Supravalvular aortic stenosis and coronary ostial stenosis in homozygous familial hypercholesterolemia

Patients with homozygous familial hypercholesterolemia exhibit severe hypercholesterolemia, cutaneous and tendon xanthomata, and premature atherosclerosis from childhood. A rare presentation of this condition with supravalvular aortic stenosis and coronary ostial stenosis is described.     

Bilateral ostial coronary stenosis and rheumatic aortic valve stenosis

A 49-year-old patient presented with angina pectoris and clinical findings of aortic valve stenosis and regurgitation. Rheumatic aortic valve stenosis and regurgitation was diagnosed on echocardiography. Coronary angiography findings showed severe calcification in the aorta root with right coronary ostial occlusion, and were suggestive of left main ostial stenosis and proximal main stem stenosis, which was confirmed on CT angiography. Curvilinear calcification of the aorta was present on CT angiography. The findings suggested syphilitic aortitis. Syphilis serology was positive (RPR titre 1/16). The angina was caused by severe coronary ostial disease likely due to syphilitic aortitis and exacerbated by the rheumatic aortic valve stenosis and regurgitation.     

Bilateral ostial coronary stenosis and rheumatic aortic valve stenosis

A 49‐year‐old patient presented with angina pectoris and clinical findings of aortic valve stenosis and regurgitation. Rheumatic aortic valve stenosis and regurgitation was diagnosed on echocardiography. Coronary angiography findings showed severe calcification in the aorta root with right coronary ostial occlusion, and were suggestive of left main ostial stenosis and proximal main stem stenosis, which was confirmed on CT angiography. Curvilinear calcification of the aorta was present on CT angiography. The findings suggested syphilitic aortitis. Syphilis serology was positive (RPR titre 1/16). The angina was caused by severe coronary ostial disease likely due to syphilitic aortitis and exacerbated by the rheumatic aortic valve stenosis and regurgitation.     

Cross sectional echocardiographic assessment of great artery diameters in infants and children.

The pulmonary trunk and aortic root were measured on cross sectional echocardiograms in 173 normal subjects aged from one day to 15 years. Fifteen neonates were reexamined 3-6 days later. The great vessels were visualised in the parasternal long axis and short axis views. All measurements were made in end diastole and end systole by the leading edge method. The internal diameter (inner surface to inner surface) of the pulmonary trunk was also measured. The diameters of the great vessels correlated best with the square root of body surface area. Individual variability in cardiac growth gave a wide scatter of normal values. This was controlled for by calculating the ratio of the pulmonary trunk to aortic root for each subject. This ratio showed little individual variability and, except for the neonatal period, was remarkably constant throughout infancy and childhood (1.06 (0.06)). In the first 24 hours of life the ratio of the pulmonary trunk to the aortic root was significantly larger (1.29 (0.12)) but within one week it decreased to the "normal" ratio found in the older age groups. These normal data should be useful in assessing patients with congenital heart disease, particularly those in whom pulmonary blood flow is abnormal.     

Bilateral coronary ostial stenosis and aortic valvular disease after radiotherapy

A 44-year-old woman with a history of chest radiotherapy developedostial coronary artery disease associated with aortic valvulardisease and a lesion of the right internal mammary artery.     

Three cases of iatrogenic coronary ostial stenosis after aortic valve replacement.

BACKGROUND: Iatrogenic coronary ostial stenosis (ICOS) is a rare but potentially life-threatening complication of aortic valve replacement (AVR). This complication is usually diagnosed by angiography and treated with aortocoronary bypass surgery. CASE REPORTS: In the present 3 cases pre-operative coronary angiography confirmed normal coronary arteries and they underwent uncomplicated AVR. Coronary lesions were clinically manifest within 4 months after surgery, and repeat coronary angiography demonstrated bilateral ostial stenosis in 1 patient and left main trunk stenosis in the other 2. Two cases were detected by multidetector computed tomography (MDCT) before angiography. MDCT and Virtual Histology suggested fibrous tissue formation in the lesions. All 3 patients were successfully underwent percutaneous coronary intervention (PCI) and stenting. The post-procedure clinical course has been uneventful, except for elective stenting of a recurrent lesion in 1 asymptomatic patient. CONCLUSIONS: The incidence of ICOS after AVR is low. Noninvasive MDCT is useful for early diagnosis and PCI is a possible alternative treatment. ICOS may be caused by fibrous tissue formation, and therefore be distinct from conventional atherosclerosis.     

Intraoperative echocardiographic assessment of the severe isolated ostial stenosis of left main coronary artery before and after surgical patch angioplasty.

Isolated critical ostial stenosis of left main coronary artery is currently treated by conventional bypass surgery. Surgical patch angioplasty in an alternative surgical approach. Transesophageal echocardiography enables visualization of proximal branches of left and right coronary artery. The report describes intraoperative echocardiographic assessment of surgical left main coronary artery angioplasty.     

Cross sectional echocardiographic assessment of great artery diameters in infants and children

The pulmonary trunk and aortic root were measured on cross sectional echocardiograms in 173 normal subjects aged from one day to 15 years. Fifteen neonates were reexamined 3-6 days later. The great vessels were visualised in the parasternal long axis and short axis views. All measurements were made in end diastole and end systole by the leading edge method. The internal diameter (inner surface to inner surface) of the pulmonary trunk was also measured. The diameters of the great vessels correlated best with the square root of body surface area. Individual variability in cardiac growth gave a wide scatter of normal values. This was controlled for by calculating the ratio of the pulmonary trunk to aortic root for each subject. This ratio showed little individual variability and, except for the neonatal period, was remarkably constant throughout infancy and childhood (1.06 (0.06)). In the first 24 hours of life the ratio of the pulmonary trunk to the aortic root was significantly larger (1.29 (0.12)) but within one week it decreased to the "normal" ratio found in the older age groups. These normal data should be useful in assessing patients with congenital heart disease, particularly those in whom pulmonary blood flow is abnormal.     

Severe syphilitic aortic regurgitation with bilateral critical coronary ostial stenosis

A forty-year-old male with syphilitic severe aortic regurgitation and critical bilateral coronary ostial stenosis, proved by cardiac catheterization and angiocardiography, is presented. He underwent successful aortic valve replacement and coronary artery bypass grafting with gratifying results.     

Color-guided Doppler echocardiographic assessment of aortic valve stenosis

The severity of valvular aortic stenosis was assessed by Doppler color flow mapping in 100 consecutive patients who underwent successful cardiac catheterization within 2 weeks of the Doppler study. The maximal width of the aortic stenosis jet seen in 61 of these patients (Group A) was measured at the aortic valve. Color-guided continuous wave Doppler examination was used to measure the mean transaortic pressure gradient, and the aortic valve area was estimated using the simplified continuity equation. The aortic stenosis jet was not seen in 39 patients (Group B), and the mean pressure gradient and aortic valve area in these patients were assessed by conventional Doppler echocardiography alone. The mean pressure gradient obtained by continuous wave Doppler study and cardiac catheterization in the 61 Group A patients correlated well (r = 0.90); the correlation was lower in the 39 Group B patients (r = 0.70). The overall correlation for the combined Groups A and B was good (r = 0.82). The aortic valve area estimated by continuous wave Doppler study and cardiac catheterization in 54 Group A patients correlated well (r = 0.92); the correlation in 22 Group B patients was lower (r = 0.71). The correlation for all 76 patients (Groups A and B) was good (r = 0.80). The maximal aortic stenosis jet width also correlated well with the aortic valve area estimated at catheterization in 54 patients (r = 0.90). Group C represented an additional 14 patients in whom the left ventricle could not be entered during cardiac catheterization.(ABSTRACT TRUNCATED AT 250 WORDS)     

Extent and severity of atherosclerotic involvement of the aortic valve and root in familial hypercholesterolaemia

OBJECTIVE: To compare the frequency of valvar and supravalvar aortic stenosis in homozygous and heterozygous familial hypercholesterolaemia (FH). DESIGN: Analysis of life time cholesterol exposure and prevalence of aortic atherosclerosis in 84 consecutive cases attending a lipid clinic. SETTING: A tertiary referral centre in London. PATIENTS: Outpatients with FH (six homozygous, 78 heterozygous). INTERVENTIONS: Maintenance of lipid lowering treatment. MAIN OUTCOME MEASURES: Calculated cholesterol x years score (CYS) and echocardiographic measurement of aortic root diameter, aortic valve thickness, and transaortic gradient. RESULTS: Four homozygotes with a mean (SD) CYS of 387 (124) mmol/1 x years had severe aortic stenosis (treatment started after seven years of age), whereas the other two had echocardiographic evidence of supravalvar thickening but no aortic valve stenosis (treatment started before three years of age). On multivariate analysis, mean transaortic gradient correlated significantly with CYS (mean = 523 (175) mmol/1 x years) in heterozygotes (p = 0.0001), but only two had severe aortic valve and root involvement. CONCLUSIONS: In patients with familial hypercholesterolaemia, aortic stenosis is common in homozygotes, and aortic root involvement is always present despite the lower CYS than in heterozygotes. It appears to be determined by short term exposure to high cholesterol concentrations in early life. Conversely, aortic root and valve involvement are rare in heterozygotes and occur only with severe, prolonged hypercholesterolaemia, possibly accelerating age related degenerative effects.     

Three-dimensional transthoracic echocardiographic assessment of aortic stenosis and regurgitation

Color-guided continuous-wave Doppler has important limitations in the assessment of aortic stenosis (AS) and aortic regurgitation (AR). This article outlines the limitations of conventional echocardiographic methods and describes the three-dimensional echocardiographic assessment of AS and AR.     

Cross sectional and Doppler echocardiographic evaluation of aortopulmonary shunts.

BACKGROUND--Shunt vessels were imaged and shunt flow was analysed by cross sectional and Doppler echocardiography in 12 patients who had had 14 shunt procedures (nine left Blalock-Taussig shunts, three right Blalock-Taussig shunts, one modified Waterston shunt, and one central shunt). METHODS--The shunt vessels were classified by echocardiography as uniformly patent, segmentally stenosed, and uniformly stenosed. These findings were compared with those of angiography. Also the peak flow velocities at the aortic and the pulmonary ends of the shunt vessels were measured by Doppler echocardiography and the ratio of these values was calculated for each shunt. RESULTS--Twelve (85.7%) of 14 shunt vessels were imaged along their entire length by cross sectional echocardiography. The two remaining shunt vessels were only partially imaged. In 10 patients who also had angiography the echocardiographic and angiographic images of the shunt vessels were identical. The ratio of the peak flow velocity measured at the aortic and the pulmonary ends of the shunt vessel was significantly larger in the segmentally stenosed shunt vessels than in the uniformly patent shunt vessels (p < 0.001). The ratio in the two shunt vessels only partially imaged by cross sectional echocardiography indicated that they were segmentally stenosed. CONCLUSION--The combination of cross sectional and Doppler echocardiography may be useful for determining either the patency or the morphology of an aortopulmonary shunt.