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A case of congenital left ventricular diverticulum with pulmonary stenosis and its scintigraphic characteristics.

We encountered a 31-year-old female patient with mild valvular pulmonary stenosis who had no abnormality in the electrocardiogram but pulmonary dilatation in the chest radiograph. Two-dimensional echocardiography and magnetic resonance imaging demonstrated two small protrusions at the interventricular septum indicating diverticula. Large perfusion defects were observed at the anterior wall in the thallium-201 myocardial tomograms. Short axial and vertical long axial images by ECG-gated blood pool tomography revealed an out-pouching best seen during diastole and a good contraction during systole in the corresponding areas. These findings suggested the presence of thin but normal myocardium in the anterior wall, i.e. a muscular type of left ventricular diverticulum. The presence of the muscular type of left ventricular diverticulum at the anterior and septal walls was confirmed by contrast left ventriculography. A congenital diverticulum at the anterior and septal walls with pulmonary stenosis is very rare. Furthermore, its scintigraphic images were quite characteristic and useful for its diagnosis.

Adult↗

[Percutaneous transluminal valvuloplasty in pulmonary stenosis. Apropos of 24 cases].

Twenty-four patients with moderate or severe valvular pulmonary stenosis were treated by percutaneous transluminal valvuloplasty (PTV). The age of the patients ranged from 24 hours to 27 years. Eighteen patients (group I) had a right ventricle-pulmonary artery (RV-PA) pressure gradient greater than 50 mmHg (measured simultaneously or during catheter withdrawal). Six patients (group II) had RV-PA pre-sure gradients of less than 50 mmHg. Twenty-seven PTV procedures were performed. Six of the 24 patients were under 2 months of age, including 4 who were less than 1 week old. The youngest patient (under 24 hours old) had valvular pulmonary stenosis, very nearly atresia. PTV was carried out using Rashkind's technique on 2 occasions and with a dilatation catheter with a balloon of 6 to 20 mm of diameter on the other 25 occasions. In 6 cases, 2 dilatation catheters were introduced and inflated simultaneously in the pulmonary valve. The overall results were satisfactory: RV pressure fell from 87.6 to 54.4 mmHg, the RV-PA gradient from 72.3 to 31.9 mmHg and the ratio of RV/systemic pressure from 104.4 to 51.1. The improvement was more marked in group I than in group II. The causes of failure are discussed: valvular dysplasia, severe infundibular hypertrophy, inadequate diameter of the balloon. Tolerance of PTV was very good. The value of this procedure is emphasised. It may save surgical valvotomy in high risk groups of patients, such as the newborn and infants. The haemodynamic results of PTV are maintained at long-term, but the number of cases is still too small.

Adolescent↗

[A case of supravalvular pulmonary stenosis with atrial septal defect].

We reported a case of supravalvular pulmonary stenosis with atrial septal defect (ASD) in 37-year-old man. Extended pulmonary arterioplasty with the technique of Doty's operation for supravalvular aortic stenosis was carried out with bovine pericardial patch. Pressure gradient between the right ventricle and the pulmonary artery reduced from 90 to 20 mmHg after this operation. Doty's technique could be modified and successfully applicable to supravalvular pulmonary stenosis.

Adult↗

Pulmonary stenosis and severe biventricular dysfunction: improvement following percutaneous valvuloplasty.

A 15-year-old boy with severe pulmonary stenosis associated with severe right and left ventricular systolic dysfunction is reported. After successful percutaneous pulmonary valvuloplasty, there was an initial and early improvement in right ventricular (RV) function, followed by a delayed and more gradual improvement in left ventricular (LV) function. At long-term follow up, both RV and LV systolic functions were nearly normalized. Several mechanisms may be implicated, including ventricular interdependence, geometric factors, altered compliance and intrinsic alteration in the LV muscle. A delayed, but sustained, improvement in LV systolic function following relief of RV pressure overload suggests that the latter mechanism must have played an important role in the genesis of the LV dysfunction. Pulmonary stenosis associated with severe biventricular dysfunction may be treated primarily by percutaneous pulmonary balloon valvuloplasty with near-total recovery of the ventricular function.

Adolescent↗

SUPRAVALVULAR PULMONARY STENOSIS IN LEVO TRANSPOSITION AND "SINGLE" VENTRICLE.

A case of supravalvular and valvular pulmonary stenosis in an L-transposition of the great arteries and "single" or double inlet left ventricle is described. Supravalvular pulmonary stenosis has not heretofore been reported in transposition of the great arteries. The clinical, hemodynamic and angiographic features are described. Surgical correction has many problems.

Journal Article↗

Results of balloon valvuloplasty in the treatment of congenital valvar pulmonary stenosis in children.

Transluminal balloon valvuloplasty was used in the treatment of congenital valvar pulmonary stenosis in 19 children, aged 5 months to 18 years. The right ventricular (RV) systolic pressure and RV outflow tract gradient decreased significantly immediately after the procedure (95 +/- 29 vs 59 +/- 14 mm Hg, p less than 0.01, and 78 +/- 27 vs 38 +/- 13 mm Hg, p less than 0.01). Seven of these patients were evaluated at cardiac catheterization 1 year after balloon valvuloplasty. No significant change occurred in RV systolic pressure or RV outflow tract gradient at follow-up evaluation compared with measurements immediately after balloon valvuloplasty (60 +/- 5 mm Hg vs 56 +/- 12 mm Hg and 39 +/- 5 vs 38 +/- 10 mm Hg). In addition, follow-up evaluation was performed using noninvasive methods and included electrocardiography (n = 13), vectorcardiography (n = 11) and Doppler echocardiography (n = 11) Doppler echocardiography in 11 patients 15 +/- 9 months after balloon valvuloplasty showed a continued beneficial effect with a mild further decrease in RV outflow tract gradient. Thus, balloon valvuloplasty is effective in the relief of pulmonary stenosis.

Adolescent↗

[Percutaneous valvuloplasty of congenital pulmonary stenosis in adults over 50 years of age].

The authors report three cases of congenital pulmonary stenosis in adults over 50 years of age treated by percutaneous balloon valvuloplasty. Three symptomatic women aged 74, 80 and 51, had systolic pressure gradients ranging from 107 to 113 mmHg between the right ventricle and pulmonary artery. After valvuloplasty with two balloons or one trefoil balloon, the transvalvular pressure gradient fell to 25 to 30 mmHg. It was only 14 mmHg in one patient controlled after one year's follow-up. The cardiac index was initially decreased and did not change very much immediately after the procedure, increasing from 1.68 1/m2/mn to 1.77 1/m2/mn. The pulmonary valve surface area increased from 0.22 to 0.43 cm2. There were no complications and in one patient, reviewed two years later, the clinical improvement was maintained. Percutaneous valvuloplasty is indicated in severe and/or poorly tolerated pulmonary stenosis. With the 10 other previously reported cases of patients over 50 years of age, the procedure was successful in 12 out of 13 patients (92%). In these patients of 51 to 80 years of age, the systolic pressure gradient between the right ventricle and pulmonary artery was reduced from 112 +/- 46 mmHg to 43 +/- 26 mmHg (-62%). Slight pulmonary regurgitation appeared in 5 out of 9 cases. Valvuloplasty was usually well tolerated and there were no fatalities. There were no signs of restenosis in 5 cases controlled 10 days to 1 year after dilatation. In the future, systematic Doppler echocardiographic examinations should help comparison of cardiac haemodynamics before, immediately after valvuloplasty and at long-term.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

[Balloon dilatation of the pulmonary valve within the first 40 days of life in critical valvular pulmonary stenosis, Fallot's tetralogy and following surgical or interventional high-frequency opening of pulmonary atresia].

A balloon-valvuloplasty of the pulmonary valve (BVP) was carried out in 14 patients within the first 40 days of life. Six infants had a critical valvular pulmonary stenosis with intact ventricular septum (group S), 5 infants had a pulmonary atresia with intact ventricular septum (group A), and 3 infants had a tetralogy of Fallot (group F). In 4 of the 5 patients of group A the BVP was preceded by operative opening of the pulmonary valve. In one case radiofrequency perforation of the valve during the first diagnostic cardiac catheterization could be carried out successfully. The average value of the ratio of the balloon diameter to the diameter of the pulmonary valve was 1.31 +/- 0.20. The systolic pressure in the right ventricle dropped 51 mm Hg (from 106 +/- 32 mm Hg to 55 +/- 9 mm Hg) in group S and 33 mm Hg (from 101 +/- 27 mm Hg to 68 +/- 19 mm Hg) in group A. In group F the arterial oxygen saturation increased 15%, however the pressure of the right ventricle was, after the BVP, slightly higher than before. The average residual gradients after BVP were in group S 28 +/- 15 mm Hg, in group A 34 +/- 12 mm Hg and in group F 48 +/- 18 mm Hg.(ABSTRACT TRUNCATED AT 250 WORDS)

Catheter Ablation↗

[Pulmonary stenosis in adults. Report of a clinical case].

The authors report a case of valvular pulmonary stenosis in a 60 years old patient, admitted to hospital with heart failure and angor pectoris. Four years previously a VVI pacemaker had been implanted for complete heart block. At the time of the admission he had two pacemakers leads in the right ventricle; one of them was retained and functionless since the changing of the generator which occurred 1 year before. The usual complications of endocardial pacemakers are discussed, as well as the natural course and surgery indications for valvular pulmonary stenosis in adults.

Humans↗

Percutaneous balloon valvuloplasty for congenital critical pulmonary stenosis in neonates.

BACKGROUND: Percutaneous balloon valvuloplasty (PBVP) was performed on 6 neonates with severe pulmonary stenosis to evaluate its efficacy. METHODS: The 6 studied neonates were aged from 1 to 30 days and weighed 2.5 to 3.3 kg. Follow-up period ranged 6-21 months with a mean of 11.5 +/- 6.3 months. RESULTS: Dramatic improvement after PBVP was obtained not only on the clinical manifestations but also on the hemodynamic data. After percutaneous balloon valvuloplasty, the aortic O2 saturation increased significantly from 71.3 +/- 14.8% to 83.2 +/- 9.3% (p = 0.001) in 5 neonates who had cyanosis before PBVP. The right to left ventricular peak systolic pressure ratio decreased significantly from 1.65 +/- 0.41 to 0.77 +/- 0.27 (p < 0.001). The peak systolic right ventricular pressure dropped significantly from 104.0 +/- 18.8 to 48.8 +/- 13.6 mmHg (p < 0.001). The peak systolic gradient between right ventricle and main pulmonary artery dropped significantly from 83.2 +/- 16.2 to 24.3 +/- 16.3 mmHg (p < 0.05). CONCLUSIONS: The percutaneous balloon valvuloplasty is safe and effective in treating the neonates with congenital critical pulmonary stenosis.

Catheterization↗

Pulmonary atresia or critical pulmonary stenosis with intact interventricular septum diagnosed in utero: echocardiographic findings and post-natal outcome.

BACKGROUND: Survival of neonates with critical pulmonary obstruction depends on cardiac morphology and function but also on proper perinatal management. METHODS: Postnatal outcome of five neonates with critical pulmonary stenosis or pulmonary atresia detected in utero is reported. RESULTS: Right ventricular morphology and ratio between tricuspid and mitral annulus at Fetal Echocardiography were the most important prognostic parameters. Increasing severity of obstruction and of right ventricular hypoplasia was documented in two fetuses. CONCLUSION: Prenatal diagnosis was useful for planning delivery; we hypothesize that it can be of benefit on postnatal outcome. In selected cases, earlier delivery and relief of obstruction could have beneficial effect on prognosis.

Female↗

Percutaneous transluminal balloon valvuloplasty: the treatment of choice for congenital valvar pulmonary stenosis.

Forty-eight children with suspected isolated pulmonary valvar stenosis were evaluated clinically and echocardiographically between January 1987 and December 1988. Fourteen patients with significant transpulmonary peak systolic pressure gradient greater than 30 mmHg on continuous-wave Doppler interrogation were subjected to right heart catheterisation. Percutaneous transluminal balloon valvuloplasty was performed in 11 of these patients with peak systolic pressure gradient than 40 mmHg across the pulmonary valve at cardiac catheterisation. Satisfactory reduction of systolic right ventricular pressure (110 +/- 43 to 46 +/- 17 mmHg, p less than 0.001) and peak systolic pressure gradient across pulmonary valve (92 +/- 44 to 27 +/- 16 mmHg, p less than 0.001), were achieved in all cases. The reduction of peak systolic pressure gradient (64 +/- 37 mmHg, range = 29-158 mmHg), was positively correlated with the initial right ventricular systolic pressure (r = 0.92, p less than 0.0002). There was no significant complication in this series and all patients were discharged the day after the procedure. Percutaneous transluminal balloon valvuloplasty is effective and safe. It should be the treatment of choice for significant valvar pulmonary stenosis in children.

Cardiac Catheterization↗

Valvuloplasty with large trefoil balloons for the treatment of congenital pulmonary stenosis.

Percutaneous balloon valvuloplasty was performed in 16 children, 1 1/2 to 14 years old, with congenital pulmonary stenosis with teh use or trefoil balloons. These balloons, which consist of 3 identical angioplasty balloons, do not interrupt completely the blood flow during inflation. The size of the trefoil balloons used was 30 to 50% larger than the valve anulus. A obtain an effective pulmonary valvuloplasty in two patients with large valve anulus. The mean prevalvuloplasty systolic gradient fo 78.2 +/- 28.9 mmHg (range 40 to 140 mmHg) was reduced to 20.3 +/- 5.7 (range 10 to 30 mmHg) after valvuloplasty. No patient developed significant hypotension or bradycardia or other complications as a result of the procedure. The findings demonstrate that percutaneous balloon valvuloplasty with oversized trefoil balloons effectively treated congenital pulmonary stenosis without complications in the cases studied. The use of large trefoil instead of single balloons of a similar diameter is advantageous since they cause no significant compromise of the cardiac output during valvuloplasty.

Adolescent↗

Neonatal Williams syndrome presenting as an isolated supravalvular pulmonary stenosis.

An infant with normal facies and none of the extracardiac anomalies usually associated with Williams syndrome presented at birth with an echocardiographic pattern of supravalvular pulmonary stenosis and displastic pulmonary valve. A clinical reappraisal was planned at 3 months of age, but the girl died suddenly at home at 2 months of age. At autopsy, both ventricles were hypertrophic, and the valves showed mild dysplasia. The walls of the great arteries were thick, with a "washed leather" consistency, but there was no gross evidence of discrete stenosis. The histologic mosaic appearance of the media of the great arteries, due to elastosis and extreme disarray of the elastic lamellae, prompted a postmortem diagnosis of supravalvar aortic stenosis and suggested a diagnosis of Williams syndrome, which was subsequently confirmed by fluorescence in situ hybridization. Pediatricians and pathologists should be alerted that Williams syndrome in the newborn may present as an isolated supravalvular pulmonary stenosis, whereas supravalvular aortic stenosis becomes clinically significant only a few months later.

Aortic Stenosis, Supravalvular↗