UMLS:C0018799 - FACTA Search

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

During the last 15 years, 34 children were treated for a brain abscess. 13 (38%) had cyanotic heart disease; 12 (35%) had an ENT infection. The standardized treatment protocol included puncture of the abscess and administration of antibiotic and antiedematous drugs. Postoperative permanent epidural monitoring of intracranial pressure and, in the last 5 years, repeated CT scans indicated if retapping the abscess or reinforcing the antiedematous treatment was necessary. In 7 out of these 34 cases the abscess had to be excised. Postoperative mortality rate was 6%. Overall mortality rate was 12%. Neurological sequelae were slight. Epilepsy frequency was 10%. Mental retardation (18%) was found only in cyanotic congenital heart disease to which it is related. Repeated CT scans demonstrated the progressive disappearance of the capsule within a few months. This study shows an improvement in the results when brain abscesses in children are punctured rather than excised.
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PMID:Brain abscess in childhood. A study of 34 cases treated by puncture and antibiotics. 688 27

The protein CD62P expressed on platelet surface membrane was measured by flow cytometry to evaluate its clinical significance. Whole blood contained 0.32% citrate from 64 patients with heart disease and 30 healthy adults were fixed with 0.1% paraformaldehyde. To 50 microliters of the fixed blood, 10 microliters of CD62 PE (Becton Dickinson) was added. After standing for 20 minutes at room temperature, the samples were washed and suspended in 1ml of PBS. Platelets were analyzed with Spectrum III (Ortho) flow cytometer. In healthy control, the percentage of platelets positive for anti-CD62P was 0.16 +/- 0.20 (mean +/- SD)%. Abnormal levels of CD62P were observed in 5 patients with unstable angina, 6 patients with acute myocardial infarction, 1 patient with old myocardial infarction, and 2 patients with mitral stenosis. These results show that activated platelets may play some roles in pathogenesis of heart disease though it is not fully clear yet.
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PMID:[Detection of CD62P (GMP-140) on peripheral blood platelet membrane in various heart diseases]. 754 Feb 29

The CHARGE association was described as a cluster of multiple congenital defects by Pagon et al in 1981. The underlining cause of this association is not yet clear. These defects include coloboma of the eye tissues (C), heart disease (H), atresia choanae (A), retarded growth and development and/or CNS anomalies (R), genital anomalies (G), ear anomalies and/or hearing loss (E). Here we report a case of CHARGE association in a 15-year-old female patient. She presented with unilateral ptosis and coloboma of the iris, choroid and retina, patent ductus arteriosus, growth and mental retardation, unilateral ear deformity and a hearing loss. A total of 124 cases have been reported up to now in the literature. We also discuss the frequency of the respective clinical symptoms and signs, and the leading causes of misdiagnosis. We emphasize the importance of ophthalmology and ENT consultations whenever a patient is noted to have multiple congenital anomalies, especially when a cardiac defect is present. Early diagnosis and treatment are essential for this rare association.
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PMID:[CHARGE association: report of a case with literature review]. 790 67

A 45-year-old male with end-stage dilative cardiomyopathy was referred for heart transplantation (HTx). Apart from severe heart disease the patient had an unremarkable medical history. Risk factors were heavy smoking and moderate consumption of alcohol. Preoperative screening including a thorough ENT status did not reveal any other risk factor or contraindication for heart transplantation. HTx was performed three months later. Immunosuppressive therapy consisted of triple-drug therapy and induction therapy with antithymocyte globulin. The patient had an uneventful perioperative course. One month after transplantation the patient developed a rapidly growing squamous cell carcinoma of the left tonsil with local metastasis. Because of the rapid growth and size of the tumor surgical treatment was already impossible at that early time. Despite a course of chemotherapy the tumor continued to grow; treatment was changed to radiation therapy resulting in partial remission. Cyclosporine and azathioprine dosages were reduced at the same time. 9 months following HTx the patient developed a rapidly growing recurrence. As there were no further therapeutic options, immunosuppressive therapy was completely discontinued with the patient's agreement. He died 2 months later. The rapid tumor growth and its early manifestation following HTx suggest a preexistent occult carcinoma. A more extensive and repetitive preoperative screening in HTx candidates who are heavy smokers should be considered.
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PMID:Tonsillar carcinoma in the early postoperative course following heart transplantation. 877 62

Between September 1996 and January 1999 we used polysomnography (PSG) to examine 473 patients (involving a total of 662 records). The diagnosis was a sleep-related breathing disorder in 256 patients, including sleep apnea syndrome (SAS) in 194 patients, sleep hypoxicemia in 18 and insomnia in the other four. The SAS consisted of three subtypes: central apnea (CA) in 56 patients, obstructive apnea (OA) in 124 and mixed apnea (MA) in eight. The ratio of central apnea was relatively higher than the national average. Among the 473 patients, the most common complication was heart disease (133 patients) while other complications included hypertension, and respiratory and cerebrovascular diseases. Concerning the therapy for these patients, continuous positive airway pressure therapy was the most commonly applied and was effective in each type of SAS (CA, OA, MA). Other therapies included prosthetic mandibular advancement, bilevel positive airway pressure, medication and ENT operations. In Koga Hospital, there are many patients with heart disease and/or respiratory disease. We examined those patients who presented with snoring and/or apnea using PSG. Among these patients, SAS was the most common sleep disorder. The relative ratio of CA was high and the average age was higher than those with OA.
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PMID:The Koga Hospital Center for studies on sleep: status report. 1118 86

Over 60 million women use highly efficient and safe modern combined oral contraceptives (OCs) every day. A women who takes the oral contraceptive for 5 years before the age of 30 will actually live 12 days longer, although a woman taking the pill for the 1st time for 5 years after the age of 30 will have her life span reduced on the average by 80 days. OC related morbidity and mortality mostly occur in women over 35 who smoke. Combined low dose OCs are safe for women who do not smoke, at least to 45 years of age and probably to the menopause. The prescription of OCs is also safe to the young adolescent. The pill does not interfere with maturation of the hypothalamic-pituitary ovarian axis and does not increase the incidence of amenorrhoea, oligomenorrhoea or infertility in later life. Patients with contraindications to estrogen therapy are excluded from OC use (history of thromboembolism, major heart disease, liver disease, breast cancer). Low-dose (30-35 mcg estrogen-containing monophasic or triphasic) pills are recommended. Combined oral contraceptives contain either ethinyl estradiol (1.7 to 2 times more potent) or mestranol. After absorption the progestagens, norethisterone acetate, ethynodiol diacetate and lynoestrenol are all metabolized to norethisterone. The progestagen-only pill has about a 2% failure rate and poorer cycle control than the combined pill, but it lacks estrogenic, progestagenic and androgenic side effects. This pill is suitable for the lactating mother, for smokers over 35, for hypertensive patients, and for those with a history of thrombosis. The efficacy of the progestagen-only pill is restored in 3 days of pill taking. Postcoital contraception is an alternative: treatment can be given for at least 72 hours after intercourse. The Yuzpe method calls for the patient to take 2 combined oral contraceptive tablets containing levonorgestrel and ethinyl estradiol (Eugynon or Ovral) followed by a further 2 tablets 12 hours later. This regimen probably reduces the risk of pregnancy about tenfold and it is generally well tolerated.
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PMID:Oral contraceptives. 1231 13

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.
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PMID:Nomenclature and databases - the past, the present, and the future : a primer for the congenital heart surgeon. 1748 90

Jervell, Lange-Nielsen syndrome is a condition that causes profound hearing loss and disruption of the normal cardiac rhythm. This disorder is a form of long QT syndrome, a cardiac disorder that causes the cardiac muscle to take longer than usual to recharge between beats. A retrospective case study was performed to document cochlear implantation in three profoundly deaf children (two of them siblings) with Jervell, Lange-Nielsen syndrome. We discuss diagnosis and management of this syndrome and also the long-term performance of cochlear implantation in these Iranian patients, referring especially to the role of the ENT specialist in diagnosis and treatment. The collected data show that cochlear implantation can be relatively safely performed in patients with Jervell, Lange-Nielsen syndrome and that these children received significant benefit from cochlear implantation.
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PMID:Cochlear implantation in children with Jervell, Lange-Nielsen syndrome. 1749 28

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. The working component of this international nomenclature society has been The International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease, also known as the Nomenclature Working Group. The Nomenclature Working Group created the International Paediatric and Congenital Cardiac Code, which is available for free download from the internet at [http://www.IPCCC.NET]. In previous publications from the Nomenclature Working Group, unity has been produced by cross-mapping separate systems for coding, as for example in the treatment of the functionally univentricular heart, hypoplastic left heart syndrome, or congenitally corrected transposition. In this manuscript, we review the nomenclature, definition, and classification of heterotaxy, also known as the heterotaxy syndrome, placing special emphasis on the philosophical approach taken by both the Bostonian school of segmental notation developed from the teachings of Van Praagh, and the European school of sequential segmental analysis. The Nomenclature Working Group offers the following definition for the term "heterotaxy": "Heterotaxy is synonymous with 'visceral heterotaxy' and 'heterotaxy syndrome'. Heterotaxy is defined as an abnormality where the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body. By convention, heterotaxy does not include patients with either the expected usual or normal arrangement of the internal organs along the left-right axis, also known as 'situs solitus', nor patients with complete mirror-imaged arrangement of the internal organs along the left-right axis also known as 'situs inversus'." "Situs ambiguus is defined as an abnormality in which there are components of situs solitus and situs inversus in the same person. Situs ambiguus, therefore, can be considered to be present when the thoracic and abdominal organs are positioned in such a way with respect to each other as to be not clearly lateralised and thus have neither the usual, or normal, nor the mirror-imaged arrangements."The heterotaxy syndrome as thus defined is typically associated with complex cardiovascular malformations. Proper description of the heart in patients with this syndrome requires complete description of both the cardiac relations and the junctional connections of the cardiac segments, with documentation of the arrangement of the atrial appendages, the ventricular topology, the nature of the unions of the segments across the atrioventricular and the ventriculoarterial junctions, the infundibular morphologies, and the relationships of the arterial trunks in space. The position of the heart in the chest, and the orientation of the cardiac apex, must also be described separately. Particular attention is required for the venoatrial connections, since these are so often abnormal. The malformations within the heart are then analysed and described separately as for any patient with suspected congenital cardiac disease. The relationship and arrangement of the remaining thoraco-abdominal organs, including the spleen, the lungs, and the intestines, also must be described separately, because, although common patterns of association have been identified, there are frequent exceptions to these common patterns. One of the clinically important implications of heterotaxy syndrome is that splenic abnormalities are common. Investigation of any patient with the cardiac findings associated with heterotaxy, therefore, should include analysis of splenic morphology. The less than perfect association between the state of the spleen and the form of heart disease implies that splenic morphology should be investigated in all forms of heterotaxy, regardless of the type of cardiac disease. The splenic morphology should not be used to stratify the form of disease within the heart, and the form of cardiac disease should not be used to stratify the state of the spleen. Intestinal malrotation is another frequently associated lesion that must be considered. Some advocate that all patients with heterotaxy, especially those with isomerism of the right atrial appendages or asplenia syndrome, should have a barium study to evaluate for intestinal malrotation, given the associated potential morbidity. The cardiac anatomy and associated cardiac malformations, as well as the relationship and arrangement of the remaining thoraco-abdominal organs, must be described separately. It is only by utilizing this stepwise and logical progression of analysis that it becomes possible to describe correctly, and to classify properly, patients with heterotaxy.
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PMID:The nomenclature, definition and classification of cardiac structures in the setting of heterotaxy. 1803 96

Deletion of chromosome 22q11 gives rise to a spectrum of anomalies, including cleft palate. These are grouped together as the DiGeorge or velocardiofacial syndrome. Patients with this chromosomal abnormality account for a small, but noteworthy proportion of patients attending our cleft service. They frequently have other significant comorbidities consistent with their diagnosis. Over a ten-year period, 16 patients within our cleft service have been diagnosed, using chromosome analysis, as having deletions at 22q11. All had either a cleft palate and/or velopharyngeal incompetence, for which they underwent repair of the cleft palate or pharyngoplasty. Several have required secondary palate surgery following initial palate surgery. Poor quality of speech was the indication for secondary procedures in the majority of cases. Fourteen of the 16 have other comorbidities, ranging from congenital heart disease to ocular abnormalities. In addition, 15 of the 16 have developmental delays and/or learning difficulties. Other specialties, such as ENT, cardiology, genetics and ophthalmology have been involved in the care of all these patients. Although comprising only a small proportion of patients attending a cleft team, the diagnosis of this chromosomal abnormality is significant, as these patients may require substantial input of resources and the expertise of several specialties. Early recognition of features of this entity and diagnosis can aid more efficient intervention.
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PMID:22q11 chromosome abnormalities and the cleft service. 1924 64

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