Congenital absence of the left circumflex coronary artery in the systolic click syndrome.

Abstract

pREVIOUS STUDIES in our laboratories haveshown that patients with the systolic click syndrome exhibit a characteristic disorder of left ventricular contraction, with impaired shortening of the inflow tract and, in many instances, systolic expansion of the region of myocardium surrounding the mitral valve ring.1 The presence of a segmental myocardial abnormality appears to provide a plausible explanation for the occurrence of regional ECG abnormalities, as well as arrhythmias commonly observed in these patients in the absence of evidence of significant mitral valve dysfunction, and may also account for the late systolic timing of mitral incompetence in patients without obvious mitral leaflet abnormality. The possibility was considered that this segmental myocardial contraction disorder, in turn, might be related to an abnormality of the coronary circulation. Accordingly, the anatomy of the coronary circulation was examined by means of selective coronary arteriography

Prolapse of the Mitral Valve: Clinical Spectrum and Coronary Arterial Distribution

“…A recent study by G entzler et (il. [1] is at variance with the present findings. These investigators found an incidence of 89.5% of absence of any arterial supply to the posterior atrioventricular groove.…”

“…Recently, Gentzler et al [1] studied the anatomy of the coronary arterial circulation in 19 patients with the systolic click syndrome and prolapse of the mitral valve. In 17/19 patients, a single high marginal branch arose from the main left circumflex artery, and the atrioventri cular branch of the circumflex artery was absent.…”

Mitral valve prolapse in patients with coronary artery disease. Echocardiographic-angiographic correlation.

“…Jeresaty (Jeresaty, 1973) believes the association of two such common diseases to be coincidental rather than causative, since he saw only 4 instances of proven coronary artery disease in 45 patients with prolapse who underwent coronary arteriography. Other authors (Davis et al, 1971;Gooch et al, 1972;Liedtke et al, 1973;Scampardonis et al, 1973;Gentzler et al, 1975). have documented coronary artery disease in a small percentage (2 to 3%) of their patients with prolapsed mitral valve.…”

“…Cheng (1972) reported atherosclerotic disease ofthe right and left circumflex coronary arteries in 50 per cent and 17 per cent of his patients, respectively, who had angiographic evidence of the posterior mitral leaflet prolapse. Congenital absence of the left circumflex coronary artery AV branch, giving rise to segmental myocardial dysfunction, was noted by Gentzler et al (1975) in 90 per cent of their patients who had abnormalities of the mitral valve apparatus and non-ejection systolic clicks. None of their study group had significant occlusive disease involving the right coronary artery.…”

“…The association of mitral valve prolapse with obstructive coronary disease is recognised yet controversial (Davis et al, 1971;Steelman et al, 1971;Cheng, 1972;Gooch et al, 1972;Liedtke et al, 1973;Scampardonis et al, 1973;Jeresaty, 1973Jeresaty, , 1975Aranda et al, 1975;Gentzler et al, 1975). Some investigators (Cheng, 1972) maintain that coronary artery disease can produce mitral ballooning by virtue of papillary muscle ischaemia or fibrosis.…”