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

A survey of 1,950 phonocardiograms recorded over a 6-year period revealed 170 (9%) with a distinct aortic ejection sound. All patients were men with a mean age of 61 years (range 29 to 88). Associated clinical features were: aortic stenosis in 28%, history of systemic hypertension in 10%, history of rheumatic fever in 4% and none of these features in 58% of patients. In 141 (83%) of 170 patients the aortic ejection sound occurred simultaneously with or 0.01 second before or after the onset of the rise of the externally recorded carotid pulse. In 37 (66%) of 56 patients who had simultaneous echocardiograms and phonocardiograms recorded, the aortic ejection sound occurred at 0.01 second before or after the maximal opening point of the aortic valve leaflets. Two-dimensional echocardiography was performed in all patients and a bicuspid aortic valve was identified in 38 patients (22%). In 83 patients (49%) 3 cusps were clearly seen. In 49 patients (29%) an accurate determination was not possible. Anatomic examination of 120 consecutive aortic valves at autopsy was performed to identify possible causes of the aortic ejection sound. In 18 (15%) of autopsies fusion of 2 aortic cusps extending greater than or equal to 5 mm from the attachment to the aorta was observed. This abnormality, aortic commissural fusion, may be congenital or acquired. It is concluded that aortic ejection sounds may occur in patients without bicuspid aortic valves and in a variety of clinical conditions. A moderate degree of cuspal fusion may be the cause of the sound.
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PMID:Prevalence and characteristics of the aortic ejection sound in adults. 333 3

We have described some of the cardiological findings in 66 patients with Williams-Beuren syndrome and analysed the two dimensional cross sectional echocardiograms in 61 of them in comparison with normal controls. Supravalvar aortic narrowing was shown in all patients examined echocardiographically and may be a useful diagnostic sign. We documented a 7.8% incidence of systemic hypertension, a 15% clinical and echocardiographic incidence of mitral valve prolapse, and a 11.6% incidence of bicuspid aortic valve.
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PMID:Cardiac anomalies in Williams-Beuren syndrome. 341 98

We report two patients with Turner syndrome who had aortic dissection and rupture, one with prior repair of coarctation. We also note the high incidence (8.8%) of unrecognized aortic root dilation in a group of 57 patients with Turner syndrome whom we prospectively evaluated by echocardiography. Our analysis and review of previously reported cases suggests that multiple risk factors may exist for aortic dissection, including coarctation, bicuspid aortic valve, and systemic hypertension, but that these need not be present. Aortic root dilation may be an additional finding that suggests the patient with Turner syndrome is also at risk. When it is present, magnetic resonance imaging visualizes the entire aorta and allows quantification of the site and degree of dilation. In patients with dissection, the aorta often exhibits pathologic evidence of cystic medial necrosis similar to the finding in patients with Marfan syndrome. Therapeutic methods to decrease risk, such as those directed toward prevention of bacterial endocarditis, blood pressure control, and perhaps prophylactic beta blockade or surgical reconstruction, may need to be considered. Patients with Turner syndrome, their families, and the physicians who care for them should be aware of the significance of unexplained chest pain, dyspnea, or hypotension as potential manifestations of aortic dissection or rupture.
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PMID:Aortic dilation, dissection, and rupture in patients with Turner syndrome. 377 61

A coarctectomy performed on infants with congestive heart failure in the first year of life is an emergency procedure due to the "coarctation syndrome". An intracardiac anomaly is also present in the majority of cases. The surgical risk of coarctectomy in the newborn infant or during the first year of life is now low (7 percent in our study), but intermediate-term mortality rates were relatively significant (24 percent in our study), due to intracardiac anomalies and their surgical correction. Without treatment, however, the coarctation syndrome proves fatal in two out of three cases before one year of age. The principal long-term complication is recurrence of the coarctation resulting from the absence of growth at the anastomosis. It occurs in 20 to 50 percent of cases depending on the study, the diagnostic criteria, and length of time since the surgery. This complication is more frequent when the coarctectomy was performed in the first month of life. Another coarctectomy must be undertaken in these cases. Persistent arterial hypertension without recurring coarctation is rarely observed, but is frequently encountered following a coarctectomy in an older child or adult. It should be noted that the systolic arterial blood pressure in almost all infants in our study was between the 50th and 97th percentile for normal children of this age. Aside from the problem of recurring coarctation, the long-term follow-up depends principally on associated valvular lesions, particularly aortic (bicuspid valves are very frequent) and mitral valvular lesions. Even though surgery has greatly improved the prognosis of coarctation in children, these infants must have long-term postoperative surveillance.
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PMID:[Outcome of patients operated on for aortic coarctation during the 1st year of life]. 397 73

A patient with XO Turner's syndrome with a 12-year history of progressive aortic root dilatation resulting in chronic aortic regurgitation is presented. Her case is unique in that it occurred in the absence of coarctation of the aorta, bicuspid aortic valve, or hypertension. Idiopathic dilatation of the aorta may be an additional risk factor to the development of aortic dissection in the setting of Turner's syndrome.
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PMID:Aortic dilatation resulting in chronic aortic regurgitation and complicated by aortic dissection in a patient with Turner's syndrome. 652 81

Among 161 necropsy cases of aortic dissection, 87 (54%) were type I, 34 (21%) type II, and 40 (25%) type III, and an intimal tear was identified in each. Systemic hypertension had been present in 63 of 121 cases (52%) with type I or II dissection and in 30 of 40 (75%) with type III dissection. Aortic dissection involved 7 of 16 cases (44%) with the Marfan syndrome. In the 154 cases without the Marfan syndrome, grade 3 or 4 medial degeneration (cystic medial necrosis) was observed in the ascending aorta in only 27 (18%). The risk of aortic dissection in persons with congenitally bicuspid and unicommissural aortic valves, respectively, was 9 and 18 times that in subjects with tricuspid aortic valves. The mean age of those with aortic dissection and tricuspid, bicuspid and unicommissural aortic valves was 63, 55 and 40 years, respectively, and aortic dissection was more common in men than in women. Grade 3 or 4 atherosclerosis involved the intimal tear in only 11 of 121 type I or II dissections (9%) but 32 of 40 type III dissections (80%). Accordingly, the major risk factors for aortic dissection were systemic hypertension, the Marfan syndrome, and, for type I and II dissections, congenitally bicuspid or unicommissural aortic valves. Aortic medial degeneration was a less important risk factor. Rupture of ulcerocalcific aortic atheromas may have initiated the intimal tear in some type III dissections.
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PMID:Risk factors for aortic dissection: a necropsy study of 161 cases. 670 37

A 6-year-old boy with a congenital bicuspid aortic valve presented with finger clubbing and hypertrophic osteoarthopathy, and subsequently he developed severe hypertension. The hypertension was successfully treated by nephrectomy, at which a thrombosed mycotic aneurysm of the renal artey was found. Echocardiography showed the presence of aortic valve vegetations. Blood cultures were sterile, but high antibody titres to the phase 1 and 2 antigens of Coxiella burneti strongly suggested Q fever infection. We believe this is the first reported case of Q fever endocarditis in early childhood.
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PMID:Q fever endocarditis in a 6-year-old child. 741 83

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

We report 2 cases of aortic coarctation in women aged 18.5 and 24 years, respectively. In 1 the malformation was found during gestation, while the other became pregnant after the diagnosis was made. Both had uneventful pregnancies and delivered healthy babies. Coarctation of the aorta is found in approximately 7% of those with congenital heart disease. The clinical diagnosis is made from the absence of femoral artery pulsations and low arterial pressure in arteries originating distal to the coarctation and hypertension in those proximal to it. Since it occurs mainly in males and is usually corrected during infancy, its incidence in pregnancy is low. Maternal morbidity may be high, since pregnancy increases the risk of aortic rupture or dissection, as well as of cerebral hemorrhage from rupture of an aneurysm of the circle of Willis. Surgical repair before conception reduces the risk of aortic complications during pregnancy, and also decreases the incidence of fetal complications, such as IUGR or congenital heart disease. The outcome of pregnancy is also influenced by coexistence of other malformations, such as bicuspid aortic valve which leads to infective endocarditis. In uncomplicated coarctation, pregnancy and delivery are usually uneventful. Cesarean section is done mainly for obstetric indications.
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PMID:[Pregnancy and delivery in women with coarctation of the aorta]. 837 67


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