from 60 to 5 mmHg. An attempt to close the 3.5 mm of diameter and concial shape duct using coil was performed, but the device embolized into the pulmonary artery. After immediate retrieval, a 12 mm Rashkind umbrella was implanted. Indication of treatment of both conditions, technical aspects and complications related to the procedures, and patients follow up are also discussed.

Cardiac Catheterization↗

Balloon pulmonary valvuloplasty for pulmonary valve stenosis with atrial septal defect.

We performed successful balloon pulmonary valvuloplasty (BPV) in 6 patients with pulmonary valve stenosis and atrial septal defect (ASD) accompanied by atrial left-to-right shunt without an increase of shunting immediately after BPV. It suggests that such patients should be treated by BPV initially and the need for ASD repair can be assessed during long-term follow-up.

Catheterization↗

Balloon angioplasty in the treatment of pulmonary valve stenosis and coarctation of the aorta.

When balloon angioplasty of the pulmonary valve was attempted in 42 children, aged 1 day to 19 years (mean, 3.1 years), immediate relief of pulmonary stenosis was achieved in 37 cases. In two groups, angioplasty was not uniformly successful: these included (1) two neonates and (2) three out of seven children with dysplastic pulmonary valves, who had an unsuccessful early result. Long-term (>1-year) follow-up of 28 successful cases supports the contention that balloon angioplasty provides effective long-term relief of pulmonary valve stenosis and that it should be considered the treatment of choice for this lesion. The results of angioplasty were also studied in 36 children who underwent this procedure to treat coarctation of the aorta (involving 21 native and 15 recurrent lesions). Angioplasty effectively relieved the obstruction in 30 cases (17 native and 13 recurrent lesions). In the six unsuccessful cases, poor hemodynamic results were due to age (three failures occurred in children less than 2 years old), tubular hypoplasia of the aorta (in one case), and the use of an undersized angioplasty balloon (in four cases, the diameter of the balloon was > or =3 mm less than that of the aortic isthmus). The 36 patients had two major complications: A cerebral vascular accident occurred in one case, and a late aortic aneurysm developed in another. Our results indicate that angioplasty is an effective form of therapy for most cases of coarctation of the aorta (whether native or recurrent).

Journal Article↗

[Application of doppler ultrasound in diagnosis of aortic and pulmonary valve stenosis].

120 children (58 boys, 62 girls) aged 2 days-12 years (mean 3.5 +/- 3 years) with pulmonary or aortic valve stenosis underwent the study. Diagnosis was proved by a cardiac catheterization and angiography. There were determinated maximal flow velocities and pressure gradients through stenosed valve using the doppler ultrasound method. Values were compared with those obtained from cardiac catheterization. There were no statistically significant differences between these two methods. Increased blood flow velocities through the aortic or pulmonary valves were stated in the examined group comparing with healthy individuals. Obtained results prove Doppler method usefulness in diagnosis of aortic and pulmonary valve stenosis. In pulmonary valvular and subvalvular stenosis the Doppler method is useful for estimation of outflow tract obstruction while in a extreme case a results can be incompetent.

Aortic Valve Stenosis↗

[Pulmonary valve stenosis. The medical solution].

Pulmonary valvular stenosis had been diagnosed since infancy in a 3 year-old girl. Cyanotic episodes occurring when the child cried led to the decision to remove the stenosis, whose gradient was 58 mmHg. Transluminal dilatation through the femoral vein, after premedication and local anesthesia induced a decrease of the gradient to 18 mmHg with a good angiographic result. This technique, which may be used in numerous types of pulmonary stenosis, allowed avoidance of the disadvantages of surgery under general anesthesia with median sternotomy and cardiopulmonary by-pass.

Angioplasty, Balloon↗