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PubMed1998

Cervical headgear usage and the bioprogressive orthodontic philosophy.

During the past 125 years, a variety of headgear appliances have been used as adjuncts during orthodontic therapy. At the outset, a headgear was usually used in combination with the extraction of the upper first bicuspid teeth which created the space required to retract protruding upper anterior teeth. It did not take long for clinicians to understand that some form of extraoral support was required to augment the somewhat-limited anchor-age provided by the maxillary bicuspid and molar teeth during incisor retraction. Thus, the headgear became the treatment auxiliary of choice during the retraction of the anterior segment of teeth. Used in this manner, the appliance became known as occipital, or headgear anchorage. After the introduction of cephalometric radiography, it became evident that headgear appliances could be used not only to move teeth, but also as orthopedic appliances to modify the development of the jaws of growing children. With time and development, headgears lost some of their early complexity and they evolved into appliances that closely resembled modern cervical headgears. The cervical pull headgear soon became involved in a controversy when some clinicians claimed that the use of a high pull, or occipital pull headgear resulted in more optimal treatment results than the cervical pull headgear. The Bioprogressive philosophy of orthodontic therapy recommends the use of cervical headgears in the unequivocal belief that, when correctly used, they produce the most advantageous and predictable orthopedic changes in growing faces.

Cephalometry↗
PubMed2001

Hypertension is a major risk factor for aortic root dilatation in women with Turner's syndrome.

Women with Turner's syndrome (TS) have a threefold increase in mortality, primarily as a result of their cardiovascular complications. Recently, the risk of fatal aortic dissection has come to light as a major cause of mortality in women with TS. The aim of this study was to assess the prevalence of aortic root dilatation in a group of women with TS and to investigate the factors contributing to its development. Thirty-eight women with TS attending a dedicated adult Turner clinic were examined clinically and by M-mode and two-dimensional echocardiography on at least one occasion. Aortic root dilatation was defined as an aortic root diameter greater than the 95th centile for body surface area. Fasting serum lipid concentrations were measured in all women. Additionally, 18 subjects underwent noninvasive assessment of central arterial stiffness using applanation tonometry. Fifty percent of subjects were hypertensive and a similar number had an abnormal echocardiogram. A bicuspid aortic valve was present in 33% of subjects, 16 women (42%) had ascending aortic root dilatation. This was associated with a bicuspid aortic valve in four women and hypertension in 11. Two women had isolated aortic root dilatation. Aortic root diameter was significantly associated with systolic blood pressure (r = 0.5, P = 0.003) and left ventricular thickness (r = 0.5, P = 0.02). There was no association with serum lipids or arterial compliance. Structural cardiac abnormalities are present in up to 50% of women with Turner's syndrome. Aortic root dilatation is a significant risk in women with Turner's syndrome and is closely dependent on blood pressure. Aortic root dilatation does not appear to be related to atherosclerosis and is more likely to be due to a mesenchymal defect. Regular surveillance of the aortic root diameter is essential in all women with Turner's syndrome and hypertension should be treated aggressively when present in order to minimize the risk of potentially fatal aortic dissection.

Adolescent↗
PubMed2001

An unusual case of aortic dissection in Turner's syndrome.

Cardiovascular malformations are common in patients with Turner's syndrome. Aortic coarctation and bicuspid aortic valve are the most frequently occurring abnormalities, and are associated with cystic medial necrosis of the aortic wall. Aortic dissection is an uncommon but catastrophic complication of the 'aortopathy' of Turner's syndrome. We report the unusual case of a Turner's syndrome patient (with a bicuspid aortic valve and previous coarctation repair) who died following an intramural haemorrhage of the aortic root that was complicated by dissection and rupture, with no evidence of aortic intimal tear. The role of intramural haemorrhage in the pathogenesis of acute aortic syndromes in Turner's syndrome patients is unclear. The condition may be associated with atypical clinical presentations, it can be difficult to confirm with imaging techniques, and it carries a high risk of progression to classical aortic dissection and death. This case therefore highlights the need for a high index of suspicion when assessing Turner's syndrome patients presenting with chest pain syndromes. Furthermore, the effective management of Turner's syndrome patients with cardiovascular abnormalities requires the development of evidence-based preventive (such as echocardiographic surveillance of aortic dilatation) and interventional strategies.

Journal Article↗
PubMed2003

Percutaneous prosthetic venous valves: current state and possible applications.

The small-intestinal submucosa square-stent bicuspid venous valve has shown the most promising results of all of the artificial venous valves developed. In long-term experimental studies in sheep, 25 valves were placed into the jugular veins and 88% exhibited good function, whereas 12% had decreased function because of valve tilting. Only 4% had partial thrombosis in the tilted valve. Valves also were placed in 3 patients. The bicuspid venous valves remained patent without thrombosis or other complications. Proper sizing and proper placement of the valves was critical to their function. The valve construction needs to be improved to prevent occasional tilting during their deployment.

Adult↗
PubMed1994

[Fine structure of conjunctival lymphatic vessel valves].

BACKGROUND: Lymphatic valves are due to ensure a zentripetal flow of lymph. The majority of the authors regard these valves as bicuspid structures with joint insertion points of the downstream localized free valvular edges. Because of that valvular structure a retrograde flow of lymph is impossible. Observations of our own however demonstrated retrograde flow of fluid under physiological conditions. In the following paper the structure of conjunctival lymphatic valves should be investigated or rather those structures, which were regarded as valves hitherto. Are there segmental connections, which allow retrograde flow of lymph? Is a regulation of lymph flow possible without self-acting valves? MATERIALS AND METHODS: In the conjunctiva of bovine post-mortem eyes (n = 100) segmental connections of lymphatic vessels with a diameter of less than 1 mm were opened specifically under control of slit lamp microscope after having undertaken an interstitial double contrast lymphography (solution of Berliner Blue/air). Some of these preparations (n = 20) could be investigated further on with the scanning electron microscope. That procedure allowed to make the patterns of flow and vessels structures in the beginnings of the lymphatic system observed with the slit lamp microscope congruent with those structures shown by the scanning electron microscope. RESULTS: The following segmental connections could be demonstrated: 1. Lymphatic vessels, which flow like a pipe into the wall of another segment. 2. Laterally arranged oval connections with valve resembling an aperture. 3. Segments of lymphatic vessels arranged in a line, which flow into one another between two hump-shaped protrusions with bicuspid valvular structures. Also the existence of different connecting structures ("valves") in one segment ("gear segment") were observed. In bulbus-like segmental connections arranged one behind the other with two valvular leaflets it could be demonstrated by scanning electron microscopy, that the two leaflets were attached apart at the vessel wall. That divergence to the previous models of lymphatic valves means, that these valvular leaflets cannot work as self-acting valves and prevent retrograde flow. CONCLUSIONS: However according to the valveless model of Libau a directed regulation of flow is possible. Perhaps the valvular structures described above play an important part by the interaction between the endothelium of lymphatic vessels and the substances transported in the lymph. The variety of segmental connections, the network of vessels with different diameters, which seem to be connected by feed-back, demand a new way of looking at the initial lymph flow.

Animals↗
PubMed2000

Sensitivity and specificity of transesophageal echocardiography for determination of aortic valve morphology.

BACKGROUND: Preoperative recognition of the presence of bicuspid aortic valve can be important in the planning of procedures. Multiplane transesophageal echocardiography may allow more accurate detection of valvular morphology than does biplane transesophageal echocardiography. METHODS AND RESULTS: The studies of 710 patients who subsequently underwent valvular or aortic surgery were reviewed in a blinded fashion. The inclusion criteria were adequate short-axis view and operative note confirmation of aortic valve morphology. Six hundred eight patients were submitted to further analysis. Four hundred three patients had aortic stenosis as the primary diagnosis. Three hundred sixty patients had biplane examinations and 248 had multiplane examinations. The sensitivity and specificity of the multiplane technique in assessing aortic valve morphology (bicuspid vs tricuspid valve) was 87% and 91%, respectively. The sensitivity and specificity of the biplane technique was 66% and 56%, respectively. Whether valves were calcified or not did not result in major changes in sensitivity and specificity for either technique. CONCLUSIONS: Multiplane transesophageal echocardiography provides a more accurate assessment of preoperative aortic valve morphology than does the biplane approach in the majority of patients.

Adolescent↗
PubMed2002

KCNJ2 mutation results in Andersen syndrome with sex-specific cardiac and skeletal muscle phenotypes.

Evaluation of candidate loci culminated in the identification of a heterozygous missense mutation (R67W) in KCNJ2, the gene encoding the inward-rectifying potassium current, Kir2.1, in 41 members of a kindred in which ventricular arrhythmias (13 of 16 female members [81%]) and periodic paralysis (10 of 25 male members [40%]) segregated as autosomal dominant traits with sex-specific variable expressivity. Some mutation carriers exhibited dysmorphic features, including hypertelorism, small mandible, syndactyly, clinodactyly, cleft palate, and scoliosis, which, together with cardiodysrhythmic periodic paralysis, have been termed "Andersen syndrome." However, no individual exhibited all manifestations of Andersen syndrome, and this diagnosis was not considered in the proband until other family members were examined. Other features seen in this kindred included unilateral dysplastic kidney and cardiovascular malformation (i.e., bicuspid aortic valve, bicuspid aortic valve with coarctation of the aorta, or valvular pulmonary stenosis), which have not been previously associated. Nonspecific electrocardiographic abnormalities were identified in some individuals, but none had a prolonged QT interval. Biophysical characterization of R67W demonstrated loss of function and a dominant-negative effect on Kir2.1 current. These findings support the suggestion that, in addition to its recognized role in function of cardiac and skeletal muscle, KCNJ2 plays an important role in developmental signaling.

Abnormalities, Multiple↗
PubMed1989

Familial aortic valve disease: evidence for a genetic influence?

It has been suggested that genetic factors may play a role in the aetiology of congenital bicuspid aortic valve disease. This report describes a family in which three male siblings with bicuspid aortic valves developed critical aortic stenosis. This provides further evidence that an hereditary mechanism may play a role in some patients with this condition.

Adult↗
PubMed1990

Calcific aortic stenosis in twins: a clue to its pathogenesis?

A pair of identical twins presented almost simultaneously at age 62 years with calcific aortic stenosis requiring surgery, one with bicuspid and one with tricuspid aortic valve. This suggests that bicuspid morphology may perhaps not be the cause, but may be a genetically linked marker of an inherited predisposition to calcific stenosis.

Aortic Valve↗
PubMed1998

Adams-Oliver syndrome associated with cardiovascular malformations.

We describe two families with Adams-Oliver syndrome (AOS), an autosomal dominant malformation syndrome (MIM No. 10030), in which cardiovascular malformations (CVMs) have been reported previously. In the first family, twin boys and their mother had the typical digital and scalp defects of AOS with various obstructive CVMs of the left heart (bicuspid aortic valve, Shone's complex). At least three other relatives not examined personally are reported to have related CVMs (aortic valve stenosis, hypoplastic left heart syndrome). In the second family, a girl had typical AOS digital and scalp defects and a bicuspid aortic valve. At least three other relatives are reported to be mildly affected. Tetralogy of Fallot had been previously reported as the most common CVM in AOS [Zapata HH, Sletten LJ, Pierport MEM (1995). J Med Genet 47:80-84.]. However, with the addition of these new patients and two other literature reports, we emphasize that approximately 20% have a CVM, frequently obstructive lesions of the left heart. Cardiology consultation should be offered to most patients with AOS.

Abnormalities, Multiple↗
PubMed2004

Approach to the dilated aortic root.

PURPOSE OF REVIEW: The dilated aortic root is often completely asymptomatic and found incidentally on routine imaging studies such as chest radiograph, echocardiography, chest computed tomography, or magnetic resonance imaging. The dilated aortic root may be associated with underlying aortic valve abnormalities as seen with bicuspid aortic valve. It may also lead to the awareness of important underlying connective tissue disorders like the Marfan syndrome. It is imperative that the dilated aortic root be observed carefully over time with serial imaging studies and that timely resection of the aneurysm be carried out before catastrophic complications such as aortic dissection, aortic rupture, or congestive heart failure from aortic insufficiency occur. RECENT FINDINGS: In recent years, the advent of molecular genetics has heightened awareness of familial aortic disease such as the Marfan syndrome, bicuspid aortic valve disease, and hereditary aortic aneurysm and dissection. In addition to hypertension and inflammatory aortic disease, these hereditary aortopathies are important to consider in the evaluation of patients with a dilated aorta and have implications for screening of the relatives of the patient with aortic aneurysm. SUMMARY: Because there is often uncertainty regarding the dilated aortic root, this review will summarize the approach to diagnosis, evaluation, and management of aortic root aneurysms. Clinical features, diagnostic approaches, screening of relatives, and long term follow-up will be highlighted.

Aortic Aneurysm↗
PubMed2006

Molecular genetics of aortic valve disease.

PURPOSE OF REVIEW: Bicuspid aortic valve is the most common type of cardiac malformation and predisposes to aortic valve calcification, which is the third leading cause of heart disease in adults. These seemingly temporally disparate diseases have been described as having genetic influences but limited data exist on the precise genetic causes in humans. Several advances in the molecular genetics of aortic valve formation and calcification have recently been identified and are described here. RECENT FINDINGS: A large family with autosomal-dominant aortic valve disease consisting of bicuspid aortic valve and aortic valve calcification was studied using genome-wide linkage analysis. This led to the identification of a nonsense mutation in NOTCH1 in affected individuals. This finding was supported by the discovery of a NOTCH1 frameshift mutation in an unrelated family with similar aortic valve disease, suggesting that NOTCH1 haploinsufficiency was a genetic cause of aortic valve malformations and calcification. The NOTCH signaling pathway was linked to a molecular pathway for aortic valve calcification, as NOTCH1 was found to repress activation of Runx2 - a transcription factor critical for osteoblast cell fate that is up-regulated in calcified human aortic valves. SUMMARY: The recent genetic and biochemical findings provide novel insights into the molecular and genetic basis for aortic valve dysmorphogenesis and calcification. Future studies focusing on the identification of additional disease-causing and susceptibility genes will aid in the development of prevention strategies. Ultimately, it will be the careful dissection of these molecular pathways that will hopefully lead to novel therapeutic options.

Adult↗
PubMed2006

Surgical pathology of the ascending aorta: a clinicopathologic study of 513 cases.

Only 2 comprehensive surgical series, published in 1977 and 1983, have evaluated clinicopathologic features of the ascending aorta. Retrospective review of medical records and microscopic slides was performed on 513 consecutive patients with surgical resection of ascending aortic tissue (1985 to 1999). Patients were 2 to 89 years old (mean 59 y), and 303 (59%) were men. Aneurysm or dissection was the indication for surgery in 479. Aortic valves were also excised in 360. Systemic hypertension was present in 279, inherited connective tissue disease (ICTD) in 67, arteritis in 33, and acquired connective tissue disease in 16. Microscopy showed cystic medial degeneration (CMD) in 209, aortic dissection (AD) in 109 (with CMD in 56), normal media in 90, aortitis in 57 (with CMD in 14), and other findings in 48. The most significant, independent risk factor of CMD and AD was ICTD (confidence interval=7.61 and 2.26, respectively). Systemic hypertension was more common in patients with AD than without (P=0.0202). Normal media was the most common histologic finding associated with bicuspid aortic valve (P<0.0001). Among 57 patients with aortitis (giant cell in 39), ages ranged from 16 to 85 years (mean 64 y), and 42 (74%) were women; only 8 had Takayasu arteritis, and 11 had temporal or systemic arteritis. In surgically resected ascending aorta, the 3 most common histologic findings were CMD, AD, and normal media. ICTD, systemic hypertension, and bicuspid aortic valve were common comorbid findings. Giant cell aortitis occured predominantly in women, usually without systemic disease.

Adolescent↗
PubMedDate not supplied

Absence of right superior vena cava and aortic annular hypoplasia in a patient with Turner's syndrome.

Cardiovascular abnormalities are frequently encountered in patients with Turner's syndrome. These include coarctation of the aorta, bicuspid aortic valve, aortic root dilatation, atrial and ventricular septal defects, but absence of the right superior vena cava (SVC) in visceroatrial situs solitus is extremely rare. We report absence of the right SVC and congenital aortic annular hypoplasia with bicuspid aortic valve stenosis in a patient with Turner's syndrome. Aortic root extension with prosthetic valve replacement was performed and absent right SVC, which was detected incidentally during operation, is confirmed by postoperative venous angiogram.

Adult↗
PubMed1977

Gingival condition in areas of minimal and appreciable width of keratinized gingiva.

Sixteen dental, dental hygiene, and dental assisting students and dental faculty members who had contralateral or unilateral areas of minimal (less than or equal to 1.0 mm) and appreciable (greater than or equal to 2.0 mm) widths of keratinized gingiva on mid-buccal plaque-free surfaces of mandibular bicuspids were examined. Gingival exudate amounts and clinical inflammation based on color change and/or swelling and bleeding on probing were evaluated. The results showed that gingiva with "appreciable" width as well as gingiva with "minimal" width of keratinized tissue exhibited only minute amounts of gingival exudate. Also, there were generally no clinical signs of inflammation for both types of tissue. From the groups of 16, six subjects were selected who had contralateral pairs of minimal and appreciable keratinized gingiva. They were instructed to cease oral hygiene in the lower bicuspid area for 25 days. At day 0, 4, 7, 11, 14, 18, 21, and 25, plaque, gingival exudate, and clinical gingival inflammation were evaluated. Results revealed increases in plaque, gingival exudate scores and clinical gingival inflammation over the 25-day period with no apparent difference between the areas with minimal and appreciable width of keratinized gingiva.

Dental Plaque↗
PubMed1983

Osseous repair in the presence of active tooth hypermobility.

The present investigation was undertaken to evaluate the periodontal response after resolution of inflammation in a situation of established marginal periodontitis, but in the presence of active, continued tooth hypermobility. Periodontitis was induced unilaterally around mandibular second and third bicuspids in 4 squirrel monkeys by tying plaque retentive silk ligatures at the gingival margins. Jiggling trauma to the periodontium between these bicuspids was begun 5 weeks later and continued for the remaining 20 weeks. Ligatures were removed 15 weeks after placement, whereupon regular oral hygiene was begun and continued. Periodontitis and trauma were produced around the corresponding contralateral teeth (control) so that the interproximal area represented the situation immediately prior to ligature removal. Infiltrated connective tissue, loss of connective tissue attachment and alveolar bone, and percentage of bone were determined histometrically for each coronal interproximal periodontium. In control specimens, 58% of the supracrestal tissue was infiltrated with inflammatory cells compared to 19% in experimental specimens. There were no differences in levels of connective tissue attachment or crestal alveolar bone. However, bone repair occurred in the experimental specimens which increased bone volume from 11 to 18% (P less than 0.05). It was concluded that osseous repair occurred in the presence of active, continued tooth hypermobility after resolution of inflammation.

Alveolar Process↗
PubMed1985

Prevalence and clinical significance of aortic valve prolapse.

The prevalence and clinical significance of aortic valve prolapse were determined prospectively in 2000 consecutive patients undergoing routine clinical cross sectional echocardiography. Two hundred and twelve patients were excluded because the aortic cusps were not adequately visualised. Aortic valve prolapse was defined as downward displacement of cuspal material below a line joining the points of attachment of the aortic valve leaflets. Twenty four cases of aortic valve prolapse (1.2%) were identified. The patients were aged 12-64 years and nine were women. All had underlying valvar heart disease and the commonest lesion (in 11 cases) was prolapse of the larger cusp in bicuspid valves. Aortic valve prolapse was seen in four patients with mitral valve prolapse (two with severe regurgitation), one of whom had marfanoid aortic root dilatation. The remaining examples of aortic prolapse were seen in patients with various disorders including one with pulmonary atresia, two with aortic root disease (one with dissection and one with idiopathic dilatation), and one case of severe mitral regurgitation. Valves destroyed by infective endocarditis were seen in two cases. Aortic valve prolapse may be detected in various cardiac disorders and does not imply the presence of aortic regurgitation, but when bicuspid aortic valves are present it may well be important in producing such regurgitation. Although aortic valve prolapse may be associated with severe forms of mitral valve prolapse, these patients rarely have aortic regurgitation.

Adolescent↗
PubMed1997

A case-control investigation of the relation between hyperlipidaemia and calcific aortic valve stenosis.

OBJECTIVE: To investigate the relation of hyperlipidaemia to calcific aortic valve stenosis. DESIGN: A case-control study designed to detect a clinically relevant difference in the fasting plasma concentrations of total cholesterol between the groups at the 5% level with a power of 90%. Predefined subgroup analyses were based on presence of significant coronary disease and valve morphology (that is, bicuspid or tricuspid). SETTING: A district general hospital. SUBJECTS: 20 patients with severe calcific aortic stenosis and 20 controls. RESULTS: Mean (SD) fasting plasma total cholesterol in patients with aortic stenosis was 0.79 (1.50) mmol/l greater than in the controls (p = 0.029). The magnitude of differences between patients with aortic stenosis and controls was similar whether the patients had coronary artery disease (0.78 (1.73) mmol/l) or not (0.80 (1.37) mmol/l). The presence of a stenosed tricuspid aortic valve was associated with a significant increase in plasma cholesterol (1.70 (0.87) mmol/l, p = 0.012). For bicuspid valves the degree of elevation of plasma cholesterol was less and not statistically significant. CONCLUSIONS: Calcific aortic stenosis is associated with hypercholesterolaemia, especially when the valve is tricuspid. Further studies are necessary to confirm that the relation is causal. This finding may have implications for measures to prevent the most common cause of cardiac valve replacement in the developed world.

Aged↗