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CLINICAL STUDY: VALVE DISEASE

Discrete subaortic stenosis in adults: increased prevalence and slow rate of progression of the obstruction and aortic regurgitation

^a Adult Congenital Heart Disease Unit, Hospital Universitario La Paz, Madrid, Spain

Manuscript received November 10, 2000; revised manuscript received March 21, 2001, accepted June 1, 2001.

Reprint requests and correspondence: Dr. José María Oliver, Unidad Médico-Quirúrgica de Cardiología, Hospital Universitario La Paz, Castellana 261, Madrid 28046
pepeoliver{at}jet.es

OBJECTIVES

We sought to determine the prevalence and rate of progression of left ventricular outflow tract obstruction (LVOTO) and aortic regurgitation (AR) in adults with discrete subaortic stenosis (DSS).

BACKGROUND

Discrete subaortic stenosis is an uncommon form of LVOTO, with rapid hemodynamic progression in children, but the prevalence and rate of progression in adults have not been studied so far.

METHODS

The prevalence of DSS was determined in 2,057 consecutive adults diagnosed with congenital heart disease (CHD). The relationship between LVOTO on Doppler echocardiography and patient age was analyzed. Sequential changes in LVOTO and AR were determined for patients with two or more Doppler echocardiograms obtained with at least a two-year interval.

RESULTS

A total of 134 adults (mean age 31 ± 17 years) were diagnosed with DSS. The prevalence was 6.5% for all adults with CHD. Sixty patients (44%) had other associated CHD. The mean age of 29 patients who had undergone an operation for DSS during their adult life (56 ± 15 years) was significantly higher than that of 64 patients (27 ± 13 years) who had not required a surgical intervention (p < 0.0001). A significant relationship between LVOTO and patient age (r = 0.61, p < 0.0001) was found: 21 ± 16 mm Hg in patients <25 years old, 51 ± 47 mm Hg for those between 25 and 50 years old, and 78 ± 36 mm Hg for those >50 years old. The LVOTO increased from 39.2 ± 28 to 46.8 ± 34 mm Hg (p = 0.01) during a mean follow-up of 4.8 ± 1.8 years in 25 patients. The slope of the change in LVOTO was 2.25 ± 4.7 mm Hg per year of follow-up. Aortic regurgitation was detected by color Doppler imaging in 109 patients (81%), but it was hemodynamically significant in <20%. An increase in the mean degree of AR over time was not significant (baseline: 1.3 ± 0.8; follow-up: 1.5 ± 0.9; p = 0.096).

CONCLUSIONS

The prevalence of DSS is increasing in adults due to the greater number of repaired CHDs that develop into evolutive DSS. In contrast to infants and children, adults with DSS show a slow rate of LVOTO progression. Aortic regurgitation is a common but usually mild and nonprogressive consequence. The current indications for surgical intervention should be revised.

Abbreviations and Acronyms   AR = aortic regurgitation   CHD = congenital heart disease   CI = confidence interval   DE = Doppler echocardiography   DSS = discrete subaortic stenosis   LVOT = left ventricular outflow tract   LVOTO = left ventricular outflow tract obstruction

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