Malignant pulmonary edema in a pregnant woman caused by cor triatriatum.

BACKGROUND: Undiagnosed maternal congenital heart disease can result in significant risks to both mother and fetus. When maternal symptoms and signs are atypical of preeclampsia, broadening the differential diagnosis and multidisciplinary consultation can lead to improved outcomes. CASE: A 24-year-old primiparous woman with a pregnancy complicated by elevated blood pressure and proteinuria presented at term with symptoms of decreased fetal movement, increasing dyspnea, and cough. Her symptoms progressively worsened until she experienced cardiorespiratory arrest during attempted intubation. Emergency cesarean delivery was performed after 5 minutes of resuscitation efforts. Echocardiogram performed after delivery revealed cor triatriatum requiring surgical excision. CONCLUSION: A complete differential diagnosis for edema, hypertension, and dyspnea near term will allow for recognition of more rare conditions. Referral to cardiology in cases in which work-up is negative for preeclampsia may aid in earlier diagnosis and management. A team approach to management involving obstetrics and medicine will improve the rapidity with which rare conditions can be managed effectively.

The postural orthostatic tachycardia syndrome: a potentially treatable cause of chronic fatigue, exercise intolerance, and cognitive impairment in adolescents.

Head upright tilt table testing has become an accepted method to measure an individual's predisposition to autonomically mediated periods of hypotension and bradycardia severe enough to cause frank syncope. At the same time it has become increasingly apparent that less profound falls in blood pressure, while not sufficient to result in loss of consciousness, may cause symptoms such as near syncope, vertigo, and dizziness. We describe a subgroup of adolescents that have a mild form of autonomic dysfunction that exhibit disabling symptoms such as postural tachycardia and palpitations, extreme fatigue, lightheadedness, exercise intolerance, and cognitive impairment. During baseline tilt table testing at a 70 degrees angle, these patients demonstrated a heart rate increase of > or = 30 beats/min (or a maximum heart rate of > or = 120 beats/min) within the first 10 minutes upright (not associated with profound hypotension), which reproduced their clinical symptom complex. Similar observations have been made in the adult population and has been termed the postural orthostatic tachycardia syndrome (POTS). We report that POTS may also occur in adolescents and represents a mild, potentially treatable form of autonomic dysfunction that can be readily identified during head upright tilt table testing.

Exercise-induced neurocardiogenic syncope: clinical data, pathophysiological aspects, and potential role of tilt table testing.

The evaluation of syncope occurring during exercise or occurring spontaneously in highly trained individuals presents a unique diagnostic challenge. It is of critical importance to exclude potential life-threatening disorders such as hypertrophic cardiomyopathy, long QT syndrome, right ventricular dysplasia, anomalous coronary artery distribution, valvular heart disease, myocarditis, or exercise-induced arrhythmia. This review is not directed towards identifying, treating, or determining athletic eligibility of individuals with such disorders. Rather, we endeavour to discuss the pathophysiology of exercise-induced neurocardiogenic syncope and to address the role of head upright tilt testing in evaluating syncope in athletic individuals in whom proper evaluation has excluded the presence of ischaemic heart disease or primary structural or electrical heart disease.

Preliminary observations on the use of midodrine hydrochloride in the treatment of refractory neurocardiogenic syncope.

Recurrent episodes of neurocardiogenic syncope that occur without warning are a common cause of recurrent syncope that can be identified during head upright tilt table testing. While the use of beta blockers, theophyllines, fludrocortisone, disopyramide, and serotonin reuptake inhibitors can be useful in the prevention of episodes, some patients are either unresponsive to or poorly tolerant of these agents. We investigated the use of the peripheral alpha stimulating agent midodrine in preventing both tilt-induced and spontaneous neurocardiogenic syncope. Twenty-five patients (16 women, 9 men, mean age 30 +/- 23 years) with severe recurrent syncope and a positive head upright tilt table study (refractory to or intolerant of standard therapies) were placed on midodrine 5-10 mg orally three times per day, (two patients required 15 mg/day). Of these, twelve became asymptomatic and five had a marked reduction in symptoms. We conclude that midodrine may be an effective therapy in patients with recurrent neurocardiogenic syncope refractory to other forms of therapy.

Clinical disorders of the autonomic nervous system associated with orthostatic intolerance: an overview of classification, clinical evaluation, and management.

The disorders of autonomic control associated with orthostatic intolerance are a diverse group of infirmities that can result in syncope and near syncope (as well as a host of other complaints). A basic understanding of these disorders is essential to both diagnosis and proper treatment. These infirmities are not new, what has changed is our ability to recognize them. It has been said that "the world undergoes change in the human consciousness. As this consciousness changes, so does the world." On going studies will continue to help better define the broad spectrum of these disorders, and to elaborate better diagnostic and treatment modalities.

Diagnosis and management of neurocardiogenic syncope.

The medical term syncope is derived from the Greek word "synkoptein," the original meaning of which was "to cut short". Because it is both a symptom and a sign, the physician may often find the evaluation of syncope a difficult and frustrating endeavor. Over the course of the past decade it has become apparent that transient alterations in autonomic nervous system activity, resulting in hypotension and bradycardia, are common causes of syncope. At the same time it has become apparent that these are a heterogenous group of disorders which, while sharing some characteristics, are nonetheless distinct maladies. This article attempts to briefly review our current understanding of these disorders and suggests guidelines on their diagnosis and management.

Reentrant tachycardias. A look at where treatment stands today.

Cardiac dysfunction is not limited to the elderly. In fact, the forms of tachycardia discussed in this article usually begin before age 40. The authors summarize the mechanisms involved and the findings on electrocardiography that help identify the type of dysrhythmia in patients presenting with palpitations and other signs of possible cardiac disease. In addition, they describe the procedure that has revolutionized treatment for reentrant tachycardia: closed-chest radiofrequency catheter ablation.

Effects of adenosine on wavelength of premature atrial complexes in patients without structural heart disease.

Intravenous adenosine produced slight decreases in conduction times for premature atrial complexes but proportionally greater shortening of the functional refractory period. Decreased wavelength may provide a basis for transient atrial fibrillation, which is sometimes observed after adenosine administration.

Back injury prevention interventions in the workplace: an integrative review.

The purpose of this integrative review is to describe the state of knowledge about the effect of worksite back injury prevention programs on selected study outcomes. Fifteen experimental and quasi-experimental studies published between 1987 and 1994 were identified through a comprehensive literature search. The research studies were reviewed and analyzed using a data collection abstracting tool. Four types of back injury prevention intervention programs were identified: back belts, back schools, exercise/flexibility training, and educational classes. Positive program outcomes were reported for all four program types; however, the back school and the exercise/flexibility training programs were studied more frequently and demonstrated a greater proportion of positive results than the other two program types. Conclusions should be viewed cautiously due to the small number of studies reviewed and their methodological limitations. Implications for occupational health nursing practice and future research related to worksite back injury prevention are offered.

Peripherally-inserted central catheters: challenges for hospital management.

The peripherally-inserted central catheter (PICC) is an established technology in home intravenous (IV) therapy and its use is increasing in hospital and home-health settings. Institutions introducing PICCs as another form of IV therapy will realize cost savings. However, challenges may be encountered. Implementation and education programs for staff, patients, and home caregivers will maximize the benefits of PICC use.

Heparinization of biological vascular graft reduces fibrin deposition.

An alternative graft is needed for coronary bypass operations in patients lacking suitable autologous vessels. We therefore studied Denaflex, a biologic graft, in a dog ex-vivo shunt model to determine whether heparin treatment makes this graft less thrombogenic. Comparison was also made to Bioflow, a nonheparinized biologic graft. Fibrinogen deposition during high flow (593 +/- 202 ml/min) decreased from 672 +/- 467 ng/mm2 in nonheparinized Denaflex grafts to 448 +/- 298 ng/mm2 (p < 0.05) in heparinized Denaflex grafts. At low flow (117 +/- 13 ml/min), heparinization of Denaflex grafts similarly decreased fibrinogen deposition from 1102 +/- 601 ng/mm2 to 703 +/- 405 ng/mm2 (p < 0.05). At both flow rates fibrinogen deposition in Bioflow grafts was less than in nonheparinized Denaflex, but was similar to heparinized Denaflex grafts. Platelet deposition was not influenced by heparinization of Denaflex grafts and was similar among Denaflex and Bioflow preparations. Whether Denaflex performs acceptably in vivo as a xenograft requires extensive study.

IUD appendicitis during pregnancy.

This case shows that abdominal and pelvic x-ray examinations may not adequately show a misplaced IUD in a gravid woman, and further workup is necessary after delivery if the IUD is not clearly visible on the initial x-ray films. It also shows that chronic inflammation may develop in the area of a misplaced IUD, producing chronic abdominal pain and complications, even after several years have transpired. Finally, patients may still be at risk for complications after an IUD has been removed because of the dense adhesions that develop due to the chronic inflammatory response elicited by the IUD.

PIP: Appendicitis caused by a misplaced IUD was found in a 29-year-old pregnant woman. The woman had had the device inserted 8 years before. About 5 months after placement and a severe experience of right lower quadrant pain, medical examination revealed that she was pregnant. Abdominal and pelvic X-ray films were thought to be consistent with IUD expulsion, a fairly common occurrence, with an estimated rate of 2-20% within 1 year of placement. Over the next 7 years, the woman continued to experience right lower quadrant pain, but the pain was mild until 20 weeks into her next pregnancy when she was hospitalized with nausea, anorexia, fever, and severe pain. Surgery revealed that her appendix and cecum were bound to an inflamed mass of tissue. During the course of an appendectomy, this tissue mass was found to contain a copper-coated IUD, which was removed by blunt dissection and gentle traction. The IUD had probably partially perforated the uterus on insertion; complete perforation followed in 2-3 months; and copper from the device caused inflammation that eventually involved the appendix. Several months after the appendectomy, it was discovered that the inflammatory mass had been replaced by dense adhesions. This case shows that abdominal and pelvic X-ray examinations may not be sufficient to locate a misplaced IUD in a pregnant woman. If a misplaced device is not clearly visible on X-ray films, further workup may be necessary to avoid the possibility of chronic abdominal pain and complications.

Pyrophosphohydrolase activity and inorganic pyrophosphate content of cultured human skin fibroblasts. Elevated levels in some patients with calcium pyrophosphate dihydrate deposition disease.

In calcium pyrophosphate dihydrate (CPPD) crystal deposition disease, metabolic abnormalities favoring extracellular inorganic pyrophosphate (PPi) accumulation have been suspected. Elevations of intracellular PPi in cultured skin fibroblasts from a single French kindred with familial CPPD deposition (19) and elevated nucleoside triphosphate pyrophosphohydrolase activity (NTPPPH), which generates PPi in extracts of CPPD crystal-containing cartilages (14) favor this suspicion. To determine whether NTPPPH activity or PPi content of cells might be a disease marker expressed in extraarticular cells, human skin-derived fibroblasts were obtained from control donors and patients affected with the sporadic and familial varieties of CPPD (CPPD-S and CPPD-F) deposition. Intracellular PPi was elevated in both CPPD-S (P less than 0.05) and CPPD-F (P less than 0.01) fibroblasts compared with control fibroblasts. Ecto-NTPPPH activity was elevated in CPPD-S (P less than 0.01) but not CPPD-F. Intracellular PPi correlated with ecto-NTPPPH (P less than 0.01). Elevated PPi levels in skin fibroblasts may serve as a biochemical marker for patients with familial or sporadic CPPD crystal deposition disease; ecto-NTPPPH activity further separates the sporadic and familial disease types. Expression of these biochemical abnormalities in nonarticular cells implies a generalized metabolic abnormality.

Ecto-nucleoside triphosphate pyrophosphohydrolase activity and calcium pyrophosphate dihydrate crystal deposition disease.

Articular cartilage contains any ectoenzyme activity, NTP-PPH, which is capable of generating PPi from NTP substrates. The PPi generated is from the cleavage of the alpha-beta pyrophosphate bond of NTP and does not result from the effects of NTP catabolites. NTP-PPH activity is expressed on human skin fibroblasts in culture and is significantly increased in subjects with CPPD deposition. In addition, cultured fibroblasts from subjects with CPPD disease have higher intracellular PPi concentrations compared to cells from normals and patients with OA. These results support the hypothesis that alterations in PPi metabolism provide the metabolic basis for CPPD deposition.

Cartilage nucleoside triphosphate pyrophosphohydrolase. II. Role in extracellular pyrophosphate generation and nucleotide metabolism.

Extracellular generation of inorganic pyrophosphate (PPi) in cartilage organ culture is markedly augmented by ATP.ATP, not an ATP metabolite (ADP, AMP, adenosine) is necessary for this augmentation. Excess PPi production is effectively blocked by known inhibitors of nucleoside triphosphate (NTP) pyrophosphohydrolase (EDTA, EGTA, dithiothreitol). Excess 32P-PPi is generated directly from gamma 32P-ATP by cartilage, as substrate and product have similar specific activities. These findings strongly favor ecto-NTP pyrophosphohydrolase as the source of extracellular PPi generation in the presence of NTP. Additionally, active nucleotide and nucleoside catabolism is demonstrated in these cartilage organ cultures.

Selenium, zinc, and copper changes with valproic acid: possible relation to drug side effects.

Side effects of treatment with the anticonvulsant valproic acid (VPA) suggested the possibility of alteration of trace metal status. Administration of VPA for 1 week produced significant depletion of zinc and selenium in plasma of rats and a one-third reduction of hepatic selenium. Patients who were treated chronically, with VPA as the sole anticonvulsant medication, had decreased plasma selenium levels. Most cases of VPA-associated hepatotoxicity occur in children. This could be due to decreased selenium concentrations when mechanisms for protection against peroxidative damage are not fully developed.

Cartilage nucleoside triphosphate (NTP) pyrophosphohydrolase. I. Identification as an ecto-enzyme.

When 1 mM ATP was added to ambient media of canine cartilage in organ culture or canine chondrocytes in monolayer culture, PPi was generated linearly over 4 hours. The appearance of PPi was related to an ectoenzyme based upon its ability to act upon extracellular substrate, to generate extracellular products, failure to detect enzyme activity in supernatant media, failure to increase activity by cell disruption, and susceptibility to digestion by extracellular trypsin. The enzyme responsible for PPi generation is nucleoside triphosphate (NTP) pyrophosphohydrolase, which acts upon a number of purine and pyrimidine nucleoside triphosphates. This enzyme may play a role in generation of extracellular PPi which participates in calcium pyrophosphate dihydrate crystal formation.