Congenital coronary artery obstruction associated with aortic anomalies in children: report of two cases.

Abstract

SUMMARY Coronary arterial obstruction associated with congenital aortic valve disease is rare in childhood. We studied two children with aortic valve disease and symptoms of coronary insufficiency. Cineangiography revealed localized obstruction of the proximal left coronary artery. In one patient, a membrane-like structure partially covered the left sinus of Valsalva, almost in our clinic soon afterward. There was a systolic thrill over the base of the heart that radiated to the neck, corresponding to a grade IV/VI aortic ejection murmur. This was followed by a grade I/VI high-pitched diastolic murmur of aortic regurgitation; no click was heard and the remainder of the physical examination was normal. He had no clinical features of William's syndrome or symptoms in infancy to suggest hypercalcemia.The chest roentgenogram and the resting ECG were normal. The M-mode echocardiogram showed concentric hypertrophy of the left ventricular wall and fine systolic vibrations of the aortic valve leaflets with mild early semiclosure of the anterior aortic valve leaflet. The left ventricular shortening fraction was 44%.A treadmill exercise test (2.5 mph, 12% grade) was abnormal, with 3-4 mm of ST-segment depression after 4 minutes of exercise ( fig. 1). Cardiac catheterization revealed a 40-mm Hg peak systolic gradient across the aortic valve and 2+ aortic insufficiency. Aortic root angiography showed an abnormal membrane-like structure that partially isolated the left coronary sinus ( fig. 2)

Misdiagnosis in Hypoplastic Sinus of Valsalva with Localized Aneurysm: A Diagnostic Pitfall in Computed Tomography

“…The most common congenital structural abnormality in the sinus of Valsalva is a diffusely dilated aneurysm, which is caused by a localized weakness at the junction of the aortic media and the annulus fibrosus cordis. Patients with a hypoplastic sinus of Valsalva are rarely reported; they are often young and present with angina or sudden death because of insufficient coronary blood flow . Our middle‐aged patient presented with minor symptoms because her smaller left aortic sinus provided sufficient diastolic coronary blood flow.…”

“…Patients with a hypoplastic sinus of Valsalva are rarely reported; they are often young and present with angina or sudden death because of insufficient coronary blood flow. [1][2][3][4] Our middle-aged patient presented with minor symptoms because her smaller left aortic sinus provided sufficient diastolic coronary blood flow. Moreover, although the outpouching morphology of the left aortic sinus indicated a localized sinus of Valsalva aneurysm, surgical correction was not necessary for the small size and functionally normal sinus of Valsalva.…”

“…LVOT = left left ventricular outow tract; NCS = noncoronary aortic sinus; NC = noncoronary cusp; R = right aortic sinus; RA = right atrium; RCA = right coronary artery; RV = right ventricle. Other abbreviations see captions forFigures 1,2.…”

Native aorto-ostial coronary lesions on CT coronary angiogram

“…AOLs may also be intrinsic from ostial hypoplasia/atresia, be consequent to circumferential sphincter-like ostial muscle or be caused by an ostial ridge 3 . More rarely, aortic valve anomalies can compromise ostial flow, as may be seen in aortic cusp hypoplasia and with supravalvular aortic membranes 4 .…”

Entrapment of the Left Coronary Artery Ostium by the Aortic Valve Leaflet, Promoting Myocardial Ischemia

“…The patient is scheduled to have corrective operation. This case illustrates that entrapment of the left coronary artery ostium can be one of the causes of ischemic heart disease (1,2).…”