Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “BICUSPID”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 775 records · Page 43Linked to original sources
PubMed2004

Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD).

OBJECTIVES: The goal of this study was to better characterize the young patient with aortic dissection (AoD). BACKGROUND: Aortic dissection is unusual in young patients, and frequently associated with unusual presentations. METHODS: Data were collected on 951 patients diagnosed with AoD between January 1996 and November 2001. Two categories of patients, <40 years and >or=40 years, were compared using chi-square cross tabulations for categorical and Student t test for continuous data. RESULTS: Sixty-eight patients (7%) with AoD were <40 years of age. Compared with patients >or=40 years, younger patients were less likely to have a prior history of hypertension (p < 0.05); however, younger patients were more likely to have Marfan syndrome, bicuspid aortic valve, and prior aortic surgery (all, p < 0.05). Clinical presentations in the two age groups were similar; however, younger patients were less likely to be hypertensive (25% vs. 45%, p = 0.003). The proximal aortas of young AoD patients were larger (all, p < 0.05) compared with older patients. These differences in aortic size between age groups were not entirely related to Marfan syndrome. Mortality among young patients was similar to patients >or=40 years of age (22% vs. 24%, p = NS), irrespective of the site of dissection. CONCLUSIONS: Compared with older patients with AoD, young patients have unique risk factors for dissection: Marfan syndrome, bicuspid aortic valves, and larger aortic dimensions. Surprisingly, the mortality risk for young AoD patients is not lower than older AoD patients.

Adult↗
PubMed2004

Risk factors for aortic complications in adults with coarctation of the aorta.

OBJECTIVES: We sought to determine the prevalence and predisposing condition for aortic wall complications in adults with either repaired or non-repaired coarctation of the aorta. BACKGROUND: Aortic wall complications may develop in adults with coarctation of the aorta, despite successful surgical repair in childhood. METHODS: A total of 235 adults with coarctation (mean age 27 +/- 13 years) were retrospectively reviewed. Treatment had been performed by surgery in 181 patients (group I) or by balloon angioplasty or stenting in 28 patients (group II). No previous intervention had been carried out in 26 patients with mild coarctation at diagnosis (group III). RESULTS: Forty-four aortic wall complications were found in 37 patients (16%). There were no differences among the three groups with respect to total complications (15%, 18%, and 15%, respectively), ascending aortic aneurysms (9%, 11%, and 12%), or descending aortic aneurysms (4% in all three groups). Multivariate analysis did not show a significant relationship between previous repair, type of repair, age at repair, residual Doppler pressure gradient, or systemic hypertension and the occurrence of aortic complications. Only aging (risk ratio [RR] 1.4 per decade of age, 95% confidence interval [CI] 1.1 to 1.8, p = 0.002) and bicuspid aortic valve (RR 3.2, 95% CI 1.3 to 7.5, p = 0.005) were significantly related to these complications. CONCLUSIONS: Aortic wall complications are frequent in adults with coarctation of the aorta beyond that attributable to associated hemodynamic derangement or previous repair. The only independent risk factors appear to be advanced age and bicuspid aortic valve.

Adolescent↗
PubMed2006

Evaluation of downsized homograft conduits for right ventricle-to-pulmonary artery reconstruction.

OBJECTIVE: Although homograft conduits are frequently used to establish right ventricle-to-pulmonary artery continuity, the limited availability of small-size homografts is a significant constraint in pediatric cardiac surgery. We compared the performance of standard homograft conduits with that of surgically reduced bicuspid homograft conduits in patients undergoing repair of truncus arteriosus. METHODS: Forty infants undergoing complete repair of truncus arteriosus with either standard homografts (n = 26) or reduced-size bicuspid homografts (n = 14) were evaluated. RESULTS: The median downsized conduit diameter (13 mm) was similar to the standard homograft diameter (12 mm, P = .52). There were 6 early deaths and 5 late deaths, representing an overall 30-day mortality of 15% and a 5-year mortality of 25%. No deaths were directly related to homograft dysfunction. Four (29%) downsized conduits and 8 (31%) standard conduits required replacement at a median interval of 18.5 months and 42.4 months, respectively. Catheter-based interventions were required in 5 (36%) patients in the downsized group and in 3 (12%) patients in the standard group. There was no difference in freedom from surgical or catheter-based reintervention between the 2 groups (P = .42). Freedom from conduit failure (severe conduit stenosis, moderate or greater regurgitation) was 55.9% and 17.2% at 3 years in the downsized and standard groups, respectively. CONCLUSION: The surgically downsized homograft is an excellent option when an appropriate-sized homograft is not available and might prevent morbidity associated with the use of an oversized conduit.

Anastomosis, Surgical↗
PubMed1997

Two-dimensional echocardiographic assessment of the progression of aortic root size in 127 patients with chronic aortic regurgitation: role of the supraaortic ridge and relation to the progression of the lesion.

Although aortic root dilation has etiologic and prognostic significance in patients with chronic aortic regurgitation (AR), no information is available regarding changes over time in aortic root size in patients with the entire spectrum of AR severity or how such changes relate to progression of the AR or to left ventricular (LV) overload. To analyze this, a total of 127 patients with chronic AR who had more than 6 months of follow-up by two-dimensional and Doppler echocardiography were included in the study (69 men and 58 women; mean age 59.3 +/- 21.2 years [range 14 to 94 years]; 67 cases of mild, 45 moderate, 15 severe, and 21 bicuspid aortic valve disease). The aortic anulus, sinuses of Valsalva, supraaortic ridge, and ascending aorta were measured in the parasternal long-axis view, LV volumes were calculated (biplane Simpson's approach), and the severity of AR was quantified based on proximal jet size and graded according to an algorithm that takes into account major color Doppler criteria. At entry to the study, significant differences between patients with mild, moderate, and severe AR were noted only in supraaortic ridge size (1.46 +/- 0.29 cm/m2 vs 1.63 +/- 0.33 cm/m2 [p < 0.006]; vs 1.67 +/- 0.43 cm/m2 [p < 0.03]). A significant increase in aortic root size at all levels was observed during the follow-up period in all three groups of severity of AR. The rate of change of the supraaortic ridge, the upper support structure of the anulus and cusps, was faster in patients with more severe degrees of AR (p = 0.013); this was not the case at the other aortic levels. No differences were observed in aortic root size or rate of progression between patients with bicuspid or tricuspid aortic valves. Patients were considered "progressive" if they lay on the steepest positive segment of the curve representing the rank order in the rate of aortic root progression. Compared with "nonprogressive" patients, patients who were progressive in supraaortic ridge size (rate >0.12 cm/yr; n = 23) had a faster rate of progression in the degree of regurgitation as assessed by the regurgitant jet area/LV outflow tract area ratio measured in the parasternal short-axis view (0.48 +/- 0.45 vs 0.24 +/- 0.5/yr; p < 0.03) and a foster rate of progression of LV end-diastolic volume (30 +/- 22.8 vs 14.4 +/- 15.6 ml/yr; p < 0.0002) and LV mass (70.8 +/- 74.4 vs 16.8 +/- 19.2 gm/yr; p < 0.0004). In conclusion, there is progressive dilation of the aortic root at all levels, even in patients with mild AR. More rapid progression in aortic root size is associated with more rapid progression of the underlying aortic insufficiency, as well as more rapid increases in LV volume and mass.

Adolescent↗
PubMed2000

Ruptured sinus of valsalva aneurysm: early recurrence and fate of the aortic valve.

BACKGROUND: We reviewed our experience with congenital ruptured sinus of Valsalva aneurysms (RSVA) to determine patterns of early recurrence and the fate of the aortic valve (AV). METHODS: Over a 28-year period, RSVA was identified in 34 patients, (mean age 31.6 years). Primary closure of the RSVA was performed in 10 patients, and a patch employed in 24. Aortic insufficiency was present in 24 patients. AV replacement (AVR) was performed in 5 patients; AV repair in 6. RESULTS: Follow-up of 9.2 +/- 8.3 years (6 months to 24 years) was complete in all but 2 patients. Five early fistula recurrences (in 4 patients) correlated with primary rather than patch closure (p < 0.03). Kaplan-Meier survival at 10 years is 90 +/- 7%. Freedom from reoperative AVR at 10 years is 83 +/- 9%. Late AVR was performed in 6 patients for progressive aortic insufficiency due to bicuspid valve (n = 3), cusp disease of affected sinus (n = 2), or aortic root dilatation (n = 2). CONCLUSIONS: Patch closure of the RSVA should be routinely employed. A bicuspid valve may be associated with the late need for AVR.

Adolescent↗
PubMed2002

Correction of leaflet prolapse in valve-preserving aortic replacement: pushing the limits?

BACKGROUND: For aortic dilatation with morphologically intact leaflets, valve-preserving aortic replacement has become an accepted treatment modality. Leaflet prolapse, however, may be present, making composite replacement the most frequent choice. Alternatively, valve preservation may be combined with correction of leaflet prolapse. The results of this approach should be comparable with those of valve-preserving aortic surgery in the presence of normal leaflets. METHODS: Between 1995 and 2002, 156 patients were treated by valve-preserving surgery. The aortic valve was bicuspid in 46, and tricuspid in 110 instances. In 88 aortic valves, apparently normal leaflet coaptation (normal, 12 bicuspid and 76 tricuspid), and in 68 instances, prolapse of one or more leaflets, was observed. Root remodeling (n = 133) or aortic replacement with valve reimplantation (n = 23) were performed. Leaflet prolapse was corrected by triangular resection (n = 16) or plicating sutures (n = 59), mostly placed in the central portion of the leaflet. RESULTS: Neither operative mortality nor 5-year survival were influenced by the additional correction of prolapse. Freedom from reoperation at 1 year (normal, 98.8%; prolapse, 96.5%) and 5 years (normal, 97.3%; prolapse, 96.5%) were comparable in both cohorts, as was freedom from aortic regurgitation > or = II at 1 year (normal, 98.8%; prolapse, 94.2%) and 5 years (94.4%). CONCLUSIONS: Surgical correction of leaflet prolapse in combination with proximal aortic replacement is feasible with good results. Midterm results are identical with those known for morphologically normal leaflets. Repair of prolapse allows for preservation of the native valve in most patients with aortic regurgitation and aortic pathology, and thus appears a beneficial addition to valve-preserving surgery.

Adolescent↗
PubMed1999

Aortic valve sparing operations: an update.

BACKGROUND: Aortic valve sparing operations in patients with ascending aorta and/or aortic root aneurysms have been performed for a decade in our institution. Initially only patients with normal aortic valve leaflets had these operations, but more recently we utilized them in patients with prolapse of a single leaflet and in those with a bicuspid aortic valve. This article is an update on the clinical results of these operations. METHODS: From May 1988 to December 1997, 126 patients with ascending aorta and/or aortic root aneurysms and aortic insufficiency underwent replacement of the ascending aorta with reconstruction of the aortic root and preservation of the native aortic valve. There were 85 men and 41 women, with a mean age of 54 years (range, 14 to 84). Thirty-two patients had the Marfan syndrome; 17 patients had acute and 10 had chronic type A aortic dissection; 23 had a transverse arch aneurysm; 26 had coronary artery disease, and 8 had mitral regurgitation. The aortic valve sparing operation consisted of simple adjustment of the sinotubular junction in 33 patients, adjustment of the sinotubular junction and replacement of one or more aortic sinuses in 60, and reimplantation of the aortic valve in a tubular Dacron (C.R. Bard, Haverhill, PA) graft in 33. Fifteen patients also had repair of aortic leaflet prolapse. Only 4 patients had a bicuspid aortic valve. RESULTS: There were 3 operative deaths due to cardiac failure. Patients were followed from 2 to 117 months, with a mean of 31. There were 11 late deaths: 7 cardiovascular and 4 from unrelated causes. The actuarial survival was 72 +/- 8% at 7 years. Two patients required aortic valve replacement; the freedom from aortic valve replacement was 97 +/- 2% at 7 years. Doppler echocardiography revealed absent, trivial or mild aortic insufficiency in most patients; only 9 patients had moderate aortic insufficiency. CONCLUSIONS: Aortic valve sparing operations are feasible in most patients with ascending aorta and/or aortic root aneurysms who have normal or near normal aortic leaflets. The functional results of the repaired aortic valve are excellent, and the repair appears to be durable.

Adolescent↗
PubMed2003

Targeted expression of androgen receptors in tooth-forming tissues of a neotropical salamander (Bolitoglossa schizodactyla) enables region-specific formation of dimorphic types of teeth in the male.

Bicuspid teeth are a morphological characteristic in adult metamorphosed urodeles. However, during the mating season the males of many species of plethodontid salamanders (Urodela, Plethodontidae) from northern America bear large monocuspid teeth on their jaws. Since androgen levels are elevated during the mating season, it is likely that the formation of monocuspid teeth is controlled by androgens. However, many plethodontid salamanders from southern Central America live in habitats which lack significant seasonal changes and therefore are no seasonal breeders. These males constantly bear monocuspid teeth, however their presence is restricted to the premaxillary bone, which forms the most frontal element of the upper jaw. Teeth of all other dentigerous bones are bicuspid. This study is based on an immunohistochemical localisation of androgen receptors and reveals a differential expression of androgen receptors in the three separate dental laminae of the upper jaw of Bolitoglossa schizodactyla (Urodela, Plethodontidae) from western Panama. Our findings suggest that the restriction of androgen receptor expression to the premaxillary dental lamina of the male and its absence in all other tooth-forming tissues leads to the differences in tooth morphogenesis. In conclusion, a differential and cell-specific expression of androgen receptors in urodeles-similar to the situation in mammalian species-allows a selective response of differentiating tissues to the hormonal status of the animal.

Animals↗
PubMed1998

Congenital heart disease in patients with Turner's syndrome. Italian Study Group for Turner Syndrome (ISGTS).

OBJECTIVE: There is a high prevalence of congenital heart defects in patients with Turner's syndrome. Few studies have reported echocardiographic data in unselected patients according to the different chromosomal patterns. The aim of our study was to evaluate a large series of patients with Turner's syndrome, comparing these data with those of the general population. METHODS: Five hundred ninety-four patients with Turner's syndrome, aged 1 month to 24 years, in the Italian Study Group for Turner Syndrome underwent full cardiologic evaluation. Karyotype distribution was: 45,X (54%), X-mosaicism (13%), and X-structural abnormalities (33%). RESULTS: The prevalence of cardiac malformations was 23%. Bicuspid aortic valve (12.5%), aortic coarctation (6.9%), and aortic valve disease (3.2%) were the most prevalent malformations. In comparison with the general population, partial anomalous pulmonary venous drainage had the highest relative risk. A correlation was found between type of congenital heart defect and karyotype. The patients with 45,X karyotype had the greatest prevalence of partial anomalous pulmonary venous drainage and aortic coarctation, whereas bicuspid aortic valve and aortic valve disease were more common in the patients with X-structural abnormalities. The patients with severe dysmorphic signs showed a significantly higher relative risk of cardiac malformations. CONCLUSION: X-linked factors may be involved in determining cardiac defects in Turner's syndrome.

Adolescent↗
PubMed2003

The influence of mechanical properties on wall stress and distensibility of the dilated ascending aorta.

OBJECTIVES: We sought to determine how intrinsic mechanical properties of dilated ascending aorta influence in vivo distensibility and wall stress, potential contributing factors to the risk of aortic rupture and dissection. METHODS: Fresh tissue from patients undergoing ascending aortic replacement for Marfan syndrome (n = 8, age 38 +/- 14 years), bicuspid aortic valve with associated aneurysm (n = 20, age 55 +/- 12 years), or idiopathic degenerative aneurysm (n = 7, age 64 +/- 9 years) was analyzed for elastic properties and circumferential residual stress. Histologic specimens were graded for elastin fragmentation, cystic changes, fibrosis, and necrosis. Distensibility and wall stresses were predicted with a cylindrical mathematical model of the aorta. RESULTS: Elastic properties of aortic tissue were nonlinear and extensibility decreased with age. Moderate or severe elastin fragmentation was prevalent in tissue from patients with the Marfan syndrome and more common in tissue from younger patients. Model-predicted mean circumferential stress did not vary significantly with age or patient group, but increased with aortic lumen diameter and systolic blood pressure. Model-predicted distensibility decreased with age in bicuspid aortic valve and degenerative aneurysm groups. Differences in distensibility were correlated with differences in elastic properties. CONCLUSIONS: The age dependence of elastic properties of dilated ascending aorta is consistent with that of normal aorta observed in previous studies. Increases in mean circumferential stress with blood pressure and diameter support the clinical importance of blood pressure control and serial evaluation of aortic diameter. Declining wall strength with age may increase the risk of rupture or dissection.

Adolescent↗
PubMed1985

Surgical pathology of combined aortic stenosis and insufficiency: a study of 213 cases.

The gross surgical pathologic features of the aortic valve were reviewed in 213 patients who had had clinically combined aortic stenosis and insufficiency and aortic valve replacement at our institution during the years 1965, 1970, 1975, and 1980. The three most common causes were postinflammatory disease (69%) and calcification of congenitally bicuspid (19%) and unicommissural (6%) aortic valves. Other causes included infective endocarditis (2%) and congenitally quadricuspid or malformed tricuspid aortic valves (1% each); the cause was indeterminate in 1%. In the postinflammatory and bicuspid states, calcification tended to be more extensive in men than in women. The relative incidence of postinflammatory disease in our study did not change appreciably from 1965 to 1980, despite the steadily decreasing incidence of acute rheumatic fever reported in western countries.

Adult↗
PubMed1984

Surgical pathology of pure aortic stenosis: a study of 374 cases.

The gross surgical pathologic features of the aortic valve were reviewed in 374 patients who had had clinically pure aortic stenosis and aortic valve replacement at our institution during the years 1965, 1970, 1975, and 1980. The most common cause of aortic stenosis, accounting for 46% of our cases, was calcification of a congenitally bicuspid valve. In the remainder, stenosis was produced by postinflammatory fibrocalcific disease (including rheumatic disease) in 35%, by degenerative calcification of an aging valve in 10%, and by calcification of a congenitally unicommissural valve in 6%. The cause of aortic stenosis was indeterminate in 4%. Valvular lesions included various degrees of dystrophic calcification, commissural fusion, and cuspid fibrosis. Calcification tended to occur more extensively and at a younger age in men than in women. Furthermore, it tended to produce stenosis and to necessitate valve replacement earliest in patients with unicommissural valves (mean age, 48 years), later in those with bicuspid or postinflammatory valves (mean age, 59 and 60 years, respectively), and latest in those with degenerative stenosis (mean age, 72 years). In our study, the relative incidence of postinflammatory aortic stenosis remained unchanged from 1965 to 1980, despite the steadily decreasing incidence of acute rheumatic fever reported in western countries. Our data suggest that (1) the incidence of chronic rheumatic heart disease has not yet begun to decrease appreciably, (2) many episodes of acute rheumatic fever may be subclinical, or (3) some forms of nonrheumatic aortic valve disease may produce gross alterations indistinguishable from those of classic chronic rheumatic valvulitis.

Adult↗
PubMedDate not supplied

[Marfan's or Marfan-like syndrome: value of echocardiography].

The purpose of this study of 6 cases of Marfan's or Marfan-like syndrome detected in 7077 echocardiographic examinations was to investigate the clinical value of echocardiography. The mean age of the patients was 40 years, and 4 of them (66 p. 100) were female. The diagnosis was based on the 4 criteria of Marfan's syndrome in 1 case, on 3 criteria in 2 cases and on 2 criteria in 3 cases. Four patients were known to have a previous cardiac murmur. Auscultation revealed a systolic murmur of mitral regurgitation in 3 cases (associated with a diastolic murmur of aortic regurgitation in 2 of them), a diastolic murmur of aortic regurgitation in 3 cases and a systolic murmur due to calcified bicuspid aortic valve in 1 case. ECG recorded a normal rhythm in 4 cases, atrial fibrillation in 2 cases of mitral regurgitation, and left ventricular hypertrophy in 3 cases. Chest X-ray showed cardiomegaly in 3 patients and severe kyphoscoliosis in one. Echocardiography visualized dilatation of the ascending aorta, severe (60 mm) in 1 case, in 3 patients; dilatation of the pulmonary artery in 1 patient; pansystolic mitral valve prolapse in 3 patients (associated with aortic and tricuspid valve prolapse in 2 of them after the disease had progressed); isolated aortic valve prolapse due to bicuspid valve in 2 patients; intracardiac calcifications in 3 patients; subaortic septal hypertrophy in 1 patient and calcified incompetent foramen ovale in 1 patient. Aortography performed in 3 patients disclosed an aneurysm of Valsalva's sinuses in 1 case and a mild aortic insufficiency in 2 cases. Two patients underwent cardiac catheterization for severe mitral regurgitation due to mitral valve prolapse requiring valve replacement, which was successfully done. Thus, echocardiography may provide an early diagnosis of Marfan's syndrome, since cardiovascular abnormalities are frequent in infancy. It also ensures a close follow-up of the disorders and it is useful in deciding whether treatment should be medical or surgical. It may detect formes frustes in a family with Marfan's syndrome, and it may define a borderline group of patients: those with Marfan-like syndrome. In these patients the cardiovascular lesions are more preponderant and appear later than in the classical Marfan's syndrome; they are often difficult to differentiate from the lesions of Barlow's syndrome.

Adult↗
PubMed2000

Adhesion molecules in nonrheumatic aortic valve disease: endothelial expression, serum levels and effects of valve replacement.

OBJECTIVES: We studied the expression of intercellular adhesion molecule 1 (ICAM-1), vascular cell adhesion molecule 1 (VCAM-1) and endothelial selectin (E-selectin) on aortic valve endothelium in patients undergoing valve replacement. We also assessed the relation between serum levels and endothelial expression and also the changes in serum levels following surgery. BACKGROUND: Nonrheumatic aortic valve disease is believed to be a degenerative condition. Increased tissue and soluble adhesion molecule levels are described in inflammatory conditions. METHODS: Aortic valves from 22 surgical (16 bicuspid, 6 tricuspid) and 6 autopsy (4 normal, 2 thickened) cases were studied by immunohistochemistry. Soluble adhesion molecules were measured in peripheral blood preoperatively, and at 6 and 18 months postoperatively, and compared with controls. RESULTS: The majority of the surgically removed tricuspid and bicuspid valves expressed adhesion molecules (E-selectin, 75% and 100%; ICAM-1, 75% and 80%; VCAM-1, 69% and 60%, respectively). The normal postmortem valves did not express these, while the diseased ones did. Endothelial expression of E-selectin correlated strongly with serum levels (r = 0.695, p = 0.004). Soluble E-selectin levels were significantly higher at baseline compared with controls (p = 0.017) and fell significantly at 18 months postoperatively (p = 0.005). CONCLUSIONS: Adhesion molecule expression on diseased valves supports an inflammatory component in "degenerative" aortic valve disease. The diseased valves may be the main source of elevated soluble E-selectin in this condition as blood levels correlate with endothelial expression and blood levels fall at 18 months postoperatively.

Aged↗
PubMed2002

The incidence of congenital heart disease.

This study was designed to determine the reasons for the variability of the incidence of congenital heart disease (CHD), estimate its true value and provide data about the incidence of specific major forms of CHD. The incidence of CHD in different studies varies from about 4/1,000 to 50/1,000 live births. The relative frequency of different major forms of CHD also differs greatly from study to study. In addition, another 20/1,000 live births have bicuspid aortic valves, isolated anomalous lobar pulmonary veins or a silent patent ductus arteriosus. The incidences reported in 62 studies published after 1955 were examined. Attention was paid to the ways in which the studies were conducted, with special reference to the increased use of echocardiography in the neonatal nursery. The total incidence of CHD was related to the relative frequency of ventricular septal defects (VSDs), the most common type of CHD. The incidences of individual major forms of CHD were determined from 44 studies. The incidence of CHD depends primarily on the number of small VSDs included in the series, and this number in turn depends upon how early the diagnosis is made. If major forms of CHD are stratified into trivial, moderate and severe categories, the variation in incidence depends mainly on the number of trivial lesions included. The incidence of moderate and severe forms of CHD is about 6/1,000 live births (19/1,000 live births if the potentially serious bicuspid aortic valve is included), and of all forms increases to 75/1,000 live births if tiny muscular VSDs present at birth and other trivial lesions are included. Given the causes of variation, there is no evidence for differences in incidence in different countries or times.

Ductus Arteriosus, Patent↗
PubMed1998

Flow dependence of valve area in aortic stenosis: relation to valve morphology.

OBJECTIVES: We sought to develop an index of flow dependence of valve area in aortic valve (AoV) stenosis and to determine whether this index is related to structural characteristics of the diseased valve. BACKGROUND: Many studies of AoV stenosis using Gorlin or continuity equation methods have demonstrated flow dependence (an increase in valve area with increased flow). Variation in flow dependence between patients despite similar flow rates remains unexplained. METHODS: Dobutamine Doppler echocardiography was used to calculate flow rate and valve area by the continuity equation in 27 patients with aortic stenosis. For each patient the slope of the regression line of valve area to flow rate was determined (slope of flow dependence). Transesophageal echocardiography was used to evaluate features of valve morphology potentially related to the etiology of AoV stenosis and the mechanism of flow dependence. RESULTS: Mean slope of flow dependence was 0.28 cm2/100 ml per s (range -0.06 to 0.53); flow dependence was significantly >0 in 21 patients and was lower for bicuspid valves (slope 0.21 cm2/100 ml per s) than for tricuspid valves with <10% commissural fusion (slope 0.35, p < 0.01). Off-center/ovoid orifices demonstrated the least flow dependence (slope 0.19), whereas star-shaped orifices showed the most (slope 0.36, p < 0.01). Greater flow dependence was related to a lower percentage of commissural fusion (r = -0.46, p = 0.02) as well as diffuse sclerosis, primarily involving the cusp bodies, rather than localized sclerosis, with involvement of cusp margins. CONCLUSIONS: The slope of flow dependence of valve area in AoV stenosis differs markedly between patients. More flow dependence was associated with tricuspid valves and the morphologic features characteristic of calcific AoV stenosis, whereas less flow dependence was associated with bicuspid valves and the features of rheumatic disease.

Adrenergic beta-Agonists↗
PubMedDate not supplied

Aortic dissection and Turner's syndrome: case report and review of the literature.

Cardiovascular abnormalities are frequently encountered in patients with Turner's syndrome. These include coarctation of the aorta, aortic root dilatation, bicuspid aortic valve, atrial and ventricular septal defects. Aortic dissection is a rare but devastating complication of Turner's syndrome that usually occurs in adulthood. We report a case of Turner's syndrome with coarctation of the aorta and chronic aortic dissection, and review the relevant literature. There have been 21 prior reported cases of aortic dissection in patients with Turner's syndrome. Possible etiologic factors contributing to the occurrence of aortic dissection in this syndrome are protean. They include the presence of cystic medial necrosis, coarctation of the aorta, bicuspid aortic valve, aortic root dilatation, and hypertension, although cases of aortic dissection and Turner's syndrome have been described in patients without any risk factors. As our knowledge of the natural history of congenital heart defects and risk factors for aortic dissection in Turner's syndrome is limited, periodic cardiac evaluation of these patients may be warranted. Early recognition and treatment of this potentially lethal complication of Turner's syndrome is essential.

Adult↗
PubMed2003

Surgery of the dilated aortic root and ascending aorta in pediatric patients: techniques and results.

OBJECTIVE: Dilatation of the aortic root is a well-known cardiovascular manifestation in children and adult patients with connective tissue disease (e.g. Marfan syndrome). Dilatation of the ascending aorta is extremely rare and may be associated with bicuspid aortic valve. This report evaluates the incidence of dilatative aortic root and ascending aortic pathology in patients younger than 18 years and analyzes the results obtained after repair and replacement strategies. METHODS: Between 1/1995 and 12/2002, a total of 752 operations on the thoracic aorta were performed in adult and pediatric patients. We present our experience with a group of 26 patients <18 years of age, who required isolated surgery of the aortic root and/or ascending aorta because of a dilatative lesion. Fifteen patients had isolated aortic root dilatation (13 of them suffered from Marfan syndrome), eight patients presented with an idiopathic dilatation of the ascending aorta and three patients had dilatation in association with a bicuspid aortic valve. Mean age was 10 +/- 4.8 years (4-18 years). Repair of the aortic root with preservation of the aortic valve (Yacoub, David or selective sinus repair) was performed in nine patients, replacement using a homograft was performed in five patients, composite graft with mechanical prosthesis in two patients, with biological prosthesis in one patient and Ross operation was performed in one case. Isolated supracoronary graft replacement was performed in eight patients. RESULTS: Two patients died during hospitalization: a 10-year old girl developed respiratory failure on the 2nd postoperative day and autopsy revealed Ehlers-Danlos syndrome with a massive intrapulmonary emphysema. A 14-year-old Marfan patient with severely depressed preoperative LV function died from low cardiac output following composite-graft, mitral and tricuspid valve repair. One patient required aortic valve replacement 7 days after an aortic valve sparing root repair. There was no additional perioperative morbidity. In the long-term, two patients died from rupture of the thoracic aorta, both following minor non-cardiovascular surgical procedures. Both had normal sized descending and abdominal aorta. CONCLUSION: Repair of the aortic root and/or ascending aorta in children and adolescent patients can be performed with acceptable early and late results. While the presence of severe comorbidity may adversely affect early outcome, long-term survival was mainly determined by rupture of the descending aorta.

Adolescent↗