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Renal artery stenosis in patients with aortic dissection: increased prevalence.

The authors retrospectively analyzed the prevalence of renal artery stenosis in 63 consecutive patients with aortic dissection who underwent thoracic and abdominal aortography. Ten patients (16%) had renal artery stenosis, five with atherosclerosis and five with fibromuscular lesions. Risk factors for aortic dissection were Marfan disease in nine patients, bicuspid aortic valve in one, and hypertension in 54 (including seven patients with Marfan syndrome). If the patients with Marfan syndrome and the patient with the bicuspid aortic valve are excluded, renal artery stenosis was present in 10 of 53 patients (19%) when the cause of dissection was presumably hypertension. This finding suggests that renovascular hypertension is a greater risk factor for aortic dissection than is essential hypertension. The success of angiotensin converting enzyme inhibitors and percutaneous transluminal renal angioplasty (PTRA) in controlling renovascular hypertension has been proved. In this series, emergent PTRA successfully controlled the hypertension in one patient with a type B dissection, resulting in an excellent clinical outcome. Angiography should be routinely performed on patients with aortic dissections to evaluate for renal artery stenosis.

Adult↗

Copper deficiency alters collagen types and covalent cross-linking in swine myocardium and cardiac valves.

Dietary copper deficiency induces alterations of connective tissue metabolism that are associated with lesions in cardiovascular and other organ systems. To determine the impact of copper deficiency on characteristics of collagen in porcine myocardium and cardiac valves, weaned pigs were fed diets with adequate or deficient levels of copper. Although dietary copper did not affect the concentration of collagen in either myocardium or bicuspid valves, the degree of collagen cross-linking, as assessed by the level of hydroxylysylpyridinoline, was lower in both tissues of copper-deficient pigs. Proportions of type III collagen were increased in the left ventricle and bicuspid valves of copper-deficient pigs. Copper deficiency induced extensive remodeling, however, of the collagen fraction of cardiac interstitium. Reduction in left ventricular collagen cross-linking may provide the stimulus for the development of cardiac hypertrophy, which characterizes severe copper deficiency, by increasing the compliance of the ventricular wall. The shift in the phenotypic profile of collagen that is associated with this cardiac hypertrophy indicates synthesis of new collagen, which could affect collagen cross-linking irrespective of copper status.

Animals↗

Fixed subaortic stenosis revisited. congenital abnormalities in 72 new cases and review of the literature.

Seventy-two patients aged 2-65 years (mean 20.2) with fixed subaortic stenosis (FSS) are reported. All patients underwent echocardiography and angiography prior to surgical intervention. Sixty-four (89%) had discrete subaortic stenosis (DSS) and 8 (11%) tunnel type stenosis (TSS). Patients with DSS aged 2-65 years (mean 29) while patients with TSS aged 10-34 years (mean 19) and had a distinct female preponderance (7:1). Twenty-five patients (39%) with DSS had other congenital abnormalities of which aortic stenosis associated with bicuspid aortic valve was slightly more common than ventricular septal defect (7:6). Five patients with TSS had a small aortic annulus and hypoplastic aorta. Our data and those in the literature indicate that TSS although related to DSS has certain features that may set it apart as a distinct entity. In addition DSS appears to be associated with aortic stenosis and bicuspid aortic valve as frequently as with ventricular septal defect.

Adolescent↗

Fate of the aortic root late after Ross operation.

BACKGROUND: The Ross operation is an alternative to mechanical aortic valve replacement in the young. Early dilatation of the pulmonary autograft root exposed to the systemic circulation has been reported. To define the prevalence of, risk factors for, and consequences of late autograft dilatation, outcome in all consecutive patients operated since May 1994 was reviewed. METHODS AND RESULTS: Ninety one patients, 77 males and 14 females, with at least 1 year of follow-up underwent cross-sectional clinical and echocardiographic examination. Age at operation was 27+/-10 years (range 6 to 49), and the indication was aortic regurgitation in 54 (59%) patients and bicuspid valve was present in 62 (68%). End-points of the study were freedom from autograft dilatation (root diameter >4 cm or 0.21 cm/m2), from (moderate) autograft regurgitation and from reoperation. Follow-up (4.0+/-1.9, range 1 to 8 years) autograft root diameters were anulus, 29+/-4 mm (18-39); sinus of Valsalva, 38+/-7 mm (24-53); sinotubular junction, 37+/-6 mm (23-54); and ascending aorta, 37+/-5 mm (27-54). Late autograft dilatation was identified in 31 (34%) patients and regurgitation in 13 (14%), 7 of whom had autograft dilatation. At 7 years, freedom from dilatation was 42+/-8%, freedom from regurgitation was 75+/-8%, and freedom from reoperation was 85+/-10%. Cox proportional hazard analysis identified younger age (P=0.05), preoperative sinus of Valsalva (P=0.02), root replacement technique (P=0.03), and absence of pericardial buttressing (P=0.04) as predictive of autograft dilatation, whereas female sex (P=0.002), follow-up sinus of Valsalva (P=0.003), and sinotubular junction diameter (P=0.02) as predictive of autograft regurgitation. CONCLUSIONS: Autograft dilatation is common late after the Ross procedure, particularly in younger patients, in those with preoperative aortic aneurysm, and those having root replacement without support of anulus and sinotubular junction. Bicuspid aortic valve is not a risk factor. Significant autograft valve dysfunction affects a minority of patients, but it is more prevalent in those with autograft dilatation.

Adolescent↗

Abnormal aortic valve development in mice lacking endothelial nitric oxide synthase.

BACKGROUND: Endothelium-derived nitric oxide (NO) is produced by an oxidative reaction catalyzed by endothelial NO synthase (eNOS). NO plays a crucial role in controlling cell growth and apoptosis, as well as having well-characterized vasodilator and antithrombotic actions. More recently, endothelium-derived NO was shown to be involved in postdevelopmental vascular remodeling and angiogenesis, as well as in the formation of limb vasculature during embryogenesis. Therefore, we investigated the role of endothelium-derived NO during cardiovascular development using mice deficient in eNOS. METHODS AND RESULTS: We examined the hearts of 12 mature eNOS-deficient and 26 mature wild-type mice. Five of the mature eNOS-deficient mice had a bicuspid aortic valve; none of the 26 wild-type animals exhibited identifiable valvular or cardiac abnormalities. Immunohistochemical analysis revealed prominent eNOS expression localized to the endothelium lining the valve cusps of the aorta in mature wild-type mice; expression was localized to the myocardium and endothelial cell monolayer lining the valve leaflets in the developing embryo. CONCLUSIONS: These results show a strong association between eNOS deficiency and the presence of a bicuspid aortic valve; they provide the first molecular insight into one of the most common types of congenital cardiac abnormality.

Animals↗

Aortic valve repair for adult congenital heart disease: A 22-year experience.

BACKGROUND: Aortic valve-preserving procedures have resulted in excellent outcomes in selected patients, particularly those with normal aortic valve leaflets and dilated aortic roots. However, several congenital heart lesions are associated with abnormal aortic valve leaflets. The long-term results of aortic valve repair for these lesions are not well defined. METHODS AND RESULTS: We reviewed the clinical records of 54 adult (age >18 years) patients who underwent repair of congenital abnormalities of the aortic valve between 1976 and September 1999. Follow-up data were available on 52 (96%) patients (mean 50+/-67 months, range 1 to 266). Patients underwent repair at a mean age of 34+/-14 years with associated diagnoses of subaortic stenosis (n=10), ventricular septal defect with prolapsing aortic valve (n=17), bicuspid aortic valve (n=23), sinus of Valsalva aneurysm (n=10), and bacterial endocarditis (n=2). There was 1 operative death (1.9%) and 3 late deaths. Survival at 5 and 10 years was 98+/-2% and 74+/-12%, respectively. Freedom from reoperation was 74+/-9% and 51+/-15% at 5 and 10 years, respectively. The presence of a ventricular septal defect predicted failure of valve repair (59% versus 22%, P:=0.01). A bicuspid aortic valve, subaortic stenosis, or the requirement for mitral valve surgery did not affect outcomes. CONCLUSIONS: Aortic valve repair in adult patients with congenital heart disease can be performed with minimal morbidity and mortality rates. The medium-term results of repair are acceptable, regardless of valvular or associated pathology. However, only 31 patients (57%) demonstrated long-term competence of the aortic valve, suggesting that most adult patients with congenital aortic valve disease will eventually require aortic valve replacement.

Adult↗

Echocardiographic studies of abnormalities associated with coarctation of the aorta.

Echocardiograms were performed in thirty-six patients (aged 4 to 36 years) with proven coarctation of the aorta. Nineteen patients (53%) were found to have marked diastolic eccentricities of their aortic valves (Eccentricity Index greater 1.5), indicating the presence of bicuspid aortic valves. One of these patients also had multilayered aortic root echoes in diastole. Five patients had angiographic proof of their aortic valve morphologies which corroborated the echo findings. Five patients with bicuspid aortic valves showed mitral valve diastolic flutter indicative of aortic regurgitation. Idiopathic hypertrophic subaortic stenosis (IHSS) was suspected in four patients (11%) with abnormal systolic anterior motion of the mitral valve; three of these patients also had asymmetric septal hypertrophy. There was catheterization proof of IHSS in one patient. Two patients (5.6%) demonstrated mitral valve proplapse.

Amyl Nitrite↗

Anomalous origin of the right coronary artery from the ascending aorta: case history.

A patient is described with an anomalous right coronary artery arising high above the left sinus of Valsalva. This patient is unique because the other 2 cases with such an anomalous origin of a right coronary artery had bicuspid aortic with such an anomalous origin of a right coronary artery had bicuspid aortic valves; this patient had a normal tricuspid aortic valve.

Aorta↗

A comparison of echocardiography and magnetic resonance imaging in cardiovascular screening of adults with Turner syndrome.

The high mortality rate from aortic dissection of women with Turner syndrome (TS) achieving ovum donation pregnancies has highlighted the need for a refinement of cardiac screening protocols. Echocardiography and magnetic resonance imaging (MRI) are used to assess the risk factors, aortic root dilatation, bicuspid aortic valve, and coarctation, but the relative merits of each modality are unclear. Cardiovascular screening was performed in 128 unselected women with TS (mean age +/- SD, 31.1 +/- 8.5 yr) using echocardiography (n = 120) and MRI (n = 115) and in 36 age-matched normal control women. Clinical history, anthropometric measurements, blood pressure, and metabolic parameters were recorded. Echocardiography was normal in 53% of women with TS; MRI was normal in 34%. Aortic root dilatation was identified in 16% of women by echocardiography, 33% on MRI criteria, and 7% by both modalities. Height-adjusted echocardiographic aortic root dimensions were greater in TS than controls (2.90 vs. 2.62 cm; P = 0.010). Bicuspid aortic valve and increasing age were associated with greater aortic dimensions; the latter effect was more marked in TS than controls. On MRI, ascending aortic diameter was greater in TS than control women (2.83 vs. 2.52 cm; P = 0.029), but descending aortic diameter and ascending/descending aortic ratio were not, because these may be affected by the presence of coarctation. The two techniques are complementary and identify different aspects of cardiovascular pathology. Ascending/descending ratio on MRI circumvents issues of stature, but may be influenced by descending aortic abnormalities. We present reference ranges for absolute aortic dimensions in a TS population to aid future interpretation of these measurements.

Adult↗

Growth hormone treatment and aortic dimensions in Turner syndrome.

BACKGROUND: In recent years many girls with Turner syndrome (TS) have been treated with supraphysiological doses of GH to increase adult height. In addition to promoting statural growth, GH may have direct effects on the cardiovascular system. OBJECTIVE: We sought to determine whether GH treatment affects aortic diameter in girls with TS because there is an increased risk for aortic dilation and dissection in this syndrome. METHODS: In a retrospective, cross-sectional study, we compared ascending and descending aortic diameters measured by magnetic resonance imaging in GH-treated (n = 53) vs. untreated (n = 48) patients with TS participating in a National Institutes of Health protocol between 2001 and 2004. RESULTS: The average duration of GH treatment was 4.7 with se 0.4 yr (range 2-11 yr). The two groups were similar in age and weight, but GH-treated subjects were on average 8 cm taller (P = 0.002). The diameter of the ascending aorta was increased by 7.3% and descending aorta by 8.9% in the GH-treated group. However, after correction for age, height, weight, and presence of bicuspid aortic valve and coarctation, using a multiple regression, neither history of GH treatment nor the length of GH treatment had an effect on the aortic diameter. Weight (P = 0.02), height (P = 0.001), and presence of bicuspid aortic valve (P = 0.0001) were associated with larger ascending aortic diameter, whereas age (P = 0.008), height (P = 0.02), and history of coarctation (P = 0.006) were associated with larger descending aortic diameter. CONCLUSIONS: GH treatment of girls with TS does not seem to affect ascending or descending aortic diameter above the increase related to the larger body size.

Adolescent↗

An autopsy case of rheumatoid arthritis with aortic steno-insufficiency, angina pectoris and severe heart failure.

Although nonspecific pericarditis, myocarditis, valvulitis, and coronary arteritis are known as cardiac lesions that accompany rheumatoid arthritis (RA), there have been few reports of the occurrence of clinically severe valvular disease. We report here the case of 69-year-old man with a 25-year history of RA who died of acute left-sided heart failure complicating to aortic steno-insufficiency and angina pectoris. Autopsy findings revealed the coincidence of a congenital bicuspid aortic valve with chronic inflammation, fibrosis and calcification; eccentric hypertrophy and myocardial fibrosis of the left ventricle; 75% luminal narrowing of the proximal portion of the coronary artery due to atherosclerosis, and narrowing of the small arteries of the cardiac muscle due to angitis. It is deduced that the coronary artery lesions, aortic valve lesions and myocardial lesions were aggravated by the bicuspid aortic valve, changes with ageing and corticosteroid therapy.

Aged↗

Contamination from possible solar light exposures in ESR dosimetry using human tooth enamel.

Electron spin resonance (ESR) measurement data of 98 teeth from atomic-bomb survivors who received various radiation doses were analyzed in terms of possible solar light exposure by tooth position. Each tooth was divided into buccal and lingual parts for independent ESR measurement. We found that average buccal doses were larger than their corresponding lingual doses by 0.48 +/- 0.30 Gy (first incisors), 0.33 +/- 0.38 Gy (second incisors), 0.20 +/- 0.23 Gy (canines), 0.24 +/- 0.26 Gy (first bicuspids), 0.17 +/- 0.51 Gy (second bicuspids) and 0.04 +/- 0.18 Gy (large molars and wisdom teeth). Such a clear declining trend following tooth position in the mouth is readily appreciated as due to solar light exposures. Comparisons of lingual doses of multiple teeth from the same five donors suggest that lingual doses of first incisors were also overestimated by 0.34 +/- 0.18 Gy. From the results presented, we deduce that the buccal doses of first incisors are, on the average, overestimated by nearly 0.8 Gy. Therefore, use of front teeth for biodosimetry requires special caution. How to estimate the contribution from exposure to solar light in ESR biodosimetry is discussed.

Dental Enamel↗

Spontaneous coronary artery embolus associated with calcific aortic stenosis.

Calcific embolization from aortic stenosis may be more frequent than commonly appreciated. Most calcific emboli are clinically silent, although transient ischemic attacks, cerebral infarcts, blindness (from central retinal artery occlusions), and myocardial infarctions have been reported. We describe a patient with calcific bicuspid aortic stenosis who presented with transient ischemic attacks and angina secondary to a calcific embolus to the second circumflex marginal coronary artery. The calcific embolus was retrieved during aortic valve replacement surgery. A review of the literature suggests that calcific embolization from calcific aortic stenosis may occur more commonly in patients with bicuspid valves.

Aortic Valve Stenosis↗

Further delineation of aortic dilation, dissection, and rupture in patients with Turner syndrome.

OBJECTIVE: Although cardiovascular malformations (CVMs) are well-recognized congenital anomalies in Turner syndrome, aortic dilation and dissection are less common and less familiar. Most of the relevant literature is limited to single cases reports or small series. We sought to increase the information available about the frequency and characteristics of aortic dilation in patients with Turner syndrome. DESIGN: A 1-page survey of cardiac abnormalities, including aortic dilation, was mailed to approximately 1000 (1040 verified) members of the Turner Syndrome Society as an enclosure in the June 1997 newsletter. We also conducted a literature review. PARTICIPANTS: A total of 245 patients or families of patient members of the Turner Syndrome Society responded to the survey ( approximately 24% response rate). RESULTS: A CVM was reported in 120 of 232 (52%) respondents to this questionnaire. Obstructive lesions of the left side of the heart predominated and included bicuspid aortic valve (38%) and coarctation (41%). Aortic dilation was reported in at least 15 of 237 respondents (6.3%; 95% confidence interval: 3.6%-10.3%); 2 of 15 (13%) had dissection. Twelve of 15 (80%) patients had an associated risk factor for aortic dilation such as a CVM or hypertension. The 3 (20%) patients who did not have a CVM or hypertension were all younger than 21 years. In the entire group with aortic dilation, 10 of 15 (67%) patients were younger than 21 years. All patients with aortic dilation had involvement of the ascending aorta, and 2 had additional involvement of the descending aorta distal to a repaired coarctation. An update of the literature revealed 68 patients with aortic dilation, dissection, or rupture. An associated CVM or hypertension was reported in 53 of 59 (90%) informative patients. At least 6 (10%) had no predisposing risk factor (information was inadequate for 9 of 68 patients). The following patterns of aortic involvement were identified: ascending +/- descending aorta with coarctation (14); ascending +/- descending aorta without coarctation (39); descending aorta with coarctation (3); descending thoracic or abdominal aorta without coarctation (4); and unspecified (8). Dissection or rupture was reported in 42/68 (62%). Two reported patients died suddenly from aortic dissection in the third trimester of assisted pregnancy. At least 20 (29%) patients were younger than 21 years. One of the 6 (17%) patients with isolated aortic dilation was in this younger group. CONCLUSIONS: More information is needed about the frequency and natural history of aortic dilation in Turner syndrome. This work contributes new patient data and increases the literature review. Despite the well-recognized limitations of self-reporting, this survey detected aortic dilation with or without dissection in approximately 6% of patients with Turner syndrome. Although rare, this is a potentially catastrophic occurrence, warranting greater awareness among health professionals. In this study and the literature, the vast majority of patients with aortic dilation have an associated risk factor such as a CVM, typically bicuspid aortic valve or coarctation, or systemic hypertension. These patients represent a higher risk group that should be followed appropriately, usually under the direction of a cardiologist. Patients undergoing assisted pregnancy also should be evaluated closely. It is generally accepted that at the time of diagnosis of Turner syndrome, all patients should have a complete cardiology evaluation including echocardiography. The small number of patients with aortic dilation without a CVM, who would not be under the long-term care of a cardiologist, makes it prudent to screen all patients with Turner syndrome for this potentially lethal abnormality. The specific timing for this screening is controversial. Our recommendations for prospective imaging do not represent a rigid standard of care.

Adolescent↗

[DMF index simplification in people aged 18 to 25 years].

The DMF index can be estimated at double the value observed in the exam of two hemiarchs (Upper Right and Lower Left), or at four times the value obtained from six teeth (First Molar and Central Incisor Upper Right, first bicuspid upper left, second molar and lateral incisor lower left and second bicuspid lower right. The estimates were made by the use of methods of descriptive statistics, averages, percentages and the coefficient of the prevalence of caries, in a sample of 100 people, aged from 18 to 25 years.

Adolescent↗

[Echocardiographic findings in children with "innocent" murmur].

OBJECTIVES: To access the frequency of abnormal echocardiographic findings in patients with diagnosis of innocent murmur. METHODS: A transversal study of 166 consecutive patients was carried out with diagnosis of innocent murmur evaluated in the pediatric cardiology outpatient clinic of "Instituto de Cardiologia/Fundação Universitária de Cardiologia", a tertiary reference center, between December 3, 2001 and December 2, 2002. History, clinical examination and echocardiogram were performed in all patients. Post-test probabilities of disease with positive and negative clinical examination were estimated, considering the echocardiogram as gold standard. RESULTS: Of the 166 patients evaluated, 11 showed some alteration in the echocardiogram: pulmonary stenosis (4), patent foramen ovale (2), atrial septal defect (1), bicuspid aortic valve (2), minimum aortic regurgitation (1) and ventricular septal defect (1). Negative post-test probability (probability of disease with diagnosis of innocent murmur) was of 6.6%. CONCLUSION: In a referral outpatient clinic the proportion of minor lesions is high in patients with diagnosis of innocent murmur. These injuries may not be related to the heart murmur, as in the patent foramen ovale and bicuspid aortic valve. However, they raise the question of the indication of echocardiogram taking into account the low risk of detected lesions and, on the other hand, the resolutive character of a daycare reference center.

Child↗

Microleakage and penetration depth of three types of materials in fissure sealant: self-etching primer vs etching: an in vitro study.

Clinical preventive procedures must be done after a risk assessment. One of the risk factors is the occlusal morphology of the posterior teeth. These caries-free fissures must be sealed. This first in vitro experimentation of the study evaluated the microleakage and the penetration depth of three types of materials by Vivadent: Helioseal F, Tetric, Tetric Flow. The teeth were etched with phosphoric acid and bonded using a one bottle bonding in order to determine the best material for the sealing of the fissure. The depth of penetration of fuschine dye as well as that of the tested material was measured with a grid. The results, compared to the depth of the fissures, are expressed in percentage of penetration. The results were as follows: penetration of fuschine dye: 0% for the 2 composites, 100% for Helioseal F; penetration of the materials: 96.90% for Helioseal F, 70.82 for Tetric and 86.10 for Tetric Flow (significant difference, Wilcoxon test = 0.0105). In this first in vitro study, Tetric Flow shows no microleakage and is more efficient when compared to Helioseal F and Tetric in obturating deep fissures of non carious bicuspids. The second experiment of the study evaluated the microleakage and the penetration depth of Tetric Flow when it is bonded by two different methods: Group 1: total etch (phosphoric acid) and Scotch-bond 1 (3M), and Group 2: self-etching primer with Prompt (Espe). There was no significant difference (p > 0.03) between classical bonding vs self-etching primer. The self-etching primer Prompt is very efficient vs phosphoric acid in obturating the fissures of non carious bicuspids with Tetric Flow. It is concluded that for prevention by sealing, using a flowable ceromer (Tetric Flow) with the self-etching (Prompt), is a really good technique.

Acid Etching, Dental↗

The effect of intrasulcular toothbrushing on the amount of sulcular fluid.

Nineteen young adult Caucasian males had an average Gingival Score of 0.47. Each was asked to demonstrate his present method of toothbrushing. Thirteen used a scrub method, and six combined the scrub and roll methods. Sulcular fluid measurements were taken from the facial aspect of the mandibular left second bicuspid using filter paper strips stained with ninhydrin. An average value of 0.94 mm was obtained. Intrasulcular brushing was performed in the mandibular left posterior segment, for 30 seconds daily for 40 days. The brushing was done by the investigator on 28 days and by the subjects on 12 days. The average Gingival Index score following intrasulcular brushing was 0.26. Sulcular fluid measurements were again taken from the mandibular left second bicuspid. A decreased amount of sulcular fluid was found in six subjects, while nine exhibited an increase and four showed no change. An average sulcular fluid measurement of 0.87 mm resulted following the experimental brushing period. From these data, it can be concluded that, within the limitations of this study, intrasulcular brushing had no significant effect on the amount of gingival fluid.

Adolescent↗