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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This two-part article examines the histologic and morphologic basis for stenotic and purely regurgitant aortic valves. Part I discusses stenotic aortic valves and Part II will discuss causes of purely regurgitant aortic valves. In over 95% of stenotic aortic valves, the etiology is one of three types: congenital (primarily bicuspid), degenerative, or rheumatic. Other rare causes of stenotic aortic valves include active infective endocarditis, homozygous type II hyperlipoproteinemia, and systemic lupus erythematosis. The causes of pure aortic regurgitation are multiple but can be separated into diseases affecting the valve (normal aorta) (infective endocarditis, congenital bicuspid, rheumatic, floppy), diseases affecting the walls of aorta (normal valve) (syphilis, Marfan's, dissection), disease affecting both aorta and valve (abnormal aorta, abnormal valve) (ankylosing spondylitis), and diseases affecting neither aorta nor valve (normal aorta, normal valve) (ventricular septal defect, systemic hypertension). Diseases affecting the aortic valve alone are the most common subgroup of conditions producing pure aortic valve regurgitation.
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PMID:Pathology of aortic valve stenosis and pure aortic regurgitation. A clinical morphologic assessment--Part I. 816 31

This two-part article examines the histologic and morphologic basis for stenotic and purely regurgitant aortic valves. Part I discussed stenotic aortic valves and Part II discusses causes of purely regurgitant aortic valves. In over 95% of stenotic aortic valves, the etiology is one of three types: congenital (primarily bicuspid), degenerative, and rheumatic. Other rare causes included active infective endocarditis, homozygous type II hyperlipoproteinemia, and systemic lupus erythematosis. The causes of pure aortic regurgitation are multiple but can be separated into diseases affecting the valve (normal aorta) (infective endocarditis, congenital bicuspid, rheumatic, floppy), diseases affecting the walls of aorta (normal valve) (syphilis, Marfan's dissection), disease affecting both aorta and valve (abnormal aorta, abnormal valve) (ankylosing spondylitis), and disease affecting neither aorta nor valve (normal aorta, normal valve) (ventricular septal defect, systemic hypertension). Diseases affecting the aortic valve alone are the most common subgroup of conditions producing purely regurgitant aortic valves.
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PMID:Pathology of aortic valve stenosis and pure aortic regurgitation: a clinical morphologic assessment--Part II. 816 82

A 56-year-old female had pure regurgitation in all cardiac valves. Color Doppler echocardiography showed a regurgitant jet in all cardiac valves. The severity of regurgitation due to the prolapse in all valves was moderate. The patient had no history of rheumatic fever, ischemic heart disease, endocarditis or hypertension. Physical characteristics of the patient were neither of Marfan's nor Ehlers-Danlos' syndrome. The etiology of regurgitation in all cardiac valves of this patient may be due to multiple valve prolapse.
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PMID:An adult case with multiple cardiac valve prolapse and regurgitation. 832 22

We report a case of rapidly fatal aortic dissection which was unusual because no intimal tear was present and no blood was present in the dissected aortic wall, although the dissection involved the entire aorta. Death was considered to be due to acute myocardial ischaemia from dissection involving the left coronary artery. Prominent cystic medial necrosis was present. There was no history of hypertension and only histological evidence of left ventricular hypertrophy. No other clinical or pathologic states associated with aortic dissection were present. Although cystic medionecrosis was the probable cause in this case, it is present in only a small proportion of patients in the absence of Marfan's syndrome, and more recently investigated defects in the composition of aortic wall connective tissue components may be more relevant factors in the causation of many cases of aortic dissection.
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PMID:Sudden death associated with bloodless aortic dissection. 833 Aug 7

Adult polycystic kidney disease is frequently associated with gastrointestinal and cardiovascular abnormalities. These include hypertension, mitral valve prolapse, mild dilation of the aortic root, abdominal aneurysms, and predisposition to aortic, mitral, and tricuspidal valve regurgitation reminiscent of Marfan's syndrome. Although the exact molecular mechanisms of adult polycystic kidney disease are not well established, a generalized defect of collagen structure is hypothesized. The most severe vascular problems, however, are typical intracranial aneurysms with a high incidence of subarachnoid hemorrhage and a high mortality rate. We report a case of dilated coronary arteries found incidentally in a patient with adult polycystic kidney disease and stress-induced angina pectoris. The typical angina pectoris of the patient is explained by left ventricular hypertrophy and coronary heart disease. Multiple liver cysts, mitral valve prolapse, and the coronary aneurysms in this patient with adult polycystic kidney disease appear to reflect the manifestation of a generalized connective tissue disorder in this syndrome.
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PMID:Aneurysms of coronary arteries in a patient with adult polycystic kidney disease: arteriosclerosis or involvement by the primary disease? 846 27

The purpose of this study was to demonstrate the special features of cardiovascular effects in connective tissue disorders through a group of fifteen cases observed over a period of ten years. The group consisted of eleven cases of Marfan syndrome (or Marfan-like syndrome), two cases of pseudoxanthoma elasticum and two cases of Ehlers-Danlos disease. The cardiovascular lesions were as follows: 1) dissection of the ascending aorta which was confirmed and had been treated surgically in 2 cases; 2) aneurysmal dilatation of the ascending aorta in 5 cases; 3) moderate mitral valve prolapse, which was isolated in 2 other cases; 4) distention of the mitral valve in 2 cases; 5) mitral valve prolapse combined with tricuspid valve prolapse in 1 case; the mitral incompetence was severe and made it necessary to carry out mitral valve replacement; 6) moderate aortic valve prolapse combined with tricuspid prolapse in a case of type-I Ehlers-Danlos disease; 7) Fallot's tetralogy combined with Marfan's syndrome and treated surgically in one case; 8) severe hypertension with abnormalities of the iliac and renal arteries in one case of elastic pseudoxanthoma elasticum. In three cases complications occurred leading to death, extension of the dissection of the abdominal aorta and global recalcitrant heart failure respectively. The outcome in the other 12 cases, with a mean follow-up time of 3 and one half years (range: 3 years to 7 years) was not marked by complications.
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PMID:[Cardiovascular manifestations of hereditary dysplasias of connective tissue]. 851 93

Several authors have reported aortic dissection in Marfan's syndrome with familial incidence. Cases of dissecting aortic aneurysms have likewise been described in families whose members had severe systemic arterial hypertension. We report 4 cases of familial aortic aneurysm, 3 of whom had a dissection, in the absence of Marfan's phenotype but with histologic findings and genetic transmission similar to those previously described as formes frustes of Marfan's syndrome.
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PMID:[Another case of dissecting aortic aneurysm with familial incidence]. 852 30

We retrospectively reviewed all patients with a final diagnosis of spontaneous thoracic aortic dissection treated at Linkou Chang Gung Memorial Hospital between January 1989 and December 1994. There were a total of 109 patients with a mean age of 55 +/- 11 years ranging from 19 to 88 years. The male-to-female ratio was 2 to 1 (73 to 36). There was a predilection to present during the colder months, with 69% seen between September 1 and February 28 and only 31% during the warmer half of the year. In most patients, hypertension (85%) was the major predisposing factor with another 7% having Marfan syndrome. The remaining 8% had no obvious underlying disease except for one patient who had an atrial septum defect. Presenting chief complaints in order of frequency included: anterior chest pain 58.7% (64/109), back pain 19.2% (21/109), abdominal pain 10.1% (11/109), consciousness change 3.7% (4/109), neck pain 2.7% (3/109), paraparesis 2.7% (3/109), dyspnea 1.8% (2/109), and hemoptysis 0.9% (1/109). The diagnostic breakdown revealed 46% to be type A (50/109) and 54% type B (59/109). A total of 26 (24%) patients died in hospital (16% were type A and 8% were type B). (Type A included all proximal dissections and those distal dissections that extend retrograde to involve the arch and ascending aorta; Type B refers to the other distal dissections without proximal extension; proposed by Daily et al.) Thoracic aortic dissection remains an important concern in patients with a history of hypertension. Patients seem particularly susceptible during cold weather months. The average age of our patients was only 55 years and 24% of them died during hospitalization. Earlier identification and more aggressive antihypertensive treatment is required.
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PMID:Aortic dissection in Taiwan. 855 68

Aortic dissection without entry and blood-flow in a false lumen was recently identified at necropsy and in vivo as intramural hemorrhage in the aortic wall (IMH). It was the purpose of the study to elucidate clinical signs and prognosis in this rare and poorly understood condition. Among 360 prospectively evaluated patients with clinical suspicion of aortic dissection, 195 patients presented with evidence of aortic disease. Of these, 25 patients (13.2%) had IMH of the thoracic aorta with no primary intimal tear, flap or overt dissection as shown by MRI (n = 12), contrast enhanced CT (n = 14) and TEE (n = 3). IMH was confirmed intraoperatively or at necropsy. There were 16 men and 9 women of ages 56 +/- 13 years; arterial hypertension was associated in 84% and Marfan's syndrome in 12%. IMH involved the ascending aorta in 12 (48%), the arch in 2 (8%), and the descending aorta in 11 cases (44%). IMH occupied 8.5 +/- 5 cm in length and 2.0 +/- 1.2 cm in aortic wall thickness. Both aortic regurgitation and pericardial/mediastinal effusion was present in 42% of type A and in 18% of type B IMH. IMH progression to overt dissection, rupture and/or acute tamponade occurred in 8/25 cases (32%) within 24 to 72 h, indicating the need for urgent surgical repair. The 30-day mortality of IMH inflicting the ascending aorta was 80% (4 of 5) with medical treatment in contrast to none (of 7) with early surgical treatment (p < 0.01). One-year survival was 71% in surgically treated patients and 20% with medical treatment (p < 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Intramural hemorrhage of the thoracic aorta as a precursor of dissection]. 857 45

By means of molecular biology and genetic research the influence of genetic factors on a great variety of human diseases could be shown. In the field of cardiovascular research the genetic defects of a few monogenetic disorders, such as Marfan's syndrome and hypertrophic cardiomyopathy, have been characterized. In addition, candidate genes for polygenetic diseases, such as arterial hypertension and atherosclerosis, have been cloned. However, the identification of a candidate gene or its mutation does not prove its influence on the phenotype or the final cause of a particular disease. Only a targeted manipulation of a defined candidate gene in a transgenic animal model helps to understand the role of the gene and its product in the whole organism. Transgenic experiments can be divided into gene-addition and gene-deletion models. In a gene-addition experiment a fusiongene is microinjected into a fertilized oocyte. The fusiongene itself consists at least of a regulatory element promoter and of a DNA sequence coding for the gene product (protein) of intended overproduction. The choice of the right promoter is important for obtaining tissue-specific gene expression. The cardiac myosin light chain-2 promoter for example leads to a ventricle-specific gene expression in cardiomyocytes from early embryogenesis through adulthood. In a gene-deletion experiment on the other hand, the target gene is selectively knocked out by homologous recombination in embryonic stem (ES) cells. The selected ES-cells are then injected into blastocysts. If the ES-cells are integrated into the germ line and transmitted to the progeny, a transgenic line is established. This review article describes planning and development of transgenic animals and discusses established transgenic animal model systems with regard to cardiovascular physiology. In addition, animal models which may provide a basis for future gene therapy will be introduced.
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PMID:[A transgenic animal model: new possibilities for cardiovascular research]. 858 70


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