Cross-sectional echocardiographic detection of aortic obstruction. 2. Coarctation of the Aorta

Cross-sectional echocardiographic studies of the aortic arch and proximal descending aorta were performed in 18 patients with coarctation of the aorta and 20 normal subjects. In normals the aortic arch and proximal descending aorta appeared as an arcuate, echo-free structure curving across the plane of the scan. There were no localized changes in aortic diameter and the amplitude of aortic systolic pulsation was symmetrically maintained throughout the scan plane. Visualization of this region was possible in 16 of 18 patients with coarctation. In each of these cases there was a localized area of decrease in aortic diameter in the region of the left subclavian artery which corresponded to the angiographic appearance of the coarctation. In addition prominent systolic pulsation of the aortic arch proximal to the region of obstruction was evident. Cross-sectional echocardiography may offer a useful noninvasive method for direct visualization of aortic coarctation.     

Cross-sectional echocardiographic localization of sites of left ventricular outflow tract obstruction

Real time two-dimensional echocardiographic studies of left ventricular outflow tract cross-sectional anatomy were obtained by the multicrystal echocardiographic method (Bom system) in 35 patients with various types of outflow obstruction as delineated by clinical, hemodynamic and angiographic studies. In each patient the noninvasive test allowed prediction of the site of obstruction. In valvular aortic stenosis, echocardiographic diagnostic findings included poststenotic dilatation of the ascending aorta, thickened aortic cusp tissue and increased superior-inferior cusp excursion (doming). The site of supravalvular aortic stenosis was readily observed although echocardiographic findings often underestimated the degree of obstruction recorded at cardiac catheterization. In patients with discrete subvalvular aortic stenosis, the major finding was a localized thickening of the septum and anterior mitral anulus producing a narrowing of the left ventricular outflow tract that was present in diastole and persisted throughout the cardiac cycle. The combination of discrete subvalvular and valvular aortic stenosis could be identified as well as mitral valve abnormalities associated with left ventricular outflow tract obstruction. Asymmetric septal hypertrophy and systolic anterior motion of the mitral leaflets were noted in six patients with idiopathic hypertrophic subaortic stenosis. This new echocardiographic approach allowed accurate localization of the site of left ventricular outflow tract obstruction and detection of associated malformations. The method has substantial merit as an initial test to establish diagnosis and allows more appropriate planning of a subsequent hemodynamic study.     

Preoperative prediction of prosthesis size using cross sectional echocardiography in patients requiring aortic valve replacement.

In 43 patients who underwent aortic valve replacement for aortic stenosis with or without regurgitation the accuracy of preoperative left ventricular angiography, parasternal long axis cross sectional echocardiography of left ventricular outflow tract and proximal ascending aorta, and M mode echocardiography of aortic root in predicting aortic root size and thereby prosthesis size was compared. Cross sectional echocardiographic measurements and angiographic measurements of aortic root correlated well with prosthesis size, with over two thirds of the indirect measurements being within 2 mm of prosthesis diameter. M mode echocardiography did not yield useful predictive information. Non-invasive preoperative evaluation of patients likely to require aortic valve replacement may be usefully extended to include aortic root dimensions measured by cross sectional echocardiography.     

Results of surgery for congenital supravalvular aortic stenosis.

Of eight children aged 3 to 15 years with surgical correction of severe supravalvular aortic stenosis, 6 were evaluated 7 to 44 months later by repeat cardiac catheterization and aortography. Prosthetic patch angioplasty was performed in all cases. Preoperative systolic gradients ranged from 40 to 90 mm Hg (average 70); postoperative gradients ranged from 0 to 20 mm Hg (average 11). The postoperative anglographic appearance of the ascending aorta was near normal in all six patients, and none had new aortic valve insufficiency. These results of surgery for supravalvular aortic stenosis are judged to be excellent.     

Cross sectional echocardiographic assessment of the aortic root and coronary ostial stenosis in familial hypercholesterolaemia.

Aortic root abnormalities (atherosclerotic thickening and obstruction) seen at necropsy may readily be detected by aortography in familial hypercholesterolaemia. We studied 35 patients with familial types IIa and IIb hyperlipoproteinaemia including three homozygotes and 32 heterozygotes. Two homozygotes showed abnormal bright echoes (atheroma) encircling the proximal aortic root, which interfered with full excursion of the aortic cusps. One homozygote showed the typical echocardiographic features of supravalvular aortic stenosis at the superior border of the sinus of Valsava with normal aortic cusps. Cardiac catheterisation showed valvular gradients of 15 and 80 mm Hg in two homozygotes and a supravalvular gradient of 40 mm Hg in the third. Left coronary artery ostial stenosis was identified by echocardiography in all three homozygotes. Echocardiographic measurements of the aortic root in the 32 heterozygotes were similar to the control group, but 10 patients showed abnormal bright echoes within the aortic cusps and four had supravalvular changes similar to, but less severe than, the homozygotes. In one severely heterozygote supravalvular atheroma prevented full aortic cusp excursion, and this finding was confirmed during coronary artery bypass surgery.     

Supravalvular aortic stenosis. A 20-year clinical perspective and experience with patch aortoplasty

Supravalvular aortic stenosis has a wide range of clinical and morphologic expression. Since 1961, 25 patients (aged 1 to 49 years) with documented supravalvular aortic stenosis have been evaluated. Seven (28%) had Williams' syndrome, 5 (20%) had a familial form of supravalvular aortic stenosis, and 13 (52%) had a sporadic form. A blood pressure difference of greater than 10 mm Hg between the arms was noted in 65% of the patients. Angiographically, 19 (76%) had segmental supravalvular narrowing; 6 (24%) had diffuse narrowing of the ascending aorta. Sixteen patients underwent patch aortoplasty. At surgery, portions of the aortic valve cusps were frequently attached to supravalvular tissue. This "cusp tuck" resulted in distinctive angiographic features and influenced the results of corrective surgery. Three surgical deaths occurred in the early 1960s-2 with diffuse narrowing of the aorta. Of the remaining 12 patients, followed for 1 to 12 years, 10 are asymptomatic, 1 has angina, and 1 died from cancer. All 8 patients who underwent postoperative catheterization had a thick band between the left and right coronary sinus which represented persistent attachment of portions of the aortic valve cusps to residual supravalvular tissue (cusp tuck). This resulted in aortic valvular gradients (23 to 48 mm Hg) in 4 patients and aortic valvular insufficiency in 2 patients. No significant supravalvular gradient was noted. The 20-year experience with supravalvular aortic stenosis reported herein emphasizes a wide range of clinical and morphologic expression, the benefits and limitations of patch aortoplasty, and the importance of postoperative cardiac catheterization, and furthers the understanding of a complex clinical syndrome.     

Congenital aortic supravalvular stenosis. Frequency and results of surgical treatment in a third level hospital

In a prospective study made between 1 September 1996 and 31 December 2001, pediatric patients with a diagnosis of supravalvular aortic stenosis confirmed by a reduction in the aortic inner diameter and a gradient > or = 50 mmHg were detected. Of 83 patients with aortic stenosis, only 7 (8.4%) had supravalvular aortic stenosis. All 7 patients underwent surgical treatment consisting of resection of fibrous tissue and reconstruction of the ascending aorta with a preclotted Dacron patch. One patient with severe, diffuse stenosis died and the another had perioperative heart failure, cardiac arrest and reversible neurological sequelae. A significant decrease in the postoperative gradient was obtained (p < 0.05). At present all surviving patients are free of symptoms. It was concluded that supravalvular aortic stenosis is infrequent in Mexico. In our experience, surgical treatment produced good results and success depended on the magnitude and type of stenosis.     

Supravalvular and valvular aortic stenosis in homozygous familial hypercholesterolemia

Premature atherosclerosis with a particular predilection to the coronary arteries is a well-known complication of homozygous familial hypercholesterolemia. However, well documented involvement of the aortic root and valve with aortic stenosis has not been recognized frequently antemortem. Clinical, echocardiographic, hemodynamic and angiographic features of a case with valvular and supravalvular aortic stenosis secondary to homozygous familial hypercholesterolemia are described. Surgical relief of the left ventricular outflow obstruction was not attempted because of the patient's refusal.     

Spectrum of findings in a family with nonsyndromic autosomal dominant supravalvular aortic stenosis: a Doppler echocardiographic study

Nonsyndromic familial supravalvular aortic stenosis is an autosomal dominant disorder. However, for many reported families, systematic study of all family members with echocardiographic or hemodynamic techniques has not been performed and degree of penetrance has not been assessed. The supravalvular stenosis in these family members usually is not associated with mental retardation or other characteristics of Williams syndrome. Although some believe that autosomal dominant supravalvular aortic stenosis is part of the spectrum of Williams syndrome, others believe that these are separate entities. Doppler echocardiograms were analyzed on 23 members of a 34 member family with several known to have supravalvular aortic stenosis; 20 studies were performed by the authors and 3 were done elsewhere and made available for review. No family member had mental retardation, characteristic facies or other findings of Williams syndrome. Three of the 34 had supravalvular aortic stenosis requiring surgery. Of 22 members examined echocardiographically who had not had prior surgical repair, 13 had supravalvular aortic stenosis. Echocardiographic findings ranged widely, from calcification of the ascending aorta in a 71 year old man with minimally increased flow velocity (1.7 m/s) to mild narrowing with mildly increased flow velocity in six members to significant narrowing with impressively increased flow velocity (2 to 4 m/s) in seven. In addition, four patients had mild narrowing of pulmonary artery branches and eight had peak pulmonary artery flow velocity above normal. This study demonstrates complete penetrance with extremely variable expression in this family with autosomal dominant supravalvular aortic stenosis and emphasizes the importance of using echocardiographic techniques in studying the family members who are suspected of having an inherited cardiovascular disease.     

Supravalvular aortic stenosis: repair with the Yacoub procedure

Preservation and restoration of the aortic valve and root, as well as relief of the stenosis, is of paramount importance during surgical treatment of supravalvular aortic stenosis. A 23-year-old woman with a history of medically treated endocarditis presented with progressive fatigue and dyspnea on exertion. A diagnostic work-up revealed multiple stenoses and a small saccular aneurysm involving the diffusely hypoplastic ascending aorta, in addition to severe mitral regurgitation. The aortic root and ascending aorta were replaced using the Yacoub remodeling technique. Hemiarch replacement was performed under hypothermic circulatory arrest, and the mitral valve replaced. By using a remodeling technique, it was possible to augment the left sinus of Valsalva, to obtain the largest diameter at the sinotubular junction, and to adjust the spatial relationship of commissures that renders good leaflet coaptation. Although extended aortoplasty has been used widely for supravalvular aortic stenosis, the Yacoub procedure is a good option in diffuse type hypoplasia with multiple stenosis of the ascending aorta.     

Cross-sectional echocardiographic recognition of interruption of aortic arch between left carotid and subclavian arteries.

Interrupted aortic arch is a rare condition, usually lethal in early infancy without treatment. The only characteristic feature on conventional non-invasive investigation is peripheral pulse inequality, which indicates ductal construction, and therefore may be absent or transient and preterminal. We report the cross-sectional echocardiographic findings in seven patients with aortic arch interruption between the left carotid and subclavian arteries. Their ages were 1 day to 7 months (median 7.5 days). The arterial connection was concordant in four, double outlet right ventricle in two, and truncus arteriosus in one. In each case the ascending aorta was small in comparison to the pulmonary trunk. From the suprasternal approach the ascending aorta could be seen to terminate in the left carotid artery, and the ductus to continue smoothly into the descending aorta, with no vestige of an aortic arch linking its ascending and descending portions. The left subclavian artery was seen to arise distal to the ductus in all but one patient. All four patients with ventriculoarterial concordance had pronounced subaortic stenosis caused by posterior displacement of the infundibular septum. Cross-sectional echocardiography therefore provides the only accurate method of non-invasive diagnosis of this condition. It permits early treatment with prostaglandins to prevent ductal closure, a planned approach to cardiac catheterisation, and a further means of investigating the nature of subaortic stenosis in this condition.     

Diagnosis of Supravalvular Aortic Stenosis by Transesophageal Echocardiography

The role of transesophageal echocardiography (TEE) in diagnosis of disorders of the thoracic aorta is well established. In this report the TEE findings in an adult patient with supravalvular aortic stenosis are presented. This showed narrowing of the ascending aorta just above the sinuses, due to fibromuscular thickening, causing an hour-glass shaped deformity. The excellent image quality obtained by TEE is far superior to transthoracic echocardiography. Coronary artery ostial obstruction a known association of supravalvular aortic stenosis can be caused by different mechanisms including adherence of the aortic valve leaflet to the ridge of obstructive muscle or premature atherosclerosis. TEE can define the mechanism of coronary artery ostial obstruction associated with supravalvular aortic stenosis.     

Echocardiographic features of supravalvular aortic stenosis.

A method for the echocardiographic detection of supravalvular aortic stenosis (SVAS) is described and the findings in a series of patients are presented. When compared to angiography, the echo tended to underestimate the severity of the supravalvular aortic obstruction. However, echocardiography appears to be a valuable, noninvasive method for detecting SVAS.     

Natural history of supravalvular aortic stenosis and pulmonary artery stenosis

Data from 35 patients with supravalvular aortic stenosis or pulmonary artery stenosis, or both, undergoing cardiac catheterization between 1973 and 1989 were analyzed retrospectively. Twenty-seven patients had supravalvular aortic stenosis: 11 required surgery after the first investigation and 8 (80%) of 10 others undergoing serial investigation showed an increase in the left ventricle to aorta pressure gradient. Angiographic measurements showed that the increase in the aortic pressure gradient was related to failure of normal growth of the ascending aorta lumen. Nineteen patients had pulmonary artery stenosis, with a right ventricular pressure greater than 33 mm Hg. At restudy, right ventricular pressure had decreased in 9 (82%) of 11 patients. This decrease in right ventricular pressure was associated with an increase in the systolic distensibility of the proximal pulmonary arteries, although there was no increase in the diastolic diameters. One patient had a rapid early increase in right ventricular pressure and no pulmonary artery growth. In two patients, multiple peripheral pulmonary artery stenoses became evident with time and produced persistent right ventricular hypertension. Supravalvular aortic stenosis is usually a progressive lesion, with an increase in left ventricular outflow tract pressure gradient related to poor growth of the ascending aorta. Pulmonary artery stenosis usually improves and only rarely limits prognosis.     

Supravalvular aortic stenosis. Echocardiographic features.

The echocardiographic manifestations of segmental supravalvular aortic stenosis are described in 2 patients. The diagnosis was confirmed by cardiac catheterization in both and at operation in 1. A systematic echocardiographic approach to such patients is described. The characteristic finding in these patients was the narrowing of the diameter of the aortic lumen at the stenotic area just distal to the aortic valve. As the transducer sweeps further cephalad the aortic lumen widens to a normal diameter. In one patient treated surgically, postoperative echogram demonstrated the narrowing to be reduced.     

Supravalvular aortic stenosis. Echocardiographic features.

The echocardiographic manifestations of segmental supravalvular aortic stenosis are described in 2 patients. The diagnosis was confirmed by cardiac catheterization in both and at operation in 1. A systematic echocardiographic approach to such patients is described. The characteristic finding in these patients was the narrowing of the diameter of the aortic lumen at the stenotic area just distal to the aortic valve. As the transducer sweeps further cephalad the aortic lumen widens to a normal diameter. In one patient treated surgically, postoperative echogram demonstrated the narrowing to be reduced.     

Supravalvular aortic stenosis. Report of clinical findings in 5 patients]

Five cases of supravalvular aortic stenosis (SAS) diagnosed by heart catheterization were studied in the Instituto Nacional de Cardiologia of Mexico. The clinic and laboratory data of interest of the differential diagnosis with other forms of obstruction of the left ventricle as follows: 1. Three cases had mental retardation and "elfin" face (SAS with specific psychophysical syndrome), the rest had a normal psyco-physical state without family antecedents (sporadic SAS). 2. The aortic focus was the epicenter of the expulsion murmur. In the phoncarodiographic study, two patients had protosistolic click and another had, in addition, a descending protodiastolic murmur (Int. I-IV). In the radial sphigmograms, one case had an amplitude difference in favor of the right side. 3. All had serum calcium figures within normal limits. 4. A chromosomatic analysis of preperipheral blood was performed on two patients, with normal results. 5. In the electrocardiogram, one case had right ventricular enlargement secondary to pulmonary arterial hypertension, due to stenosis of the main pulmonary arteries. 6. The radiologic study did not show dilatation of the ascending aorta and aortic bud in any case. 7. The angiocardiography showed: stenosis directly above the Valsalva sinuses; absence of dilatation or hypoplasia of the aorta above the stenosis; and the coronary network, indirectly opaqued, showed no abnormalities. One case had aortic coarctation and abnormal implantation of the right sublaviar artery, and another, stenosis of the right and left branch of its origen of the truncus of the pulmonary artery. The literature up to the present is reviewed and an anatomo-functional classification is proposed with the objective of including new varieties.     

The aetiology and course of isolated severe aortic regurgitation: a clinical, pathological, and echocardiographic study.

Seventy two consecutive patients with severe isolated aortic regurgitation were evaluated by preoperative echocardiographic and angiographic assessment of the aortic root. Biopsy specimens of the aortic wall were taken at operation. Two major groups of patients were found: those with cusp derangement but normal aortic roots and those with normal cusps but dilated aortic roots. Of the 42 cases of abnormal cusps, 20 were rheumatic, 15 were infective, and six were bicuspid. One patient had a tear in an otherwise normal cusp. Of the 30 cases of abnormal roots but normal cusps, six had inflammatory changes (syphilis, Reiter's disease, giant cell aortitis) and 24 had root dilatation caused by non-inflammatory destruction of elastic laminae. Echocardiographic measurement of the aorta at the level of the top of the commissures predicted the findings at pathology. In 37 of 39 patients with cusp disease the measurement was less than 37 mm. In 27 of 33 patients with root disease the measurement was greater than or equal to 37 mm. This difference was statistically significant. There was no difference in the sizes of the prosthesis used in each group, suggesting that it was the diameter of the junction of the aorta with the sinuses rather than the junction of the sinuses with the ventricle that was important in aortic regurgitation. Clinical progression in patients with non-inflammatory aortic root disease is slower than in patients with infective disease but faster than in those with rheumatic cusp disease.     

Isolated supravalvular aortic stenosis with left ventricular diverticulum and cleft mitral valve: Surgical repair in adulthood

Supravalvular aortic stenosis is an uncommon but well characterized congenital narrowing of the ascending aorta above the level of the coronary arteries. It can be a familial disorder, can occur sporadically, or can be associated with Williams syndrome. We are reporting a very rare presentation of supravalvular aortic stenosis with associated left ventricular diverticulum and cleft mitral valve. Repair consisted of resection of the ascending aorta, patch augmentation of the aortic root, and mitral valve repair. Follow-up echocardiography demonstrated normal mitral and aortic valve function and a postoperative three-dimensional computed tomographic scan showed a normal shape of the reconstructed ascending aorta.     

Degenerative aortic regurgitation

In view of the growing incidence of degenerative origin among the aetiologies of aortic regurgitation, we felt it interesting to report our experience of the surgical treatment of such patients. Out of 313 patients operated on for isolated chronic aortic regurgitation between 1974 and 1989, 102 (32.6%) had aortic regurgitation of degenerative origin and form the basis of this study. The patients were divided into group I (n = 48) without aneurysm of the ascending aorta and group II (54 patients) with anulo-aortic ectasia. The first group was further subdivided according to the diameter of the ascending aorta: in group Ia (23 patients) the aorta was normal with a root diameter of less than 40 mm, while in group Ib (25 patients) the aorta was enlarged (root diameter between 40 and 54 mm). There was no statistically significant difference between the pre-operative and operative parameters of patients in groups Ia and Ib. All underwent aortic valve replacement without associated surgery of the ascending aorta. During a mean follow-up of 58 months, five patients from group Ib had aortic dissection and in another three, an ascending aorta aneurysm developed. Reoperation was performed in six cases. No such complications occurred in group Ia. At 6 years, the proportion of patients free from complications related to the ascending aorta was 100% in group Ia vs 63 +/- 6% in group Ib (P less than 0.05). In group II, all the patients underwent aortic valve replacement. The modalities of repair of the ascending aorta differed with time.(ABSTRACT TRUNCATED AT 250 WORDS)