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PubMed1996

Morphologic and hemodynamic consequences after percutaneous balloon valvotomy for neonatal pulmonary stenosis: medium-term follow-up.

OBJECTIVES: This study sought to evaluate ventricular and valvular morphologic changes, hemodynamic consequences and clinical outcomes of pulmonary balloon valvotomy performed in the neonatal period. BACKGROUND: Previous studies support percutaneous balloon valvotomy as the management option of choice for infants and children with valvular pulmonary stenosis. Less information is available to define the impact of valvotomy on the clinical course when performed in the neonatal period. METHODS: Patient records, catheterization data, cineangiograms and selected echocardiograms (initial and most recent studies) of 37 consecutive neonates undergoing attempted balloon dilation were reviewed. RESULTS: Dilation was accomplished in 35 (94%) of 37 attempts. Immediately after dilation, the transvalvular peak to peak systolic gradient decreased from 60 +/- 22 mm Hg (mean +/- SD, range 20 to 100) to 11 +/- 10 mm Hg (range 0 to 45) (p < 0.0001), and the right ventricular/aortic systolic pressure ratio decreased from 1.25 +/- 0.43 (range 0.5 to 2.6) to 0.66 +/- 0.22 (range 0.2 to 1) (p < 0.0001). Oxygen saturation measured by percutaneous oximetry increased from 80 +/- 7% to 92 +/- 4% (p < 0.0001). Three patients died (8%), and two required repeat balloon dilation. At the follow-up visit (median 31 months, range 6 months to 8 years), the estimated peak instantaneous Doppler gradient was 15 +/- 9 mm Hg (range 6 to 36). Thickening of valve leaflets, initially present in 93% of patients, was found in only 4%, and leaflet mobility improved in all. Hypoplasia of the right ventricle, initially present in 31%, was found in only 4% at the latest evaluation. Pulmonary annulus diameter Z score increased from -3 +/- 1.0 to 0 +/- 0.1 (p < 0.0001). Freedom from reintervention was 90%, 84% and 84% at 1, 2 and 8 years, respectively. CONCLUSIONS: These data support the application of balloon valvotomy as the initial intervention in the treatment algorithm for neonates with critical pulmonary valve stenosis. Medium-term follow-up observations demonstrate sustained hemodynamic relief and support maturation of the right ventricle and pulmonary valve annulus, with the expectation of a good long-term outcome.

Algorithms↗
PubMed1986

Can the technique for Doppler estimate of pulmonary stenosis gradient be simplified?

Although Doppler echocardiography has been demonstrated to accurately predict the pressure drop across the pulmonary valve in patients with pulmonary valve stenosis, prior reports have stressed the need to correct for beam-flow intercept angles, to use simultaneous imaging, and to utilize the subcostal approach. The purpose of this study was to determine the accuracy of estimating the pressure drop in pulmonary stenosis patients by means of nonimaging Doppler applied without angle correction from precordial examination. Pressure drop estimated by Doppler was compared to that measured by strain gauge manometry at catheterization. Data for 39 patients (21 simultaneous measurements; 18 nonsimultaneous) were evaluated. Results for the entire group showed a good correlation (r = 0.94; SEE = 7.9 mm Hg). The correlation for simultaneous measurement improved somewhat (r = 0.95; SEE = 5.9), but the difference was not significant. Comparison of the slope and intercept of data of this study to those of prior studies, which advocated more complex methodology, indicated that results were essentially similar and that use of the additional steps did not confer a significantly improved result. We conclude that the simplified methodology utilized in this study provides accurate Doppler estimates of pressure gradient in patients with pulmonary stenosis.

Adolescent↗
PubMed1998

Catheterization of the pulmonary artery using a 3 French catheter in patients with congenital heart disease.

A 3 French (3F) coaxial polyurethane catheter was utilized for catheterization of the pulmonary artery in patients with complex congenital heart disease with concomitant stenosis or atresia of the pulmonary valve, with or without Blalock-Taussig shunts. Mean aortic and atrial pressures measured with the 3F catheter were compared with those measured with 5-6F conventional catheters. The values measured with the 3F catheter were identical to those measured with 5-6F catheters. Mean pulmonary arterial pressure measured using the 3F catheter was significantly higher than that measured using the 5-6F catheters, but it was not significantly different from mean pulmonary venous wedge pressure. Of 43 patients, 5-6F conventional catheters could be inserted into the branch pulmonary artery in 22 patients, but the 3F catheter could be inserted into the pulmonary artery in all patients. These data suggest that the 3F catheter is useful for catheterization in patients with complex congenital heart disease associated with pulmonary atresia or severe pulmonary stenosis.

Adolescent↗
PubMed1993

[Percutaneous balloon valvotomy in neonatal obstructive cardiopathy. I: critical pulmonary valvular stenosis].

Percutaneous pulmonary balloon valvotomy using one balloon or more in sequential approach was attempted in 9 no selected neonates with critical pulmonary valve stenosis between March 1985 and October 1990 (mean age 10 + 8.6 days). The procedure was successful in seven of them (78%) in whom with a 1.22 + 0.2 balloon/annulus ratio the right ventricular systolic pressure decreased from 96 to 45 mmHg (p < 0.001), the transvalvular gradient from 65 to 23 mmHg (p < 0.001); and the systemic oxygen saturation increased from 69 to 86% (p < 0.001). In one patient the valve was not crossed. Two patients underwent surgery because unsuccessful balloon valvotomy result in the immediate and mid term period: hypoplastic right ventricle in one, and inferior vena cava thrombosis that prevent a second dilation in the other respectively. The mid term follow up showed a 62% (5/8) of successful result. Although the complex methodology and complications are not uncommon, the balloon pulmonary valvotomy is a safely and effective therapy for neonates with critical pulmonary stenosis.

Angiography↗
PubMed1998

[Short and long term results of pulmonary balloon valvuloplasty in children].

Percutaneous balloon valvuloplasty is an alternative to surgical valvotomy for congenital pulmonary valve stenosis. Between 1985 and 1997, 73 valvuloplasties were performed in 70 patients, age range 1 day to 24.7 years. We reviewed the short- and long-term results in regard to transvalvular gradient and pulmonary insufficiency. The gradient was measured pre- and immediately post-valvuloplasty at catheterization and by Doppler echocardiography, and by echocardiography only at follow-up. Valvuloplasty was successful in 61/70 patients whereas surgical valvotomy was necessary in 9/70 because of a very dysplastic valve. A second valvuloplasty was performed in 3 patients presenting with restenosis. A 60% decrease in the gradient was noted immediately after the procedure, from 78.3 +/- 24.5 to 33.7 +/- 19.7 mm Hg (p < 0.05). A follow-up of more than 2 years (4.2 +/- 2.2 years) was obtained in 35 patients. A further decrease in the gradient was noted, to 20 +/- 12 mm Hg, and the degree of pulmonary insufficiency was mild in all patients except 2 with a moderate leak. The mortality was 0% and only 1 patient had a complication (endocarditis). The short- and long-term results of pulmonary percutaneous balloon valvuloplasty are excellent. It can be considered the therapy of choice. The gradient shows a further decrease during follow-up due to regression of the infundibular muscular stenosis. Failure is attributed to very dysplastic valves and these patients may require, in a second stage, a surgical approach (patch enlargement and/or valvulectomy).

Adolescent↗
PubMed1993

Balloon pulmonary valvuloplasty for infants with severe tetralogy of Fallot.

Balloon pulmonary valvuloplasty was performed in 3 infants with severe tetralogy of Fallot at days 24, 54 and 86 because of progressive hypoxemia. In two patients, the balloon catheter (4 cm long, 5-8 mm diameter) could not pass through the pulmonary valve. This necessitated a smaller balloon and required a two-step procedure. Initially, a coronary artery balloon (2 cm long, 3.5 mm diameter) was used. Following balloon valvuloplasty, arterial oxygen saturation increased from 63 to 83% in case 1, from 69 to 85% in case 2 and 63 to 86% in case 3. Immediate postvalvuloplasty right ventricular cineangiography revealed that the maximal opening diameter of the pulmonary valve leaflets increased from 1-2 mm to 3-4 mm in cases 1 and 3, and from 2-3 mm to 4-5 mm in case 2. No significant complications occurred. Echocardiographic follow-up data showed that the diameter of the right ventricular outflow tract and pulmonary arteries increased with age. The present results show that the pulmonary valvuloplasty is an effective procedure for relief of pulmonary valve stenosis in tetralogy of Fallot and to improve oxygenation and growth of the pulmonary arteries and right ventricular outflow tract without the need of an immediate aortopulmonary shunt.

Cardiac Catheterization↗
PubMed1993

Diagnosis of stenosis in a pulmonary valve-conduit using an angioplasty guide and probing catheter.

A 42-year-old man who had undergone previous Blalock-Taussig anastomoses and placement of a homograft aortic valve-root conduit for tetralogy of Fallot presented with dyspnea at rest. Previous catheterization had shown homograft stenosis at the level of the pulmonic valve. Repeat catheterization necessitated the use of angioplasty guide and probing catheters to cross the stenosis, and pullback documented stenoses both across the homografted valve and within the homograft aortic root-native pulmonary artery conduit.

Adult↗
PubMed1987

The pathology of balloon pulmonary valvoplasty.

The pulmonary valve was examined following balloon dilation of pulmonary valve stenosis in six children. Stenosis was due to commissural fusion in two. In these patients, cineangiography showed that the valve was slightly thickened and "domed" during systole. Balloon valvoplasty produced complete separation of fused commissures in one and partial separation in the other. Commissural splitting is likely to be the most common mechanism of relief of pulmonary stenosis by balloon dilation. Incomplete separation could account for the partial reduction of the gradient in most children treated with this technique. Four children had pulmonary valve dysplasia. Balloon valvoplasty did not produce haemodynamic improvement or morphological changes that were identifiable at the time of surgical correction. Tearing of pulmonary valve tissue and avulsion of pulmonary cusps were not seen in this series.

Adolescent↗
PubMed1991

[Percutaneous transluminal pulmonary valvuloplasty in children --5 years' experience].

OBJECTIVE: Assessment of the results of percutaneous transluminal balloon pulmonary valvuloplasty (BPV) in children. DESIGN OF THE STUDY: Retrospective analysis of the methodology and results between October 1985 and October 1990. SITE OF THE STUDY: Pediatric Cardiology Service, Santa Marta Hospital, Lisbon. PATIENTS: 43 consecutive children with pulmonary valve stenosis. 4 children with Noonan's Syndrome were excluded from the study and the results of 45 valvuloplasties in 39 patients are analysed. METHODS: Full diagnostic catheterization and right ventricular cineangiography were performed. One or two balloon dilatation catheters were inserted percutaneously in one or both femoral veins and positioned across the pulmonary valve under fluoroscopic control. The balloons were then inflated to their maximum pressure with diluted contrast medium. In 18 children follow up cardiac catheterization was performed 3 to 12 months after valvuloplasty (mean 6.7 /+- 2.7). RESULTS: A satisfactory dilatation was achieved in 35 patients (89%) with a substantial decrease in right ventricular systolic pressure (RVP) and in transvalvar peak systolic gradient (GR) (p less than 0.0001). A further significant spontaneous reduction in RVP and GR was recorded on follow up catheterization (p less than 0.01). A repeat BVP was performed in 6 children and was successful in 5. In cases a satisfactory relief of the stenosis was not achieved and 3 of them were submitted to surgery. There were no deaths. One child had cardiac arrest leading to successful surgical valvotomy and with no sequelae. Another child had cerebral embolism with hemiparesis. Ligation of a femoral vein was required in one case for haemorrhage. Mild pulmonary insufficiency resulted in 15 children. CONCLUSIONS: Our experience confirms that BPV is the method of choice for the treatment of children with pulmonary valve stenosis either isolated or associated to small ventricular septal defect. It proved to be a safe and effective technique for reduction of GR. Mortality, morbidity, costs of the treatment and hospitalisation time are lower for BPV than for surgery with comparable short term results.

Adolescent↗
PubMed2000

Cardiac malformations associated with the congenital nephrotic syndrome.

The association of cardiac malformation with the congenital nephrotic syndrome (CNS) has been previously reported in only one family. We report four patients with CNS: three with pulmonary valve stenosis (one requiring valvuloplasty) and one with discrete subaortic stenosis requiring surgical resection. We conclude that the cardiac status of all patients with CNS should be reviewed regularly by a paediatrician, with a low threshold for referral to a cardiologist, as flow murmurs due to chronic anaemia may obscure cardiac pathology. It is important to diagnose any associated cardiac lesions as these may require intervention, and may also predispose to the development of bacterial endocarditis if surgical or dental procedures are undertaken without appropriate antibiotic prophylaxis.

Aortic Valve Stenosis↗
PubMed1983

Noninvasive prediction of transvalvular pressure gradient in patients with pulmonary stenosis by quantitative two-dimensional echocardiographic Doppler studies.

Recent studies suggest that maximal Doppler velocities measured within the jets that form downstream from stenotic valves can be used to predict aortic valve gradients. To test whether the Doppler method would be useful for evaluation and management of pediatric patients with right ventricular outflow obstruction, we evaluated pulmonary artery flow before catheterization in 16 children with pulmonary valve stenosis. We used a 3.5-MHz, quantitative, range-gated, two-dimensional, pulsed, echocardiographic Doppler scanner with fast Fourier transform spectral output and a 2.5-MHz phased array with pulsed or continuous-mode Doppler. Peak systolic pulmonary artery flow velocities in the jet were recorded distal to the domed pulmonary valve leaflets in short-axis parasternal echocardiographic views. The pulsed Doppler scanner, because of its limitations for resolving high velocities, could quantify only the mildest stenoses; but, especially with the continuous Doppler technique, a close correlation was found between maximal velocity recorded in the jet and transpulmonary gradients between 11 and 180 mm Hg. A simplified Bernoulli equation (transvalvular gradient = 4 x [maximal velocity]2) proposed by Hatle and Angelsen could be used to predict the gradients found at catheterization with a high degree of accuracy (r = 0.98, SEE = +/- 7 mm Hg). Our study shows that recording of maximal Doppler jet velocities appears to provide a reliable measure of the severity of valvular pulmonic stenosis.

Adolescent↗
PubMed1995

[Pulmonary valvuloplasty in childhood: the immediate results and long-term follow-up].

BACKGROUND: Pulmonary balloon valvuloplasty is the treatment of choice of pulmonary valve stenosis, but early results and long-term follow-up in a numerous and homogeneous pediatric population are poorly known. MATERIAL AND METHODS: From April 1984 to April 1994, 202 valvuloplastic procedures were performed in 197 patients (age 52.5 +/- 150 months). Hemodynamic data were analysed according with valvular morphology and patient's age at procedure. During a follow-up period of 50 +/- 38 months, clinical and instrumental data were collected. RESULTS: After pulmonary valvuloplasty RV pressure decreased from 86 +/- 28 to 41 +/- 21 mm Hg (p < 0.001), transvalvular pressure gradient from 67 +/- 27 to 24 +/- 14 mm Hg (p < 0.001) and RV/LV pressure ratio from 1.01 +/- 0.29 to 0.53 +/- 0.19 (p < 0.001). Overall success rate was 95% (187/197 patients). Pulmonary valve dysplasia and/or annulus hypoplasia significantly influenced the efficacy of the procedure and/or the recurrence of valvar stenosis (18.8% vs 2.7%, p < 0.01). During the follow-up period, clinical examination was unremarkable, and transvalvular pressure gradient did not change (23 +/- 9 vs 24 +/- 6 mm Hg at discharge, p = NS). Freedom from restenosis was 98% at 3 years and 96% at 5 and 10 years. CONCLUSIONS: Pulmonary balloon valvuloplasty is a safe, effective and possibly definitive treatment for isolated pulmonary valve stenosis in infants and children. Age at valvuloplasty does not influence the success of the procedure, as opposed to valvular dysplasia and/or annulus hypoplasia that positively relate to the need of surgical valvotomy and/or the recurrence of stenosis during a long-term follow-up. In conclusion, prognosis of the majority of infants and children after a successful valvuloplasty is excellent during a long-term follow-up period.

Adolescent↗