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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Angiocardiography is a standard procedure for quantification and evaluation of congenital heart disease. Knowledge of the diameters at the level of aortic valve ring, ascending and descending aorta can contribute to the decision in repair of congenital heart disease. Moreover, they are helpful for postoperative follow-up studies. In order to establish normal standards, analysis was performed on 51 cineangiograms obtained from infants, children and adolescents with no evidence of heart disease (n = 16) or only hemodynamically irrelevant cardiovascular malformations (n = 35). The individual diagnoses in these patients appear in the accompanying publication in this issue. Patients with bicuspid aortic valve or subvalvular aortic stenosis were excluded from measurements of the aortic valve ring, the sinus and the ascending aorta. Angiocardiography was performed in a fasting state, after sedation and in the supine position with injections of contrast material in the pulmonary artery and or in the left ventricle and ascending aorta. For purposes of calibration, a grid or the known diameter of the catheter was used. Systolic and diastolic diameters of the aortic valve ring (AO-R), sinus (AO-S) and distal region, between sinus valsalvae and ascending aorta (AO-D), as well as those of the isthmus (DAO-I), postisthmic region (DAO-PI) and descending thoracoabdominal aorta (DAO-T) were measured. To obtain a mean value for each vascular structure, the corresponding systolic and diastolic diameters were averaged. The mean diameter was referenced to body length and body surface area (BSA).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Normal values of the growth of the aorta in children. An angiography study]. 262 Aug 98

The clinical and necropsy findings in four cases of interrupted right aortic arch and right descending aorta associated with DiGeorge syndrome (congenital absence or hypoplasia of the thymus and parathyroids) are described. All patients had a mirror image of type B interruption, namely a right aortic arch with reversed branching pattern and an interruption between the right common carotid and right subclavian artery. In two patients there was a doubly committed subarterial ventricular septal defect and in the two other patients there was a perimembranous septal defect. Three patients had a bicuspid aortic valve. In a consecutive series of 185 necropsies in infants and children with congenital heart disease there were no cases of interrupted right aortic arch that were not associated with DiGeorge syndrome. These observations and previous reports indicate that the concurrence of these two rare conditions is more than fortuitous. In patients with an interrupted aortic arch the clinician should be aware of the common association with DiGeorge syndrome. If the interruption is associated with a right-sided descending aorta it is highly probable that the patient has DiGeorge syndrome.
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PMID:Interrupted right aortic arch in DiGeorge syndrome. 366 29

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.
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PMID:Prevalence and clinical significance of aortic valve prolapse. 401 27

During a 28-month period, consecutive 2-dimensional echocardiograms were reviewed to determine the prevalence of left ventricular (LV) false tendons, their associated anomalies and clinical significance. LV false tendons were found in 31 (0.8%) of 3,847 consecutive 2-dimensional echocardiograms. Of 31 LV false tendons, 30 passed longitudinally from papillary muscle to septum and 1 went from free wall to free wall. The 31 patients were aged 1 day to 15 years. Associated heart disease, most often ventricular septal defect, bicuspid aortic valve and coarctation of the aorta, was present in 48%, of whom 73% were girls. Of those without heart disease, 69% were boys. In patients with heart disease, precordial murmurs were due to the underlying cardiac anomaly. Of those without heart disease, 15 of 16 (94%) had a precordial murmur, usually of the Still's type over the lower left sternal border. Four of 31 (13%), 1 with and 3 without heart disease, had unifocal premature ventricular contractions that were rate-dependent in the 2 patients undergoing stress testing. LV false tendons appear to occur in 0.8% of pediatric patients and usually are accompanied by a Still's type innocent murmur if unassociated with heart disease. Some LV false tendons are associated with rate-dependent premature ventricular contractions.
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PMID:Left ventricular false tendons in children: prevalence as detected by 2-dimensional echocardiography and clinical significance. 665 Apr 14

Sixteen patients with serologically proven Q-fever infection are reviewed. Fifteen had significantly raised antibody titres to both phase I and phase II Coxiella burneti antigens, indicating persistent or chronic infection. One patient, a premature infant who died, had raised phase II titres only, but is included together with the mother who had chronic Q-fever and was the presumed source of infection. Chronic Q-fever infection has previously been regarded as virtually synonymous with Q-fever endocarditis, but only seven of the patients in this survey had evidence of valvular endocarditis. In those who did, the infection had arisen on prosthetic valves or those affected by rheumatic or syphilitic heart disease. One patient had inexorably progressive destruction of an infected congenitally bicuspid aortic valve. Eight patients had infections associated with extra-valvular sites and several of these associations have not been previously described. These include extreme prematurity with perinatal death, possibly following transplacental infection, the sudden infant death syndrome (SIDS), multiple lower limb emboli from endocarditis of an abdominal aortic dacron graft, and colonization of ventricular endocardium following left ventricular myotomy/sub-aortic diaphragm resection. The current concept that chronic Q-fever is invariably associated with endocarditis is therefore untenable and the indications for phase I antibody screening should be extended to include patients other than those under investigation for 'culture-negative' endocarditis, for example those with unusual osteomyelitis of vertebrae.
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PMID:Chronic or fatal Q-fever infection: a review of 16 patients seen in North-East Scotland (1967-80). 687 20

A decrease in severity of rheumatic fever, in the number of recurrences of the disease, a relative decrease in prevalence of rheumatic heart disease compared to other forms of heart disease are well documented or suggested by the data collected in the last decades. The reasons of the decline are uncertain. Prevalence surveys in more recent years indicate that, while at one time almost all valvular heart disease was considered to be rheumatic in origin, current data suggest that a significant proportion of damaged valves may be attributed to congenital defects or to acquired disease of other etiologies. Bicuspid aortic valve and mitral valve prolapse deserve special mention. The main clinical and echocardiographic features of bicuspid aortic valve and mitral valve prolapse are discussed.
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PMID:[Rheumatic cardiopathy in childhood. Epidemiological aspects, and differential diagnostic problems as compared with congenital bicuspid aortic valve and mitral valve prolapse]. 743 84

Sudden death is a frequent mode of fatal outcome in cardiac disease and does not exclude young people. The aim of this investigation was to establish whether and to what extent sudden death in the young may be ascribable to the substrate of underlying congenital heart disease. Among 182 young people (< or = 35 years) who died of cardiac sudden death and underwent postmortem examination, 58 (32%) had congenital heart disease. Seven showed an intrapericardial rupture of aortic dissection, in the setting of Marfan syndrome in two, isolated bicuspid aortic valve in two, and bicuspid aortic valve and isthmic coarctation in three; all exhibited equally severe degeneration of the aortic wall. Sixteen cases had conduction system anomalies, mostly bypass tracts; 15 coronary artery anomalies (three ostial valve-like stenosis, five origin from the wrong aortic sinus, and seven deep intramyocardial course); 12 hypertrophic cardiomyopathy; five postoperative congenital heart disease including scar following ventriculotomy, conduction system injury, and defects left unrepaired; and three congenital aortic valve stenosis. One third of sudden deaths in the young was ascribable to structural defects present since birth. A large spectrum of congenital heart disease involves the risk of sudden death, but most structural defects are usually not considered to be life threatening. Some of these concealed defects are potentially detectable in life by clinical imaging techniques.
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PMID:Congenital heart disease and sudden death in the young. 755 38

The many changes in classification of cardiovascular disease during the twentieth century reflect changing etiology of diseases, clinical comprehension and technological advances. In particular, the etiology of valvular heart disease has changed dramatically in the last five decades. The significant reduction of acute rheumatic fever and its sequelae, and the recognition of non-rheumatic causes of valvular disease are responsible for the metamorphosis in the etiology of valvular disorders. Valvular heart disease can be classified as follows: 1) Heritable-congenital causes of valvular heart disease e.g., floppy mitral valve with mitral valve prolapse, bicuspid aortic valve, and the Marfan syndrome; 2) Inflammatory-immunologic causes such as rheumatic fever, acquired immune deficiency syndrome, endocardial proliferative disorders, and antiphospolipid syndrome; 3) Myocardial dysfunction-ischemic cardiomyopathy, dilated or hypertrophic cardiomyopathy-resulting in valvular heart disease; 4) Diseases and disorders of other organs as causes of valvular heart disease, e.g., chronic renal failure and carcinoid heart disease; 5) Valvular heart disease related to aging: calcific aortic stenosis and mitral annular calcification; 6) Valvular disease following interventions such as valvuloplasty, valve reconstructive surgery and valve replacement; and 7) Valvular disease related to drugs and physical agents, such as chronic ergotamine use, radiation therapy and trauma. In clinical practice the most common causes of mitral regurgitation are floppy mitral valve with mitral valve prolapse, ischemic heart disease, dilated cardiomyopathy and mitral annular calcification, while the most common cause of mitral stenosis is rheumatic fever. The most common causes of isolated aortic regurgitation are bicuspid aortic valve and floppy aortic valve, while the most common causes of isolated aortic stenosis are related to the bicuspid aortic valve and the development of calcific senile aortic stenosis. The most common causes of tricuspid regurgitation are dilated cardiomyopathy, ischemic cardiomyopathy, floppy tricuspid valve with tricuspid valve prolapse and infectious endocarditis. Combined mitral and tricuspid regurgitation occur with heritable connective tissue disorders, dilated or ischemic cardiomyopathy, while the most common cause of mitral stenosis plus aortic regurgitation is rheumatic fever. Statistics obtained from cardiac surgery and necropsy may underestimate the true incidence of certain valvular diseases by selection bias. This is particularly so with valvular disease associated with significant ventricular dysfunction, or in the elderly who may not be surgical candidates, or in cases where the valvular disease is not severe enough to require surgical intervention. Recent advances in hemodynamic and imaging technology allow clinicians to define valvular structure and function and to accurately classify valvular heart disease in clinical practice.
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PMID:Valvular heart disease: the influence of changing etiology on nosology. 800 Jun 16

Among 236 aortic valves surgically excised at the Mayo Clinic in 1990 (mean patient age, 66 years; age range, 10 to 92 years), 154 (65%) were stenotic, 58 (25%) were insufficient, and 24 (10%) were both stenotic and insufficient. Pure stenosis was related to calcification, and causes included degenerative (51%), bicuspid (36%), post-inflammatory (9%), and other (4%) reasons. Fourteen (9%) valves with pure stenosis also underwent ventricular septal myectomy, 12 for hypertrophy and two for co-existent hypertrophic cardiomyopathy. Pure insufficiency was not related to calcification, and causes included aortic root dilatation (50%), bicuspid valve (14%), post-inflammatory (14%), post-therapeutic (14%), and other (8%) reasons. Combined stenosis and insufficiency was secondary to degenerative calcification (46%), bicuspid and post-inflammatory etiologies (17% each), post-therapeutic (13%), and indeterminate (8%) causes. New observations include the following findings: (1) degenerative (senile) disease is the most common cause of aortic stenosis and combined stenosis and insufficiency at the Mayo Clinic, (2) aortic root dilatation is the most common cause of pure aortic insufficiency, (3) post-therapeutic aortic valve disease now leads to valve replacement in a substantial percentage of patients, particularly among those with insufficiency, (4) post-inflammatory (presumably rheumatic) disease is relatively uncommon in all three functional categories, (5) septal myectomy may be performed for hypertrophic states other than hypertrophic cardiomyopathy, and (6) adults with operated congenital heart disease are undergoing valve replacement for annular dilatation with insufficiency. Because of the increasing age of the general population, the prominence of age-related degenerative aortic valve calcification and aortic root dilatation may have important implications concerning future health care costs.
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PMID:New observations on the etiology of aortic valve disease: a surgical pathologic study of 236 cases from 1990. 827 80

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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