Images in cardiovascular medicine. Anomalous origins of the left main coronary artery from the noncoronary sinus and of the right coronary artery from the left sinus of Valsalva.
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Publications and source records attributed to E Mayer.
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The insulin resistance syndrome (IRS) is characterized by a combination of interrelated coronary heart disease (CHD) risk factors, including low high-density lipoprotein cholesterol (HDL-C) levels, obesity and increases in triglyceride (TG), blood pressure, small low-density lipoprotein particles (LDL), and both fasting and postload plasma insulin and glucose. Using factor analysis, we previously identified 3 uncorrelated factors that explained 66% of the variance among these variables, based on data from women participating in examination 2 of the Kaiser Permanente Women Twins Study in Oakland, CA during 1989-1990. The factors were interpreted as: 1) body mass/fat distribution, 2) insulin/glucose, and 3) lipids: TG, HDL-C, LDL peak particle diameter. In this analysis, heritability of each of the factors was estimated based on data from 140 monozygotic and 96 dizygotic pairs of non-diabetic women twins. Heritability estimates were calculated using the classical approach, the analysis of variance (ANOVA) approach, and the maximum likelihood approach. For the body mass/fat distribution factor heritability estimates suggest moderate genetic influences; 0.61 (P < 0.001), 0.14 (P > 0.05), and 0.71 (P < 0.001), respectively. The insulin/glucose factor appeared to be highly heritable, with estimates of 0.87, 0.92, and 0.57 (all P < 0.001), respectively. The heritability estimates for the lipid factor were moderate and consistent across methods: 0.25 (P < 0.10), 0.32 (P < 0.05), and 0.30 (P < 0.05), respectively. These results are consistent with genetic influences on each of the 3 "factors," and suggest that both genetic and environmental effects are involved in the clustering of IRS risk factors.
Intermanual transfer for a skilled motor task was studied in two patients with total callosal agenesis, and one with an acquired partial callosal lesion and clinical evidence for disturbed transfer of motor signals. Patients had to draw meaningless figures with one upper extremity (original learning, OL) and to reproduce their mirror-reversals thereafter with the other side (transfer learning, TL). Both directions of intermanual transfer were tested in two conditions, that is, between either proximal or distal muscle groups. Transfer was evaluated by comparing OL and TL performance at the same effector. The main variable of interest was movement time during the first eight trials of OL and TL. All three patients displayed a significant benefit for transfer from the dominant to the non-dominant hand but not vice versa during proximal motor activity. When compared with the performance of healthy subjects tested in almost identical conditions in a previously reported study, the proximal transfer behavior was found to be similar for all patients and the normal group. Although patients exhibited no significant benefit for distal transfer, their non-dominant-to-dominant distal transfer was above the normal range. The similar transfer pattern of the patients and healthy subjects when using proximal musculature suggests that proximal transfer may be subserved by identical extracallosal pathways, most probably by the ipsilaterally descending motor systems. Since non-dominant-to-dominant distal transfer was found to be disadvantageous in healthy subjects, the patients' relative superiority in this condition may reflect missing callosal influences of an inhibitory nature.
The sensitivity of MR imaging for detection of pulmonary metastases was evaluated in 23 patients. The MR imaging was performed with T2-weighted turbo-spin-echo (TSE) sequences at 1.5 T. The MR images were compared with spiral volumetric CT which served as the radiological standard. All MR images were interpreted by two radiologists without knowledge of CT findings. The MR imaging technique initially enabled correct identification of altogether 286 of 340 metastases (84 %) resulting in sensitivities of 36 % (size of nodules < 5 mm), 83 % (size of nodules 5-10 mm), 92 % (size of nodules 10-15 mm), and 100 % (size of nodules > 15 mm). Retrospectively with knowledge of CT findings an additional 39 metastases were visible; 15 remained undetected even retrospectively. The results of this paper suggest that MRI still does not have a role for screening for pulmonary metastases. It proves, however, that the incidental diagnosis of a pulmonary lesion on T2-weighted TSE images is highly reliable in representing a pulmonary nodule also visible on CT. However, thus far the malignant nature especially of small nodules detected by MRI (as by CT) remains uncertain even in patients with known malignant neoplasm.
Pulmonary thromboendarterectomy (PTE) is a potentially curative procedure in chronic thromboembolic pulmonary hypertension. From June, 1989, to December, 1994, we performed PTE in 109 consecutive patients. Multiple changes in surgical approach and postoperative management have been implemented since January, 1995. We report the early results of 32 thromboendarterectomies performed from January, 1995, to January, 1997. Thirty-two patients (16 females, 16 males; mean age 55 years) were operated using cardiopulmonary bypass, deep hypothermia and circulatory arrest. Preoperative NYHA functional class was III in 21 and IV in 11 patients. Pulmonary vascular resistance (PVR) and mean pulmonary artery pressure (mPAP) were elevated to 967 +/- 238 dynes.s.cm-5 and 51 +/- 11 mm Hg respectively. The perioperative mortality rate was 9.3% (3 of 32). Twenty-nine survivors were weaned from mechanical ventilation and extubated after a mean of 35 hours (12 to 190 hours). PVR was reduced to 301 +/- 151 dynes.s.cm-5 (p < 0.001) and mPAP was reduced to 28 +/- 10 mm Hg (p < 0.001). Pulmonary thromboendarterectomy is an effective surgical procedure for chronic thromboembolic pulmonary hypertension. By means of modifications in surgical approach and postoperative management, early results can be improved and perioperative mortality can be decreased to less than 10%.
Pulmonary thromboendarterectomy (PTE) leads to an acute decrease of right ventricular (RV) afterload in patients with chronic thromboembolic pulmonary hypertension. We investigated the changes in right and left ventricular (LV) geometry and hemodynamics by means of transthoracic echocardiography. The prospective study was performed in 14 patients (8 female, 6 male; age 55 +/- 20 years) before and 18 +/- 12 days after PTE. Total pulmonary vascular resistance and systolic pulmonary artery pressure were significantly decreased (PVR: preoperative 986 +/- 318, postoperative 323 +/- 280 dyn x s/cm5, p < 0.05; PAP preoperative 71 +/- 40, postoperative 41 +/- 40 mm Hg + right atrial pressure, p < 0.05). End diastolic and end systolic RV area decreased from 33 +/- 12 to 23 +/- 8 cm2, respectively, from 26 +/- 10 to 16 +/- 6 cm2, p < 0.05. There was an increase in systolic RV fractional area change from 20 +/- 12 to 30 +/- 16%, p < 0.05. RV systolic pressure rise remained unchanged (516 +/- 166 vs. 556 +/- 128 mm Hg/sec). LV ejection fraction remained within normal ranges (64 +/- 16 vs. 62 +/- 12%). Echocardiographically determined cardiac index increased from 2.8 +/- 0.74 to 4.1 +/- 1.74 l/min/m2. A decrease in LV excentricity indices (end diastolic: 1.9 +/- 1 vs. 1.1 +/- 0.3, end systolic: 1.7 +/- 0.6 vs. 1.1 +/- 0.4, p < 0.05) proved a normalization of preoperatively altered septum motion. LV diastolic filling returned to normal limits: (E/A ratio: 0.62 +/- 0.34 vs. 1.3 +/- 0.8; p < 0.05); Peak E velocity: 0.51 +/- 0.34 vs. 0.88 +/- 0.28 m/sec, p < 0.05; Peak A velocity: 0.81 +/- 0.36 vs. 0.72 +/- 0.42 m/sec, ns; E deceleration velocity: 299 +/- 328 vs. 582 +/- 294 cm/sec2, p < 0.05; Isovolumic relaxation time: 134 +/- 40 vs. 83 +/- 38 m/sec, p < 0.05). We could show a marked decrease in RV afterload shortly after PTE with a profound recovery of right ventricular systolic function--even in case of severe pulmonary hypertension. A decrease in paradoxic motion of the interventricular septum and normalization of LV diastolic filling pattern resulted in a significant increase of cardiac index.
PURPOSE: To analyze the potential cause of retinal arterial occlusion in a 9-year-old child. METHODS: Case report. Antithrombin III, protein C, free protein S, activated protein C resistance, and antiphospholipid antibodies in plasma were determined. Determination of factor V R506Q (Leiden mutation), thermolabile methylene tetrahydrofolate reductase by polymerase chain reaction, and restriction enzyme analysis were performed. RESULTS: The patient was found to be heterozygous for factor V R506Q (Leiden mutation) and homozygous for thermolabile methylene tetrahydrofolate reductase. CONCLUSION: Coexistence of two mild hereditary thrombophilic states may result in severe thrombotic manifestations in young people.
Electrophysiologic tests may be abnormal in asymptomatic HIV-1-infected individuals. Our study was aimed at determining whether these findings have a prognostic value and could be corrected by antiviral treatment. In 18 patients, followed for 34 or 43 months, these findings were not progressive. Only one patient developed Aids dementia complex (ADC). Three have died (one with normal, two with abnormal tests at baseline). To study the effect of antiviral treatment, another group of seven asymptomatic patients was included into a cross-over double-blind study with either eight weeks zidovudine or eight weeks placebo, separated by eight more weeks without treatment. Electrophysiological evaluation was also performed in a group of 15 patients before antiviral therapy with zidovudine or didanosine was started and again after a mean of three and 13 months treatment. Results did not suggest that treatment reverses early electroencephalographic and otoneurological changes seen in HIV-1 infection.
AIM: In this study of patients with irritable bowel syndrome (IBS), we evaluated the relationship between patient-rated severity of IBS and patients' physical and psychological symptoms, health care resource use and quality of life. METHODS: One hundred and twenty-six patients diagnosed with IBS were administered a series of questionnaires, including the Bowel Symptom Checklist, the Symptom Checklist-90-R (a psychological symptom checklist), the IBSQOL (a disease-specific quality of life instrument), the SF-36 (a general health status instrument), and a health resource utilization assessment that measured health care use, time loss from work, impact on productivity, and days worked with symptoms. RESULTS: No relationship was found between IBS severity and gastrointestinal symptoms, except for a feeling of unpassed stool. IBS severity was also not related to psychological symptom severity. Direct traditional indicators of resource use (e.g. physician visits, hospital admissions and emergency room visits) were not significantly associated by severity level; however, indirect measures of resource use (e.g. number of days with pain, productivity and number of bed days) were related to severity. Quality of life was clearly associated with perceived IBS severity. Patients who rated themselves as very severe reported the lowest scores and had the poorest health for all quality of life dimensions measured. CONCLUSIONS: These findings suggest that perceived IBS severity is defined by the limitations the disease imposes, rather that by the symptoms. Patients with reduced productivity and decreased functioning for most of the quality of life indicators were those who rated their IBS as very severe.
BACKGROUND: We describe the development and evaluation of a new disease-specific instrument, the Irritable Bowel Syndrome Quality of Life Questionnaire (IBSQOL), which was designed for use in patients with irritable bowel syndrome. The IBSQOL measures 10 domains found to be relevant to patients with irritable bowel syndrome: emotional health, mental health, health belief, sleep, energy, physical functioning, diet, social role, physical role, and sexual relations. METHODS: During its development and evaluation, the IBSQOL was administered to over 500 patients with irritable bowel syndrome--two groups of patients from tertiary care centres, three focus groups of 8-12 patients each, and 287 patients in a national irritable bowel syndrome support network. As a control, the IBSQOL was also administered to 37 patients who did not have irritable bowel syndrome but had other gastrointestinal disorders. Statistical analyses to test the reliability and validity of the IBSQOL were performed using Cronbach's alpha coefficient. RESULTS: Responses from the focus groups indicated that the IBSQOL was easy to complete and did not require too much time to fill out (approximately equal to 25 min). Statistical analyses of the final 30-item version of the IBSQOL demonstrated that it had both adequate validity and reliability (alpha > or = 0.60). A comparison of mean IBSQOL scores of persons with and without irritable bowel syndrome (but with other gastrointestinal conditions) showed no difference between the two groups with irritable bowel syndrome; however, scores for both irritable bowel syndrome groups were considerably lower than for the non-irritable bowel syndrome group, suggesting better health-related quality of life in patients who do not have irritable bowel syndrome. This further demonstrated the validity of the IBSQOL in targeting questions and domains specific to patients with irritable bowel syndrome. CONCLUSIONS: Evaluation of the IBSQOL included testing the questionnaire in a large number of patients, which resulted in a revised and well-constructed instrument that demonstrated both adequate validity and reliability. The IBSQOL is currently being used in large-scale clinical trials to measure changes in quality of life in patients with irritable bowel syndrome following treatment intervention.
Exercise effects on natural killer cell (NK) activity in men appear to be intensity dependent, but there is very little data in women. We tested the effect of high versus moderate-intensity exercise relative to non-exercising controls on NK cytolytic activity (NK activity) in women using oral contraceptives. Subjects (n = 8) participated in 3 treatments consisting of 25 min of cycle ergometer exercise at 80% (HI-INT) and 40% (MOD-INT) VO2max, and a 25 min control (CON) session in which the subject remained seated on the cycle ergometer, but did not exercise. Blood was obtained prior to exercise, immediately after, and at 90 min and 3 h after exercise. During CON, NK activity gradually increased and cortisol gradually decreased during the approximately 3.5 h experimental period. Relative to CON, HI-INT increased NK activity, %CD56+ (NK) cells, and plasma norepinephrine immediately post exercise (p < or = 0.05). There was also a trend for decreased NK activity at 90 min (p = 0.075). No differences among treatment groups were found by 3 h post exercise. In MOD-INT, there were no differences from CON in any variable at any time. These data suggest that the typical NK response to intensive exercise in men, which consists of a brief increase followed by a more prolonged suppression, also occurs in women using oral contraceptives. However, it is important to use time-matched control measurements in determining this response.
NIDDM is usually characterized by beta-cell failure and decreased insulin sensitivity. It has been reported that a high proportion of African-American NIDDM subjects are insulin sensitive. To examine this issue, we determined insulin sensitivity (S(I)) in 479 NIDDM subjects by minimal model analyses of frequently sampled intravenous glucose tolerance (FSIGT) from the Insulin Resistance Atherosclerosis Study (IRAS), a large multicenter study of insulin sensitivity and cardiovascular risk in African-Americans, Hispanics, and non-Hispanic whites. The African-Americans and non-Hispanic whites were sampled in Los Angeles and Oakland, California. The non-Hispanic whites and Hispanics were sampled in San Antonio, Texas, and San Luis Valley, Colorado. We defined the proportion of insulin-sensitive (S(I)) subjects as > or =1.61 min-1 x microU-1 x ml-1, which is above the median for nondiabetic subjects of all ethnic groups in the IRAS. Using this definition, the proportion of insulin-sensitive diabetic subjects was very low in all ethnic groups (non-Hispanic whites [14.3%] vs. African-Americans [6.5%], P = 0.039 in Los Angeles and Oakland; non-Hispanic whites [6.8%] vs. Hispanics [4.9%], P = 0.737 in San Luis Valley and San Antonio). These results were also similar in newly diagnosed mildly hyperglycemic diabetic subjects. In addition, these results were not affected by the adjustment for differences in obesity, body fat distribution, and severity of hyperglycemia. Even in nonobese subjects (with BMI <30 kg/m2), the proportion of insulin-sensitive subjects (S(I) > or =1.61 min-1 x microU-1 x ml-1) was low (3.6-9.7%). The acute insulin response (AIR) was significantly higher in African-Americans than in non-Hispanic whites; there were no ethnic differences in AIR between Hispanics and non-Hispanic whites. There were no significant ethnic differences for non-insulin-mediated glucose disposal (S(G)). We conclude that the number of insulin-sensitive NIDDM subjects is low and similar among non-Hispanic whites, Hispanics, and African-Americans in the U.S.
As the U.S. Department of Veterans Affairs (VA) makes the change over to Veterans Integrated Service Network (VISNs) the need for new and better leadership is warranted if VA wants to not only survive, but thrive in the emerging twenty-first century healthcare system. VA can prepare for the future and meet the challenges facing them by adopting a system of visionary leadership. The use of scenarios and vision techniques are explained as they relate to VA's efforts to move toward their new system of VISNs. The four scenarios provide snapshots of possible futures for the U.S. healthcare system as well as the possible future role and mission of VA--from VA disappearing to its becoming a premier virtual organization.
Pulmonary thromboendarterectomy is an accepted operative procedure for treatment of pulmonary hypertension due to chronic embolism. Despite its proven value this procedure has been established at very few centers worldwide. In this paper we report our actual operative concept and operative results. Between 8'89 and 4'96 127 patients were operated with use of extracorporeal circulation, deep hypothermia and circulatory arrest. After analysis of the initial high perioperative mortality (26%, 29/108) our operative and postoperative concept changed since 11'94: 1. central incision of both pulmonary arteries, 2. endarterectomy exclusively during circulatory arrest, 3. prolonged reperfusion to 37 degrees C, 4. pressure controlled ventilation, NO-inhalation, early extubation, and 5. modified vasopressor therapy. Preoperatively 12 of the 19 patients were in NYHA class III and 6 in class IV. Mean pulmonary artery pressure was 52(17) mmHg with a calculated pulmonary resistance of 1013(579) dynes.s.cm-5. Mean circulatory arrest time was 37 min (19-57 min) (bypass time 345 min, (240-430 min)). Perioperatively two patients (11%) died (multiorgan failure; rethrombosis of pulmonary artery/right heart failure), all other patients survived (89%). Perioperative complications included reversible renal failure, delirium and postcardiotomy syndrome (1/2/1). Mean pulmonary resistance was postoperatively significantly reduced (362(124) dynes.s.cm-5) (p < 0.01). Early results of pulmonary thromboendarterectomy can be improved by consequent modifications of the intra- and postoperative concept.
Myasthenia gravis is a relatively uncommon autoimmune disorder of neuromuscular transmission. Surgical therapy plays an important role in addition to medical treatment. Follow-up results of 52 patients with thymectomy are presented. Between 1984-1996 thymectomy via median sternotomy was performed in 52 patients with myasthenia gravis (female = 28, male = 24). The score described by Ossermann and Genkins was used for classification. According to this classification, we found 12 patients in class II(I), 21 in class IIA, 17 in class IIB and 2 in class III, respectively. A thymoma was found in 19, follicular lymphoid hyperplasia in 24 and an atrophic thymus in 9 cases, respectively. There was no mortality. Severe postoperative complications consisted of bleeding and reoperation in one patient and another patient developed a sternal instability with consecutive operative refixation. Follow-up evaluation after a mean period of 36 months (min. 6 months, max. 130 months) revealed a relief of myasthenic symptoms in 37 patients. Thymectomy is effective in the treatment of myasthenia gravis with a low complication rate.
BACKGROUND: Pulmonary angiography is the gold standard for the diagnosis of chronic thromboembolic pulmonary hypertension; however, major complications have been reported. This study evaluates the hemodynamic effects of direct pulmonary nonionic contrast bolus injection and oxygen inhalation in patients with chronic thromboembolic pulmonary hypertension. METHODS AND RESULTS: In 33 patients, hemodynamic parameters were measured after oxygen inhalation and during bolus injection of nonionic contrast medium in a control group (group 1. n = 11), in a group of patients with moderately severe pulmonary hypertension (group 2, n = 9), and in a group with severe pulmonary hypertension (group 3, n = 13). Oxygen inhalation significantly improved oxygen supply. Pulmonary artery pressure and heart rate were reduced, but pulmonary vascular resistance and total pulmonary resistance were not significantly affected. One hundred ninety-eight angiograms were performed selectively on both pulmonary arteries in the posterior-anterior, oblique, and lateral views. Before contrast bolus injection, RAP and PAP significantly increased because of initial inspiration. Contrast bolus injection caused only a minor pressure increase (delta PA systolic, 2.3 +/- 1.4, 2.5 +/- 1.8, and 5.0 +/- 5.2 mm Hg, groups 1, 2, and 3, respectively) without significance between the groups. After the angiography, pulmonary artery pressure was moderately increased, predominantly in group 3, but pulmonary vascular resistance was not significantly changed. Systemic vascular resistance was decreased. Cardiac index increased in groups 1 and 2 but was unchanged in group 3. Systemic pressure therefore decreased in group 3. CONCLUSIONS: We concluded that bolus injection of nonionic contrast medium causes no major hemodynamic effects even in patients with severe chronic thromboembolic pulmonary hypertension. Oxygen contributes to safety during the procedure.
Expression of the mRNAs of the proto-oncogenes pp60c-src+, c-fos and jun D were studied using in-situ hybridisation histochemistry in the developing striatum and in striatal grafts. The temporal patterns of mRNA expression were monitored in the striatum of the normal developing rat from the 12th day of gestation (E12) to 10 days postnatally, and were compared to the changes in gene expression observed in E13-E14 primordial striatal tissue grafts 7, 15 and 30 days after implantation in the ibotenic acid-lesioned striatum of adult rats. During development, all three proto-oncogenes were most highly expressed just before birth, at E19. Striatal expression of all three proto-oncogenes was markedly reduced after birth and remained at a low level through to adulthood. A different mode of expression was observed in the transplanted striatum which was unique to each particular gene. jun D and pp60c-src+ were expressed for a longer time period in the grafted primordial cells than in normal development, whereas no c-fos expression could be detected in the grafts. These results suggest that transplantation of embryonic neural cells into the host brain may affect the normal developmental regulation of such cells and their expression of some proto-oncogenes.
Vapor-deposited amorphous solid and hyperquenched glassy water were found to irreversibly transform, on compression at 77 kelvin, to a high-density amorphous solid. On heating at atmospheric pressure, this solid became viscous water (water B), with a reversible glass-liquid transition onset at 129 +/- 2 kelvin. A different form of viscous water (water A) was formed by heating the uncompressed vapor-deposited amorphous solid and hyperquenched liquid water. On thermal cycling up to 148 kelvin, water B remained kinetically and thermodynamically distinct from water A. The occurrence of these two states, which do not interconvert, helps explain both the configurational relaxation of water and stress-induced amorphization.