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

A prospective study of 26 patients undergoing cardiac operation requiring cardiopulmonary bypass was undertaken to determine the incidence and etiologic factors leading to post-operative neuro-psychiatric complications. Detailed evaluation of the patients showed the neuro-psychiatric complications after surgery occurred in 8 of the 26 patients (30.77%). If primitive reflexes were included, the cases increased to 10 (38.46%). Delirium was the most common complication, which was noted in 6 cases (23.08%). Other manifestations were Babinshski sign (2 cases), coma and seizure (1 case), and major depressive symptoms (1 case). Factors evaluated were: age, sex, heart disease, disease severity, type of surgery, combined physical illness, duration of anesthesia, duration of surgery, duration of bypass, mean systolic BP during operation, and mean BP during bypass. The following factors tended to be related to the occurrence of neuro-psychiatric complications: 1) duration of bypass, 2) mean BP during bypass, and 3) mean systolic BP during operation.
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PMID:[Post cardiopulmonary-bypass neuropsychiatric complications]. 849 Jul 76

In 1981, the Food and Drug Administration approved prostaglandin E1 (PGE1) for use in the treatment of neonates with congenital heart disease. PGE1 is commonly used in neonatal and pediatric intensive care units to maintain patency of the ductus arteriosus in those cardiac lesions that depend on the ductus for either systemic or pulmonary blood flow. Early recognition of hemodynamic instability and prompt initiation of PGE1 therapy is vital to survival in neonates with ductal-dependent cardiac lesions. Administration of PGE1 allows delay of palliative or corrective surgery until stabilization or transfer of the neonate to a tertiary care facility is achieved. It is also used as a bridge to heart transplantation in neonates in whom this treatment is an option. Congenital anomalies requiring treatment with PGE1 are those that restrict pulmonary blood flow (cyanotic or right-sided outflow tract obstructions) and systemic blood flow (acyanotic or left-sided outflow tract obstructions). Prostaglandin E1 is excreted by the kidneys, and elimination is almost complete within 24 hours after administration; 80 percent of it is rapidly metabolized after one pass through the pulmonary bed. Therefore, a continuous infusion and adequate intravenous access are necessary. Nurses caring for these neonates must have knowledge of all its potential side effects. Some of the most common side effects include cutaneous vasodilation, bradycardia, tachycardia, hypotension, seizure-like activity, hyperthermia, and apnea.
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PMID:Nursing care of the neonate receiving prostaglandin E1 therapy. 851 Jun 22

Long QT syndrome (LQT) is an inherited cardiac disorder that causes syncope, seizures and sudden death from ventricular tachyarrhythmias. We used single-strand conformation polymorphism (SSCP) and DNA sequence analyses to identify mutations in the cardiac sodium channel gene, SCN5A, in affected members of four LQT families. These mutations include two identical intragenic deletions and two missense mutations. These data suggest that SCN5A mutations cause LQT. The location and character of these mutations suggest that this form of LQT results from a delay in cardiac sodium channel fast inactivation or altered voltage-dependence of inactivation.
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PMID:Cardiac sodium channel mutations in patients with long QT syndrome, an inherited cardiac arrhythmia. 854 46

Recent important technical developments in the field of surgery for congenital heart disease have included the Ross pulmonary autograft replacement of the aortic valve, video-assisted thoracoscopic surgery, and the double-switch and switch-Rastelli procedure for congenitally corrected transposition. Although the growth potential of the pulmonary autograft has been confirmed, an important incidence of late aortic regurgitation has been noted. Expanding indications for video-assisted thoracoscopic surgery have been described, including division of the vascular ring. Late hemodynamic assessment after the double-switch or switch-Rastelli procedure will encourage increased application of this repair. Both clinical and laboratory studies have focused on neurologic and developmental outcome after cardiac surgery. Perioperative seizures in young infants have been found to be associated with impaired psychomotor development at 1 year of age. In the area of perioperative management, the antifibrinolytic agents tranexamic acid, epsilon-aminocaproic acid, and aprotinin have been found to be useful in reducing postoperative hemorrhage. Nitric oxide is useful in reducing postoperative pulmonary hypertension. Late clinical follow-up studies of patients with single ventricle have revealed an important incidence of pulmonary arteriovenous malformations after a bidirectional Glenn shunt and atrial flutter after a Fontan procedure. Late assessment of patients after the arterial switch procedure for transposition has revealed preservation of ventricular function and an extremely low incidence of late arrhythmias.
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PMID:Advances in surgical care of infants and children with congenital heart disease. 854 59

Sixty-seven patients with brain abscess were managed over 19 years (1975-1993). Our series had a 2.5 to 1 male predominance; the age distribution was from 3 days to 81 years. The underlying conditions of hematogenic brain abscesses (n = 33; 49%) included lung infections (n = 16), heart disease (n = 4), sepsis (n = 10), and other foci (n = 3). Otolaryngologic infections led to the abscess in 10 cases; there were 9 traumatic abscesses. The causes remained unknown in 15 cases. There were 47 solitary abscesses (70%) and 20 multiple abscesses. The most frequent presenting signs and symptoms were neurologic deficits (n = 17), disturbances of consciousness (n = 14), seizures (n = 6), and headaches, meningism and vomiting (n = 13). Causative organisms were isolated in 39 cases (58%) and included staphylococci (n = 6), streptococci (n = 6), enterobacteriae (n = 2), and anaerobic pathogens (n = 9). The most reliable laboratory sign of inflammation was an elevated ESR (52/59 patients). With the advent of computed tomography, burr hole aspiration of the abscess with or without drainage was possible in 30 cases; the mortality in this subgroup was 9%. All 4 patients with surgical excision in the pre CT-era died. The mortality of patients treated with antibiotics only was 62% (18/29). Overall mortality was 37% (25/67), including 5 cases with post mortem-diagnosis of brain abscess. Good recovery was achieved in 29/42 survivors. Predictors of a poor outcome were the patient's age, the level of consciousness, multiple abscesses, polybacterial cultures, and a hematogenic etiology, but not the size of the abscess.
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PMID:[Bacterial brain abscess--experiences with 67 patients]. 880 80

In order to evaluate the neonatal outcomes of infants who had prolonged fetal heart rate (FHR) deceleration during the second stage of labor, the neonatal outcomes of 24 infants born after vaginal delivery at 37 to 42 weeks of gestation with prolonged FHR deceleration during the second stage of labor were compared with the outcomes of 28 infants of a similar gestational age who had normal FHR patterns. No differences in the Apgar scores, mean umbilical PaCO2, PaO2, HCO3- or base deficit values were observed between the two groups, but the mean pH values differed significantly. The occurrences of a 1-minute Apgar score < 7, umbilical arterial pH < 7.20, presence of meconium and admission to the neonatal intensive care unit were higher in the study group, but were not significantly different. None of the 24 infants with prolonged FHR deceleration experienced birth trauma, meconium aspiration, neonatal seizure or neonatal death, but three were found to have congenital heart disease. We conclude that prolonged FHR deceleration during the second stage of labor without FHR abnormalities during the first stage of labor is not always associated with an adverse neonatal outcome and does not mandate the need for surgical or immediate vaginal delivery. Their appearance on FHR tracings requires the implementation of additional methods to assess fetal well-being and also to diagnose fetal distress.
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PMID:Implications of prolonged fetal heart rate deceleration during the second stage of labor. 885 56

The disease is named after George H. Whipple who, in 1907, was the first to describe an intestinal "lipodystrophy". Although Whipple's disease is generally recognized as a multisystem chronic granulomatous disease, primarily involving the digestive system, it can also appear as a primary neurological disorder in rare cases. Most often it is manifested with loss of weight, diarrhea, malabsorption, abdominal pain, lymphadenopathy, cardiopathy, hyperpigmentation and hypotension. The presence of periodic acid-Schiff (PAS)-positive macrophages in biopsy specimens (not only jejunal) and demonstration of "Whipple's bacilli" visible by electron microscopy, are diagnostic signs of active Whipple's disease. Whipple's disease confined to the CNS is rare. It is rarely found in the differential diagnosis of patients with progressive neurological deterioration. The most common neurological picture includes progressive dementia, external ophalmoplegia, myoclonus, seizures, ataxia, hypothalamic dysfunction (sleep disorders, hyperphagia, polydipsia) and meningitis. Oculofacial-skeletal myorhythmia as a movement disorder, associated with Whipple's disease, is reported. Fulminant course of cerebral Whipple's disease is unusual and unfavourable. The confusing and nonspecific clinical appearance is typical for primary CNS involvement. It has recently been suggested that CNS involvement occurs in all cases, although only 10-20% of patients may show it. The CNS is the most common site of disease relapse. The CT scans and MRI of the brain are often normal, but may show cortical/subcortical atrophy, hydrocephalus, focal or intracerebral mass lesions. The cerebrospinal fluid can sometimes contain PAS-positive macrophages. Brain biopsy is suggested as a diagnostic method in cases of high suspicion of CNS Whipple's disease. However, the lesions are frequently inaccessible and false negative. Without extended antibiotic therapy, the course of Whipple's disease is lethal. Now, the prognosis is good, although the optimal antimicrobial regimen is not clearly established. Initial parenteral therapy (tetracycline, penicilline, streptomycine, chloramphenicol, ampicilline) and peroral long-term treatment with trimetoprime-sulphametoxasole, are recommended. As CNS relapse of Whipple's disease may occur after several years, long-term treatment should include antibiotics that are able to cross the blood-brain barrier. The CNS relapse, in contrast to the systemic ones, is resistant to the treatment. Appropriate therapy instituted earlier in the course of the disease is associated with a better neurological outcome. Early recognition can be critical in Whipple's disease because of irreversible neurological sequelae seen later in the course of this potentially treatable condition. In cases with high clinical suspicion in which Whipple's disease cannot be diagnosed with procedures such as jejunal biopsy, antibiotic therapy is recommended. Recovery of an established neurological deficit may rarely occur. Longterm follow-up studies would help to identify the optimal antibiotic regimen and duration of treatment.
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PMID:[Neurologic disorders in Whipple's disease]. 910 28

Effective management of the syncope patient is critically dependent on excluding conditions in which altered consciousness is not due to syncope (e.g., seizure and sleep disorders) then establishing the basis for syncopal symptoms. The initial diagnostic step in syncope patients is differentiation of those individuals with normal cardiovascular status from those with structural heart disease. In the former, tilt-table testing and related studies of autonomic nervous system function are usually the most productive direction in which to proceed. In patients with structural heart disease, a functional assessment of the suspected structural disturbance (i.e., hemodynamic, angiographic, imaging as appropriate) and evaluation for susceptibility to symptomatic arrhythmias by monitoring or conventional electrophysiologic testing is appropriate. Autonomic function testing should follow if the diagnosis remains unclear. In only a few instances should specialized neurologic studies be undertaken as an initial step. The ultimate objective is always to obtain a sufficiently strong correlation between syncopal symptoms and detected abnormalities to feel confident in the diagnosis, permit an accurate assessment of prognosis, and develop an appropriate treatment plan.
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PMID:Clinical approach to diagnosis of syncope. An Overview. 916 8

Recently, there has been intense excitement in the field of cardiac arrhythmias. Molecular genetic studies have led to significant progress in characterizing molecular mechanisms underlying long QT syndrome, an inherited cardiac disorder that causes syncope, seizures, and sudden death from ventricular arrhythmias. Three long QT syndrome genes have been identified: SCN5A on 3p21-24, HERG on 7q35-36, and KVLQT1 on 11p15.5; all encode cardiac mycote ion channels. Molecular and electrophysiological characterization of these three long QT syndrome genes has led to identification of three critical electrical currents in the human heart (INa, IKr, IKa) and provides insight into our fundamental understanding of cardiac function. Genetic testing and gene-specific therapies are now available for some families with long QT syndrome.
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PMID:Molecular genetics of long QT syndrome from genes to patients. 924 89

The "CHIME" syndrome (MIM#280000) is a rare neuroectodermal disorder comprised of Colobomas of the eye, Heart defects, Ichthyosiform dermatosis, Mental retardation, and Ear defects. We report on the sixth child with this syndrome and the first of these to develop acute lymphoblastic leukemia at age 4 1/2 years. Her major problems included a migratory ichthyosiform dermatosis, multiple skin infections and infestations, bilateral retinal coloboma, developmental delay, seizures, infantile macrosomia, facial anomalies, a duplicated renal collecting system, and conductive hearing loss. Histologic examination of the skin demonstrated findings of an epidermal nevus with deep rete pegs, hyperkeratosis, and a markedly increased granular layer. The cause of the CHIME syndrome is unknown, but the disorder is easily recognized because of the striking phenotype. The diagnosis is important to make because of the potential for associated congenital heart disease, neurologic compromise, possible autosomal recessive inheritance, and possible association with malignancy.
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PMID:Acute lymphoblastic leukemia in a child with the CHIME neuroectodermal dysplasia syndrome. 929 69


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