UMLS:C0018799 - FACTA Search

Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Target Concepts:

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

The clinical presentation of right ventricular (RV) dysfunction due to congenital heart disease (CHD) is similar to that of cor pulmonale. RV volume and pressure loads, and primary RV myocardial dysfunction are mechanisms by which CHD affects right heart function. RV volume load may arise from pre-tricuspid left to right shunts (e.g., atrial septal defect) or regurgitant lesions in the right heart (e.g., Ebstein's anomaly of the tricuspid valve and pulmonary insufficiency after repair of tetralogy of Fallot). RV pressure load may be caused by anatomic obstruction to RV outflow and by pulmonary arteriolar hypertension. The latter can result from Eisenmenger syndrome secondary to congenital and postoperative left to right shunts or from defects that cause pulmonary venous hypertension (e.g., pulmonary vein stenosis, cor triatriatum, or mitral stenosis). The RV myocardium may be affected by a primary cardiomyopathy or by congenital abnormalities of the coronary vessels. Finally, CHD may be associated with airway obstruction, scoliosis, or pulmonary emboli, which, in turn, may lead to the development of cor pulmonale. Congenital heart disease, therefore, must be included in the differential diagnosis of patients who present with right ventricular dysfunction.
...
PMID:The right heart in congenital heart disease. 1608 51

Congenital heart disease will be the next frontier for prevention by periconceptional management of homocysteine and its metabolites by folate supplementation. Evidence for the connection between maternal and fetal folate metabolism and congenital malformations of the cardiovascular system is reviewed including possible applications to the large population of patients at risk for a child with congenital heart disease.
...
PMID:Homocysteine, folate, and congenital heart defects. 1624 51

Congenital heart disease (CHD) has a major impact on the survival of babies with esophageal atresia (EA). The present study assesses whether early diagnosis influences the management strategies in a large series of EA. Cases of EA treated between 1982 and 2002 were retrospectively divided into groups according to the presence or absence of CHD and to whether this was diagnosed or not prior to tracheo-esophageal fistula repair. Patients were also staged according to Spitz's classification in which major congenital heart disease (MCHD) are those associated with cyanosis and/or heart failure requiring surgery. Comparisons between groups of patients were made by standard statistical tests. Among 195 babies with EA (99 boys and 96 girls), 82 (42%) had CHD (31 boys, 39% and 51 girls, 61%, chi2 P < 0.05) and 43 out of these 82 (52%) had MCHD. Six children died without treatment. CHD was diagnosed prenatally in six cases and before EA repair in 26 cases. The diagnoses were missed in 12 instances (in five it was a MCHD). Regardless of preoperative diagnosis of right aortic arch in 3/6 cases, the oesophagus was approached from the right thorax in all cases and only in one of them the operation was not completed due to hemodynamic instability. There were 145/195 (75%) Spitz group I patients; 44/195 (22%) were group II and 6/195 (3%) group III. The mortality was respectively 9.5, 59 and 83%. We lost 15/113 (13%) babies with EA without CHD, 4/39 (10.2%) with EA and CHD and 26/43 (60%) with EA and MCHD. In children with CHD diagnosed prior to the esophageal operation 8/26 (30.7%) died and among those without diagnoses 3/12 (25%) died, (chi2 P > 0.1). 1. CHD was diagnosed after EA repair in one-third of cases. 2. Preoperative diagnosis of CHD changed the operative indication only in one case. 3. Survival after treatment for EA was not influenced by the moment or the accuracy of the diagnosis of CHD in this series. 4. With only a few exceptions, associated CHD should not change the strategies of EA repair.
...
PMID:Impact of preoperative diagnosis of congenital heart disease on the treatment of esophageal atresia. 1631 63

Congenital heart disease (CHD) is the most common form of cardiovascular diseases in children. In Thailand, there has not been available information about congenital heart disease in neonates. Between January 1st and December 31st, 2000, all full-term babies born at Siriraj Hospital with detected heart murmur within the first week of life were consulted to pediatric cardiologists. Echocardiography was performed for diagnosis in every baby. Total livebirths during that period were 11,245 cases. Heart murmurs were detected in 83 cases. The incidence of heart murmur within the first week of life was 7.38:1,000 livebirths. Innocent murmurs were found in 34 cases and echocardiogram revealed no detectable cardiac anomalies (2 cases), mild tricuspid regurgitation (2 cases), physiologic branch pulmonary stenosis (4 cases), and small size PDA (< 2 mm., 26 cases). Forty-nine cases had CHDs. The incidence of CHD was 4.36:1,000 livebirths. At the time of initial diagnosis, 22 cases (44.8%) were asymptomatic. Among these patients, 1 case had serious cardiac anomaly, i.e., tetralogy of Fallot. There were 27 cases with symptoms, including 15 cases (30.6%) with tachypnea, 8 cases (16.4%) with cyanosis and 4 cases (8.2%) with congestive heart failure. The 3 most common cardiac diseases were ventricular septal defect (9 cases, 18.4%), patent ductus arteriosus greater than 2 mm. (8 cases, 16.3%), and atrial septal defect (8 cases, 16.3%). Those with CHDs were treated with anticongestive medications (22 cases, 44.8%), prostaglandin E1 (5 cases, 10.2%), laser pulmonary vulvulotomy (1 case, 2%), palliative surgery within the first week of life (4 cases, 8.2%) and corrective surgery (4 cases, 8.2%). During follow-up for the period of 1 year, 2 cases died from sepsis. Early diagnosis and proper management are important to reduce morbidity and mortality in the newborn with CHD.
...
PMID:Heart murmur in the first week of life: Siriraj Hospital. 1685 37

Heart transplantation is a recognised treatment for end-stage heart failure of any cause including congenital heart disease. Congenital heart disease has contributed relatively little to the adult heart transplant activities in the past two decades. However, this is likely to change as an increasing number of children with congenital heart disease reach adulthood because of the advances in paediatric cardiology and surgery. Some of these grown-ups with congenital heart disease (GUCH patients) will need transplantation for late myocardial dysfunction either secondary to uncorrected lesions, or despite previous repair or palliative surgery. These patients are managed along the same clinical principles as those with cardiac failure of other aetiologies, despite the lack of any evidence to support this approach. Nevertheless, they introduce new challenges. First, some may have pulmonary vascular disease and require heart-lung transplantation, or lung transplantation combined with repair of their cardiac defects. Second, those with failing Fontan circulation are usually much sicker than other transplant candidates, with protein-losing enteropathy along with renal and hepatic dysfunction. Third, a suitable donor organ may not be found due to elevated levels of antibodies in response to previous blood transfusions and possibly the previous implantation of homografts. Fourth, the operation may be technically difficult because of the presence of adhesions secondary to previous operations, collaterals, and unusual anatomy. Fifth, postoperative care may be complicated because of predisposition to bleeding, infection and pulmonary hypertension, and the presence of residual aortopulmonary collaterals resulting in a significant left-to-right shunt. Despite a higher early mortality, the overall results of heart transplantation so far have been encouraging with survivals similar to that of adults with acquired heart disease and that of the paediatric population. However, this may change as the proportion of high-risk patients (failing Fontans) increases. GUCH patients with Eisenmenger's syndrome may be offered lung transplantation with repair of the cardiac defect or heart-lung transplantation. However, because of the limited success of these approaches, and improved management of pulmonary hypertension, patient selection remains difficult.
...
PMID:Transplantation for adults with congenital heart disease. 1685 76

Congenital heart diseases (CHD) are the most frequent malformation at birth. The aims of this study were to assess the prevalence of congenital heart disease, their different types, and the detection rate among children in the central Anatolian region in Turkey. The study was conducted during an eight-year period (March 1995-December 2002). The prevalence of CHD in a large tertiary care hospital in the central Anatolian region in Turkey was studied. The diagnosis of a structural defect was based on echocardiographic study. The following age groups were considered: neonates, infants and toddlers, preschool children, schoolchildren, and adolescents. In the study period, 1,693 children were found to have CHD; 1253 patients were neonates and infants. Total prevalence of CHD over the study period was 7.77 per 1000 live-born. The prevalence increased from 6.35 to 9.65 per 1000 live births between 1995 and 2002 (p < 0.05). The average age at diagnosis was 2.2 +/- 3.64 years (1 day to 18 years, median 5 months). There were 863 (51%) boys and 830 (49%) girls, with a male/female ratio of 1:1. Isolated ventricular septal defect (32.6%) was the most frequent acyanotic anomaly, and tetralogy of Fallot (5.8%) was the most frequent cyanotic anomaly. The commonest non-cardiac anomalies with CHD were musculoskeletal anomalies. Down syndrome was determined in 83 patients (78.3%) from all syndromic CHD cases. Congenital heart disease is a very significant health problem. It requires urgent measures in terms of organization of early diagnosis and proper management. The prevalence rate is comparable to that of similar developed countries. Increasing incidence of CHD might be attributed to more diagnoses with new technologic development or it may indicate a real increase in the defects.
...
PMID:Prevalence and distribution of children with congenital heart diseases in the central Anatolian region, Turkey. 1717 68

Congenital heart disease is usually regarded as an esoteric field of medicine, dealt with primarily by dedicated specialists. However, over the last two decades, increased attention has been given by the medical profession, the media and the general public to the possible association between a minor and common congenital heart defect, namely patent foramen ovale, and stroke. In recent months, unusual and unfortunate circumstances have made this topic one of the most fiercely debated medical issues in Israel. It is the belief of the authors of this paper that the association of PFO and stroke can be better understood if the PFO is viewed as part of the broader context of congenital heart disease, and as such it will be presented. Paradoxical embolism is a mechanism of stroke unique to congenital heart disease. The direction and volume of shunted blood in various conditions have a central role in determining the risk of stroke, as will be explained. With this basic knowledge in mind, we shall critically assess the potential role of PFO in stroke patients, suggesting that each case be evaluated individually using the above-mentioned principles. Conditions that enhance the formation of clot or other embolic material will be discussed briefly. The review will conclude with the various treatment options and our center's own experience with this challenging topic.
...
PMID:Stroke in congenital heart disease and patent foramen ovale. 1718 Aug 34

The adult congenital heart disease (ACHD) patient population has numerous and distinctive needs and comprise a rapidly growing group within the modern day healthcare arena. Current treatment provisions along with recommendations for future specialized multidisciplinary ACHD teams are discussed. The exclusive needs of this patient population have forced professional groups to reconsider the availability and appropriateness of current healthcare delivery to this group of complex patients, whose survivability and life expectancy have continued to improve as a direct result of enhanced research, technology, medical, surgical, and nursing care. General care considerations including psychosocial issues and patient education are presented, and reveal the challenges and strengths of caring for this adult patient population within a pediatric facility. Congenital heart disease is a lifelong illness that requires a dedicated, consistent approach to healthcare delivery in order to guarantee quality care throughout the lifespan.
...
PMID:Care of the patient with adult congenital heart disease. 1719 32

Congenital heart disease likely results from a complex mixture of environmental and genetic factors. Recent work has elucidated rare single gene mutations that cause a variety of cardiac defects, but the etiologies of more common disease remains unknown. Here, we review the known genetic causes of cardiac malformations and discuss future approaches for addressing sporadic congenital heart disease as a complex trait.
...
PMID:The genetics of cardiac birth defects. 1724 Jan 75

This review discusses the historical aspects, current state of the art, and potential future advances in the areas of nomenclature and databases for congenital heart disease. Five areas will be reviewed: (1) common language = nomenclature, (2) mechanism of data collection (database or registry) with an established uniform core data set, (3) mechanism of evaluating case complexity, (4) mechanism to ensure and verify data completeness and accuracy, and (5) collaboration between medical subspecialties. During the 1990s, both the Society of Thoracic Surgeons (STS) and the European Association for Cardiothoracic Surgery (EACTS) created congenital heart surgery outcomes databases. Beginning in 1998, the EACTS and STS collaborated in the work of the International Congenital Heart Surgery Nomenclature and Database Project. By 2000, a common congenital heart surgery nomenclature, along with a common core minimal data set, were adopted by the EACTS and the STS and published in the Annals of Thoracic Surgery. In 2000, the International Nomenclature Committee for Pediatric and Congenital Heart Disease was established; this committee eventually evolved into the International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD). The working component of ISNPCHD is the International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease, also known as the Nomenclature Working Group (NWG). By 2005, the NWG cross-mapped the EACTS-STS nomenclature with the European Paediatric Cardiac Code of the Association for European Paediatric Cardiology and created the International Paediatric and Congenital Cardiac Code (IPCCC) ( http://www.IPCCC.NET ). This common nomenclature (IPCCC), and the common minimum database data set created by the International Congenital Heart Surgery Nomenclature and Database Project, are now utilized by both EACTS and STS; since 1998, this nomenclature and database have been used by both the STS and EACTS to analyze outcomes of more than 75,000 patients. Two major multi-institutional efforts have attempted to measure case complexity; the Risk Adjustment in Congenital Heart Surgery-1 and the Aristotle Complexity Score. Efforts to unify these two scoring systems are in their early stages but are encouraging. Collaborative efforts involving the EACTS and STS are under way to develop mechanisms to verify data completeness and accuracy. Further collaborative efforts are also ongoing between pediatric and congenital heart surgeons and other subspecialties, including pediatric cardiac anesthesiologists (via the Congenital Cardiac Anesthesia Society), pediatric cardiac intensivists (via the Pediatric Cardiac Intensive Care Society), and pediatric cardiologists (via the Joint Council on Congenital Heart Disease). Clearly, methods of congenital heart disease outcomes analysis continue to evolve, with continued advances in five areas: nomenclature, database, complexity adjustment, data verification, and subspecialty collaboration.
...
PMID:Nomenclature and databases - the past, the present, and the future : a primer for the congenital heart surgeon. 1748 90

<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>