Congenital severe aortic stenosis with congestive heart failure in late childhood and adolescence: effect on left ventricular function after balloon valvuloplasty.

Left ventricular (LV) dysfunction with congestive heart failure (CHF) resulting from severe congenital aortic stenosis (AS) is a well-described condition in infancy, but it is rarely found in older children and adolescents. Aortic valve surgery in such cases may be associated with higher rates of morbidity and mortality. Aortic valve balloon dilatation (AVBD) is a viable alternative, but its effect on LV function has not been evaluated. We describe follow-up results of AVBD in 10 cases of severe congenital AS in older children and adolescents with CHF and LV dysfunction. The ages of these patients ranged from 5 to 18 yr (mean +/- SD: 10.8 +/- 4 yr), and nine were males. The follow-up period after AVBD ranged from 3 mo to 7 yr (mean +/- SD: 2.93 +/- 2.1 yr). Success was achieved in all cases, with no immediate complications. After valvuloplasty, the peak-to-peak systolic gradient declined from 74.7 +/- 30.8 to 33.9 +/- 18.2 mm Hg (P < 0.0001). The cardiac index increased slightly but significantly, from 1.9 +/- 0.27 to 2.2 +/- 0.5 L/min/m(2) (P < 0.015). Hemodynamic improvement was also confirmed by a significant decrease in mean pulmonary artery and pulmonary artery wedge pressures from 41.9 +/- 9 to 32.6 +/- 6.6 and from 25.5 +/- 2.9 to 19.3 +/- 3.4 mm Hg, respectively. The echocardiographically derived left ventricular ejection fraction (LVEF) improved from 21.6 +/- 5. 37% to 31 +/- 6.5% within 24 hr after AVBD, and it further improved in all cases on follow-up. Mean LVEF at last follow-up was 59.4 +/- 11.4%. The Doppler instantaneous peak systolic gradient (IPSG) increased from 37.3 +/- 18.8 to 64.8 +/- 30.7 mm Hg at late follow-up. Significant aortic regurgitation (AR) developed in 20% of patients. The Doppler IPSG across the aortic valve was > 60 mm Hg in five cases on follow-up. Two of these patients underwent another AVBD successfully 4 and 16 mo later, respectively. Aortic valve replacement was done in two patients, one for severe restenosis with mild AR 12 mo after AVBD and another for severe re-restenosis with moderate AR 21 mo after a second AVBD. Severe congenital AS can be associated with LV dysfunction and CHF in late childhood and adolescence. AVBD results in good palliation with improvement in LV function on follow-up.