The diagnosis and treatment of congenital pulmonary stenosis.
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Humans↗
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Results for “PULMONARY STENOSIS”
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[Diagnosis and treatment of congenital pulmonary stenosis. II..].
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Humans↗
Comparison of types of pulmonary stenosis with the state of the ventricular septum in complete transposition of the great arteries.
The pulmonary valve and left ventricular outflow tract of 214 hearts with d-transposition of the great arteries (d-TGA) were visually inspected (126 of 214 with intact ventricular septum and 88 of 214 with ventricular septal defect [VSD]). Pulmonary stenosis (PS), either valvular, subvalvular, or in combination, was present in 26 cases and was found to be more common in the presence of a VSD (20.5%) than intact ventricular septum (6.3%). PS occurred more commonly in the presence of a supracristal VSD than an infracristal VSD (70%, 7 of 10 vs 15%, 10 of 66). Further, infracristal or supracristal VSDs were associated with different types of pulmonary obstruction. In seven of ten cases with infracristal VSD and PS, the stenosis was caused by an anomaly of an atrioventricular valve. In six of seven cases with supracristal VSD and PS, the latter was caused by membranous encirclement of the left ventricular outflow tract.
Cardiomyopathy, Hypertrophic↗
Severe pulmonary stenosis and interruption of the aortic arch.
A case of severe pulmonary stenosis associated with a subpulmonary ventricular septal defect and interruption of the aortic arch is described in a 5-month-old girl. This combination defies explanation by current theories of the development of obstructive abnormalities of the aortic arch secondary to reduced aortic flow during fetal life, and, to the best of our knowledge, is the first reported case with this particular association.
Angiography↗
Progressive pulmonary stenosis in the fetus: two case reports.
Fetal echocardiography is a helpful tool in diagnosing and following the natural history of fetuses with congenital heart disease. This report describes two fetuses with complex congenital heart disease that developed progressive pulmonary stenosis in utero. On fetal echocardiogram, one of the fetuses had tetralogy of Fallot and the other had a single ventricle. Both fetuses, when followed serially by fetal echocardiography, showed pulmonary stenosis that progressed in severity. These findings confirm that certain cardiac defects progress in severity in utero.
Adult↗
[Peripheral pulmonary stenosis due to tumour compression (author's transl)].
A report is presented of the occurrence of peripheral pulmonary stenosis due to tumour compression in a 12 year-old boy. The tumour was identified as a liposarcoma of the mediastinum, which caused complete occlusion of the left pulmonary artery and stenosis of the right pulmonary artery. This was followed by an extreme increase in prestenotic pressure with right heart failure.
Angiocardiography↗
Isolated congenital pulmonary stenosis; report of a case complicated by rheumatic endocarditis and pulmonary tuberculosis.
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Endocarditis↗
Aneurysm in the pulmonary trunk associated with atrial septal defect, a left coronary artery fistula to the pulmonary trunk, and valvular pulmonary stenosis.
A 78-year-old woman with an aneurysm in the pulmonary trunk associated with an atrial septal defect, left anterior descending coronary artery fistula to the pulmonary trunk and valvular pulmonary stenosis is reported. The aneurysm showed gradual dilatation over 16 years and was successfully treated using aneurysmorrhaphy. Although there has been some controversy regarding the optimum management for a pulmonary artery aneurysm, surgical correction is thought to be essential for aneurysms associated with congenital cardiac anomalies because of the high incidence of rupture.
Aged↗
Right ventricular pressure dynamics after operation for pulmonary stenosis.
Right ventricular pressure dynamics were monitored during the first 24 hours after operation for relief of pulmonary stenosis in 9 patients with dysplastic pulmonary valves who underwent total valvectomy. Rather than the expected immediate drop in pressure after total removal of the obstruction, right ventricular pressures required 18 to 24 hours to decrease to physiological values. In 7 patients, the pressures decreased steadily, but in 2, there was an increase in pressure immediately postoperatively, followed by a gradual decrease.
Adult↗
Rest and exercise hemodynamics in pulmonary stenosis: comparison of children and adults.
To better understand the hemodynamics of pulmonary stenosis (PS), 24 adults and 53 children with similar degrees of PS who had undergone cardiac catheterization at rest and during supine exercise were retrospectively studied. Three groups were defined. Group I consisted of 9 adults and 18 children with a pulmonary valve area of less than 0.5 cm2/m2; group II, 6 adults and 25 children with a pulmonary valve area of 0.5 to 1.0 cm2/m2; and group III, 9 adults and 10 children with pulmonary valve area of more than 1.0 cm2/m2. The mean ages of the adults were 29, 26 and 22 years for groups I, II, and III, respectively. The mean ages of the children were 11, 10 and 9 years for groups I, II and III, respectively. The pertinent data collected from catheterization included oxygen consumption, cardiac rate and index, arterial venous oxygen difference, stroke index, right ventricular (RV) systolic pressure and RV end-diastolic pressure. Adults and children in groups II and III had an appropriate response to exercise. Group I children responded abnormally by increasing their RV end-diastolic pressure and decreasing their stroke index. In group I adults both of these variables increased. Group I adults exhibited a significantly lower cardiac index at rest and exercise secondary to a significantly lower absolute cardiac rate. Long-standing severe PS results in hemodynamic compromise. Hence, early relief of PS is recommended.
Adolescent↗
Second shunting operations for pulmonary stenosis with cyanosis following failure of original systemic-pulmonary anastomoses.
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Constriction, Pathologic↗
Hemodynamic findings during exercise on a bicycle ergometer following balloon valvuloplasty of pulmonary stenosis in children and adolescents.
Eleven patients (4 female, 7 male), age range 3.3 to 24.8 years (mean 11.10 years) treated for isolated pulmonary stenosis underwent cardiac catheterization and percutaneous transluminal balloon valvuloplasty (PTVP). The right ventricular systolic pressure (RVSP) before valvuloplasty ranged from 31 to 127 mmHg (mean 79 mmHg) decreasing to 28 to 62 mmHg (mean 42 mmHg) immediately after the dilatation. The peak systolic gradient of the pulmonary valve (delta p RV-PA) before valvuloplasty ranged from 22 to 107 mmHg (mean 61 mmHg) and decreased to a range of 14 and 45 mmHg (mean 23 mmHg) immediately after the dilatation. Balloon valvuloplasty was performed using balloons of 13 to 31 mm in diameter. On 11 patients cardiac catheterization and Doppler echocardiography were repeated between 11 months and 5.3 years (mean 3.11 years) after the balloon valvuloplasty showed a further significant fall in the gradient of pressure. The right ventricular systolic pressure ranged from 20 to 51 mmHg (mean 31.7 mmHg) while the transpulmonary gradient varied from 3 to 24 mmHg (mean 11.6 mmHg). At the time of follow-up examination the patients were aged between 7.2 and 25.7 years (mean 15.9 years). On average the second catheterization was performed 3.11 years following the first hemodynamic study. The follow-up examination encompassed clinical examination, electrocardiogram, Doppler echocardiography, and right heart cardiac catheterization. During right heart cardiac catheterization the children exercised on a bicycle ergometer for three min at 50 or 100 W depending on their body surface area. During this exertion, pressures of the right ventricle and the pulmonary artery as well as heart rate and oxygen saturation were recorded.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
Balloon dilation angioplasty of peripheral pulmonary stenosis associated with Williams syndrome.
BACKGROUND: Experience of balloon dilation of peripheral pulmonary stenosis (PPS) in Williams syndrome (WS) is limited. METHODS AND RESULTS: Catheterizations in all patients with WS undergoing therapy for PPS from 1984 to 1999 were reviewed. Criteria for successful dilation included an increase >50% in predilation diameter and a decrease >20% in ratio of right ventricular (RV) to aortic (Ao) systolic pressure. Median age and weight were 1.5 years and 9.5 kg. There were 134 dilations during 39 procedures in 25 patients. The success rate for initial dilations was 51%. In multivariate analysis, successful dilation was more likely (1) in distal than in central pulmonary arteries (P=0.02), (2) if the balloon waist resolved with inflation (P=0.001), and (3) with larger balloon/stenosis ratio (P<0.001). RV pressure was unchanged after dilation (96+/-30 versus 97+/-31 mm Hg), primarily because of failure to enlarge central pulmonary arteries. The Ao pressure increased (102+/-14 versus 109+/-19 mm Hg, P=0.03), and the RV/Ao pressure ratio decreased (0.97+/-0.34 versus 0.91+/-0.30, P=0.05). Aneurysms developed after 24 dilations (18%) and were not related to balloon/stenosis ratio. Balloon rupture in 12 dilations produced an aneurysm in all 7 cases when rupture was in a hypoplastic segment. Three patients died, none from pulmonary artery trauma, and all before 1994. CONCLUSIONS: Mortality occurred early in our experience. Despite successful dilation of distal pulmonary arteries, there was modest initial hemodynamic improvement, mainly because of persistent central pulmonary artery obstruction. A serial approach of distal dilations followed by surgical repair of proximal obstruction may be a rational and successful therapy.
Adolescent↗
Late (five to nine years) follow-up after balloon dilation of valvular pulmonary stenosis in adults.
The transvalvular gradient was investigated in 14 patients with pulmonary stenosis 5 to 9 years after balloon valvuloplasty. None of the patients had developed restenosis, and in those who had a peak gradient >100 mm Hg before valvuloplasty, the gradient decreased further due to resolution of subvalvular muscular hypertrophy within 3 months after intervention.
Adult↗
[Treatment of supravalvular pulmonary stenosis after arterial switch operations (ASO)].
The aim of this study was to evaluate the incidence of postoperative pulmonary supravalvular stenosis in patients with d-TGA and to assess the rate of success or failure of balloon angioplasty. Out of 70 patients with d-TGA 67 patients underwent successful arterial switch operation. Twelve children developed severe supravalvular pulmonary stenosis with a peak gradient above 50 mmHg (range: 50-120 mmHg). In these patients 19 high pressure dilatations were performed up to a diameter of 130% of the native valve dimension. The mean age at angioplasty was 17 months (range: 3-36 months). Successful intervention was defined as a > 50% decrease of predilatation peak pressure gradient or right ventricular pressure < 50 mmHg. Dilatations were performed without complications. Complete resolution was primarily achieved in 1 patient. In 7 patients the pressure gradients could be reduced to 10-45 mmHg (mean: 25 mmHg). In another two patients a palliative stent-implantation into the pulmonary trunk was necessary to reduce the pressure gradient. Because unsuccessful intervention, two patients needed subsequent operation. During follow-up of 6-9 months after intervention severe restenosis occurred in 3 patients (2 after stent-implantation; 1 after re-re-dilatation) who then also needed operation. Balloon dilatation should be the first treatment in patients with pulmonary stenosis after ASO in TGA owing to the low complication rate and the potential benefit of this procedure. Recurrent and combined stenoses with narrow pulmonary valve annulus should be treated surgically.
Age Factors↗
Does mild pulmonary stenosis progress during childhood? A study of its natural course.
BACKGROUND: Increase in the gradient in congenital pulmonary valvular stenosis during follow-up is a subject of controversy and could determine the need for treatment in pediatric patients. HYPOTHESIS: It is postulated that a gradient <50 mmHg shows a stable or decreasing tendency at follow-up for congenital pulmonary valvular stenosis. METHODS: Thirty-five patients with pulmonary stenosis, isolated and not treated, were followed for 7 years (interquartilic rank 5.7 years) at 1.5-year intervals. Clinical and complementary tests (electrocardiogram, x-ray, Doppler echocardiogram) were undertaken. The gradient was measured by Doppler and by using the clinical formula derived from the New England Study (Ellison). The changes observed from the initial to the final consultation were analyzed by means of the Student's t-test, paired Wilcoxon, and Pearson correlation coefficient. RESULTS: No significant changes were noted on symptoms or physical examination. Signs of cardiac enlargement diminished on both ECG (R wave in V1, p<0.0001) and x-ray (cardiothoracic ratio, p<0.0007), with a decreasing gradient trend during the follow-up period (p<0.026) as well. CONCLUSIONS: The gradient trend confirms the stable nature of mild pulmonary stenosis. In our study, we found that patients aged >6 months, whose gradients were below 40 mmHg at the time of diagnosis, remained stable and required no treatment. Furthermore, the follow-up control and clinical management of these patients may then be performed at intervals of 2 years or more.
Child↗