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The underlying principle of the Fontan procedure, perfusing the lungs without benefit of a ventricular pump, has been reinforced by its successful application in many children with complex cardiac anomalies involving only one functional ventricle. Several different techniques that direct systemic venous return through the right atrium directly to the pulmonary arteries can be used. By separating the pulmonary and systemic circulations, reducing ventricular volume overload, and relieving cyanosis, improved cardiac function and hemodynamics can be achieved. This article reviews the surgical techniques, perioperative nursing care with particular attention to the assessment, and management of systemic venous hypertension and the long-term outlook for these children. The collaboration of physicians and critical care nurses in the postoperative care of these children and their families is vital to a successful outcome.
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PMID:The child following the Fontan procedure: nursing strategies. 219 57

A case of severe vasoconstriction treated as cardiorespiratory collapse in a woman given extraamniotic PGF2alpha for midtrimester abortion is described, with comments on management of this rare reaction. The patient was having elective termination because of confirmed spina bifida with hydrocephalus by ultrasound and elevated AFP at 18 weeks gestation. She was given a 4 mg test dose of PGF2alpha (Dinoprost, Upjohn Pty, Ltd) in viscous gel (Tylose MH300, Hoechst Australia Ltd) via extraamniotic Foley catheter. She immediately developed dyspnea, abdominal and breast pain, hypotension of 50 mm Hg systolic, peripheral vasoconstriction, cyanosis and confusion. She was treated with iv Hartmann's solution 600 ml, oxygen 8 1/min, and sc adrenaline 1/1000 0.5 ml. She seemed to improve after receiving 500 ml 3.5% polygeline colloid (Haemaccel, Behringwerke AG), and 5 ml 1/10,000 adrenaline iv, as her systolic blood pressure rose to 70 mm Hg measured indirectly. 500 ml more iv colloid was given, and blood pressure rose to 90 mm Hg. Then she suddenly deteriorated with florid pulmonary edema. Oxygen saturation fell and positive pressure ventilation was begun. She was given furosemide 160 mg iv and hydrocortisone 500 mg iv. Anaphylactic reaction was ruled out on the basis of blood count; amniotic fluid embolism was ruled out because of minor changes in clotting parameters. The events seen here most likely occurred as a result of inadvertent injection of PGF2alpha into the arterial circulation, causing increased pulmonary arterial pressure and vascular resistance, systemic vasoconstriction interpreted as hypotension, all exacerbated by adrenaline and exogenous fluid load. Severe hypertension after extraamniotic PGF2alpha has been reported before in a similar case of apparent hypotension treated with agents to increase blood pressure. PGF2a should not be used without facilities to treat such adverse reactions.
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PMID:Cardiorespiratory collapse and pulmonary oedema due to intravascular absorption of prostaglandin F2 alpha administered extraamniotically for midtrimester termination of pregnancy. 260 61

Severe reactions in exercise-induced asthma (EIA) seem to be underestimated in the published literature. We report two cases of near-miss death from EIA that occurred after a short run. We review 364 exercise tests that were performed between September 1987 and October 1988 by a standardised protocol on a treadmill, on patients with possible EIA. A positive test, defined by a fall of FEV1 of at least 20% was found in 173 patients. From 21 patients with a fall of greater than 50%, 4 presented severe signs of: Cyanosis. Intense dyspnea with impediment of speech. General malaise with hypertension. These 4 patients were not greatly different from patients of the 50% fall group when compared for FEV1 before the test and for heart-rate during the test. They differed in the duration of the asthma attack, which was more protracted, despite the use of beta-2 agonists. The onset of severe reactions is 2.3% of positive tests and seems to be unpredictable.
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PMID:[Severe forms of effort-induced asthma]. 271 39

Antoantibodies to cholesterol were detected and purified from normal (nonimmunized) pig serum. The antibodies were assayed by ELISA with crystalline cholesterol as an Ag and by C-dependent damage to cholesterol-laden liposomes. Intravenous injection of liposomes containing cholesterol into anesthetized animals caused decreased hemolytic complement titers, and induced a reaction consisting of transient neutropenia, thrombocytopenia, respiratory distress, cyanosis, pulmonary and systemic hypertension, and decreased cardiac output. Plasma levels of thromboxane B2 and 6-keto-prostaglandin F1 alpha increased 1300 and 200%, respectively, and leukocyte and platelet counts decreased by 36 and 38%, respectively. Injection of cholesterol-free liposomes did not induce the reaction. These results show that naturally occurring autoantibodies to cholesterol can initiate C activation and can be associated with anaphylactoid reaction to exogenously administered cholesterol in pigs.
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PMID:Anaphylactoid reactions mediated by autoantibodies to cholesterol in miniature pigs. 280 13

The authors report the case of a 2-year-old infant who presented with paroxysm and short changes characterized by acute drowsiness, cold sweats, ocular reversion, facial cyanosis, and bradycardia. Between these attacks, the condition was normal, suggesting diencephalic seizures. Over 2 months five fits were observed by the parents when some to-and-fro bobbing of the head onto the trunk appeared during drowsiness. One electroencephalogram was normal without a slow background or spikes discharges. As the skull radiographs showed erosion of the jugum and chronic intracranial hypertension features, a CT scan was performed and showed hydrocephalus associated with a congenital suprasellar cyst. The cyst was opened into basal cisterns with cystoperitoneal shunt. The histological examination revealed that it was an arachnoid cyst. Six months later, the infant was free of diencephalic seizures and head bobbing. Thus, we can assert that there was a direct relationship between this cyst and the diencephalic seizures. From this case, the authors make a review of the clinical features of diencephalic epilepsy, and their different causes and show that both diencephalic epilepsy and suprasellar arachnoid cysts are not common.
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PMID:Diencephalic epilepsy with congenital suprasellar arachnoid cyst in an infant. 316 81

Traumatic asphyxia is an uncommon syndrome of craniocervical cyanosis, facial petechiae, and subconjunctival hemorrhages following severe crush injury to the thorax. Ocular manifestations of the syndrome have rarely been reported. A 42-year-old male sustained temporary blindness in association with traumatic asphyxia. Completely normal vision returned within 24 hours of injury. The pathogenesis of this unique finding probably involves transient retinal vein hypertension and reflex vasospasm in the retina.
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PMID:Temporary blindness associated with traumatic asphyxia. 322 44

Ischemic myocardial and papillary muscle dysfunction has considerable implication in newborn infants and children with normal or malformed hearts. Papillary muscle dysfunction in adults primarily involves coronary artery occlusion and ischemic necrosis in the left ventricle and papillary muscles. Infants and children rarely develop coronary artery occlusion. Their myocardial dysfunction and injury occurs with nearly equal frequency in both ventricles as a result of underperfusion from a wide range of causes, including severe birth asphyxia, congenital heart disease, and complications of premature delivery. A history of cardiogenic shock, acute congestive heart failure with cyanosis and atrioventricular murmur, or persistent fetal circulation in a newborn without congenital heart disease should alert the pathologist to the possibility of ischemic myocardial necrosis (IMN). Older infants with ventricular hypertrophy, persistent pulmonary hypertension (PPHN), bronchopulmonary dysplasia (BPD), and those with malformed hearts involving severe ventricular hypertension due to outflow obstruction or pulmonary hypertension may have IMN, fibrosis, or dystrophic calcification alone or in combination. Animal models of adult ischemic cardiac injury may not be suitable for study of the newborn.
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PMID:Ischemic myocardial necrosis and papillary muscle dysfunction in infants and children. 333 39

Univentricular heart is a complex congenital anomaly with an extremely dismal prognosis. Left untreated the majority of patients will die in infancy. This poor outlook has prompted an aggressive surgical approach consisting of early palliation followed by eventual complete repair. The initial palliative efforts are aimed at providing adequate arterial oxygen saturation and normalizing pulmonary artery pressure. Infants with severe cyanosis due to diminished pulmonary blood flow are best treated with a systemic to pulmonary artery shunt. Those with excessive pulmonary flow and hypertension must be banded early to prevent pulmonary vascular occlusive disease. Most patients who are properly palliated will become acceptable candidates for an eventual corrective procedure. For the majority, this will be a direct atriopulmonary connection, known as the Fontan procedure. In this operation, systemic venous blood is routed directly to the pulmonary artery, excluding the ventricular mass. Although not corrective in the usual sense, this procedure provides normal arterial saturation without ventricular volume overload. A small group of patients with univentricular heart may best be treated by partitioning the ventricular chamber into 2 halves. Septation procedures, however, carry a high operative mortality and are likely to result in complete heart block. These surgical options, when properly selected and timed, have improved the outlook for many children with univentricular heart.
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PMID:Cardiac surgery for the adolescent with univentricular heart. 358 51

A phase I and pharmacokinetic study of recombinant tumor necrosis factor (rH-TNF Asahi) was carried out in 29 patients, who received a total of 72 courses with doses ranging from 1 to 48 X 10(4) units/m2. Drug was given as 1-h i.v. infusions. Acute toxicities, taking the form of fever, chills, tachycardia, hypertension, peripheral cyanosis, nausea and vomiting, headache, chest tightness, low back pain, diarrhea and shortness of breath were seen, but were not dose-limiting or dose-related. Some early rise in SGOT, without any change in serum bilirubin, was noted at the highest doses. Eosinophilia, monocytosis, mild hypocalcemia and an increase in fibrin degradation products were seen in a few patients. The dose-limiting toxicity was hypotension, which occurred after the end of the drug infusion and was seen in all 5 patients treated at the highest dose. There was no mortality or long-term morbidity. There were no responses. Pharmacokinetic studies indicated a rapid plasma clearance and a short plasma half-life, generally less than 0.5 h.
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PMID:Phase I clinical trial of recombinant human tumor necrosis factor. 366 33

Hypersensitivity reactions to heparin preparations with a wide spectrum of clinical manifestations have been reported frequently in the past, but are a rarity now. A 88 year old man was admitted for physical therapy of a collum femoris fracture. Treatment with a diuretic, Reserpine and Verapamil was continued. Chest x-ray revealed a large thoracic aortic aneurysm. From the 12th to the 18th day of low dose heparin prophylaxis with calcium heparin, 7500 U twice daily, at least eight attacks of asthma or cyanosis were observed, starting about two hours after heparin injection. The last attack began suddenly with wheezing, tachypnoea and cough and was associated with apprehension, a sudden blood pressure increase and severe cyanosis. Ventilation improved with oxygen and a beta 2-stimulator, but hypertension and cyanosis lasted for three hours. After discontinuation of heparin no further attacks occurred. Causes other then heparin could not be found. Despite the use of porcine mucosa heparin, avoidance of preservatives and use of low doses a hypersensitivity reaction occurred in our case. The delayed onset after preceding subcutaneous application as well as difficulties in separating the reaction from complications of underlying disease may delay heparin discontinuation.
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PMID:[Asthma attacks in low-dose preventive use of heparin in a male with femoral neck fracture and aortic aneurysm]. 366 60


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