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PubMedDate not supplied

Acquired pulmonary stenosis: ultrasonographic diagnosis.

A 15-year-old boy presented with chest pain and a new heart murmur. The clinical diagnosis of pulmonary stenosis was confirmed by two-dimensional and Doppler echocardiographic examinations. A large, encapsulated, partly solid and partly cystic tumor in the anterior mediastinum, visualized by ultrasonography, was compressing the main pulmonary artery and producing the right ventricular outflow tract obstruction. The tumor was removed surgically and was found to be a benign teratoma. Postoperative ultrasound examination of the right ventricular outflow tract showed no evidence of obstruction. We conclude that: (1) there are subtle clinical diagnostic differences between intrinsic and extrinsic pulmonary stenosis; (2) ultrasound examination can make cardiac catheterization unnecessary; and (3) relief of the extrinsic cause of pulmonary stenosis should be curative.

Adolescent↗
PubMed1996

[A case of congenital tricuspid regurgitation associated with atrial septal defect and peripheral pulmonary stenosis].

This report describes a 5-year-old girl with congenital tricuspid regurgitation associated with an atrial septal defect and peripheral pulmonary stenosis. The girl was diagnosed with the heart murmur at birth and recently developed the cardiomegaly. Cardiac echocardiography and catheterization showed severe tricuspid regurgitation, an atrial septal defect of the secundum type and peripheral pulmonary stenosis. In the operative findings, the tricuspid annulus was dilated to 33 mm in diameter, and leaflets were attached normally to the antomic annulus. There was a large cleft of the anterior leaflet of the tricuspid valve. Suture of the cleft and annuloplasty of the tricuspid valve, suture closure of the atrial septal defect and patch dilatation of peripheral pulmonary stenosis were successfully performed. Including this case, 19 other cases with congenital tricuspid regurgitation undergoing surgery were reported to date.

Child, Preschool↗
PubMed2005

Long-term outcome after surgery for pulmonary stenosis (a longitudinal study of 22-33 years).

AIMS: Long-term (>20 years) survival and clinical outcome are only partly documented in patients who underwent surgical repair for isolated pulmonary stenosis. Yet, such data are of critical importance for the future perspectives, medical care, employability, and insurability of these patients. METHODS AND RESULTS: Ninety consecutive patients underwent surgery for pulmonary stenosis between 1968 and 1980 at the Thoraxcenter. A systematic follow-up study was performed in 1990 and again in 2001. Survival after 25 years was 93%. Re-intervention was necessary in 15% of the patients, mainly for pulmonary regurgitation. Right atrial and ventricular dilatation and paradoxical septal motion were associated with the need for reoperation. No major ventricular arrhythmias occurred. Supraventricular arrhythmias occurred, only in patients with severe pulmonary regurgitation and disappeared after reoperation. At last follow-up, 67% of the patients was in NYHA Class I and maximal exercise capacity was 90% of normal. Moderate or severe pulmonary regurgitation was present in 37% of the patients. CONCLUSION: Although long-term survival and quality of life are good, pulmonary regurgitation is found in a third of the patients 22-33 years after surgical repair for isolated pulmonary stenosis and reoperation for pulmonary regurgitation was necessary in 9%, especially after the transannular patch technique.

Adolescent↗
PubMed2005

Aortic translocation in the management of transposition of the great arteries with ventricular septal defect and pulmonary stenosis: results and follow-up.

BACKGROUND: The surgical management of patients with transposition of the great arteries, ventricular septal defect, and pulmonary stenosis remains a challenge. The Rastelli operation is the preferred surgical procedure, but its long-term results are not optimal. The objective in this retrospective study was to review our experience using aortic translocation and biventricular outflow tract reconstruction as an alternative surgical procedure for the management of these patients. METHODS: Since January 1996, 12 patients have undergone aortic translocation and biventricular outflow tract reconstruction for the management of transposition of the great arteries, ventricular septal defect, and pulmonary stenosis at our institution. All patients had ventriculoarterial discordance; 9 had atrioventricular concordance and 3 atrioventricular discordance. Associated lesions included a straddling atrioventricular valve in 3 patients. An inlet ventricular septal defect was present in 4 patients. The median age at operation was 2 years. Eight patients had previous palliative procedures. The surgical technique used was a modification of the Nikaidoh procedure. The 3 patients with atrioventricular discordance required a Senning procedure. RESULTS: There was one hospital death (8.3%) as a result of a massive cerebrovascular accident. The median intensive care unit and hospital stays were 15 and 18 days, respectively. At a median follow-up of 33 months, all patients are alive. Four late reoperations occurred in 3 patients, including two reoperations for conduit obstruction. CONCLUSIONS: Aortic translocation and biventricular outflow tract reconstruction is a valuable surgical option for the surgical management of patients with transposition of the great arteries, ventricular septal defect, and pulmonary stenosis, especially in the presence of "inadequate anatomy" for a Rastelli repair.

Abnormalities, Multiple↗
PubMed2000

Pulmonary Stenosis.

Balloon valvuloplasty provides optimal treatment for moderate and severe pulmonary valve stenosis. Dysplastic pulmonary valves may not respond to balloon dilation and frequently require surgical treatment. Balloon angioplasty with or without stenting is the preferred treatment of peripheral pulmonary stenosis.

Journal Article↗
PubMed1995

[Adult pulmonary stenosis: percutaneous balloon valvuloplasty].

The results of balloon valvuloplasty as treatment for pulmonary stenosis in 8 adults (6 male) whose ages ranged from 17 to 58 years are presented. Three patients had been subjected to cardiac surgery 32, 40 and 10 years before. The procedure was performed through the right femoral vein using one or two balloons with a diameter of not less than 20 mm, without complications and excellent tolerance. The pulmonary gradient and the right ventricular pressure were reduced in 7 patients from 81.4 +/- 26.9 to 26.6 +/- 11.3 and from 99.3 +/- 25.5 to 47.7 +/- 13.6 mm Hg respectively (p < 0.0005). During follow up, 6 of the patients have remained in functional capacity I and in two, Doppler ultrasound examination showed a further reduction in valvular gradient. These results are in accordance with other reports and confirm that this technique is the treatment of choice for adult pulmonary stenosis.

Adolescent↗
PubMed1999

[Percutaneous valvotomy in critical neonatal pulmonary stenosis. Results and mid-term follow up].

INTRODUCTION: Catheter-balloon valvotomy has been proposed as an alternative to surgical valvotomy in the critical pulmonary stenosis in newborns. OBJECTIVES: The present report describes the results, complications and follow-up of this technique at our center. METHODS AND RESULTS: From May 1993 to June 1998, fourteen consecutive patients with critical pulmonary stenosis underwent balloon valvotomy. Their mean ages were 8.7 +/- 5.8 days. Each of them was cyanotic and ten of the fourteen were on prostaglandin E1 therapy and three of them required endotracheal intubation and ventilation. The diameters of balloons were 9.6 +/- 2.1 mm (range, 5 to 12 mm) and the balloon/annulus diameter ratio was 1.3 +/- 0.2 (range, 0.8 to 1.5). By means of this technique, right ventricle systolic pressure decreased from 95.2 +/- 22.4 mmHg to 51 +/- 23.2 mmHg (p < 0.001) and the right ventricle/aortic pressure ratio decreased from 1.5 +/- 0.4 to 0.8 +/- 0.3 (p < 0.001). Surgical valvotomy was performed in two patients (cases 1 and 8) at 5-months and 3 days, respectively, from the balloon valvotomy. One patient (case 4) required a blalock-taussig shunt. Complications included cardiac tamponade in one patient. One patient died seven days after successful valvotomy by extracardiac complications. All eleven patients are currently symptom free 26.6 +/- 17.1 months later (range, 5 to 56) and Doppler residual gradient ranges from 0 to 29 mmHg (11.1 +/- 10.4 mmHg). CONCLUSIONS: Balloon pulmonary valvotomy is an effective and relatively safe technique for treatment of critical pulmonary stenosis in newborns with low index complication and mid-term relief of valvular obstruction.

Age Factors↗
PubMed1976

Pulmonary valvulotomy under inflow stasis for isolated pulmonary stenosis.

One hundred ten patients were operated upon between 1961 and 1972 for isolated pulmonary stenosis by the inflow stasis technique. Analysis of the preoperative and postoperative clinical and catheterization data define the role of inflow stasis as an acceptable method of pulmonary valvulotomy except in relieving stenosis due to a dysplastic pulmonary valve. Patient ages ranged from 2 days to 36 years. All underwent preoperative catheterization and 69 (63%) were restudied postoperatively. Mean preoperative and postoperative peak systolic gradients were 93 and 23 mm Hg, respectively. Mean valve areas before and after operation were 0.38 and 1.10 cm2/m2. Operative mortality was 3.6% (4 patients), and there was 1 late death. Two of the dead were children 2 and 9 days old, respectively. Four patients required reoperation for residual gradients; 2 had dysplastic pulmonary valves. The overall results were excellent or good in 78%, fair in 15%, and poor in 7%. Patients with dysplatic pulmonary valves were in the poor or fair group, and it is recommended that the inflow stasis technique not be used for this type of pulmonary stenosis. The excellent surgical exposure, adequate time for valvulotomy, low morbidity, and freedom from problems of cardiopulmonary bypass are reasons for continued use of this technique for treatment of selected patients with pulmonary valve stenosis.

Adolescent↗
PubMed1996

Sympathetic activity in children undergoing balloon valvuloplasty of pulmonary stenosis.

We studied the influence of balloon valvuloplasty on alpha- and beta-adrenoceptor densities, plasma catecholamine, and cAMP levels in children and infants with pulmonary stenosis before and 10 min after balloon dilatation, employing as controls children undergoing transcatheter occlusion of patent ductus arteriosus (PDA) with Qp/Qs ratio < 1.5. In the PDA group, the alpha-adrenoceptor density (Bmax) was 3.75 +/- 0.72 fmol/10(7) cells (n = 15) before occlusion and remained unchanged at 3.35 +/- 0.47 fmol 10 min thereafter. In the pulmonary stenosis patients (n = 31), the receptor density was 59% higher (p < 0.05) before, and decreased to PDA levels 10 min after, the procedure. The control beta-adrenoceptor density was 64.8 +/- 11.0 fmol/10(6) cells before, and 71.2 +/- 13.2 fmol 10 min after, occlusion. In the study group, the density was 23% lower (p < 0.07) and increased to the PDA levels 10 min after the dilatation. Compared with the PDA, pre- and postdilatation plasma norepinephrine levels were not significantly changed; epinephrine was slightly elevated before, but increased by 73% after, dilatation; dopamine was 80% (p < 0.05); and cAMP was 37% higher before, and remained elevated at 70 and 23% above the PDA values after, the procedure. Accordingly, alpha-adrenoceptor density is significantly elevated in children with pulmonary stenosis and decreases significantly immediately after balloon valvuloplasty. On the other hand, beta-adrenoceptor density is attenuated and increases toward normal levels after the procedure. The immediate reversal of the receptor levels after balloon valvuloplasty suggests that this procedure exerts acute effects on the sympathetic functional level in this disease.

Adolescent↗
PubMed2001

Pulmonary stenosis and reactive right ventricular hypertrophy in the recipient fetus as a consequence of twin-to-twin transfusion.

The present study describes an association between adverse outcome in the twin-to-twin transfusion syndrome (TTTS) and pulmonary stenosis or reactive right ventricular hypertrophy. Six discordant monozygotic twin pregnancies with TTTS are described. Ventricular hypertrophy and atrioventricular valvular regurgitation occurred in all the recipient twins with pulmonary valvular stenosis in three cases and infundibular stenosis in one case. The recipient twin in one pair and both twins in another pregnancy died as a consequence of immaturity but the remaining twins all survived. Surgical intervention was required in one baby for valvular pulmonary stenosis. Our observations suggest that elevated blood pressure in the transfusion recipient may play an important role in pathogenesis. We hypothesise that both pulmonary stenosis and right chamber hypertrophy are secondary to hemodynamic changes. Although we have found valvular pulmonary stenosis in three recipients and infundibular stenosis in only one, this (obstruction to outflow) could be due to right chamber hypertrophy.

Adolescent↗
PubMed1975

Familial pulmonary stenosis with underdeveloped or normal right ventricle.

A family with 3 cases of pulmonary stenosis with underdeveloped or normal right ventricle is described. In the family there have also been some spontaneous abortions and many infant deaths, and it seems possible that these were also related to congenital cardiac abnormalities. Recognized syndromes with pulmonary stenosis and reports on familial occurrence of this malformation are reviewed. The cases reported in this paper differ from these previously described syndromes. The possibility of sex-influenced monogenic inheritance is discussed.

Chromosome Aberrations↗
PubMed1997

[Redo operation for recurrent pulmonary artery aneurysm associated with pulmonary stenosis and regurgitation].

A 63-year-old woman who underwent surgical correction of a recurrent pulmonary artery aneurysm associated with pulmonary stenosis and regurgitation is reported. On April 1986, she underwent commissurotomy of pulmonary valve, reconstruction of right ventricle out flow tract using a Polystan monocusp patch and pulmonary aneurysmorrhaphy for pulmonary artery aneurysm. Pathological examination of the resected pulmonary arterial wall revealed mucoid degeneration of media and fragmentation of elastic fiber. Nine years after the operation, recurrence of pulmonary artery aneurysm, pulmonary stenosis and regurgitation were recognized. On September 1995, she underwent redo operation with graft replacement of pulmonary artery and pulmonary valve replacement using woven Dacron prosthesis containing a Carpentier-Edwards bioprosthetic valve. We should choose as an initial procedure with graft replacement for pulmonary artery aneurysm with fragility of the pulmonary arterial wall. She is now doing very well at one year and 5 months after the redo operation.

Aneurysm↗
PubMedDate not supplied

Bilateral coronary artery dilatation and supravalvular pulmonary stenosis in a child with noonan syndrome.

Noonan syndrome is the second most frequent congenital malformation syndrome, after Down syndrome, associated with cardiovascular abnormalities. The most prevalent cardiovascular abnormalities in Noonan syndrome are pulmonary stenosis and hypertrophic cardiomyopathy. We report the case of a 12-year-old girl with Noonan syndrome who had multiple cardiovascular abnormalities, including extensive bilateral coronary artery dilatation, valvular and supravalvular pulmonary stenosis, atrial septal defect, and mitral valve prolapse. Both coronary artery dilatation and supravalvular pulmonary stenosis, although rarely reported, are abnormalities of the cardiovascular system that may occur in Noonan syndrome.

Cardiomyopathy, Hypertrophic↗
PubMed1997

Surgical repair of pulmonary stenosis with intact ventricular septum in a 68-year-old woman.

Patients with mild pulmonary stenosis after infancy rarely have symptoms or develop increasing obstruction. We experienced a 68-year-old woman with severe pulmonary valvar and infundibular stenosis (peak to peak pressure gradient = 80 mmHg). She had been pointed out heart disease at the age of six. Endocarditis at the age of 17 might induce calcification of valve and affect the progression of pulmonary stenosis, and moreover, which might gradually develop severe subvalvar obstruction and poststenotic aneurysm of pulmonary trunk. She refused operative intervention because of mild clinical symptoms (NYHA class II), but we recommended surgical repair due to repeated transient ischemic attacks, which were suspected paradoxical embolism through persistent foramen ovale. She underwent pulmonary valvotomy and infundibular resection and is doing well.

Aged↗
PubMed1977

Bacterial endocarditis in patients with pulmonary stenosis, aortic stenosis, or ventricular septal defect.

Bacterial endocarditis developed in 24 patients during follow-up in the Natural History Study. It occurred significantly more often in patients with ventricular septal defect (1.5/1000 patient-years) and aortic stenosis (1.8/1000 patient-years) than in those with pulmonary stenosis (0.2/1000 patient-years). Complications, especially emboli and aortic regurgitation, occurred in 50% of the patients. Overall mortality was 25%. Incidence rates were significantly greater for males than for females and greater for patients over 20 years of age than for younger patients. Patients who had had ventricular septal defect treated surgically had a lower incidence of endocarditis than non-surgical patients, whereas surgery did not reduce the incidence in patients with aortic stenosis. The estimated risk of contracting bacterial endocarditis prior to 30 years of age in a patient with medically-treated ventricular septal defect is 9.7%; the risk estimate for surgical patients in 2.0%. Although data regarding the use of prophylactic antibiotics were not available for patients in the Natural History Study, these results certainly suggest that continued antibiotic prophylaxis for dental and certain surgical procedures is mandatory for patients with aortic stenosis, even after successful cardiac surgery. It is the authors opinion that prophylaxis is probably less necessary for patients who have pulmonary stenosis, whether surgically treated or not, and for patients with documented complete closure of VSD.

Adolescent↗
PubMed1992

[A case of supravalvular pulmonary stenosis associated with Noonan's syndrome: usefulness of Doty's operation].

A twelve-year-old boy associated with Noonan's syndrome is reported. He had supravalvular pulmonary stenosis with a very high right-ventricular pressure, higher than that of systemic. Surgical treatment was performed to the supravalvular pulmonary stenosis with the technique of Doty's operation for supravalvular aortic stenosis, brought an excellent results. The gradient between the right ventricle and the pulmonary artery fell from 97 mmHg to 30 mmHg. The residual gradient is caused by functional infundibular stenosis. Doty's operation is a useful method for the operation of severe supravalvular pulmonary stenosis, too.

Aortic Valve Stenosis↗