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Surgical intervention in neonates with critical pulmonary stenosis.

The surgical experience with 18 consecutive neonates with critical pulmonary stenosis (PS) and intact ventricular septum was reviewed. All patients had cardiac catheterization with calculation of right ventricular volume. Group A patients (N = 8) had a small right ventricular end-diastolic volume (RVEDV less than 72% of predicted). Group B patients (N = 10) had a normal or enlarged RVEDV. All patients had a closed pulmonary valvotomy. Five Group A patients required a systemic-pulmonary shunt or prostaglandin (PGE1) after operation; one patient died. Nine Group B patients did well after valvotomy; one moribund patient died after valvotomy and shunt. Six of 16 survivors required reoperation: valvectomy in four patients and shunt takedown in two patients. Four of the six patients who had reoperation also had a transannular patch. There was one unrelated late death. All long-term survivors are asymptomatic. Recatheterization in four patients with a small right ventricle (RV) documented significant RV growth. In conclusion, most neonates with critical PS can be managed with closed valvotomy. Patients with a small RV may require PGE1 or a shunt after operation for persistent hypoxemia. Some patients with a small RV will have significant RV growth after valvotomy.

Heart Defects, Congenital↗

[Palliative surgery of transposition of great vessels associated with pulmonary stenosis].

Palliative treatment for transposition of the great vessels with pulmonary stenosis (combined or not with ventricular septal defect) was studied in relation with 24 patients operated upon at Laennec's Hospital in Professor Mathey's section between January 70 and January 74. Three therapeutic attitudes are possible according to whether or not an atrial septal defect is created (Blalock-Hanlon's operation) in combination with the systemic-pulmonary artery anastomosis, and according to whether the operations are performed in one or many steps. It seems that for the badly-tolerated forms of the new-born, combination of both operations seems to be the best attitude, the curative treatment being impossible at this age.

Blood Pressure↗

Cor triatriatum associated with severe valvular pulmonary stenosis and patent ductus arteriosus: an unreported constellation.

A divided left atrium because of cor triatriatum is a relatively rare cardiac anomaly requiring corrective surgery. Although association of cor triatriatum with different congenital heart diseases had been reported, to our knowledge, its association with severe valvular pulmonary stenosis and patent ductus arteriozus had not been reported previously. In this paper, an asymptomatic case with cor triatriatum, severe valvular pulmonary stenosis and patent ductus arteriosus is defined.

Child↗

Transaortic closure of ventricular septal defect in patients with corrected transposition with pulmonary stenosis or atresia.

In 12 patients having corrected transposition with pulmonary stenosis (n = 8) or atresia (n = 4), the associated ventricular septal defect was repaired transaortically. Ages ranged from 4 to 50 years (mean 14.3). The hemoglobin ranged from 13.2 to 22.4 g/dl (mean 16.6), the hematocrit ranged from 40.2% to 68.2% (mean 49.0%), and the arterial oxygen saturation ranged from 63% to 97% (mean 83.8%). Associated anomalies included atrial septal defect (n = 11), double-outlet right ventricle (n = 7), dextrocardia (n = 4), and mesocardia (n = 2). Ten patients received an extracardiac conduit. There were two in-hospital deaths from low cardiac output. None of the 10 survivors had complete heart block, residual ventricular septal defect, aortic incompetence, or coronary arterial injury. Transaortic closure of ventricular septal defect is preferred in corrected transposition because it minimizes complete heart block (the conduction system courses subendocardially in the pulmonary ventricle), allows secure placement of the patch (on the high-pressure side of the defect), and limits coronary arterial and anterior papillary muscle injury (the pulmonary ventriculotomy for conduit anastomosis may be made in an apical location).

Aortic Valve↗

Pulmonary stenosis with intact ventricular septum: assessment and indication of reconstructive surgery for residual right-ventricular outflow tract obstruction.

If not all of the right ventricular outflow tract obstruction (RVOTO) is removed in the operation for pulmonary stenosis, high right ventricular pressure can sometimes occur afterward. However, it is not easy to assess the amount of RVOTO that is to be removed, and there is no quantifiable method for selecting operative procedures. The aim of this report is to discuss the formulation of a numerical indicator, based on the parameters peak systolic right-ventricular pressure (RVP), systolic systemic arterial pressure (AP), pulmonary valvular orifice size (VS), and body surface area (BSA), from the results obtained in sixty-four open heart surgeries for pulmonary stenosis with intact ventricular septum. A group, in which an outflow tract patch was not used and which had a higher pre-operative RVP/AP ratio, had a tendency to have a correspondingly higher RVP/AP ratio one month after the operation. Most patients with a high RVP/AP ratio one month after the operation showed a significant decrease in this ratio a long time after the operation. But, there were exceptions to this rule where the ratio remained high. Reconstruction of the right ventricular outflow was considered for some patients whose RVP/AP ratios remained high. Reconstruction of the RVOT by using an outflow tract patch worked well for patients with an associated infundibular stenosis. A subannular patch was used for patients with infundibular stenosis, and a transannular patch was used for patients with annular stenosis. The optimal annulus size was such that VS/BSA was not less than 2 cm2/m2. A transannular monocusp patch was applied to an area which was more than 10 mm wide.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[Natural history and surgical treatment of pulmonary stenosis. Review of 91 cases].

The natural history of valvular pulmonary stenosis is modified by the surgical treatment of these patients. In order to assess these changes we studied the clinical and hemodynamic features of 91 patients that were treated surgically. Hemodynamic studies were performed postoperatively in 62 of them. All patients had preoperatively a right ventricular systolic pressure above 70 mm Hg and were divided into three groups according to the transvalvular gradient. The first group had less than 50 mm Hg, the second group between 50 and 80 mm Hg, and the third group more than 80 mm Hg gradient. Correlation of these values with asymptomatic (49 patients) and with symptomatic (42 patients) cases showed significant values (p less than 0.001). Postoperatively only 2 patients remained with mild degree effort dyspnea. There was also a significant correlation (p less than 0.005) between the systolic gradient and the cardiothoracic ratio in the studied groups. The T wave in lead VF showed a predictive value in relation to the systolic gradient (p less than 0.001). Reduction of the systolic gradient after surgery showed very significant values when compared to the preoperative gradient (p less than 0.001). The overall mortality was 5.5%.

Adolescent↗

Critical pulmonary stenosis with an aortopulmonary window.

In neonates with severe aortic or pulmonary stenosis, the obstructed circulation is usually supported through patency of the arterial duct. We describe a neonate in whom an aortopulmonary window supported a critically obstructed pulmonary circulation.

Aorta↗

[Acquired pulmonary stenosis caused by mediastinal mature teratoma: a case report].

A 13-year-old girl was admitted with a sudden onset of chest oppression. Mediastinal teratoma was suspected on chest X-ray and CT scan. A grade 2/6 systolic murmur was heard at the upper left sternal area and cardiac catheterization showed mild pulmonary stenosis. After resection of the tumor, the murmur disappeared and histopathological diagnosis was mature teratoma. This is a rare case report of pulmonary stenosis caused by mediastinal mature teratoma.

Adolescent↗

Management of pulmonary atresia or critical pulmonary stenosis and intact ventricular septum with a small or hypoplastic right ventricle.

Twenty-one neonates and infants less than 3 months old undergoing cardiac surgery for an obstructed right ventricular outflow tract, intact ventricular septum and a small or hypoplastic right ventricle were retrospectively analyzed, in order to assess the effects of a change in management protocol. Seven of the 8 patients with critical pulmonary stenosis survived surgery using a transannular outflow patch, whereas only 1 of the 8 patients with pulmonary atresia survived the same operation. Two patients in the latter group died 2 and 3 months after surgery but with complications arising from surgery. Of 5 patients with pulmonary atresia who had a modified Blalock Taussig shunt, 3 patients survived the surgery and were discharged home. These results significantly indicate that there is an unacceptably high mortality for the relief of pulmonary atresia (with intact septum) using a transannular outflow patch, and a Blalock Taussig shunt is the preferred operation. The transannular outflow patch is a safe operation for neonates with critical pulmonary stenosis, irrespective of the size of the right ventricle.

Cardiac Catheterization↗

[Dilatation of supravalvular pulmonary stenosis in transesophageal echocardiographic monitoring--the kissing-balloon technic].

A successful dilatation of a supravalvular pulmonary stenosis after banding operation is described in a 19-year-old male. The dilatation was performed during transesophageal echocardiographic monitoring. Dilatation with a 15-mm and a 19-mm valvuloplasty catheter was not successful. Using the "kissing-balloon" technique with a 15 and 19 mm balloon together, the pressure gradient could be reduced from 59 mm Hg to 10 mm Hg (mean 41 and 8 mm Hg). Pulmonary artery diameter increased from 17 to 24 mm without intima damage or dissection. Thus pulmonary artery stenosis caused by banding operation can be dilated successfully. Monitoring during the procedure quickly demonstrates the changes in the dilated vessel. Angiography 6 months later showed slight restenosis of the dilated vessel with a maximum pressure gradient of 27 mm Hg (mean 17).

Adult↗

Surgical management of congenital and acquired pulmonary vein stenosis.

Pulmonary vein stenosis describes a variety of entities with a variable clinical course. The basic pathologic process appears to be fibrous intimal thickening, which can gradually cause obliteration of the lumen of the pulmonary veins at the atrial junction. In its most severe form, congenital pulmonary vein stenosis is a progressive disease with rapid pulmonary hypertension and rare survival beyond the first year of life. Surgical intervention has not been successful in this group. Other forms of pulmonary vein stenosis, including unilateral types associated with congenital heart disease, may be managed by various surgical treatments or pneumonectomy in some cases. Although multiple surgical techniques have been described for repair of congenital vein stenosis, the "sutureless" techniques recently described may have a place in the surgical treatment of this condition. In the most severe forms, lung transplantation appears to be an important option that should be considered early in the course of the disease. Copyright 1999 by W.B. Saunders Company

Journal Article↗

Evolving management for critical pulmonary stenosis in neonates and young infants.

Over the years, management of critical pulmonary stenosis in young infants has evolved from surgical reconstruction of the right ventricular outflow tract and closed pulmonary valvotomy to transcatheter balloon valvoplasty. Our study aimed at evaluating how the changing policy for management had affected the immediate and long term outcomes of babies with this cardiac lesion. Interventions were made in 34 infants at a median age of 8.5 days (2-90 days). Reconstruction of the right ventricular outflow tract reconstruction was performed in 10 patients, closed pulmonary valvotomy in 13, and balloon valvoplasty in 11. Initial procedure-related mortality was 50%, 15% and 0% respectively. Multivariate analysis revealed transannular patching of the right ventricular outflow tract, and male sex, to be significant factors for death. For the 27 survivors, the ratio of right ventricular to systemic systolic pressure decreased from 1.6 +/- 0.3 to 0.3 +/- 0.2 after reconstruction of the outflow tract, 1.8 +/- 0.5 to 0.8 +/- 0.4 after closed valvotomy, and 1.8 +/- 0.6 to 0.9 +/- 0.3 after balloon valvoplasty. The decrease was significantly greater after patch reconstruction (p=0.025) that required no further reinterventions. The overall rate of reintervention for the survivors was 37% (10/27). The freedom from reintervention after closed valvotomy was 82%, 64% and 51% at 1, 5 and 10 years respectively. The figure remained at 78% at both 1 and 5 years (p=0.66) after balloon valvoplasty. The higher reintervention rate for closed valvotomy corresponded to the significantly greater residual gradient across the pulmonary valve noted on follow-up (p=0.01). Reinterventions included balloon dilation (n=6), reconstruction of the outflow tract (n=4), and 1 each of ligation of an arterial duct and systemic-pulmonary arterial shunting. The risk factor for reintervention was a hypoplastic right ventricle. In conclusion, transcatheter balloon valvoplasty appears to be the optimum initial approach in view of its low mortality, efficacy at relieving the obstruction, and low rate of reintervention.

Cardiac Surgical Procedures↗

Transcatheter double-blade valvotomy for the treatment of valvar pulmonary stenosis.

A transcatheter double-blade valvotome for cutting a stenotic pulmonary valve has been devised. The valvotome consists of a retractable rhomboid structure at its tip, with a blade on each side of its proximal half. By tugging the extended blades from the pulmonary artery to the right ventricle it is possible to tear the stenotic valve. After animal experiments proved the feasibility and safety of this method, it was used in three children with pulmonary stenosis. The results were encouraging.

Adolescent↗

Use of balloon dilatation to treat supravalvar pulmonary stenosis developing after anatomical correction for complete transposition.

Eight balloon dilatations were performed in five patients (aged from 10 to 37 months) in whom supravalvar pulmonary stenosis developed after anatomical correction for complete transposition. The ratio of the maximum diameter of the inflated balloon to the narrowest pulmonary arterial diameter varied from 1.6 to 3.3. In three patients with an initial ratio of less than or equal to 2 dilatation was repeated with a larger balloon. The right ventricular systolic pressure ranged from 0.70 to 1.25 of the simultaneously measured femoral arterial systolic pressure. The major stenosis was in the main pulmonary artery in four patients and at the pulmonary arterial bifurcation in one. After balloon angioplasty in the four patients there was no significant improvement in the ratio of right ventricular to femoral arterial systolic pressure or in the angiographic appearance. There was no change in the pressure ratio after angioplasty in the patient who had a major stenosis of the pulmonary arterial bifurcation and mild main pulmonary artery narrowing. There seemed to be a slight angiographic improvement in the bifurcation stenosis but at restudy two months later the angiographic improvement had disappeared. Balloon angioplasty of supravalvar pulmonary stenosis developing after anatomical correction for complete transposition was not successful in eight procedures. This lack of success may be attributable to a small pulmonary annulus with consequent multiple levels of stenosis and distortion of the main pulmonary artery.

Catheterization↗