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)

In Western countries, sudden cardiac death (SCD) is closely related to coronary artery disease, but in Korea the clinical characteristics of SCD are not well determined. Over a 4-year period (June 1995 to May 1999), 186 cases of SCD, ranging in age from 16 to 75 years, were admitted to the Chonnam National University Hospital. In 82 (44.1%) of these, neither symptoms nor evidence of structural heart disease was found and so their clinical characteristics were investigated. There were 66 (80.5%) men and 16 (19.5%) women (male/female ratio = 4.1:1). The mean age was 50 +/- 14 years: 19 (23.2%) were in their 40s, 21 (25.6%) in their 50s, and 17 (20.7%) in their 60s. The time of circulatory collapse witnessed in 68 cases of SCD showed 2 peaks: between midnight and 03.00h (n=16, 23.5%) and between 09.00h and midday (n=15, 22.1%). Unexplained SCD occurred at home in 48 (64.9%) cases and on the street in 12 (16.2%); it occurred during normal daily routine activity in 23 (39.6%) and during sleep in 15 (25.9%). Thirty-three patients (40.2%) experienced various prodromal symptoms, including chest discomfort (n=13, 15.9%) and dyspnea (n=8, 9.8%). The electrocardiogram taken on arrival recorded asystole in 65 (79.3%) and ventricular fibrillation in 17 (20.7%). Idiopathic ventricular fibrillation was diagnosed in 14 (10 men, 4 women; 45 +/- 11 years) of 21 patients who recovered spontaneous circulation. Five (6.1%) patients were discharged alive, and an implantable cardioverter-defibrillator was implanted in 2. Unexplained SCD is common in Korea and develops predominantly in middle-aged males around midnight or in the late morning usually with no prodromal symptoms (59.8%). Idiopathic ventricular fibrillation is thought to be one of the important causes.
 ...
PMID:Clinical characteristics of unexplained sudden cardiac death in Korea. 1115 16

Successful management of pediatric cardiac emergencies requires an accurate diagnosis to institute an appropriate plan of therapy. The diagnosis, however, is not always straightforward, as evidenced by the nonspecific clinical picture that can be presented by congenital heart defects. Entertaining the possibility of a cardiac problem in neonates with pulmonary symptoms unresponsive to standard therapies is crucial for successful management of patients with congenital heart disease. In addition to ventilatory support, prostaglandin E1 infusions or emergency interventional cardiac catheterization is often a life-saving initial measure in patients with acutely decompensated congenital cardiac lesions that require a patent ductus arteriosus for survival. Pericardial tamponade is associated with various acquired and iatrogenic causes. Emergent pericardiocentesis is mandatory when cardiovascular compromise occurs. The goal of anesthetic management is to maintain cardiac output. With the increasing use of central venous catheters in neonatal ICUs and the high mortality rate for central venous catheter-related cardiac tamponade, the diagnosis must be considered in any patient with a central venous catheter in situ who acutely develops unexplained hypotension, bradycardia, and diminished pulses. Arrhythmias also can cause hemodynamic instability in infants and children. Supraventricular tachycardia is by far the most common emergently presenting arrhythmia in the pediatric population. Unstable patients require immediate intravenous adenosine or synchronized cardioversion. Complete heart block is rare, but it can lead to congestive heart failure and occasionally to cardiovascular collapse and sudden death. Emergency treatment of complete heart block includes pharmacologic support and temporary or permanent pacemaker placement as indicated. In infants, congestive heart failure usually is related to congenital heart disease, whereas in older children, it tends to be secondary to an acquired cause. Supportive measures, fluid restriction, and inotropic support are the principles of initial treatment. Prompt recognition and initiation of appropriate therapy in pediatric cardiac emergencies are essential for favorable outcomes.
 ...
PMID:Pediatric cardiac emergencies. 1146 66

We report on a female preterm infant with hepatic failure and neonatal tissue siderosis of hemochromatotic type diagnosed by using both histochemistry and atomic absorption spectroscopy. The infant presented with meconium ileus, signs of rapidly progressive hepatic failure, and hyperferritinemia (7132 ng/ml). Despite surgery and intensive care the infant died 32 days after birth. Postmortem examination showed a wrinkled liver with extensive collapse of the hepatic architecture and regenerating nodules as well as hepatic and extrahepatic iron accumulation of hemochromatotic type, sparing the reticuloendothelial system. Atomic absorption spectroscopy confirmed an increase in the iron content of various organs: liver, heart, pancreas, oral salivary gland, kidney, and adrenal gland. The increase in the iron content of various organs was determined by comparing the analysis of the propositus with those of 5 gestationally age-related preterm infants who had died in the intensive care unit: 2 died of meconium aspiration syndrome, the other 3 of hyaline membrane disease, bronchopulmonary dysplasia, and immaturity, respectively. We also compared the analysis of 15 fetuses having a a condition predisposing to iron accumulation (trisomy 21, trisomy 18, cytomegalovirus, amnion infection syndrome, Rhesus- and ABO-incompatibility, congenital hemolysis, anti-phospholipid syndrome, congenital heart disease). Delta F508, the most frequent mutation seen in cystic fibrosis patients, was excluded by gene sequencing. Different noxae causing iron accumulation in the neonatal period have led to the statement that neonatal hemochromatosis may collect different etiologies, such as metabolic disorders, infections, chromosomal aberrations, and immunological disorders. In this study, we report the singular evidence of neonatal iron accumulation of hemochromatotic type in an infant presenting with meconium ileus and propose a classification of the neonatal disorders associated with iron accumulation.
 ...
PMID:Hepatic failure with neonatal tissue siderosis of hemochromatotic type in an infant presenting with meconium ileus. Case report and differential diagnosis of the perinatal iron storage disorders. 1170 Aug 92

Venoarterial extracorporeal membrane oxygenation (VA ECMO) has become a valuable technique in the critical care of children with congenital heart disease who require mechanical cardiorespiratory support. The use of VA ECMO in cardiac patients has expanded from an extension of intraoperative cardiopulmonary bypass and now includes rescue therapy during cardiopulmonary resuscitation, temporary circulatory support for reversible heart failure, and bridge support preceding heart or heart/lung transplantation. In the majority of clinical applications VA ECMO is used in reaction to impending or ongoing cardiorespiratory failure and not in anticipation of an induced change in clinical status. We describe the anticipatory use of VA ECMO to prepare a patient with complex cyanotic congenital heart disease for a high-risk interventional cardiac catheterization. A 2.5 kg neonate with severe Ebstein's anomaly of the tricuspid valve and recurrent episodes of life-threatening supraventricular tachycardia was electively cannulated for VA ECMO in the cardiac intensive care unit. She underwent successful electrophysiologic mapping and transcatheter radiofrequency ablation of an accessory conduction pathway, resulting in termination of the tachycardia. Following an uncomplicated ECMO course she was decannulated in the cardiac intensive care unit and subsequently discharged home in stable condition. The case illustrates the proactive use of ECMO during a procedure in which severe hemodynamic instability could be predicted. We discuss this concept of ECMO use in the context of accepted indications for ECMO in cardiac patients and encourage an expanded role for its use to prevent cardiorespiratory collapse in planned interventions on compromised patients who are at risk of acute deterioration.
 ...
PMID:Anticipatory use of venoarterial extracorporeal membrane oxygenation for a high-risk interventional cardiac procedure. 1218 35

We assessed the spectrum of airway disorders in children with congenital cardiac anomalies, and reviewed our experience in using flexible bronchoscopy for assessment of airway problems in this patient group. The clinical records, flexible bronchoscopic findings, and cardiac imaging results of pediatric cardiac patients who presented with either clinical or radiological signs of airway obstruction between 1992-1999 were reviewed. Flexible bronchoscopic assessment was performed with the patients under sedation and topical anesthesia, using one of two bronchoscopes, i.e., an Olympus BFN20 or Olympus BF3C20. Of a total of 52 patients, 33 had acyanotic cardiovascular lesions, the commonest being left-to-right shunts (61%), while 19 had cyanotic heart lesions, with right ventricular outflow obstruction being the commonest (63%). Twenty-seven patients had undergone either surgical or transcatheter interventions. The median age at bronchoscopic assessment was 6 months (range, 4 days to 6 years). None of the patients developed significant procedural complications. A definitive diagnosis was made in 48 (92%) patients, 8 of whom had abnormalities involving only the upper airways, 35 only the lower airways, and 5 both. Abnormalities of the upper airway included laryngomalacia (n = 6), subglottic stenosis (n = 3), pharyngeal collapse (n = 2), and 1 each of choanal stenosis and supraglottitis. Extrinsic compression was the commonest lower airway abnormality that was found in 27/40 patients (67%), with a predilection for the left main bronchus (18/27, 67%). The structures that caused extrinsic compression included dilated pulmonary arteries with or without left atrial dilation (n = 20), an anomalous aortic or pulmonary arterial course (n = 3), a dilated aorta (n = 1), and a shunt (n = 1), but were not obvious in 2 patients. Intrinsic lower airway abnormalities included bronchomalacia (n = 4), tracheal stenosis (n = 4), and one each of variant bronchial bifurcation and a pouch arising from the tracheal wall. Intraluminal mucus plugging of the lower airways occurred in the remaining 3 patients. Children with congenital heart disease are at risk of airway obstruction both before and after surgery. Flexible bronchoscopy, being safe and effective in diagnosing airway disorders in this patient group, should be considered as the first line of investigation.
 ...
PMID:Airway obstruction in children with congenital heart disease: assessment by flexible bronchoscopy. 1220 72

The aim of this study was to determine community application of cardiopulmonary resuscitation (CPR) skills in an emergency, and, thus, assess the value of training programmes in raising community competence. A cross-sectional telephone survey of the Western Australian population was chosen randomly (n = 803). An urban sub-sample (n = 100) performed a practical demonstration of CPR skills using a simulated collapse scenario using a recording manikin as the victim. Performance was assessed by two observers using pre-determined criteria. Of all respondents, 64% had been trained in CPR. Practical and theoretical assessment scores were significantly better in trained versus untrained participants. The number of times a person was trained in CPR was more effective for retention and competence than time since last trained. Degree of training and theoretical competence were less in those aged over 65 years or with heart disease in the household. Theoretical competence poorly reflected practical performance in many tasks. This study provides a comprehensive database of CPR training and performance, and highlights future directions to ensure appropriate and cost-effective training. Specific factors to be addressed include increasing frequency of training, targeting of high-risk groups, simplification in teaching, and emphasising early activation of the emergency medical system.
 ...
PMID:Community competence in cardiopulmonary resuscitation. 1241 53

Aortic dissection is uncommon in young women and is associated with clinical conditions such as pregnancy and Marfan's syndrome. Owing to the low incidence, diagnosis of acute aortic dissection in young women might be missed or delayed in patients who have neither risk factors nor typical clinical manifestations. We report the case of a 28-year-old postpartum woman with aortic dissection. The patient complained of abdominal discomfort, transient back pain, and general malaise at our emergency department 1 week after delivery of a healthy baby. She had no history of hypertension, connective tissue disease or congenital heart disease. Cardiovascular insult was not considered until the patient developed shock. Myocarditis or peripartum cardiomyopathy with left ventricular dysfunction was diagnosed based on imaging studies and cardiac enzyme levels. Finally, computed tomography revealed acute aortic dissection after hemodynamic collapse occurred. This case suggests that acute aortic dissection can be associated with left ventricular dysfunction, and non-specific clinical symptoms in young, normotensive, and postpartum women. A high index of clinical suspicion and alertness are needed to identify this condition.
 ...
PMID:Acute aortic dissection associated with left ventricular dysfunction in a postpartum and normotensive young woman. 1287 72

Necrotizing enterocolitis (NEC) has widespread implications for neonates. While mostly affecting preterm neonates, full-term neonates, especially those with congenital heart disease, are also at risk. Although the exact pathogenesis of NEC remains elusive, three major factors, a pathogenic organism, enteral feedings, and bowel compromise, coalesce in at-risk neonates to produce bowel injury. Initiation of the inflammatory cascade likely serves as a common pathway for the disorder. Clinical signs and symptoms range from mild feeding intolerance with abdominal distension to catastrophic disease with bowel perforation, peritonitis, and cardiovascular collapse. Vigilant assessment of at-risk neonates is crucial. When conservative medical management fails to halt injury, surgical intervention is often needed. Strategies to decrease the incidence and ultimately prevent NEC loom on the horizon, such as exclusive use of human breastmilk for enteral feedings and administration of probiotics.
 ...
PMID:Current controversies in the understanding of necrotizing enterocolitis. Part 1. 1289 35

Syncope is a frequent problem in childhood; generally, it is an isolated event and the common causes are benign. However, in some circumstances, syncope can herald a potentially lethal problem, especially when occurring during exercise. Routine evaluation includes history, physical examination and a 12-lead standard ECG should be performed in all cases. Worrying features which should be an indication for further investigation include syncope during exercise, collapse in a swimming pool, history of familial sudden death, and abnormalities on clinical exam or ECG. Structural cardiac abnormalities that may cause syncope and sudden death include aortic stenosis, hypertrophic cardiomyopathy and coronary malformations. All children with unrepaired or repaired congenital heart disease who experienced a syncope should be referred to a specialist. Primary arrhythmias that are easily diagnosed on ECG are the long QT syndrome, complete atrio-ventricular block and Wolff-Parkinson-White syndrome; ST elevation in V1-V3 may reveal a Brugada syndrome. Another arrhythmia which is known to be potentially fatal if undiagnosed is the catecholaminergic ventricular tachycardia; the baseline ECG is normal but the arrhythmia is easily reproduced during exercise testing. Finally, vasovagal syncope is the most likely cause of syncope in the young and it usually easily recognized.
 ...
PMID:[Cardiac syncope in children]. 1476 42

A 21-year-old patient with repaired double-outlet right ventricle and normal ventricular function underwent internal cardioverter defibrillator implantation for primary prevention of sudden death. First occurrence of spontaneous ventricular tachycardia resulted in hemodynamic collapse and syncope but the internal cardioverter defibrillator rescued the patient. ICD implantation for primary prevention may be an appropriate goal in adults with repaired congenital heart disease, even in the setting or normal ventricular function.
 ...
PMID:Defibrillator for primary prevention in congenital heart disease. 1530 72


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