Use of propranolol for severe dynamic infundibular obstruction prior to balloon pulmonary valvuloplasty (a brief communication)

A case of severe pulmonary valvar stenosis and infundibular obstruction has been reported. Infundibular obstruction was so severe that no catheter could be advanced into the pulmonary artery. Propranolol, 0.5 mg given intravenously, reduced the obstruction and allowed the balloon dilatation of the pulmonary valve to be carried out without complication. Subsequently oral propranolol helped to remove the infundibular obstruction. We strongly recommend the use of propranolol when infundibular obstruction is present prior or after the balloon pulmonary valvuloplasty.     

Development of infundibular obstruction after percutaneous pulmonary balloon valvuloplasty

A 14 month old boy with suprasystemic right ventricular pressure secondary to pulmonary valvular stenosis and anular size of 10 mm underwent percutaneous balloon valvuloplasty with a 12 mm balloon. Right ventricular pressure almost doubled after valvuloplasty and the electrocardiogram revealed development of severe right ventricular strain. Both findings persisted on the following day. A postvalvuloplasty right ventriculogram demonstrated a severe systolic infundibular obstruction not present before. The patient underwent surgical relief of infundibular obstruction; successful opening of the pulmonary valve by the balloon valvuloplasty was observed. It is concluded that a balloon size 20% larger than anular size can be safe in human subjects and that infundibular obstruction may appear or even worsen after balloon valvuloplasty. Such an obstruction may be related to the severity of pulmonary valvular obstruction and a hypercontractile infundibulum.     

Double Balloon Technique for Percutaneous Balloon Pulmonary Valvuloplasty: Comparison with Single Balloon Technique

This study was designed to document our results of double balloon pulmonary valvuloplasty and to evaluate if the double balloon technique is preferable to single balloon valvuloplasty in the relief of isolated valvar pulmonic stenosis. Double balloon valvuloplasty in twelve patients reduced the pulmonary valvar gradient from 100.5 ± 28.0 (mean ± SD) to 26.6 ± 12.5 mm Hg (P < 0.001) which remained improved, 17.5 ± 10.2 mm Hg (P < 0.001) at 6 to 14 month follow-up. Single balloon valvuloplasty with equivalent balloon/pulmonary valve annulus ratios (1.19 ± 0.14 vs 1.19 ± 0.15, P > 0.1) in another 12 patients also reduced (P < 0.001) the gradient from 96.3 ± 40.1 to 28.3 ± 17.3 mm Hg immediately following the procedure which at 6 to 13 month follow-up was 12.8 ± 9.9 mm Hg. The right ventricular peak systolic pressures (116.6 ± 24.5 vs 113.8 ± 41.5 mm Hg) and pulmonary valvar gradients (100.5 ± 28.0 vs 96.3 ± 40.1 mm Hg) prior to valvuloplasty, residual right ventricular pressures (47.4 ± 12.2 vs 54.3 ± 14.2 mm Hg) and pulmonary valve gradients (26.6 ± 12.5 vs 28.3 ± 17.3 mm Hg) immediately after valvuloplasty, and residual right ventricular pressures (43.1 ± 10.9 vs 41.1 + 12.5 mm Hg) and pulmonary valvar gradients (17.5 ± 10.2 vs 12.8 ± 9.9 mm Hg) on follow-up were similar (P ± 0.1) in both groups.
These data indicate that results of double balloon valvuloplasty are excellent but not superior to single balloon valvuloplasty. It is suggested that the double balloon valvuloplasty should be used if the pulmonary valve annulus is too large to dilate with a single balloon. There is no need for double balloon valvuloplasty if the pulmonary valve can be dilated with a commercially available single balloon.     

Balloon aortic valvuloplasty: results of the Valvuloplasty and Angioplasty of Congenital Anomalies Registry

Data from 204 children and infants who underwent aortic balloon valvuloplasty between 1982 and 1986, reported to the Valvuloplasty and Angioplasty of Congenital Anomalies Registry, were reviewed. Valvuloplasty was successful in 192 of 204 children, reducing the peak systolic left ventricular ejection gradient from 77 +/- 2 to 30 +/- 1 mm Hg, p less than 0.001. The same degree of aortic stenosis gradients reduction was noted in both the 38 children under 1 year of age and in the 166 children over 1 year of age. Significant complications included death, aortic regurgitation and femoral artery thrombosis or damage. The incidence of these complications correlated with the age of the child, the ratio of valvuloplasty balloon size/anulus size, or both. The data suggest that percutaneous balloon valvuloplasty provides effective acute relief of valvar aortic stenosis in both infants and children. However, long-term follow-up data are necessary before balloon valvuloplasty can be established as a treatment of choice for congenital valvar aortic stenosis.     

Transcatheter treatment of pulmonary outflow tract obstruction: A review

The role of transcatheter methods in the management of pulmonary outflow tract obstruction are discussed in this review. Balloon pulmonary valvuloplasty for relief of isolated pulmonary valve stenosis has been successfully used by many investigators and is the procedure of choice for the management of these lesions. Supravalvar pulmonic stenosis, if discrete, can be relieved by balloon dilatation. Cyanotic children with interatrial right-to-left shunts secondary to severe valvar pulmonary stenosis respond in a manner similar to that observed with isolated pulmonary valve stenosis. In these patients, balloon valvuloplasty is the treatment of choice and may be corrective in most patients. In patients with interventricular right-to-left shunting secondary to pulmonary outflow tract obstruction and in patients with narrowed BT shunts, balloon dilatation may be an effective palliative procedure in a substantial proportion of patients obviating the need for an initial or second palliative shunt. Balloon dilatation is recommended if the patient's size or cardiac anatomy make them unsuitable for safe total surgical correction. In patients with pulmonary atresia, either initial opening of the atretic pulmonary valve by laser or by surgery with subsequent balloon dilatation are potentially beneficial in reducing the total number of surgical procedures that these children are likely to require. However, further clinical trials are needed before their general use.     

Balloon pulmonary valvuloplasty: a review

Balloon pulmonary valvuloplasty has been used successfully over the last few years for the relief of moderate to severe valvar pulmonic stenosis in neonates, infants, children, and adults. Both immediate and intermediate term follow-up results have been well documented by cardiac catheterization studies. Electrocardiographic and echo-Doppler evaluation at follow-up is reflective of the results and may avoid the need for recatheterization. The results of balloon valvuloplasty are either comparable to or better than those reported with surgical valvuloplasty. The causes of restenosis have been identified, and appropriate modifications in the technique, particularly the recommended use of a balloon/annulus ratio of 1.2 to 1.5, should give better results than previously documented. Complications of the procedure have been minimal. Further refinement of the catheters and technique may reduce the complication rate even further. The indications for balloon valvuloplasty have not been clearly defined but should probably be similar to those used for surgical valvotomy; only patients with moderate to severe valvar pulmonic stenosis are candidates for balloon valvuloplasty. Previous surgery and pulmonary valve dysplasia are not contraindications for balloon valvuloplasty. The procedure is also applicable to pulmonary stenosis associated with other complex cardiac defects and stenosis of bioprosthetic valves in pulmonary position. Miniaturatization of balloon/catheter systems to further reduce the complication rate and documentation of favorable result at 5- to 10-year follow-up are necessary.     

Semilunar Valve Interventions for Congenital Heart Disease: JACC State-of-the-Art Review

Transcatheter balloon valvuloplasty for the treatment of aortic and pulmonary valve stenosis was first described nearly 40 years ago. Since that time, the technique has been refined in an effort to optimize acute outcomes while reducing the long-term need for reintervention and valve replacement. Balloon pulmonary valvuloplasty is considered first-line therapy for pulmonary valve stenosis and generally results in successful relief of valvar obstruction. Larger balloon to annulus (BAR) diameter ratios can increase the risk for significant valvar regurgitation. However, the development of regurgitation resulting in right ventricular dilation and dysfunction necessitating pulmonary valve replacement is uncommon in long-term follow-up. Balloon aortic valvuloplasty has generally been the first-line therapy for aortic valve stenosis, although some contemporary studies have documented improved outcomes following surgical valvuloplasty in a subset of patients who achieve tri-leaflet valve morphology following surgical repair. Over time, progressive aortic regurgitation is common and frequently results in the need for aortic valve replacement. Neonates with critical aortic valve stenosis remain a particularly high-risk group. More contemporary data suggest that acutely achieving an aortic valve gradient <35 mm Hg with mild aortic regurgitation may improve long-term valve performance and reduce the need for valve replacement. Continued study will help to further improve outcomes and reduce the need for future reinterventions.     

Balloon pulmonary valvuloplasty in the management of cyanotic congenital heart defects

Twenty-three children with cyanotic congenital heart defects, aged 3 days to 11.5 years, weighing 2.9 to 30 kg, underwent percutaneous balloon pulmonary valvuloplasty to improve pulmonary oligemia. The patients were divided into two groups: group I with intact ventricular septum and group II with ventricular septal defect. In 12 group I patients, there was an increase of systemic arterial oxygen saturation [83 ± 8% (mean ± SD) vs. 94 ± 5%, P < 0.001] and pulmonary-to-systemic flow ratio (0.7 ± 0.1 vs. 1.0 ± 0.2, P < 0.001). Peak systolic pressure gradient across the pulmonary valve decreased (P > 0.001) from 105 ± 48 to 25 ± 18 mm Hg. In 11 group II patients, arterial oxygen saturation (67 ± 13 vs. 83 ± 13%, P < 0.01) and pulmonary-to-systemic flow ratio (0.7 ± 0.4 vs. 1.2 ± 0.5, P < 0.02) increased following valvuloplasty. Peak systolic pressure gradient across the pul-monic valve (52 ± 16 vs. 32 ± 22 mm Hg, P < 0.05) decreased while infundibular and total pulmonary outflow tract gradients were unchanged (P > 0.1). Immediate surgical intervention was avoided in all cases in both groups. On follow-up, 1 to 36 months after valvuloplasty, arterial oxygen saturation, pulmonary-to-systemic flow ratio, and pulmonary valve gradients remain improved in both groups. However, in group I, repeat balloon valvuloplasty was required in two children. In group II, six children with tetralogy of Fallot (TOF) underwent successful total surgical correction 4 months to 2 years after valvuloplasty. Two children with transposition of the great arteries required Blalock-Taussig shunts 6 and 18 months after valvuloplasty for further improvement of hypoxemia. Demonstrable increase in pulmonary artery diameter occurred in several children. These data suggest that pulmonary valvuloplasty offers an excellent relief of pulmonary valve obstruction in the intact ventricular septum group and palliation of pulmonary oligemia in the ventricular septal defect group. In the latter group, avoiding immediate surgical shunts in all patients and achieving growth in pulmonary arterial size in many patients, thus making them suitable for further surgical palliation or correction, are additional advantages.     

Transcatheter management of cyanotic congenital heart defects: a review.

In this review, the role of transcatheter methods in the management of cyanotic congenital heart defects is discussed. In patients with interventricular right-to-left shunting secondary to pulmonary outflow tract obstruction (most commonly tetralogy of Fallot), balloon dilatation may be an effective palliative procedure in a substantial proportion of patients, obviating the need for a palliative shunt. We would recommend this if the patient's size or cardiac anatomy makes that patient an unsuitable candidate for safe total surgical correction. Infundibular myectomy with atherectomy catheter in tetralogy of Fallot patients may become a useful adjunct in the management of these infants. Cyanotic children with interatrial right-to-left shunt secondary to severe valvar pulmonary stenosis respond to balloon pulmonary valvuloplasty in a manner similar to that seen with isolated pulmonary valve stenosis. In these patients, balloon valvuloplasty is the treatment of choice and may be corrective in most cases. In patients with a narrowed Blalock-Taussig shunt, balloon angioplasty may improve pulmonary oligemia and systemic arterial hypoxemia and may obviate the need for a second systemic-to-pulmonary artery shunt. Balloon angioplasty is recommended if the patient's cardiac defect is not amenable to surgical correction at a low risk either because of the size of the patient or because of the complexity of the cyanotic heart defect. In patients with pulmonary valve atresia, initial opening of the atretic pulmonary valve by either laser or surgery with subsequent balloon dilatation is potentially beneficial in reducing the total number of surgical procedures that these children are likely to require. However, further clinical trials are needed prior to their general use.     

Balloon aortic valvuloplasty for aortic stenosis in neonates, children, and young adults

Balloon aortic valvuloplasty is a safe and effective treatment for aortic stenosis in neonates, children, and young adults. The indication for balloon valvuloplasty in children is a peak systolic gradient > 70 mmHg or > 50 mmHg in any patient with symptoms. Published results indicate that the procedure results in an acute reduction in gradient ranging from 49%-70%, and this reduction appears to persist through at least an intermediate follow-up. The percentage reduction in gradient is similar for neonates as well as older children. In neonates, it is imperative to evaluate the size of the left ventricle prior to balloon valvuloplasty since mortality is highest in those with variants of hypoplastic left heart syndrome. Though morbidity and mortality is higher in the neonatal age group, the results are similar to those following surgical intervention. In children older than 1 month of age, the major complication is the development of aortic regurgitation, although it usually appears to be well-tolerated. Previous surgical valvotomy is not a contraindication to balloon aortic valvuloplasty. In the current era, we believe that balloon aortic valvuloplasty should be considered as the first option in neonates, children and young adults with significant aortic valve obstruction.     

Balloon pulmonary valvuloplasty: results of the Valvuloplasty and Angioplasty of Congenital Anomalies Registry

Eight hundred twenty-two balloon pulmonary valvuloplasties were reported to the Valvuloplasty and Angioplasty of Congenital Anomalies Registry. Before and after systolic outflow gradients were recorded in 784 valvuloplasties, and the gradient decreased from 71 +/- 33 to 28 +/- 21 mm Hg. The sites of residual obstructions could be ascertained in 196 patients. In these, the total systolic outflow gradients decreased from 85 +/- 41 mm Hg to 33 +/- 27 mm Hg. Of this total residual gradient, 16 +/- 15 mm Hg was transvalvar and 18 +/- 24 mm Hg was infundibular. The degree to which infundibular obstruction subsequently resolved was not determined in this study. The procedure was less effective in reducing outflow gradients in patients with dysplastic valves with or without Noonan's syndrome. There were 5 major complications (0.6%), including 2 deaths (0.2%), a cardiac perforation with tamponade (0.1%) and 2 tricuspid insufficiencies (0.2%). There were 11 minor complications (1.3%) and 21 incidents (2.6%). The incidence of major complications, minor complications and incidents was inversely related to age; it was substantially higher in infants and, in particular, neonates. Balloon pulmonary valvuloplasty is a safe and effective method of lowering pulmonary outflow gradients in infants, children and adults. Small transvalvar and varied infundibular gradients commonly are present at the end of the procedure. Assessing the full effect of the procedure requires intermediate-term follow-up and assessing the duration of relief requires long-term follow-up.     

Results of valvuloplasties with balloon catheter in children. Follow-up using pulsed Doppler. Experience with our first cases

Our series includes the first 19 consecutive patients with pulmonary valvar stenosis who underwent balloon valvuloplasty in our hospital. All of them have been evaluated by an echo-Doppler study at different follow-up times, and in 10 patients the study was also performed prior to the dilatation. The ages ranged between 0.4 and 10 years (mean 3.5 years; standard deviation [SD] 2.53 years). The prevalvuloplasty degree of pulmonic stenosis found at catheterization was quantified as severe in 8 cases (42.11%) with a right ventricular systolic pressure (RVSP) in excess of 90 mmHg, and moderate in 11 cases (57.89%) with an RVSP from 70 to 90 mmHg. Transvalvular gradients were registered between 38 and 110 mmHg (m: 69 mmHg and SD: 15 mmHg). According to catheterization data obtained at the time of valvuloplasty, the procedure was considered ineffective in 2 cases (10.52%), scarcely effective in four (21.05%), and successful in the remaining 13 cases (68.42%). Doppler follow-up was carried out between 1.6 and 31.9 months after valvuloplasty (mean 12.9 months and SD: 10 months). Residual stenoses were estimated by Doppler as mild in 13 cases (68.42%), moderate in four (21.05%) and severe in two (10.52%). Mild pulmonary regurgitation was detected in 10 patients (52%). The 13 patients with a satisfactory result had slight residual gradients at the valvular level that had not been modified with the course of time. Of the 4 cases with a poorly effective dilatation the gradients noted by Doppler increased in three of them, being valvular in all but one in whom the obstruction was catalogued as infundibular.(ABSTRACT TRUNCATED AT 250 WORDS)     

Therapeutic cardiac catheterization for pulmonary valve and pulmonary artery stenosis

At C.S. Mott Children's Hospital, percutaneous balloon angioplasty for pulmonary valve or pulmonary artery stenosis accounts for approximately 40 per cent of all therapeutic transcatheter procedures performed in our laboratory. Percutaneous balloon valvuloplasty offers a highly successful nonsurgical approach to the treatment of children with isolated congenital valvar pulmonary stenosis. In these children, balloon valvuloplasty generally reduces the peak systolic pressure gradient by more than 50 per cent and should be considered the treatment of choice. Balloon angioplasty provides substantial anatomic and hemodynamic benefit in approximately 50 to 60 per cent of children with peripheral pulmonary artery stenosis. It is most often successful in treating naturally occurring pulmonary artery stenoses in children with tetralogy of Fallot. Since the surgical alternatives are difficult and often unsuccessful, balloon angioplasty offers a valuable form of treatment for many children with significant pulmonary artery stenosis or hypoplasia.     

Electrocardiographic Changes Following Balloon Dilatation of Valvar Pulmonic Stenosis

The purpose of this paper was to study the electrocardiographic changes following balloon pulmonary valvuloplasty for pulmonic stenosis and to see if such changes reflect improvement in pulmonary valve gradient following balloon valvuloplasty. Forty-one patients, ages 7 days to 20 years, underwent balloon valvuloplasty for severe valvar pulmonic stenosis. In 35 of these patients ECGs were available 3 to 34 months (mean 11) following valvuloplasty and were compared with pre-valvu-loplasty electrocardiograms. In 30 children with excellent relief of pulmonic stenosis (group I), frontal plane mean QRS vector moved toward the left from 127 ±25° to 81 ±47°as did the horizontal plane mean QRS vector, 88 ± 36° to 27 ±51°. The amplitude of R wave in V_1, 19 ± 11.6 mm, and V₂, 19.7 ± 12.2 mm, decreased respectively to 9.5 ± 5.9 mm and 11.3 ±6.1 mm. S wave amplitude in V₅ and V₆ also decreased. The improvement in the electrocardiogram is associated with a decrease in pulmonary valve gradient from 95 ± 50 to 29 ± 23 mm Hg. In five children with significant residual pulmonary valve gradient (group II), the electrocardiograms did not show any significant change. Evaluation of the time course of ECG changes in group I revealed that recognizable electrocardiographic improvement was first observed at 6 months following successful balloon pulmonary valvuloplasty. Normal electrocardiogram suggests minimal residual pulmonary valve gradient while right ventricular hypertrophy suggests significant residual obstruction unless the electrocardiogram was recorded at or before six months following balloon valvuloplasty. These data suggest that electrocardiogram is a good indicator of improvement following balloon pulmonary valvuloplasty. (J. Interven Cardiol 1988:1:3)     

Incidence and treatment of reactive infundibular obstruction after balloon dilatation of critical pulmonary valve stenoses]

Seventeen consecutive newborn and premature babies with critical pulmonary stenosis underwent a technically successful balloon valvuloplasty at our institution from March 1991 to February 1998. The only major complication was a thrombosis of the femoral vein in one patient, causing no clinical problems. Four patients (24%) showed a reactive infundibular obstruction after balloon valvuloplasty. The outflow tract obstruction became evident immediately after successful dilatation of the pulmonary valve with persistently high pressures in the right ventricle. Pathognomonic was a typical notch in the ascending part of the right ventricular pressure curve. We were unable to predict this reaction based on echocardiography or angiography. To relieve the muscular subvalvar obstruction, we treated the first two patients with i.v. Propranolol (0.05 mg/kg over 2 min). The last 2 patients received Esmolol (0.5 mg/kg over 2 min followed by a continuous infusion with 100 micrograms/kg/min), a very short acting beta-blocker. In the medium-term follow-up, all 17 patients had a very good result with only mild pulmonary valve regurgitation. All 4 patients with a reactive infundibular obstruction required no repeat intervention. In the medium-term follow-up there were no differences between these 4 patients and the whole group.     

Intermediate-term effectiveness of balloon valvuloplasty for congenital aortic stenosis. A prospective follow-up study.

BACKGROUND. Percutaneous balloon valvuloplasty has proven to be acutely effective in the treatment of congenital valvar aortic stenosis; however, the intermediate- and long-term effectiveness of the procedure remain to be documented. METHODS AND RESULTS. To assess the intermediate-term effectiveness of balloon valvuloplasty, repeat catheterization was performed in 27 of 30 children 1.7 +/- 0.1 years after balloon valvuloplasty for congenital aortic stenosis (AS). In 33 children the peak AS gradient was reduced acutely by 55% from 77 +/- 4 to 35 +/- 3 mm Hg (p less than 0.001), and left ventricular systolic pressure was reduced from 176 +/- 4 to 138 +/- 4 mm Hg (p less than 0.001). Despite a technically adequate valvuloplasty procedure, three patients had inadequate relief of obstruction and required complex surgical intervention. Twenty-seven of the 30 patients available for late reevaluation (90%) enrolled in the follow-up study. The peak AS gradient remained significantly reduced compared with that present before valvuloplasty (29 +/- 3 versus 77 +/- 4 mm Hg, p less than 0.001). Furthermore, there was no difference in peak AS gradient at follow-up compared with that immediately after valvuloplasty. The greatest increase in gradient at reevaluation was 14 mm Hg. Twenty of 27 patients (74%) had no change in the degree of aortic insufficiency at follow-up compared with that present before valvuloplasty. At follow-up, 16 patients had no aortic insufficiency at all, and only two had moderate-to-severe (3-4+) insufficiency. Femoral artery injury was documented in four patients, three of whom were under 12 months of age at valvuloplasty. CONCLUSIONS. Balloon aortic valvuloplasty provides safe and effective intermediate-term gradient relief without early restenosis in children and adolescents with congenital AS.     

Current treatments for congenital aortic stenosis

PURPOSE OF REVIEW: Congenital valvar aortic stenosis is a challenging disease that often requires repeated palliative procedures. Stenosis can range from mild and asymptomatic, not requiring intervention, to severe, as seen in hypoplastic left heart syndrome. New advances such as fetal balloon valvuloplasty, improvements in the Ross technique, and long-term studies of trans-catheter balloon valvuloplasty and surgical valvotomy warrant a review of the outcomes and optimal timing of the various interventions. RECENT FINDINGS: Fetal balloon valvuloplasty has shown promise. Despite some mortality and morbidity, some fetuses are showing significant growth in left ventricular structures, allowing biventricular repair. In neonates and infants with congenital aortic stenosis, excellent initial results are obtained with trans-catheter balloon valvuloplasty, although stenosis resistant to further balloon dilation or regurgitation may develop, necessitating surgical intervention. Midterm results from the Ross procedure are encouraging, demonstrating low rates of mortality, aortic insufficiency and re-intervention. Stenosis of the pulmonary allograft may be inevitable, and recent long-term follow-up suggests an increase in aortic insufficiency. SUMMARY: While availability of fetal balloon valvuloplasty is limited, it has promise for promoting in-utero left ventricle growth and improving function. The optimal procedure for infants and neonates is trans-catheter balloon valvuloplasty. For older patients, the Ross procedure is the repair of choice, although more long-term studies are needed to assess the natural course of the autograft. Outcomes should improve with advances in pulmonary allografts.