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To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.jacr.2008.05.015 Byline: Richard B. Gunderman (a), Jeffrey C. Weinreb (b), Arl Van Moore (c), Bruce J. Hillman (d), Harvey L. Neiman (e), James H. Thrall (f) Keywords: Radiology; radiologists; practice models; socioeconomic issues; ethics; professionalism Abstract: The 2008 ACR Forum brought together a diverse group of participants from clinical radiology, radiology leadership and practice management, managed care, economics, law, and entrepreneurship in Washington, DC, in January 2008 to discuss current models of radiology practice and anticipate new ones. It addressed what forces shape the practice of radiology, how these forces are changing, and how radiology practices can most effectively respond to them in the future. Author Affiliation: (a) Department of Radiology, Indiana University School of Medicine, Indianapolis, Indiana (b) Yale University School of Medicine, New Haven, Connecticut (c) Charlotte Radiology PA, Charlotte, North Carolina (d) University of Virginia Health System, Charlottesville, Virginia (e) American College of Radiology, Reston, Virginia (f) Department of Radiology, Massachusetts General Hospital, Boston, Massachusetts

To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.jacr.2006.08.020 Byline: Richard B. Gunderman (a), Jeffrey C. Weinreb (b), James P. Borgstede (c), Bruce J. Hilman (d), Harvey L. Neiman (e) Keywords: Cardiovascular imaging; radiology; radiologists; education; economics Abstract: This paper summarizes the 2006 ACR Forum, which explored the history of the relationship between radiology and cardiovascular imaging and sought to explore strategies by which radiology could cope with similar challenges in the future. Key topics include: competition between radiology and other medical specialties, the importance of cardiac imaging, the relative merits of cardiologists and radiologists as cardiovascular imagers, and specific recommendations for radiology leaders in the areas of education, research, clinical practice, and policy. Author Affiliation: (a) Indiana University School of Medicine, Indianapolis, Ind. (b) Yale University School of Medicine, New Haven, Conn. (c) University California, San Diego, San Diego, Calif. (d) University of Virginia Health System, Charlottesville, Va. (e) American College of Radiology, Reston, Va.

Pregnant women with phenylketonuria who eat a diet free of phenylalanine can substantially reduce the risk of damage to their baby. This was the conclusion of a 12-year follow-up of 576 women with phenylketonuria, a metabolic disease that can cause mental retardation.

Byline: Brian L. Ganzel, Harvey L. Edmonds, John R. Pank, L.Jane Goldsmith Abstract: Background: Patients undergoing complex aortic procedures performed with deep hypothermia and circulatory arrest have a significant risk of an adverse neurologic event when the arrest period is prolonged. Retrograde cerebral perfusion appears to improve cerebral protection, although collapsed cortical veins or functional jugular venous valves may restrict flow at the frequently recommended maximum pressure of 25 mm Hg. Therefore, the purpose of this study was to demonstrate the benefit of multimodality neurophysiologic monitoring in assuring delivery of retrograde cerebral perfusion. Methods: Electroencephalography, cerebral blood flow velocity, and regional cerebral venous oxygen saturation were used to quantify the intraoperative neurophysiologic changes accompanying retrograde cerebral perfusion. The magnitude of changes was compared with those previously observed during arrest without retrograde cerebral perfusion. Results: Thirty patients underwent complex aortic procedures necessitating circulatory arrest, 22 with retrograde cerebral perfusion. The mean retrograde perfusion pressure of 40 mm Hg (30 to 49 mm Hg, 95% confidence interval) and flow rate of 1.2 L/min (0.9 to 1.6 L/min) necessary to achieve documented retrograde cerebral perfusion was much higher than previously recommended. During both retrograde cerebral perfusion and rewarming, cerebral oximetric monitoring guided adjustments in perfusion parameters to limit the rate of desaturation to 0.4% per minute (0.3% to 0.6%). With retrograde cerebral perfusion there was a rapid (1) recovery of electroencephalographic activity during rewarming (21 minutes [range 16 to 26 minutes]) and (2) return of consciousness after the operation (81% [58% to 95%, 95% confidence interval] awake by 12 hours). There was no transcranial Doppler evidence of cerebral edema with retrograde cerebral perfusion. Two neurologic complications occurred in the 22 patients managed with retrograde cerebral perfusion and one in the eight patients managed with arrest only. Conclusions: Multimodality neurologic monitoring assured optimal brain cooling and bihemispheric delivery of retrograde cerebral perfusion. Necessary retrograde pressure and flow were often higher than values previously reported. Avoidance of profound cerebral venous oxygen desaturation during retrograde cerebral perfusion and rewarming was associated with rapid recovery of neurologic function. (J Thorac Cardiovasc Surg 1997;113:748-57) Article History: Received 6 May 1996; Revised 13 June 1996; Revised 25 November 1996; Accepted 23 December 1996 Article Note: (footnote) [star] From the Division of Thoracic and Cardiovascular Surgery,a Departments of Surgery, Anesthesiology,b and Biostatistics Center,c University of Louisville School of Medicine, Louisville, Ky., [star][star] Supported in part by the Mary and Mason Rudd Endowment of Jewish Hospital, Louisville, Ky., a Read at the Seventy-sixth Annual Meeting of The American Association for Thoracic Surgery, San Diego, Calif., April 28-May 1, 1996., aa Address for reprints: B. L. Ganzel, MD, Department of Surgery, University of Louisville, Louisville, KY 40292., acents 0022-5223/97 $5.00 + 0 12/6/80035

To evaluate the efficacy and safety of oral amiodarone in the treatment of recurrent, sustained, refractory ventricular arrhythmias, rapid high-dose oral loading was used to treat 12 critically ill patients with frequent, sometimes incessant, sustained ventricular arrhythmias refractory to 2 to 6 antiarrhythmic agents. Presenting arrhythmias included sustained monomorphic ventricular tachycardia and ventricular fibrillation associated with cardiac arrests in 6 patients. Patients experienced 2 to 10 episodes (mean 5 [+ or -] 2) of sustained ventricular arrhythmias over a mean period of 6.2 [+ or -] 5.0 days (range 1 to 14) before oral amiodarone was initiated at 1,200 to 1,400 mg/day. This included at least 1 to 4 episodes (mean 2.2 [+ or -] 1.1) within 24 hours before amiodarone. One to 4 antiarrhythmic drugs were administered concurrently during amiodarone loading. Sustained ventricular arrhythmias no longer occurred after a mean of 5.2 days (range 1 to 22) with amiodarone. Arrhythmias were controlled in 4 patients within 24 hours, 5 patients within 48 hours, 7 patients within 4 days and 10 patients within 6 days. Patients experienced a mean of 0.6 [+ or -] 0.8 episodes within 24 hours after amiodarone. Nine patients survived to hospital discharge. No patient had significant adverse effects during high-dose loading. In conclusion, high-dose oral amiodarone loading, when added to previously unsuccessful conventional antiarrhythmic therapy, is safe and often rapidly effective for at least short-term control of frequent, refractory, sustained ventricular arrhythmias. (Am J Cardiol 1993;72:1395-1399)

Cardiac tamponade, a potentially lethal complication following cardiac surgery, may present either early or late postoperatively and may be difficult to diagnose due to atypical clinical, hemodynamic, or echocardiographic findings. [...]

The pharmacokinetics, antiarrhythmic activity and safety of intravenously administered recainam were evaluated in 15 men and 3 women. All patients had frequent (>30/hour) ventricular premature complexes (VPCs) and unsustained ventricular tachycardia. Recainam was administered at a loading dose of 4.5 mg/kg/hour over 40 minutes, followed by a maintenance infusion f 0.9 mg/kg/hour for 23 hours and 20 minutes. Sixteen patients had satisfactory efficacy data. The mean frequency of total VPCs decreased by 92.6% and the mean frequency of runs decreased by 99.9% during the maintenance infusion. Suppressions of [is greater than or equal to] 70% of total VPCs and [is greater than or equal to]90% of runs were maintained over the 23-hour, 20-minute maintenance infusion period in 16 of he 18 patients. During the maintenance infusion, hourly group plasma recainam concentrations ranged from mean [+ or -] SD 2.6 [+ or -] 0.7 to 3.4 [+ or -] 0.9 [mu]g/ml. Patients were observed for 24 hours after termination of the infusion. Periodic blood samples were obtained during and after termination of the infusion to determine recainam concentration. Urine specimens were collected over scheduled intervals to determine urinary excretion of recainam. A 2-compartment pharmacokinetic model was used to analyze the data. The following pharmacokinetic parameters were obtained: terminal elimination half-life, 5.0 [+ or -] 0.8 hours; systemic clearance, 0.27 [+ or -] 0.08 liter/hour/kg; and central and steady-state volume of distribution, 0.32 [+ or -] 0.11 and 1.4 [+ or -] 0.4 liter/kg, respectively. Adverse experiences were reported in 4 of the 18 patients, possibly drug-related in 2; none was considered severe or required discontinuation of recainam. Mean electrocardiographic PR and QRS intervals (but not QTc interval) were prolonged 20 to 25% (p [is less than or equal to]0.01) compared with baseline. No proarrhythmic response to recainam was observed. No clinically significantly changes in vital signs or in laboratory test results occurred. This study suggests that intravenous recainam is highly active in suppressing ventricular ectopy and may be safely administered. However, compete safety profiling and determination of its clinical role require additional studies.

OBJECTIVE: The purpose of this study was to evaluate the efficacy of a phenylalanine-restricted diet in reducing fetal morbidity associated with maternal hyperphenylalaninemia in women of childbearing age with blood phenylalanine levels >240 [Mu] mol/L (>4 mg/dl) on an unrestricted diet. STUDY DESIGN: Two hundred thirteen pregnant women with hyperphenylalaninemia that resulted in 134 live births have been enrolled in the study. Outcome measures were subject to the [[Chi].sup.2] test, Fisher exact test, analysis of variance, t test, or Wilcoxon nonparametric test for analysis. RESULTS: Optimal fetal outcome appeared to occur when blood phenylalanine levels

Evaluation of patients with syncope often includes a battery of noninvasive tests. In the study, 45 patients (26 with suspected neurologic and 19 with suspected cardiac syncope) were evaluated with simultaneous 24-hour electroencephalographic (EEG) and 2-channel electrocardiographic (ECG) recordings. Isolated cardiac rhythm abnormalities were noted in 21 patients, but none of these was symptomatic and no definitive arrhythmias occurred. Isolated EEG abnormalities were noted in 11 patients, 5 of whom had EEG abnormalities consistent with seizure disorders. Simultaneous EEG and ECG abnormalities were seen in 4 patients. In 2 cases, a previously unsuspected etiology of syncope was found: seizures in 1 patient with heart disease, and sinus pauses in another thought to have a seizure disorder. Thus, combined ambulatory EEG/ECG monitoring may prove useful in the evaluation of some patients with syncope., The diagnosis of the cause of syncope, fainting, usually involves evaluation of both cardiac and neurologic (nervous system) functions. Eliciting a good history of the symptoms is important, as features suggesting heart arrhythmias or seizures may be identified. The cause is not always evident, though, so that tests may be inconclusive and some may be repeated. A new technique has allowed continuous recording of electroencephalographic (EEG) information plus electrocardiographic (ECG) data while the patient is ambulatory, similar to that which has been available for ECG alone. Problems may be identified while the patient is normally active that may not be apparent if the patient is confined to bedrest. The use of combined ambulatory EEG and ECG recording in evaluating syncope in 45 patients was assessed. Neurologic problems were suspected in 26, while heart-related syncope was suspected in 19 patients. Heart arrhythmias, most of which were minor, were observed in 21 patients, while 12 patients had neurologic problems shown by the EEGs. Four patients had simultaneous EEG and ECG problems. The combined recordings identified problems in two patients which were not detected by other tests. The report indicates that combined EEG and ECG ambulatory monitoring is useful in evaluating patients with fainting due to unknown causes. (Consumer Summary produced by Reliance Medical Information, Inc.)

The case report is presented of an infant with homocystinuria (increased levels of homocysteine, an amino acid, in the urine). This condition results from a deficiency of the enzyme cystathionine beta-synthase (CBS), which normally catalyzes the manufacture of cystathionine from homocysteine. When CBS is deficient, levels of homocysteine and methionine (a product of homocysteine) build up. CBS deficiency is associated with mental retardation, abnormalities of the lens of the eye and of the bones, and blood clots that can lodge in arteries or veins. The infant described in the case report also had pancytopenia, a severe decrease in the numbers of all types of blood cells, and a deficiency of folate (commonly associated with blood cell deficiency). Treatment with folic acid, and the administration of a low-methionine diet with cysteine supplementation (the usual treatment for CBS deficiency), led to the normalization of the patient's high methionine levels and the resolution of many other symptoms. A discussion is presented of the infant screening processes that normally detect CBS deficiency; in this case, the patient probably had a genetically determined error of homocysteine metabolism as well as an acquired folate deficiency because he was fed boiled cow's milk instead of formula. The suggestion is made that infants or children with severe folate deficiency should be evaluated for CBS deficiency even if the reasons for their folate deficiency seem clear. (Consumer Summary produced by Reliance Medical Information, Inc.)

The most common type of inherited metabolic liver disease in children is alpha-1-antitrypsin deficiency (lack of an enzyme important for several physiological functions), a serious disorder often necessitating liver transplantation. To learn more about factors that affect the course and outcome of this condition, the medical records were reviewed from 98 patients treated at one medical institution during a 20-year period. Patients were classified according to whether they had liver disease when first examined (group 1, 85 patients) or they were siblings of group 1 patients identified as having liver disease during screening (group 2, 13 patients). Symptoms of group 1 patients included jaundice (yellow skin and body fluids due to excess bilirubin in the blood), enlarged liver and/or spleen, failure to thrive, gastrointestinal disorders, lethargy or irritability, and diarrhea. Many patients had more than one symptom. Their average age when diagnosed was 2.3 years. Results showed that 43 group 1 patients had poor outcomes, defined as either death or liver transplantation. Good outcomes (survival without transplantation) were experienced by the other 42 group 1 patients. Persistent hyperbilirubinemia (elevated bilirubin levels in blood) determined the need for liver transplantation. Girls had a poorer prognosis than boys. All group 2 patients remained symptom-free during follow-up, although four have abnormally high levels of alanine aminotransferase (an enzyme). Liver transplantation is currently the only treatment for the severe liver disease than can develop with alpha-1-antitrypsin deficiency. It should be performed early in the course of the disease, and is recommended for children with a particular physiological profile, which is discussed. (Consumer Summary produced by Reliance Medical Information, Inc.)

The clinical, angiographic and demographic characteristics of 42 patients with low-grade (0.10) but were significantly older (52 [+ or -] 12 vs 56 [+ or -] 10 years, p = 0.02). Prior acute MI or angina was unusual in group I. Sixty percent had no significant (>50%) residual coronary artery disease while 25% had residual single artery disease. Average significant (>50% diameter stenosis) residual vessel disease was 0.6 [+ or -] 1.0 for group I and 1.9 [+ or -] 0.9 for group II (p (Am J Cardiol 1990;66:773-778), Most heart attacks are caused by a clot blocking a coronary artery narrowed by atherosclerotic (fatty) plaques. It is not known how well patients recover after acute myocardial infarction (AMI) when they have arterial narrowing of less than 50 percent and receive thrombolytic (clot-dissolving) therapy. Although occlusion does not usually occur until the inner diameter of the artery is narrowed by 70 percent or more, 10 to 20 percent of patients requiring thrombolytic therapy after AMI have noncritical stenosis (narrowing) of 50 percent or less. This is an indication of the limitations of coronary angiography (an invasive technique for X-ray visualization of the heart and blood vessels) for predicting the potential for AMI. To measure the effects of low-grade versus critical residual stenosis after AMI, 198 patients with AMI who received thrombolytic therapy were divided into two groups, based on degree of stenosis. The 42 patients with low-grade (less than 50 percent) stenosis were predominantly male smokers; these patients are discussed. Follow-up was terminated in the event of subsequent coronary bypass, angioplasty (mechanical dilatation of the affected artery), total blockage of the infarct site, or death. Patients were monitored for a minimum of 6 months, and an average of 18 months. Data was gathered on symptoms, including typical and atypical angina (chest pain due to inadequate oxygen supply to the heart), dyspnea (difficulty breathing), medical regimen, other risk factors for heart disease, subsequent hospitalizations, and repeat coronary angiography or exercise testing. Although only 1 of the 42 patients died during follow-up, the data suggest a strong likelihood of further heart problems for these patients. Compared with greater atherosclerosis, recurrent clotting at the site of the infarction is more likely to explain total blockages or rapid narrowing of the affected artery. Because this is a small retrospective study, and variables such as type, dosage, and route of thrombolysis, duration and intensity of heparinization (anti-clotting medication), and time to angiography were not measured, it is difficult to draw firm conclusions. The authors report that they found no clear advantage with any given combination of treatments. (Consumer Summary produced by Reliance Medical Information, Inc.)

Phenylketonuria (PKU) is a birth defect in which the enzyme responsible for breaking down phenylalanine, an amino acid required for the growth of a fetus, is lacking. The phenylalanine builds up in the blood and deposits in the brain of affected fetuses. Infants born to mothers who were born with classic PKU are at risk for complications such as mental retardation, small head size, congenital heart disease and low birth weight. Women who have a blood phenylalanine of 1,200 micromoles per liter or more are highly likely to give birth to an affected fetus. In women with less severe disease, the risk is lower. Although a phenylalanine-free diet is effective in reducing the amount of circulating phenylalanine, some women remain untreated. Previous studies suggest that there might be a threshold level of 600 micromoles per liter, above which phenylalanine begins to affect the brain. A study of children born to untreated mothers with varying levels of PKU is reported. Intelligence tests were given to 24 eight-year-old children. The IQ scores were plotted against the mother's phenylalanine levels. Children born to mothers who had mild PKU (165 to 540) had children of normal or superior intelligence. Children born to mothers with atypical or severe PKU generally had lower intelligence scores. The scores did fall below normal until the blood level of phenylalanine rose above 600. It is not clear whether infants who are born to mothers with severe but treated PKU have physical reactions similar to the infants born to untreated mothers. (Consumer Summary produced by Reliance Medical Information, Inc.)

Previous studies of the value of electrophysiologic studies in patients with nonsustained ventricular tachycardia (VT) have been hampered by the inclusion of a small number of patients with various types of heart disease. This retrospective study was designed to assess the value of programmed stimulation in 205 asymptomatic patients who had had an acute myocardial infarction > 1 month before study. inclusion was based on 24-hour Holter monitoring during which patients had to manifest >3 consecutive ventricular beats at a rate >135 beats/min. Forty-seven (23%) patients had normal, 70 (34%) mildly impaired and 88 (43%) severely impaired left ventricular function. Programmed stimulation, using up to 3 extrastimuli, was used in each. Seventy-five patients (36%) were noninducible, 59 (29%) had nonsustained VT ( (Am J Cardiol 1990;65:594-598), Nonsustained ventricular tachycardia (NVT) is a common cardiac dysfunction in which the ventricles, the major pumps of the heart, beat abnormally rapidly, but in a non-sustained fashion. NVT has been difficult to study, as patients at risk for NVT are not easily identified. The effects of treatment of NVT with antiarrhythmic drugs has been equivocal. The records from 205 patients found to have NVT who were studied by electrophysiology were reviewed. During electrophysiological studies, hearts were stimulated with a programmed series of electrical stimuli. Stimulation caused no arrhythmia in 36 percent of patients. NVT was found in 29 percent of patients, while sustained VT was induced in 33 percent of patients. Left ventricular dysfunction was only slightly associated with sustained VT. Of the 205 patients, 82 were left untreated, 66 were treated with medication on the basis of the electrophysiological tests, and 57 were treated with medications but not on the basis of the electrophysiological tests. The type of treatment did not alter morbidity (illness) and mortality risk from 1 to 36 months later among groups with nonsustained, sustained, or no VT, except that of three patients with sustained VT who were not treated, only one remained alive and without illness. Left ventricular function was the only important predictor of outcome. The study suggests that invasive electrophysiological testing is not predictive of further arrhythmias. The benefits of antiarrhythmic drug therapy were not apparent in this study, except possibly for sustained VT. The retrospective design (using records of past patients) limits the usefulness of conclusions, and a prospective randomized trial is needed to provide more appropriate evaluation of this condition. (Consumer Summary produced by Reliance Medical Information, Inc.)

Infants of mothers with phenylketonuria (PKU), an inborn error of metabolism, may develop microcephaly, an abnormal smallness of the head; mental retardation; and congenital heart defects. They also tend to have low birthweight. There is limited information about the development of abnormalities in children of fathers with PKU. One study showed that men with PKU had lower sperm counts and reduced semen volume, suggesting a limited reproductive potential. However, men with PKU are capable of fathering children, and these children have a lower frequency of abnormalities than children of mothers with PKU. Previous studies examining the outcome of children of fathers with PKU have produced inconsistent results. Six families of fathers with PKU are described and include nine offspring without PKU. The results suggest that paternal PKU does not adversely affect the outcome of offspring. None of the nine children had microcephaly, low birthweight, or congenital heart disease. In addition, the study showed that men with PKU have normal fertility. The greater frequency of abnormalities associated with maternal PKU may result from the transfer of biochemical abnormalities from the mother to the fetus during pregnancy, not from damage to the fertilized egg caused by genetic factors. (Consumer Summary produced by Reliance Medical Information, Inc.)

The effect of cigarette smoking on heart rate has not been clearly established. The actual act of inhaling smoke may produce changes in heart rate patterns. The sinus node is the heart's pacemaker; it is an area of specialized heart tissue involved in the conduction of electrical impulses through the heart. The firing of these impulses creates the rhythm of heart contractions. To see if smoking affects the sinus node, 16 patients who smoked were told to abstain from smoking for 12 hours, at which point their heart beats were recorded. They were then given a drug (propranolol) to inactivate the sinus node, and then they smoked two cigarettes in a five-minute period. The length of the sinus node cycle, the normal heart beat, was then measured. The heart rate increased after smoking by shortening the length of a sinus cycle. It is not clear whether smoking affected the sinus node directly or affected other areas involved in pacing the heart. (Consumer Summary produced by Reliance Medical Information, Inc.)

The case of a 43-year-old woman is reported who had an endometrial tumor called an endometrioma that grew in a scar from a previous cesarean delivery. Her tumor was about five inches in diameter and was surgically removed. These tumors occur when tissue from inside the uterus occurs outside the uterus.