Tunnel subaortic stenosis: left ventricular outflow tract obstruction produced by fibromuscular tubular narrowing

Abstract

The clinical and morphologic features of tunnel subaortic stenosis, an unusual form of obstruction to left ventricular outflow, are described in 11 patients. Although patients with tunnel subaortic stenosis demonstrate a variety of cardiovascular malformations, the most characteristic anatomic feature is fibromuscular tubular narrowing of the outflow tract that remains relatively unchanged during the cardiac cycle. The aortic anulus was abnormally small in six of the 11 patients, including one who also had a hypoplastic ascending aorta. Evidence of a small mitral orifice was present in two patients, and two other patients had asymmetric septal hypertrophy. Although operation was successful in significantly reducing the outflow gradient in two of the seven operated patients, all seven patients had gradients of 50 mm Hg or more at the most recent postoperative evaluation. Three patients (two with previous operation) died suddenly; each of these patients had mild or no symptoms. Because of the apparent ineffectiveness of current operative methods in patients with tunnel subaortic stenosis, it is important to differentiate this condition from obstructions to left ventricular outflow.