Discrete subaortic stenosis (DSS) in childhood: a congenital or acquired disease? Follow-up in 65 patients

Abstract

Discrete subaortic stenosis (DSS) is a frequent malformation easily diagnosed by echocardiography; surgical resection of the membrane is the most suitable treatment. However, some evolutive aspects of the lesion remain unexplained (the presence of aortic dysfunction, associated malformations, etc.). With a view to analyse these aspects, we have studied 65 patients with DSS and divided them into two groups: Group I, 37 patients without surgery, and Group II, 28 patients treated by resection of the membrane. During follow-up we observed: (a) development of aortic regurgitation in operated and non-operated cases; (b) frequent association of DSS with closed or closing ventricular septal defect; (c) the absence of DSS in neonates and (d) the progressive growth of the membrane. These aspects give this malformation an evolutive character that requires observation and management even after resection of the DSS.

Short- and mid-term results of resection of discrete subaortic stenosis in children

“…[9,10] Among these, ventricular septal defect (VSD), coarctation of aorta, atrioventricular septal defects, and aortic valve stenosis are the most commonly seen conditions. [1,11] In the present study, concomitant congenital cardiac disease was detected in 28 patients (38.8%) and the most common condition was VSD in 22 patients (30.5%).…”

“…[3,14,15] Although this gradient decreases to hemodynamically insignificant values, patients should be followed for aortic regurgitation and subaortic gradient after surgery. [1] Although the aortic valve functions might be affected by more than one factor during follow-up, we consider that early surgery slows the rate of degeneration of the aortic valve and, thus, decreases the need for valvular replacement.…”

“…[1] It is rarely diagnosed antenatally or during infancy. [2] It often manifests in the first decade of life with features of progressive LVOT obstruction.…”

“…[2] It often manifests in the first decade of life with features of progressive LVOT obstruction. [1,2] Discrete subaortic stenosis may lead to left ventricular hypertrophy and regurgitation, as well as arrhythmias, aortic valve regurgitation, and endocarditis. [3,4] Although surgical treatment is recognized as an effective method of treatment, the rates of recurrence of stenosis and requirement of reoperation have been reported to be up to 50% with varying figures according to the results from two studies [5,6] In this study, we report our short and mid-term surgical results in patients with discrete subaortic stenosis.…”

Left Ventricular Outflow Obstruction

“…The clinical course of SAS is generally progressive, with increasing obstruction and progression of aortic regurgitation in Ͼ80% of untreated patients, but the degree of aortic regurgitation is mild in the majority of patients. [5][6][7][8][9] Membranes located immediately adjacent to the aortic valve or extending to the anterior leaflet of the mitral valve are more likely to lead to progressive obstruction as well as more likely to cause aortic valve damage with aortic regurgitation. Severe forms of SAS result in an increased gradient across the area of stenosis and are usually evidenced on physical examination by a harsh late-peaking systolic murmur, delayed and diminished peripheral pulses, and a displaced and sustained left ventricular systolic impulse (Table).…”