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Rationale and Objectives: Patient-centered outcome measures have become an essential focus in research methodology in recent years. This may be particularly challenging in imaging research at the technology assessment level to incorporate patient-centeredness. A primary issue in this field is designing a reference standard that is applicable to the entire study population., Materials and Methods: This important element is necessary for translation of findings into clinical practice. In our work, computed tomographic perfusion imaging is being evaluated as a new technology used in aneurysmal subarachnoid hemorrhage patients to detect cerebral vasospasm. We have developed a new reference standard employing a multistage hierarchical design incorporating both clinical and imaging criteria to determine a diagnosis of vasospasm., Results: A flowchart of the reference standard levels is provided for illustration. The limitations and potential biases that may occur using this reference standard are discussed., Conclusions: This reference standard will be applicable to the entire study population, including those with and without symptoms or further imaging with digital subtraction angiography.

Rationale and Objectives: Patient-centered care has become a primary focus in clinical practice. In developing practice guidelines for clinical care, the patients' perspective is an important component., Materials and Methods: Patients' preferences are represented in a decision analytic model as quality-of-life weights for different health states associated with the aneurysmal subarachnoid hemorrhage population. The time-tradeoff method is used to obtain the individual patients' preferences, which are directly measured in quality-adjusted life years. An individualized care model is explained as a means of implementing a patient-centered approach into practice guidelines for clinical care. A method for calculating the expected value for societal benefit from improved decision making using an individualized care model is reviewed., Results: We discuss our work-in-progress towards incorporating patients' preferences in a decision analytic model for aneurysmal subarachnoid hemorrhage patients. The main methodologic concerns for using patients' preferences in cost-effectiveness analyses for developing practice guidelines are discussed., Conclusion: Emphasis is placed on using patients' preferences and patient-centered outcome measures in cost-effectiveness analyses.

Background: It is known that many housestaff physicians are unable to demonstrate perfect metered dose inhaler (MDI) technique., Objective: This study assessed whether a single teaching session for house staff physicians would significantly improve their MDI technique., Methods: Thirty-eight pediatric house staff physicians were asked to demonstrate MDI technique with a placebo MDI. The physicians were evaluated on the following seven steps: (1) shaking the MDI and removing the cap, (2) exhaling prior to MDI use, (3) holding the MDI upright, (4) proper timing of actuation, (5) a slow inspiratory effort, (6) one MDI actuation per breath, and (7) holding the breath > or = 5 seconds. A 20-minute teaching session and demonstration of proper MDI technique was then given. At the end of this session all residents were eventually able to demonstrate proper technique. Two months following this educational session the same house staff physicians were re-evaluated on their MDI technique., Results: Initially, ten participants (26%) demonstrated perfect technique. Two months postinstruction the same number of physicians (ten) demonstrated perfect technique. Only six physicians demonstrated perfect technique at both evaluations. Three of the seven steps showed enough change from the first evaluation to the second to permit statistical analysis. Step 4 (timing of actuation) had 11 Physicians' performances improve while three worsened (P=.03). Step 5 (a slow inspiratory effort) had nine physicians' performances improve while three worsened (P=.073). For step 7 (holding the breath > or = 5 seconds), 11 physicians improved while 2 worsened (P=.006). Comparing global performance, there were 17 physicians that improved, 8 that worsened, and 13 with no change (P=.054)., Conclusions: This study confirmed that many housestaff physicians do not demonstrate optimal MDI technique. While one educational session may somewhat improve their future performance, it is not sufficient to guarantee perfect technique. This suggests that repeated education needs to be given to housestaff physicians.

We present a case of prenatally diagnosed interrupted aortic arch with a ventricular septal defect in the presence of maternal congenital heart disease, which led to the detection of segmental monosomy of chromosome 22q11.2 in both patients. The implications of detecting a microdeletion and the importance of a multidisciplinary approach to prenatal diagnosis and counseling are discussed.

Clinical and electrophysiologic data from 51 consecutive patients with sustained monomorphic ventricular tachycardia inducible during programmed ventricular stimulation were evaluated to determine what variables predict the response to intravenous class IA antiarrhythmic agents. All patients received acute drug testing in the electrophysiologic laboratory with either intravenous procainamide or intravenous quinidine. Ventricular tachycardia suppression was achieved in 9 out of 51 patients (18%). The age, gender, left ventricular ejection fraction, baseline right ventricular effective refractory period, baseline HV interval, and baseline ventricular tachycardia cycle length were not predictive of ventricular tachycardia suppression with intravenous procainamide or quinidine during programmed ventricular stimulation. The degree of prolongation of the right ventricular effective refractory period after drug administration did not predict success or failure to suppress inducible ventricular tachycardia. The degree of prolongation of the HV interval was also not predictive. In addition, the degree of prolongation of the right ventricular effective refractory period or the HV interval did not predict the change in the ventricular tachycardia cycle length after drug administration in patients who remained inducible. These data indicate that the response to class IA antiarrhythmic agents in patients with inducible sustained monomorphic ventricular tachycardia cannot be predicted on the basis of various clinical and electrophysiologic parameters.

To assess the effects of digoxin as single therapy and in combination with quinidine in the treatment of atrial fibrillation, the atrial fibrillation threshold was determined from the right atrial appendage and Bachmann's bundle in 11 open chest dogs. In group 1 (six dogs), the atrial fibrillation threshold was determined at baseline, post-quinidine (10 mg/kg intravenously) and then post-digoxin (50 micrograms/kg intravenously). In group 2 (five dogs), the order of drug administration was reversed. The results of this study were: 1) Digoxin had no significant effect on the atrial fibrillation threshold when given alone. 2) Quinidine significantly increased the atrial fibrillation threshold (p less than 0.002) and the addition of digoxin resulted in a further increase in threshold (p less than 0.002). 3) Quinidine produced greater suppression of atrial fibrillation induction at the right atrial site than at the Bachmann's bundle site, suggesting differential effects of quinidine on atrial fibers.

A 73-year-old man was noted to have atrioventricular (AV) nodal reentry tachycardia, which was induced during programmed electrical stimulation. After 1 month of oral amiodarone therapy, AV nodal reentry tachycardia was prevented by the prolongation of atrial refractoriness and not by direct action on the AV node itself.

Fifty-four patients with a previous myocardial infarction and drug-refractory symptomatic ventricular tachycardia (VT) were treated with amiodarone on a long-term basis (range 6 to 54 months, mean 26) irrespective of the results of programmed ventricular stimulation, which was performed after high-dose oral amiodarone loading for more than 4 weeks. VT was rendered noninducible in 6 of 54 patients (11%) taking oral amiodarone. During a mean follow-up of 32 months, these 6 patients remained free of VT or sudden cardiac death. Forty-eight patients (89%) continued to have VT inducible by programmed ventricular stimulation. However, they could be separated into 2 groups: VT-modified (20 patients) and VT-unchanged (28 patients). In the VT-modified group, the induced VT with amiodarone was slowed or rendered nonsustained, and only 3 of 20 (15%) patients during a mean follow-up of 23 months had well tolerated VT recurrences. In the VT-unchanged group, 16 of 28 patients (57%) had recurrences of VT or ventricular fibrillation during a mean follow-up of 24 months. Sudden cardiac death occurred in 6 of these 16 patients. Thus, programmed ventricular stimulation in patients with VT taking long-term amiodarone may have prognostic implications.

The syndrome of sudden cardiac death in southeast Asians has only recently been given attention in the American medical literature. This case report describes a patient who presented with this rare syndrome. The physical examination, Holter monitor, 2-D echocardiogram, exercise treadmill test, radionuclide ventriculogram, coronary angiography, and endomyocardial biopsy were all normal. Programmed ventricular stimulation reproducibly induced sustained polymorphic ventricular tachycardia. Oral procainamide, oral quinidine and oral quinidine plus propranolol were not successful in suppressing inducible polymorphic ventricular tachycardia. The arrhythmia remained inducible after six weeks of oral amiodarone therapy. However, he has no clinical recurrences while on amiodarone after one year of follow-up.

Suberythemogenic exposure of human skin treated with aqueous 8-MOP to radiation greater than 380 nm prolongs photosensitization to subsequent UVA from 6 to 24-72 h. One hypothesis for prolonged photosensitization is that greater than 380 nm irradiation forms 8-MOP-DNA monoadducts, which are removed more slowly than free 8-MOP and serve as a substrate for crosslinking by further UVA exposure. Sufficient DNA crosslinking results in erythema. We have examined this hypothesis by measuring the action spectrum for induction of prolonged photosensitization. Skin of normal volunteers was treated with aqueous 8-MOP (0.003%) and immediately received suberythemogenic monochromatic exposures between 334 and 430 nm. twenty-four hours later, the presence of prolonged sensitization was tested by small exposures of UVA. Erythema was evaluated 3 and 5 d later, and an action spectrum for prolonged sensitization was determined. The minimum phototoxic dose (MPD) was also determined at each wavelength. The action spectrum for prolonged photosensitization declined gradually between 334 and 425 nm. The action spectrum for delayed erythema induced by a single exposure of 8-MOP-treated skin declined rapidly from 334-390 nm. These findings are consistent with prolonged binding of 8-MOP in the skin by an initial exposure, possibly as 8-MOP-DNA monoadducts, allowing the second exposure to induce an erythemogenic event, possibly crosslinking of DNA.

An abused 10-year-old girl with a family history of Huntington's disease developed incapacitating abdominal pain with concomitant behavioral symptomatology suggestive of dementia. The pseudoneurologic nature of her symptoms was clarified through exhaustive evaluation and did not appear to be that of early-onset Huntington's disease. Assessment included pediatric, psychiatric, neurologic, and gynecologic examination; extensive radiologic and laboratory tests; and chronobiology studies. Successful treatment necessitated the integration of numerous therapeutic modalities including dynamically oriented psychotherapy, psychopharmacologic intervention, physical therapy, behavior modification, and electroconvulsive therapy.

The efficacy and safety of a new antitachycardia pacing technique, self-adapting decremental overdrive pacing, was assessed in patients with clinical ventricular tachyarrhythmias who underwent programmed ventricular stimulation and serial drug testing. The three phases of this study involved a learning/experience phase, followed by intrapatient comparison of decremental overdrive pacing with conventional antitachycardia pacing modalities of overdrive burst ventricular pacing, and diastolic scanning with single (S2) and double (S2S3) ventricular extrastimuli. The final phase involved an intrapatient comparison of automated decremental overdrive pacing with overdrive burst ventricular pacing in patients with ventricular tachycardia (VT) cycle lengths of 280 msec or greater. Decremental overdrive pacing was superior to overdrive burst pacing and diastolic scanning (S2S3 and S2) (83% vs 38%, 50%, 9%) in patients with VT cycle lengths of 280 msec or greater. Automated decremental overdrive pacing as applied in the final phase was the most efficacious modality, terminating 92% of VT episodes compared with 56% for overdrive burst pacing in the same patients.

Unlabelled: The electrophysiologic and electrocardiographic effects of intravenous pirmenol were compared with intravenous procainamide in 17 patients with symptomatic ventricular tachycardia. Pirmenol was found to prolong the PR interval, the QRS duration, the QTc interval, the HV interval, the atrial effective refractory period, and the ventricular effective refractory period. The sinus cycle length decreased following pirmenol administration. The sinus node recovery time, the PA interval, the AH interval, the Wenckebach cycle length, and the AV nodal ERP were unchanged. In patients whose ventricular tachycardias remained inducible on pirmenol, the cycle length was significantly prolonged compared to baseline. These changes were similar to those seen following the administration of procainamide. All 17 patients had sustained ventricular tachycardia inducible during programmed ventricular stimulation in the baseline state. In four patients the ventricular tachycardia was suppressed with both primenol and procainamide. In the remaining 13 patients ventricular tachycardia remained inducible on procainamide. Of these 13 patients, an additional two patients had their ventricular tachycardias rendered noninducible on pirmenol., In Conclusion: (1) the electrophysiologic and electrocardiographic effects of pirmenol are similar to those of procainamide; (2) although ventricular tachycardia inducibility following procainamide was similar to that of pirmenol, an occasional patient with ventricular tachycardia inducible on procainamide had ventricular tachycardias suppressed on pirmenol.

To determine if programmed electrical stimulation (PES) could be utilized to identify patients with high-grade ventricular ectopy at low- or high-risk for sudden cardiac death, we performed PES in 40 patients with high-grade ventricular ectopy refractory to conventional antiarrhythmic agents. Twenty-one patients had a previous myocardial infarction, five had cardiomyopathy, six had hypertension, three had valvular heart disease and five had no known structural heart disease. The mean age was 50 years (range, 18 to 76). During programmed ventricular stimulation, eight patients had inducible sustained (more than 30 seconds) monomorphic ventricular tachycardia (Group I) but in 32 patients sustained ventricular tachycardia was not inducible (Group II). None of the five patients without structural heart disease were inducible while seven out of 21 (33%) patients with previous myocardial infarction had inducible ventricular tachycardia (VT). Antiarrhythmic therapy was instituted in patients with inducible VT; patients without inducible VT did not receive antiarrhythmic agents. In Group I, seven of the eight patients are alive (mean follow-up, 16 months) and in Group II, 28 of the 32 patients are alive (mean follow-up, 17 months). None of the five deaths were sudden. We conclude that in the absence of antiarrhythmic therapy, the incidence of sudden cardiac death is very low in patients with high-grade ventricular ectopy who do not have inducible monomorphic ventricular tachycardia during programmed ventricular stimulation.

Atrial fibrillation (AF) is a difficult arrhythmia to manage with antiarrhythmic agents. Amiodarone is highly effective in restoring and maintaining normal sinus rhythm in patients with AF. However, the mechanism and predictors of efficacy for amiodarone in treating AF have not been adequately addressed. Various measures of success or failure of amiodarone therapy were examined in 68 patients who had paroxysmal or chronic, established AF refractory to conventional antiarrhythmic agents. The patients were 25 to 75 years old (mean 59) and mean follow-up was 21 months (range 3 to 56). Maintenance amiodarone dosages were 200 to 400 mg/day. Overall, amiodarone therapy was effective long term in 54 of the 68 patients (79%). Left atrial diameter, age, gender and origin of AF were not helpful in predicting success or failure of amiodarone therapy. The presence of chronic AF for longer than 1 year was an adverse factor in maintaining normal sinus rhythm (p = 0.007), although the success rate even in this group was relatively high (57%). Thirty-five percent of the patients had adverse effects, which precluded long-term therapy with amiodarone in 10%.