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

Congenital heart disease complicates the management of most accessory pathway-mediated tachycardias and also increases the challenge of radiofrequency ablation. Since 1990, radiofrequency ablation of accessory atrioventricular (AV) pathways has been attempted in 10 patients (age range 3.5 months to 30 years) with congenital heart disease: Ebstein's anomaly (n = 5), heterotaxy with AV discordance (n = 3), tetralogy of Fallot (n = 1) and total anomalous pulmonary venous return (n = 1). Eight patients had manifest Wolff-Parkinson-White syndrome and 2 had concealed pathways. Five patients had multiple pathways including 4 of the 5 with Ebstein's anomaly. Of 16 pathways total, 15 were associated with the tricuspid valve including all pathways in the patients with Ebstein's anomaly and heterotaxy. The His bundle area was identified in all patients and involved an anterior AV node in 2 of 3 with heterotaxy. Ablation was performed on the atrial side of the AV ring in all cases. No instance of AV block was encountered. Complete success was achieved in 6 patients including the 3 with heterotaxy. In 2 patients, manifest preexcitation was eliminated and clinical symptoms were greatly modified. The procedure was transiently successful in 1 patient who later had surgical interruption of the accessory pathway during tetralogy of Fallot repair. Ablation was unsuccessful in 1 patient. Thus, the overall success was 80%.
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PMID:Radiofrequency ablation of accessory pathways associated with congenital heart disease including heterotaxy syndrome. 824 46

The authors examined the level of the atrio-natriuretic factor (ANF) in 7 infants with inborn heart disease at the age of 3 weeks to 5.5 months. As controls they examined 9 convalescent infants aged 1-10 months. Congenital heart disease was confirmed by clinical methods (ECG and X-ray) and echocardiographically (one-dimensional, two-dimensional and pulsed Doppler echocardiography, 5MHz probe, Kontron Co.). Although a small group is involved, elevated ANF values were recorded in infants with inborn heart disease, as compared with controls.
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PMID:[Atrial natriuretic factor in congenital heart defects in infants]. 840 46

Common pulmonary vein atresia is a rare form of cyanotic congenital heart disease in which the pulmonary veins join to form a blind confluence that does not communicate with the heart or the major systemic veins. Twenty-one cases have been reported since the lesion was first described in 1962; only two patients with this lesion have survived. Over a 4-year period, common pulmonary vein atresia was diagnosed in five newborns referred to the San Diego Regional Extracorporeal Membrane Oxygenation Program. All five improved dramatically as a result of venoarterial bypass. Congenital heart disease was diagnosed at autopsy in the initial case and by cardiac ultrasound and/or catheterization in the others. Surgical repair was attempted in three neonates; all three required continued extracorporeal membrane oxygenation support postoperatively because of pulmonary hypertension and severe pulmonary parenchymal disease. One infant died of respiratory insufficiency at 3 months of age. The other two survived and were discharged from the hospital. The diagnostic and therapeutic dilemmas posed by this lesion and the life-saving potential for extracorporeal membrane oxygenation in this rapidly fatal cardiac anomaly are the bases of this report.
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PMID:Common pulmonary vein atresia: the role of extracorporeal membrane oxygenation. 842 18

In adults with congenital heart disease who are confronted with noncardiac surgery, perioperative risks can be reduced, often appreciably, when problems inherent to this patient population are anticipated. The first necessity is to clarify the diagnosis and to be certain that appropriate information is obtained from a cardiologist with adequate knowledge of congenital heart disease in adults. Physiology and anatomy can vary significantly among patients who superficially carry identical diagnoses and would seem to fit under the same rubric. Elective noncardiac surgery should be preceded by clinical cardiovascular assessment, including reviewing clinical and laboratory data and securing necessary diagnostic studies. Preoperative assessment should be performed far enough in advance of the anticipated surgery to allow for critical assessment of the data. Appropriate cardiovascular laboratory studies to be obtained or reviewed include electrocardiograms, chest radiographs, echocardiograms, and cardiac catheterization data (which may include specialized intracardiac electrophysiologic testing). Congenital heart disease in adults is a new and evolving area of special interest and expertise in cardiovascular medicine. Multidisciplinary centers for the care of these patients are being developed. The 22nd Bethesda Conference recommended that these centers include anesthesiologists with special expertise in managing patients with congenital heart disease among their consultants. These anesthesiologists can function either as attending anesthesiologists when patients require noncardiac surgery, or as consultants and resource individuals to other anesthesiologists. Adults with congenital heart disease may present with age-related acquired cardiovascular and noncardiovascular disorders in addition to postoperative cardiac residua and sequelae, all of which require meticulous preoperative planning and consultation before noncardiac surgery is performed. We recommend that, particularly in larger departments of anesthesiology, select members of the department serve as consultants to anesthesiologists and to other members of the medical staff for these cases, especially when large numbers of adults with congenital heart disease are to be treated.
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PMID:Anesthetic implications of adults with congenital heart disease. 849 76

Pacemaker lead removal using interlocking stylets and dilator sheaths has greatly reduced the need for major surgical intervention when lead extraction is required. Previous reports have shown the utility of this method in older patients, most of whom have anatomically normal hearts. The purpose of this study is to report the results of this technique in young patients with and without congenital heart disease. There were 13 patients (M:F = 7:6) aged 9-26 years (median 13). Congenital heart disease was present in 8 of 13 patients. A total of 17 leads required removal; they had been implanted for 54 +/- 24 months (range 19-94). Leads were removed from the left subclavian vein (13) or right subclavian vein (4) only. Seventeen of 18 leads were completely removed and one partially retained in the left subclavian vein. New leads were implanted from the same vein in 11 of 13 patients. Interlocking stylets and metal or flexible dilator sheaths were used in all cases except two. There was one surgical complication: a late wound dehiscence, which was easily managed. No patient required a transfusion, and there was no structural damage noted in any patient on the postoperative echocardiogram. We conclude that lead removal using interlocking stylets and dilator sheaths from the subclavian approach is an effective technique that can be used in young patients, including those with congenital heart disease.
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PMID:Lead extraction in young patients with and without congenital heart disease using the subclavian approach. 873 44

In adults with congenital heart disease who are confronted with noncardiac surgery, perioperative risks can be reduced, often appreciably, when problems inherent to this patient population are anticipated. The first necessity is to clarify the diagnosis and to be certain that appropriate information is obtained from a cardiologist with adequate knowledge of congenital heart disease in adults. Physiology and anatomy can vary significantly among patients who superficially carry identical diagnoses. Elective noncardiac surgery should be preceded by clinical assessment including review of clinical and laboratory data and securing the results of necessary diagnostic studies. Preoperative assessment should be performed far enough in advance of the anticipated date of surgery to allow critical assessment of the data and potential discussions with colleagues. Appropriate cardiovascular laboratory studies to be obtained or reviewed include electrocardiograms, chest radiographs, echocardiograms, and cardiac catheterization data, which may include specialized intracardiac electrophysiologic testing. Congenital heart disease in adults is a new and evolving area of special interest and expertise in cardiovascular medicine. Multidisciplinary centers for the care of these patients are being developed. The 22nd Bethesda Conference recommended that these centers include among their consultants anesthesiologists with special expertise in managing patients with congenital heart disease. These anesthesiologists can have the option of serving either as the attending anesthesiologists when patients require noncardiac surgery or as consultants and resource individuals to other anesthesiologists.
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PMID:The adult patient with congenital heart disease. 885 Apr 11

Congenital heart disease in adults has become a special area of cardiovascular interest, but remains a relatively unfamiliar discipline. Advances in diagnostic and surgical techniques have had a striking impact on longevity in infants and children with congenital heart disease. Nevertheless, since true cures are rare, long-term care is obligatory for most if not all patients. In parallel with diagnostic and surgical developments, advances in medical management have had a major impact on life span in both postoperative and unoperated patients. Who will assume responsibility for the long-term care of these adult patients? Relatively few medical cardiologists are equipped to do so and relatively few pediatric cardiologists are sufficiently well versed with the accrued problems of aging. Accordingly, the current approach involves collaboration between pediatric and medical cardiologists. The purpose of this report is to underscore the importance of this collaboration, and to focus on 2 particular issues-the management of cyanotic congenital heart disease and the management of congenital heart disease and pregnancy.
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PMID:Congenital heart disease in adults--collaboration between pediatric and medical cardiologists. 895 98

Congenital heart disease (CHD), cardiomyopathy, and vasculopathies are common causes of mortality and morbidity in pediatrics, including the perinatal period. This article reviews evidence that single gene defects cause many of the pediatric heart diseases. Vasculopathies discussed include Marfan's syndrome, supravalvar aortic stenosis and Williams' syndrome, Alagille's syndrome, and hereditary telangiectasia, the Osler-Weber-Rendu syndrome. Genetic causes of hypertrophic cardiomyopathy caused by sarcomeric protein mutations (beta-cardiac myosin heavy chain) and of dilated cardiomyopathy secondary to structural protein deficiencies (dystrophin) are presented. Defects in proteins essential for myocardial energy production such as oxidative phosphorylation proteins and fatty acid oxidation genes that cause cardiomyopathy or sudden death are described. Gene ablation models in mice, such as RXR alpha and homeobox gene knockouts, which result in cardiac phenotypes resembling human congenital heart disease, are described. Familial types of human CHD which are being investigated for genetic causes by positional cloning methods and known cytogenetic causes of CHD, including the CATCH-22 syndrome and monosomy at 22q11, are presented. General lessons and principles derived from these new and exciting discoveries in human cardiovascular development are surmised.
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PMID:The genetic basis of pediatric cardiovascular disease. 909 Jul 81

Congenital heart disease is an important cause of infant mortality and disability. The frequency, spectrum and contributory risk factors for significant cardiovascular malformations among live-births was retrospectively evaluated at the Aga Khan University Hospital. Of a total of 8331 live births between July, 1987 and December, 1992 34 babies were diagnosed to have congenital heart disease in the neonatal period giving a prevalence of 4 per 1000 live births. Ventricular septal defects was the most common (n = 10, 29%) abnormality. Eight cases had associated chromosomal abnormality, the most common being Trisomy 21. Maternal abortions, still-births, consanguinity and diabetes mellitus were not found to be significant risk factors for congenital heart disease in this survey.
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PMID:Profile and risk factors for congenital heart disease. 913 58

We surveyed the incidence of congenital heart disease in 49887 native live born children in the period between 1984 to 1994 in Qatar. Each child with clinically suspected congenital heart disease underwent echocardiographic examination. Magnetic resonance imaging, cardiac catheterization and surgical intervention were done at the discretion of the patient's pediatric cardiologist. Virtually no postmortem examinations were performed. Children with congenital heart disease were entered into a computerized database and were then followed for 1-11 years. Congenital heart disease was diagnosed in 610 of 49,887 children for an incidence of 12.23/1000 live births. The reasons for the high incidence were high proportion of small muscular ventricular septal defects discovered before the time of their spontaneous closure, referral to and follow up by a single group of pediatric cardiologists, location of the pediatric cardiology service in the same setting where nearly all of the deliveries took place, freely available health care service, and echocardiographic examination of every child with a clinical diagnosis of congenital heart disease.
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PMID:Incidence of congenital heart disease in Qatari children. 920 35


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