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At least 73 records · Page 4Linked to original sources
PubMed2002

Supravalvar aortic stenosis with supravalvar pulmonary stenosis and peripheral vascular stenoses.

A non-dysmorphic 10 month old female was discovered at surgery to have severe vasculopathy of both the systemic and pulmonary arteries. These findings were confirmed by pathologic examination. Follow-up angiography has confirmed multiple sites of vascular obstruction which appear to be worsening. Angioplasty has only partially relieved these obstructions. The pathology and possible etiology are reviewed.

Aortic Stenosis, Supravalvular↗
PubMed1989

[A case of supravalvular aortic stenosis with spontaneous remission of peripheral pulmonary stenosis].

A 14-year-old junior high school boy was admitted to our institute. Previously he had been diagnosed as having peripheral pulmonary stenosis (Gay's classification, type IV) at the age of 2 years and 10 months. On this occasion, however, a diagnosis of supravalvular aortic stenosis was made, with a pressure gradient of about 120 mmHg, and all examinations showed spontaneous remission of peripheral pulmonary stenosis. He underwent a successful standard aortoplasty. This is the first reported case of spontaneous remission of peripheral pulmonary stenosis.

Adolescent↗
PubMedDate not supplied

Ventricular diastolic filling indices in pulmonary stenosis.

Children with valvar pulmonary stenosis have right ventricular diastolic filling abnormalities that may be due to either right ventricular hypertrophy or right ventricular outflow obstruction. In order to investigate the reason for this abnormality, 23 consecutive cases with pulmonary stenosis (mean age 7.94 +/- 3.33 years) undergoing transluminal pulmonary balloon valvuloplasty without significant tricuspid or pulmonary valvar regurgitation were studied prospectively. Right ventricular diastolic filling indices and pulmonary valvar systolic gradients were measured in these children one day before and after pulmonary balloon valvuloplasty and were re-examined six months later. Right ventricular diastolic indices based on rapid early diastolic filling peak velocity (peak E), peak velocity during atrial contraction (peak A), and ratio of E/A were determined by pulsed Doppler echocardiography. In conclusion, right ventricular diastolic filling indices in patients with pulmonary stenosis did not improve after pulmonary balloon valvuloplasty in the first day but when re-examined by the sixth month there was a significant improvement. These data suggest that diastolic filling abnormalities are more likely a result of right ventricular hypertrophy than of right ventricular outflow obstruction.

Adolescent↗
PubMed1985

Pulmonary stenosis in infants and young children.

Twenty-four patients less than 3 years old underwent operation for pulmonary stenosis. Pulmonary dysplasia was diagnosed preoperatively in only 4 patients; in 20 patients the lesion was categorized simply as pulmonary stenosis. At operation, more severe valve deformities were often present in patients less than 2 years of age. Preoperative evaluation did not reveal the extent of the deformity in 7 additional patients. The deformities included not only valvular dysplasia (thickened redundant valve cusps) but also supravalvular and annular abnormalities. Relief of obstruction was obtained only when all components of the obstructive abnormality were relieved. Patch angioplasty of the right ventricular outflow tract was necessary in 13 patients with complex morphology. Valvotomy was effective only for pulmonary stenosis due to pure commissural fusion. A spectrum of the morphology of pulmonary stenosis is recognized, with more complex lesions than simple commissural fusion identified in younger children. The more complex lesions may require more extensive operations (outflow tract patch) to completely relieve the obstructive pathological condition in the outflow tract.

Cardiac Catheterization↗
PubMed1999

Ebstein's anomaly associated with severe valvar pulmonary stenosis: successful palliation with balloon pulmonary valvuloplasty in an adult.

We report a 52-year-old adult with Ebstein's anomaly associated with severe valvar pulmonary stenosis, secundum atrial septal defect, polycythemia, hypoxemia, and severe exertional limitations. Balloon pulmonary valvuloplasty relieved the valvar obstruction, reduced the degree of tricuspid regurgitation and polycythemia, and improved systemic oxygenation and exercise abilities. Use of this technique in patients with this combination of lesions can postpone the need for surgical intervention.

Catheterization↗
PubMed2000

Accuracy of MRI evaluation of pulmonary blood supply in patients with complex pulmonary stenosis or atresia.

Detailed imaging of pulmonary artery (PA) anatomy and significant aorto-pulmonary collaterals (APCs) is crucial for surgical planning and follow-up in patients with complex congenital heart disease (CHD) and pulmonary stenosis or atresia. Because examination by echocardiography is often technically limited and catheterization is invasive, this study evaluated the diagnostic accuracy of magnetic resonance imaging (MRI) as an alternate non-invasive tool. Thirteen patients (median age 28 years, range: 1-44 years) underwent both cardiac catheterization and MRI within a median of two months (range 0.1-8 months). Diagnoses included tetralogy of Fallot (TOF) with pulmonary atresia (n = 8), TOF with pulmonary stenosis (n = 2), single left ventricle with pulmonary stenosis (n = 2), and complex heterotaxy with pulmonary stenosis (n = 1). The MRI sequences used in this study were ECG-gated spin echo and gradient echo sequences acquired in multiple planes. Compared to catheterization, MRI had 100% sensitivity and specificity for the diagnosis of main PA (n = 6) and branch PA (n = 13) hypoplasia or stenosis, as well as discontinuous (n = 4) or absent (n = 10) branch PAs. There was complete agreement between catheterization and MRI identification of significant APCs (n = 18). Main PA atresia was noted by MRI in four patients but was not definitively seen by catheterization in any. MRI but not catheterization precisely defined the distance between discontinuous PAs and their relationship to other mediastinal structures. In conclusion, cardiac MRI is a reliable non-invasive imaging modality to define PA and APC anatomy in patients with complex pulmonary stenosis or atresia.

Adolescent↗
PubMed1995

[Postoperative pulmonary stenosis after arterial switch operation, comparison in three methods of pulmonary reconstruction: modified Pacifico, autologous pericardial patch, and equine pericardial patch].

To prevent postoperative pulmonary stenosis in arterial switch operation for transposition of the great arteries, pulmonary reconstruction without patch augmentation (modified Pacifico method: Pa-group) and reconstruction using W-shaped fresh autologous pericardium (AW-group) were introduced instead of the former method using equine pericardium (XW-group). Postoperative pulmonary stenosis (RV-PA pressure gradient greater than 30 mmHg) was not seen among the 17 cases in the Pa-group (0%), and was seen in 2/14 (14.3%) in the AW-group and in 4/14 (29%) in the XW-group. Progressive increase in pressure gradient was seen in the XW-group in the midterm cardiac catheterization (p < 0.01), but such tendency was not observed in the Pa-group and the AW-group. Sectional area at the pulmonary valve ring, at the anastomotic site in the main pulmonary artery, and at the pulmonary branches just distal to the bifurcation, standardized by the body surface area, increased in the Pa-group and the AW-group, but were unchanged or slightly decreased in the XW-group in the midterm cardiac angiography. We believe that postoperative pulmonary stenosis can be best avoided if modified Pacifico method is used. The cases in which postoperative pulmonary stenosis or coronary artery compression is anticipated such as those with side-by-side aortopulmonary relationship or after pulmonary artery banding, should be operated upon using fresh autologous pericardial patch.

Humans↗
PubMed1996

[Supravalvar and valvar aortic stenosis associated with valvar and subvalvar pulmonary stenosis. Coexistence of two clinical syndromes].

A 4 year old patient with congenital rubella syndrome, confirmed serologically, presents with neurosensorial deafness and a rare association of cardiac anomalies: supravalvar and valvar aortic stenosis and subvalvar pulmonary stenosis. Bidimensional echocardiography and angiography confirmed the diagnosis and the surgical treatment was successful. Due to the presence of somatic characteristics of Williams's syndrome, mental retardation and supraortic stenosis, the authors postulate that there is a coexistence of clinical syndromes responsible for the malformations of this case. This fact is rare on clinical settings, requiring accurate diagnosis and treatment.

Angiocardiography↗
PubMed1997

Natural history of asymptomatic valvar pulmonary stenosis diagnosed in infancy.

BACKGROUND AND HYPOTHESIS: Valvar pulmonary stenosis is a common congenital heart defect. Progression of stenosis over time, even when mild initially, has been shown by serial cardiac catheterization studies in children and adults. We studied the natural history of asymptomatic valvar pulmonary stenosis diagnosed in infancy with two-dimensional echocardiography and Doppler method. METHODS: Between November 1986 and March 1993, 51 infants in the Northeast Tennessee and Southwest Virginia region were clinically diagnosed to have isolated valvar pulmonary stenosis. In 40 patients, the diagnosis was confirmed by two-dimensional echocardiogram/Doppler and color-flow mapping study at the time of presentation, and only their course is reported. Of 40 infants, six asymptomatic infants (15%) showed rapid progression of pulmonary stenosis over a relatively short period of time. Within the first 6 months of life, three of the six infants showed worsening of the stenosis needing intervention (one had surgical valvectomy and the others had percutaneous balloon valvuloplasty). The three other infants showed a more gradual increase of pulmonary stenosis over the first 2 years of life. RESULTS: Pulmonary stenosis even when mild can worsen in infancy, and it is not possible to predict which patients will follow this course. In our group of asymptomatic infants with initial mild pulmonary stenosis, 15% developed significant stenosis that needed intervention. CONCLUSION: We recommend frequent follow-up of asymptomatic infants with mild pulmonary stenosis during the first 2 years of life to detect rapid progression that may need intervention.

Disease Progression↗
PubMed2003

Pulmonary stenosis and atresia with intact ventricular septum during prenatal life.

AIM: To identify fetal echocardiographic characteristics predictive of perinatal outcome in cases with a prenatal diagnosis of pulmonary stenosis or pulmonary atresia. PATIENTS AND METHODS: We retrospectively reviewed the records and the videotapes of all the cases of pulmonary stenosis and pulmonary atresia diagnosed at our institutions between 1990 and 1999. The following measurements were obtained: diameters of right and left atria and ventricles and ventricular wall thickness; main pulmonary artery and aortic root diameter; direction of flow through the atrioventricular, aortic and pulmonary valves and through the ductus arteriosus. Perinatal outcome and follow-up of the survivors were available in each case. RESULTS: There were 21 cases of pulmonary atresia. Eleven were diagnosed before 24 weeks and nine of them (82%) underwent termination of pregnancy. The survival rate was 50% among the 12 fetuses born at term. None of the fetuses that survived had a large right ventricle, while this was a finding in 50% of those that died. Among the fetuses that died, 83% had a hypertrophic right ventricular wall compared to 33% of the survivors. There were 12 cases of pulmonary stenosis. Three cases were diagnosed before 24 weeks but none underwent termination of pregnancy. All the fetuses with pulmonary stenosis were born at term and four died in the perinatal period. The survival rate was thus 66.6% (8/12). Three (75%) of the fetuses that died had reversed flow in the ductus arteriosus compared with one of the fetuses that survived. CONCLUSION: Our data suggest that a grossly enlarged right ventricle and/or a hypertrophied right ventricular wall in cases of pulmonary atresia and reversed flow in the arterial duct in cases of pulmonary stenosis are likely indicators of a poor prognosis.

Cardiomyopathy, Dilated↗
PubMed1991

Oral prostaglandin E1 derivative (OP-1206) in an infant with double outlet right ventricle and pulmonary stenosis. Effect on ductus-dependent pulmonary circulation.

A small-for-gestational-age infant with cyanosis due to double outlet right ventricle with severe pulmonary stenosis and patent ductus arteriosus was treated with oral prostaglandin E1 derivative (OP-1206). The constricting ductus arteriosus dilated and the ductus-dependent pulmonary blood flow increased. The recommended dosage was 1.5-2.0 micrograms/kg/day which was lower than that of intravenous PGE1 or of oral PGE2. The administration interval was 6 hours, which was longer than that of oral PGE2. The patient was treated as an out-patient because continuous intravenous infusion was not necessary. Treatment was continued for 2 months without complication, at which time a Blalock-Taussig shunt operation was performed. Orally administered PGE1 derivative (OP-1206) was found to be equally effective to intravenous infusion of PGE1 for both short and long-term management of cyanotic heart disease in which the pulmonary blood flow is mostly dependent on the patency of the ductus arteriosus. Oral PGE1 derivative (OP-1206) may be a possible substitute for intravenous PGE1 infusion therapy.

Administration, Oral↗
PubMed1981

[Blood flow analysis with pulsed echo Doppler cardiography in valvular pulmonary stenosis (author's transl)].

Blood flows in the main pulmonary artery, right pulmonary artery and right ventricular outflow tract were analyzed in 11 cases of valvular pulmonary stenosis and 10 healthy subjects by pulsed echo Doppler cardiography (two-dimensional) with the parasternal and suprasternal approaches. 1) The systolic flow in the right pulmonary artery was detected in 7 cases of valvular pulmonary stenosis, in which the flow of both right and main pulmonary arteries was detected in only one case. The flows seemed to be turbulent. These abnormal signals were never detected in healthy subjects and considered to be caused by the narrowing of the pulmonic orifice. 2) Abnormal flow signals were also detected in the right ventricular outflow tract in patients of pulmonary stenosis. Their features were as follows: (1) A systolic turbulent flow was detected in a case with severe hypertrophy of the wall and narrowing of the lumen of the right ventricular outflow tract. (2) A/S ratio, which is a ratio of the peak velocity in atrial contraction (A) to the peak velocity in systole (S), was larger in cases with pulmonary stenosis than in healthy subjects (p less than 0.05). It was considered that the atrial component in the right ventricular filling was augmented in pulmonary stenosis. (3) The PEP/ET (pre-ejection period/ejection time) of the right ventricle was smaller in cases with pulmonary stenosis than in healthy subjects (p less than 0.05). The ratio exhibited a reverse correlation with the pressure gradient between the right ventricle and pulmonary artery (r = 0.74, p less than 0.025). (4) Acceleration time index, a ratio of the time interval between the upstroke and the peak velocity of ejection flow to the ejection time, as a parameter indicating the time delay of the peak velocity exhibited a significant correlation with the pressure gradient between the right ventricle and pulmonary artery (r = 0.67, p less than 0.05). (5) No correspondence was revealed between the time interval of Q-peak velocity in systole and that of Q-peak intensity of the murmur during systole. It was remained to be clarified.

Adult↗
PubMed1981

Echocardiographic assessment of valvular pulmonary stenosis in children.

It has been suggested that the maximal amplitude of the pulmonary valve motion following atrial contraction (Amax) may be useful in the clinical evaluation of patients with valvular pulmonary stenosis. To evaluate the specificity and sensitivity of this measurement in children, we reviewed the echocardiograms of the pulmonary valve of 120 subjects: 57 normal individuals, 25 patients with secundum atrial septal defect and without pulmonary hypertension, and 32 patients with pulmonary stenosis proven at catheterisation (mild in 16 patients, moderate in nine, and severe in seven). Amax ranged from 0 to 12 mm in the normal subjects, and from 2 to 16 mm in those with atrial septal defect. In mild pulmonary stenosis, Amax ranged from 2 to 12 mm, in moderate pulmonary stenosis from 2 to 14 mm, and in severe pulmonary stenosis, from 3 to 12 mm. Though mean Amax was significantly larger in patients with moderate and severe pulmonary stenosis compared with normal subjects, there was much overlap between the two groups so that individual cases could not be identified correctly from this measurement. No significant difference was observed when comparing Amax values of patients with atrial septal defect and those of patients with pulmonary stenosis of various severity, nor were any observed between the pulmonary stenosis groups. These findings indicate that Amax is neither specific nor sensitive for the presence or severity of valvular pulmonary stenosis in children, and that it cannot be used to evaluate non-invasively the results of pulmonary valvotomy.

Adolescent↗