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

We present our experience in the diagnostic assessment of a wide spectrum of cardiovascular disorders using multiplane transesophageal echocardiography (MP-TEE). Two hundred and seventeen patients in the age range of 11-71 years were subjected to MP-TEE from January to November 1993. The male:female ratio was 1.1:1. One hundred and ten patients had predominantly mitral valve disease of rheumatic origin, eleven had mitral valve prolapse, twenty patients had aortic valve disease and thirty seven patients had more than one valve involvement. Six patients with suspected prosthetic heart valve dysfunction and ten patients of hypertrophic cardiomyopathy were also studied. Two patients had unexplained pulmonary hypertension, three had pericardial disease and three had proximal aortic dissections. Twenty six patients with congenital heart disease were studied of which nineteen had atrial septal defects, one had corrected transposition of great vessels with pulmonic stenosis and one adult had Ebstein's anomaly of the tricuspid valve. In our experience, MP-TEE enhances the versatility of TEE by providing incremental diagnostic information and enhancing delineation of pathology. The procedure was well-tolerated and no complications occurred.
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PMID:Multiplane transesophageal echocardiography: our initial experience. 782 33

Fifty children with established congenital heart disease (CHD) were surveyed for the immune profile. Ventricular septal defect (VSD) was the commonest lesion (56%) followed by Tetralogy of Fallot (ToF; 16%), atrial septal defect (ASD; 8%), patent ductus arteriosus (PDA; 4%), transposition of great arteries (TGA; 4%), aortic stenosis (AS; 4%), and pulmonic stenosis (PS), tricuspid atresia (TA), single ventricle with pulmonic stenosis (SV with PS) and dextrocardia with ToF (2% each). Immunoglobulins (IgG, IgA and IgM) were estimated. IgG and IgA levels were significantly reduced in all children with congenital heart disease, whereas IgM levels were increased in cyanotic but unaffected in the acyanotic group. Complement C3 and C4 levels were reduced in all, more so in cyanotics. T-helper cells were decreased and T-suppressor cells were increased in all groups with congenital heart disease as compared to controls. B-cell percentage was increased in cyanotics but not affected in the acyanotics.
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PMID:Immunological profile in congenital heart disease. 789 64

Cardiopulmonary exercise capacity is a significant criterion of life quality. The evaluation of the exercise capacity is important to answer patient-questions concerning every day activity, choice of profession, sports-activity etc. We performed cardiopulmonary exercise testing in 38 patients (age 33.6 +/- 12.0 years, 18 women, 20 men) with different congenital heart disease (5 after surgical repair of tetralogy of fallot, 2 after Mustard-operation in transposition of the great arteries (TGA), 1 single ventricle, 14 atrial septal defect (ASD), 8 ventricular septal defect (VSD), 8 pulmonary valve stenosis (PS)) during outpatient routine control. All tests were performed on upright bicycle with continuous ramp program of 20 Watt increase/minute. Ventilatory values as O2-uptake, CO2-production, minute ventilation (VE) were measured breath-by-breath. Max. VO2 was reduced as average value for every patient group (tetralogy of fallot 60.2 +/- 20.3% pred., TGA 53.0 +/- 0.0% pred., single ventricle 35% pred., closed ASD 71.9 +/- 23.8% pred., ASD 62.7 +/- 30.0% pred., VSD 64.1 +/- 11.7% pred., PS 73.2 +/- 16.0% pred.). Anaerobic threshold was reduced in tetralogy of fallot (35.9 +/- 12.2% pred. max. VO2) and in single ventricle (28.3% pred. max. VO2). In comparison with clinical classification of exercise capacity we found for max. VO2 differences in 23/38 patients. 22/23 patients reported no exercise limitation but had reduced max. VO2. One patient had a normal max. VO 2 but complaints of exercise dyspnoea. For anaerobic threshold 18/38 patients had discrepancies in objective and subjective estimation of their exercise capacity.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Cardiopulmonary capacity of patients with congenital heart defects in childhood, adolescence and adulthood]. 794 60

For 17 years, 4150 infants and children under 13 years of age with final diagnosis of well defined congenital heart disease were studied and the pattern of sex differences are reported. In general, the sex distribution was equal: 2108 males (50.8%) and 2042 females (49.2%) with a male to female ratio of 1.03. Male sex predominance was marked for the following specific lesions: aortic stenosis (valvar and subvalvular) (70%), coarctation of the aorta (66%), transposition of the great arteries (60%), univentricular heart (76%), tricuspid atresia (63%), Ebstein anomaly (76%), hypoplastic left heart syndrome (85%), vascular rings (77%) and right midventricular stenosis (70%). Female sex predominance was marked for the following specific lesions: Persistent ductus arteriosus (72%), atrioventricular septal defects (62%) supravalvular aortic stenosis (71%) and isolated infundibular pulmonic stenosis (80%). A more balanced sex distribution was confirmed for cases of congenital rubella syndrome with persistent ductus arteriosus as isolated cardiovascular malformation (56%). The Importance to establish the sex dominance is emphasized as predictive of recurrence risk of congenital heart disease.
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PMID:[The sex distribution of congenital cardiopathies]. 799 42

Anatomic evidence of intrauterine closure of ventricular septal defects (VSDs) has been reported rarely. Between 1985 and 1990, 112 autopsies were performed at the Mayo Clinic on third trimester stillborns and infants who died during the first week of life. There were 21 (19%) cases of congenital heart disease. VSD was found in 12 (11%) cases: in eight (7%) as a part of a more complex heart defect and in four (4%) as an isolated lesion. Two cases with membranous VSDs with tricuspid valve tissue partially occluding the ventricular septal defect were found. A 2280-g female infant (case 1) with trisomy 18 died at 4 days of age. Autopsy revealed bilateral superior venae cavae, a large atrial septal defect, cor triatriatum, an atypical tricuspid valve with large septal leaflet partially obstructing a large membranous VSD, a hypoplastic right ventricle, and severe pulmonic stenosis. A 2610-g female infant (case 2), born with congenital heart block died at 4 days of age. Autopsy revealed cor triatriatum dexter obstructing the tricuspid orifice, a large membranous VSD partially obstructed by the septal leaflet of the tricuspid valve, four small muscular VSDs, and pulmonic stenosis. These cases suggest that closure of membranous VSDs may begin in utero and the mechanism of closure is similar to that reported postnatally.
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PMID:Intrauterine closure of membranous ventricular septal defects: mechanism of closure in two autopsy specimens. 811 70

The paediatric therapeutic heart catheterization becomes more and more important in the treatment of congenital heart disease. Depending on the situation, interventional therapy can become an alternative or a replacement or supplement to surgical intervention. The methods of interventional therapy include the balloon valvuloplasty of stenotic valves or vessels, the implantation of stents and the occlusion of defects as well as of unwanted vascular structures with coil embolisation or umbrella devices. The balloon valvuloplasty of pulmonary valve stenosis or the embolisation of small vessels have become the treatment of choice. Valvuloplasty of aortic stenosis, angioplasty of residual coarctation, balloon dilatation of peripheral pulmonic stenosis and occlusion of a patent ductus arteriosus with a Rashkind PDA-occluder are now a valuable alternative to surgical procedure although studies of long-term results are not yet available. Occlusions of atrial or ventricular septal defects as well as the implantation of stents are still investigational tools, but give enormous hope for the future.
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PMID:[Interventional catheter therapy in childhood]. 827 83

Over the last 10 years, several advances have been made in paediatric cardiology and cardiac surgery. However, the major developments have been in non-surgical attempts at transcatheter treatment of congenital heart disease. Initially these concerned some simple defects such as pulmonary valve stenosis but lately much more high-risk and complex defects have been treated.
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PMID:Catheter treatment of congenital heart disease. 828 51

Aerobic capacity of patients with different forms of congenital heart disease was serially evaluated in 79 patients and the evolution was correlated with the lesion and the level of daily activity. The patients were divided into six groups: patients with a small ventricular septal defect (VSD) with mini shunt (n = 14), mild pulmonary valve stenosis with gradient < 40 mm Hg (PS) (n = 12), mild to moderate aortic valve stenosis (gradient 36 +/- 17 mmHg) (AS) (n = 12), patients 4.7 +/- 2.1 years after repair of tetralogy of Fallot (PO-TF) (n = 16), patients 2.2 +/- 2.9 years after closure of a high flow/high gradient VSD (PO-VSD) (n = 13), and patients 2.6 +/- 1.7 years after Fontan repair (Fontan-PO) (n = 12). Aerobic capacity was assessed by determination of the ventilatory anaerobic threshold (VAT). VAT reflects the highest aerobic exercise level prior to a disproportionate increase of CO2 and ventilation relative to O2 uptake; it is independent of patient motivation. Data are expressed as percentage of normal O2 uptake at VAT, determined in 234 age/gender matched controls. The habitual level of physical activity was assessed by a standardised questionnaire. Aerobic capacity in all subgroups of patients, even with very mild defects, was at or below the lower limit of normal. Children left unrestricted from physical exercise (VSD, PS, PO-VSD) had no change over the study period. However, aerobic capacity of patients with medically imposed physical restrictions (AS) and significant residual haemodynamic lesions (PO-TF, Fontan) decreased with age.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Serial cardiorespiratory exercise testing in patients with congenital heart disease. 852 76

Retinoid-dependent pathways play a central role in regulating cardiac morphogenesis. Recently, we characterized gene-targeted RXR alpha -/- embryos, which display an atrial-like ventricular phenotype with the development of heart failure and lethality at embryonic day 14.5. To quantitate the frequency and complexity of cardiac morphogenic defects, we now use microdissection and scanning electron microscopy to examine 107 wild-type, heterozygous, and homozygous embryos at embryonic day 13.5, 14.5, and 15.5. RXR alpha -/- embryos display complex defects, including ventricular septal, atrioventricular cushion, and conotruncal ridge defects, with double outlet right ventricle, aorticopulmonary window, and persistent truncus arteriosus. In addition, heterozygous RXR alpha embryos display a predisposition for trabecular and papillary muscle defects, ventricular septal defects, conotruncal ridge defects, atrioventricular cushion defects, and pulmonic stenosis. Lastly, we show that the intermediate anatomic phenotype displayed by heterozygous embryos is mirrored in the molecular marker MLC-2a. The intermediate phenotype of RXR alpha heterozygous embryos documents a gene dosage effect for RXR alpha in maintaining normal cardiac morphogenesis. In addition, some defects in RXR alpha mutant mice are phenocopies of human congenital heart defects, thereby suggesting that a relative deficiency in RXR alpha or molecules downstream in its signaling pathway may represent congenital heart disease-susceptibility genes.
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PMID:RXR alpha deficiency confers genetic susceptibility for aortic sac, conotruncal, atrioventricular cushion, and ventricular muscle defects in mice. 882 98

The case records of 151 dogs diagnosed with congenital heart disease were reviewed retrospectively. The most common defect was aortic stenosis, accounting for 35 per cent of all cases, followed by pulmonic stenosis (20 per cent), ventricular septal defect (12 per cent), patent ductus arteriosus (11 per cent), mitral valve dysplasia (8 per cent), tricuspid valve dysplasia (7 per cent), endocardial fibroelastosis (1.9 per cent) and tetralogy of Fallot (0.6 per cent). Fifty-one breeds were represented, with golden retrievers, German shepherd dogs and boxers predominating. No overall sex predilection was obvious. Seventy-five per cent of the dogs were asymptomatic at presentation. The defects most often associated with presenting symptoms, such as dyspnoea, syncope, ascites, failure to grow and depression, were mitral valve dysplasia, atrial septal defect, tricuspid valve dysplasia and endocardial fibroelastosis. The latter presented with the most severe signs of heart failure. In some cases of aortic stenosis and pulmonic stenosis, where the defect could not be accurately visualised with two-dimensional echocardiography, Doppler echocardiographic examination was needed for definitive diagnosis.
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PMID:Retrospective study of congenital heart defects in 151 dogs. 909 39


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