Coexistence of asymmetric septal hypertrophy and aortic valve disease in adults.

Abstract

Echocardiography detected asymmetric septal hypertrophy (ASH) in five of 200 adults being assessed for aortic valve surgery. Four of these were among 119 patients with dominant aortic stenosis, which was severe in three. ASH was confirmed at the time of aortic valve replacement in two of these patients; the third declined operation.The finding of ASH in only one of 81 patients with free aortic reflux is consistent with chance association. While the same explanation could apply to the higher prevalence in those with aortic stenosis, it may be that a long-standing pressure overload can trigger inappropriate septal hypertrophy in predisposed individuals.Brock (1957)

Combined supravalvular aortic stenosis and hypertrophic subaortic stenosis: Coexistence or association?

“…Since hypertrophic subaortic stenosis was first clinically described in 1957 (Brock, 1957) and defined in detail in 1964 (Braunwald et al, 1964) many authors have reported on its association with other anoma li es such as Friedreich's ataxia (Smith et al ., 1977), cutaneous neurofibromatosis (Elliot, et al ., 1976), fragmentation hemolysis (Solanki and Sheikh, 1978), ventricular preexcitation syndrome (Kerin et al, 1979) , heart block (Spi lkin et al, 1977), aortic valvu lar stenosis (Harrison et al ., 1977;Raj et al , 1979), aortic valve deformity with aortic reg).lrgitation (Somerville and Ross, 1977), coronary arterial anomalies (Walston and Behar, 1976), mitral ring calcification (Kronzon and Glassman, 1978), dextroversion (Buxton et al, 1976), dex trocardi a (Cochran and Wanamaker, 1975), and hypercalcemi a (McFarland et al, 1978;Malcolm et al, 197 5). In most cases, a common pathogenetic mechanism was not defin able.…”

“…Since its original description (Braunwald et 01.,1964;Brock, 1959), hypertrophic subaortic stenosis has been subsequently linked to several other cardiac and systemic anomalies (Buxton et al, 1976;Cochran and Wana-maker, 1975;Elliot et 01.,1976;Harrison et al, 1977;Kerin et al, 1979;Kronzon and Glassman, 1978;McFarland et 01., 1978;Malcolm et al, 1978;Raj et al, 1979;Smith et al, 1977;Solanki and Sheikh, 1978;Spilkin et al, 1977;Walston, and Behar, 1976). A common pathogenesis, however, could not be clearly defined in most instances.…”

“…A common pathogenesis, however, could not be clearly defined in most instances. Most interesting were the described associations first with conditions that increase left ventricular afterload (Bloom et al, 1975;Harrison et al, 1977;Raj et al, 1979), and second with hypercalcemia and valvular calcification (Harrison et 01., 1977;Kronzon and Glassman, 1978;McFarland et al, 1978;Malcolm et al, 1978). Supravalvular aortic stenosis, a syndrome frequently related to hypercalcemia of infancy (Garcia et 01., 1964), was found in association with hypertrophic subaortic stenosis in the patient to be reported.…”

Dynamic Left Ventricular Outflow Obstruction and Systolic Anterior Motion of the Mitral Valve Complicating Surgical Aortic Valve Replacement

“…12,14À20 Most cases occur after AVR for AS; however, cases have been reported in those presenting with aortic insufficiency. 12,21,22 An integrated mechanism including ventricular anatomy and the mitral apparatus has been described to explain the occurrence of dLVOTO across different patient types. 3À5,23 Two of the unifying features include a short distance between the mitral coaptation point and the ventricular septum (C-Sept distance <2.5 cm) and ventricular hypertrophy (>15 mm).…”

“…19,20,31 The reported incidence of dLVOTO after surgical AVR varies significantly depending on ventricular and mitral anatomy, and the hemodynamic conditions. 12,13,19,20,32,33 Its occurrence may be less likely in the immediate post-cardiopulmonary bypass period 32 than the postoperative period. 19,20 A 25% incidence of dLVOTO was reported, on average, 6 days after AVR with provocative studies using dobutamine or nitroprusside.…”