Short- and Intermediate-Term Follow-up Results of <br />
Percutaneous Transluminal Balloon Valvuloplasty in Adolescents and Young Adu

PVS, usually due to dome-shaped valve apparatus, resulting from commissural fusion, comprises approximately 10% of all congenital heart diseases1–3 including 10–15% dysplastic valves,4–6 which are due to markedly thickened immobile cusps with variably reduced mobility. In moderate-to-severe valvular pulmonary stenosis (PS), subvalvular hypertrophy can cause infundibular narrowing and obstructive hemodynamics, which regresses after correction of valvular stenosis.7–9 Similarly, cyanosis can occur with right-to-left atrial shunting through a patent foramen ovale due to severe PS and decreased right ventricular chamber compliance. Kan et al.10 introduced PBV in 1982, and since then this has become the treatment of choice for both children and adults. The short and long-term results of PBV are excellent and comparable to surgery.6,11–14 However, data from our country is scarce, therefore, we conducted a prospective study to determine the feasibility as well as the non-invasive intermediate-term follow-up results of PBV of isolated congenital pulmonary valve stenosis. Methods Patients. Between June 1997 and December 2001, 25 patients, ages 14–20, diagnosed on two-dimensional Doppler echocardiograms as having moderate-to-severe PVS, at the National Institute of Cardiovascular Diseases (NICVD) Karachi, Pakistan, were enrolled. Only patients with a right ventricular systolic pressure > 50 mmHg were selected for balloon valvuloplasty.15,16 However, cyanosed patients with right-to-left atrial shunt through patent foramen ovale due to severe PS and right ventricular compliance failure were also included. All patients had clinical and echocardiographic evidence of moderate to severe PVS with transvalvular pressure gradient > 50 mmHg. Patients with associated congenital malformations like large atrial septal defects, ventricular septal defects, tetralogy of fallot, patent ductus arteriosus and pulmonary incompetence, diffuse pulmonary artery branch stenosis, ebstein anomaly, severe infundibular stenosis, or significant medical complications such as infective endocarditis were excluded from the study. All patients had an ECG, chest x-ray, and two-dimensional color Doppler echocardiography done before and after cardiac catheterization and cineangiography. A fixed-size Mansfield Catheter (Mansfield Scientific, Watertown, Massachusetts) was used for PBV, due to its cost-effectiveness; a major concern in developing countries. The technique was similar to that reported earlier.3,17,18 The acceptable endpoint was an appropriately-sized balloon inflation without difficulty and without a discernible waist. Significant acute reduction in the peak systolic pulmonary valve gradient to 50 mmHg at follow-up.9 Figure 1 shows the right ventriculogram before dilation, Figure 2 shows a fully inflated blalloon wihtout discernible waist, and Figure 3 shows a post-procedure right ventricular cineangiogram showing successful PBV. Two-dimensional Doppler echocardiography was done in all patients on the next day of procedure and then on follow-up visits. Follow-up. There is an acceptable correlation between the measured pulmonary valvular pressure gradients both from cardiac catheterization and Doppler echocardiogram during the follow-up period;6 therefore, repeated cardiac catheterization for follow-up was not done, and only a Doppler echocardiogram was performed initially on next day of procedure (PBV). This was then done after the first six months and later yearly (up to five years), in order to evaluate the residual transvalvular pressure gradient. Patients were also advised to continue beta-blockers during follow-up period for the regression of residual infundibular hypertrophy. Statistical analysis. Continuous variables were presented as means ± standard deviation (SD). Two-tailed Students ‘t’ test for paired data was used for comparison of pressure gradients. A p-value of Complications. No major complication like persistent arrhythmias and bradycardia was noted. Only 3 patients had developed mild pulmonary valve regurgitation on the Doppler echocardiography and 2 patients had premature ventricular contraction or short episodes of bradycardia during or immediately after balloon dilation, which resolved spontaneously. The patients were discharged on next morning after echocardiography. Discussion PBV provides an effective relief of critical pulmonary valve stenosis in children, adolescents and adults.6,15,20–23 This is the first study on PBV in adolescents and young adults from Pakistan showing intermediate-term follow-up. We demonstrated significant reduction in pre-dilatation gradient in 80% of our patients. The presence of dysplastic valves affected the successful dilatation in our series of patients, as they are less liable to successful dilatation and frequently require surgical treatment.24 Another factor may be the balloon size that we used (balloon/annulus ratio = 1.2–1.4), it is possible that larger size balloon might have achieved more optimal results in patients with dysplastic valves.25,26 During the follow-up period we demonstrated further decrease in transpulmonic valve peak-to-peak pressure gradient, a finding similar to previously published reports on follow-up data.6,26,27 This reduction of residual pressure-gradient was mainly due to regression of reactive infundibular hypertrophy, as proven in earlier studies,6–9,13,14,16,27 requiring no more balloon dilatation. Twelve percent of our patients with severe RV dysfunction and cyanosis, due to a R-L shunt at the atrial level, showed marked improvement of right ventricular function after successful PBV as both cyanosis and right heart failure resolved completely during follow-up period. The reported experience showed that once successful dilatation has initially been achieved, then the chances of restenosis are very rare9,13 Thus, patients with successful PBV with good intermediate-term follow-up results are expected to remain asymptomatic for long-term as well without the need for further dilatation. In summary, the study showed successful results of BPV in adolescents and young adults with good intermediate-term follow-up results. Therefore, a fixed-size Mansfield Catheter can still be used successfully, especially in developing countries where procedural cost are a major concern, as Invoue balloon catheters are costly. Also, the non-invasive studies with Doppler echocardiogram avoid the need of invasive studies like cardiac catheterization for follow-up.

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