Magnetic resonance imaging in the assessment of metacarpophalangeal joint disease in early psoriatic and rheumatoid arthritis.

OBJECTIVES: The aim of this study was to determine whether magnetic resonance imaging (MRI)-related entheseal changes including osteitis and extracapsular oedema could be used to differentiate between metacarpophalangeal (MCP) joint involvement in rheumatoid arthritis (RA) and psoriatic arthritis (PsA). METHODS: Twenty patients (10 each with early RA and PsA) had dynamic contrast-enhanced MRI (DCE-MRI) of swollen MCP joints. Synovitis and tenosynovitis was calculated using quantitative analysis including the degree and kinetics of enhancement of gadolinium diethylenetriaminepentaacetic acid (Gd-DTPA). Periarticular bone erosion and bone oedema were scored using the Outcome Measures in Rheumatology Clinical Trials (OMERACT) proposals. Entheseal-related features including extracapsular soft tissue enhancement or regions of diffuse bone oedema were also evaluated. RESULTS: MRI was not able to differentiate at the group level between both cohorts on the basis of entheseal-related disease but a subgroup of PsA patients had diffuse extracapsular enhancement (30%) or diffuse bone oedema (20%). The RA patient group had a greater degree of MCP synovitis (p<0.0001) and tenosynovitis than PsA patients (p<0.0001). There were no significant differences in either the total number of erosions (p = 0.315) or the presence of periarticular bone oedema (p = 0.105) between the groups. CONCLUSION: Although conventional MRI shows evidence of an enthesitis-associated pathology in the MCP joints in PsA, this is not sufficiently common to be of diagnostic utility.

Mode of action of abatacept in rheumatoid arthritis patients having failed tumour necrosis factor blockade: a histological, gene expression and dynamic magnetic resonance imaging pilot study.

OBJECTIVES: Abatacept is the only agent currently approved to treat rheumatoid arthritis (RA) that targets the co-stimulatory signal required for full T-cell activation. No studies have been conducted on its effect on the synovium, the primary site of pathology. The aim of this study was to determine the synovial effect of abatacept in patients with RA and an inadequate response to tumour necrosis factor alpha (TNFalpha) blocking therapy. METHODS: This first mechanistic study incorporated both dynamic contrast-enhanced (DCE) magnetic resonance imaging (MRI) and arthroscopy-acquired synovial biopsies before and 16 weeks after therapy, providing tissue for immunohistochemistry and quantitative real-time PCR analyses. RESULTS: Sixteen patients (13 women) were studied; all had previously failed TNFalpha-blocking therapy. Fifteen patients completed the study. Synovial biopsies showed a small reduction in cellular content, which was significant only for B cells. The quantitative PCR showed a reduction in expression for most inflammatory genes (Wald statistic of p<0.01 indicating a significant treatment effect), with particular reduction in IFNgamma of -52% (95% CI -73 to -15, p<0.05); this correlated well with MRI improvements. In addition, favourable changes in the osteoprotegerin and receptor activator of nuclear factor kappa B levels were noted. DCE-MRI showed a reduction of 15-40% in MRI parameters. CONCLUSION: These results indicate that abatacept reduces the inflammatory status of the synovium without disrupting cellular homeostasis. The reductions in gene expression influence bone positively and suggest a basis for the recently demonstrated radiological improvements that have been seen with abatacept treatment in patients with RA.

The relationship between limited MRI section analyses and volumetric assessment of synovitis in knee osteoarthritis.

AIM: To assess whether simple, limited section analysis can replace detailed volumetric assessment of synovitis in patients with osteoarthritis (OA) of the knee using contrast-enhanced magnetic resonance imaging (MRI). MATERIALS AND METHODS: Thirty-five patients with clinical and radiographic OA of the knee were assessed for synovitis using gadolinium-enhanced MRI. The volume of enhancing synovium was quantitatively assessed in four anatomical sites (the medial and lateral parapatellar recesses, the intercondylar notch and the suprapatellar pouch) by summing the volumes of synovitis in consecutive sections. Four different combinations of section analysis were evaluated for their ability to predict total synovial volume. RESULTS: A total of 114 intra-articular sites were assessed. Simple linear regression demonstrated that the best predictor of total synovial volume was the analysis containing the inferior, mid and superior sections of each of the intra-articular sites, which predicted between 40-80% (r(2) = 0.396, p < 0.001 for notch; r(2) = 0.818, p < 0.001 for medial parapatellar recess) of the total volume assessment. CONCLUSIONS: The results suggest that a three-section analysis on axial post-gadolinium sequences provides a simple surrogate measure of synovial volume in OA knees.

The validation of simple scoring methods for evaluating compartment-specific synovitis detected by MRI in knee osteoarthritis.

OBJECTIVES: Synovitis is common in osteoarthritis (OA) of the knee. In order to evaluate its importance, valid and reliable quantification methods are required. The aim of this study was to compare simple, semiquantitative synovitis scores with detailed volume assessments in subjects with knee OA using magnetic resonance imaging (MRI) as the investigative tool. METHODS: Thirty-five subjects with clinically diagnosed OA of the knee were assessed for synovitis using gadolinium-enhanced MRI. The volume measurement of synovitis was assessed at four sites (medial and lateral parapatellar recesses, intercondylar notch and suprapatellar pouch). The semiquantitative scoring of synovitis was scored on a 0-3 scale. Establishment of a correlative relationship was undertaken using Spearman's rho for the individual site-specific measurements. Ordinal regression modelling was undertaken in order to establish the capacity of the synovitis scoring to predict the volume measurement. RESULTS: A total of 140 sites were assessed. There were good correlations between the semiquantitative scores and the volume measurements, ranging from rho = 0.86 (P < 0.00) for the medial parapatellar recess to rho = 0.71 (P < 0.00) for the lateral parapatellar recess. The ordinal regression suggested that, while the synovitis scores predicted between 50 and 70% (R2 = 0.53, P < 0.00 to R2 = 0.71, P < 0.00) of the volume measurements, there was some minor overlap, particularly in the mid-range synovitis scores. CONCLUSIONS: These results suggest that semiquantitative synovitis scores are valid and will enable feasible evaluation of the synovium in OA cohorts.

Further evidence that a cartilage-pannus junction synovitis predilection is not a specific feature of rheumatoid arthritis.

BACKGROUND: Qualitative differences in synovitis between the cartilage-pannus junction (CPJ) region and the adjoining suprapatellar pouch (SPP) have been reported in rheumatoid arthritis and the spondyloarthropathies. OBJECTIVE: To determine if the distribution of synovitis is the same in osteoarthritis (OA) using sensitive measures of inflammation derived from dynamic, contrast enhanced magnetic resonance imaging (DEMRI). METHODS: 20 subjects with established OA of the knee were recruited. Conventional MR images together with the DEMRI measurements were obtained. Areas of synovitis at the CPJ region and at a distant site in the SPP were calculated; differences in CPJ and SPP synovitis were determined using DEMRI parameters: the initial rate of contrast enhancement (IRE) and maximal enhancement (ME). RESULTS: The area of synovitis was significantly greater adjacent to the CPJ than in the SPP. IRE and ME measures were greater at the CPJ than the SPP. CONCLUSIONS: The magnitude of synovitis at the CPJ is not disease-specific and applies across the spectrum of degenerative disease as well as inflammatory diseases.

Effects of beta-adrenergic antagonists on the QT measurements from exercise stress tests in pediatric patients with long QT syndrome.

It has been proposed that beta-adrenergic antagonist protection against cardiac events in patients with long QT syndrome (LQTS) may be related to a decrease in baseline QTc dispersion. To determine the effects of beta-blocker therapy on QT measurements, we evaluated the exercise tests of 25 pediatric patients with LQTS. Measurements were made of the maximum QTc interval and QTc dispersion during the various segments of the exercise test. There was no statistically significant difference between the pre-beta-blocker and post-beta-blocker maximum QTc interval during the supine (0.473 +/- 0.039 vs 0.470 +/- 0.038 sec), exercise (0.488 +/- 0.044 vs 0.500 +/- 0.026 sec), or recovery (0.490 +/- 0.031 vs 0.493 +/- 0.029 sec) phases of the exercise stress test. There was also no statistically significant difference between the pre-beta-blocker and post-beta-blocker QTc dispersion during the supine (0.047 +/- 0.021 vs 0.058 +/- 0.033 exercise vs 0.063 +/- 0.028 sec), or recovery (0.045 +/- 0.023 vs 0.052 +/- 0.026 sec) phases of the exercise stress test. Therefore, the protection that beta-blockers offer appears not to be related to a reduction of the baseline QTc interval or a decrease of QTc dispersion.

Unusual presentation of long QT syndrome.

We describe the case of a 9-yr-old child with undiagnosed long QT syndrome who experienced an intraoperative cardiac arrest after accidental intravascular injection of bupivacaine with epinephrine via a misplaced epidural catheter.

Resolution of electrical storms after discontinuation of ICD therapy in a child with long QT syndrome.

We report a case of a child with familial long QT syndrome (Jervell Lange-Nielsen) who had multiple electrical storms in the presence of b blocker and implantable cardioverter device (ICD) therapy. Discontinuation of ICD therapy and addition of oral amiodarone to b blockade therapy resulted in freedom from electrical storms.

The incidence of arrhythmias in a pediatric cardiac intensive care unit.

A pediatric cardiac intensive care unit (CICU) manages critically ill children and adults with congenital or acquired heart disease. These patients are at increased risk for arrhythmias. The purpose of this study was to prospectively evaluate the incidence of arrhythmias in a pediatric CICU patient population. All patients admitted to the CICU at the Cardiac Center at The Children's Hospital of Philadelphia between December 1, 1997, and November 30, 1998, were evaluated prospectively from CICU admission to hospital discharge via full disclosure telemetry reviewed every 24 hours. Arrhythmias reviewed included nonsustained and sustained ventricular tachycardia (VT), nonsustained and sustained supraventricular tachycardia (SVT), atrial flutter and fibrillation, junctional ectopic tachycardia, and complete heart block. We reviewed 789 admissions consisting of 629 patients (age range, 1 day-45.5 years; median, 8.1 months). Hospital stay ranged from 1 to 155 days (total of 8116 patient days). Surgical interventions (n = 602) included 482 utilizing cardiopulmonary bypass. During the study period, there were 44 deaths [44/629 patients (7.0%)], none of which were directly attributable to a primary arrhythmia. The operative mortality was 5.1%. Overall, 29.0% of admissions had one or more arrhythmias the most common arrhythmia was nonsustained VT (18.0% of admissions), followed by nonsustained SVT (12.9% of admissions). Patients admitted to a pediatric CICU have a high incidence of arrhythmias, most likely associated with their underlying pathophysiology and to the breadth of medical and surgical interventions conducted.

Family physicians' personal experiences of their fathers' health care.

OBJECTIVES: The American health care system is complicated and can be difficult to navigate. The physician who observes the care of a family member has a uniquely informed perspective on this system. We hoped to gain insight into some of the shortcomings of the health care system from the personal experiences of physician family members. STUDY DESIGN: Using a key informant technique, we invited by E-mail any of the chairpersons of US academic departments of family medicine to describe their recent personal experiences with the health care system when their parent was seriously ill. In-depth semi-structured telephone interviews were conducted with each of the study participants. The interviews were transcribed, coded, and labeled for themes. POPULATION: Eight family physicians responded to the E-mail, and each was interviewed. These physicians had been in practice for an average of 19 years, were nationally distributed, and included both men and women. Each discussed their father's experience. RESULTS: All participants spoke of the importance of an advocate for their fathers who would coordinate medical care. These physicians witnessed various obstacles in their fathers's care, such as poor communication and fragmented care. As a result, many of them felt compelled to intervene in their fathers' care. The physicians expressed concern about the care their fathers received, believing that the system does not operate the way it should. CONCLUSIONS: Even patients with a knowledgeable physician family member face challenges in receiving optimal medical care. Patients might receive better care if health care systems reinforced the role of an accountable attending physician, encouraged continuity of care, and emphasized the value of knowing the patient as a person.

Role conflicts of physicians and their family members: rules but no rulebook.

OBJECTIVES: To elucidate the difficulties physicians have when a family member becomes ill and to elicit their underlying causes. DESIGN: Using a key informant technique, we solicited chairs of family medicine departments for their experiences with the health care provided to seriously ill family members. We then conducted in-depth, semistructured telephone interviews that were then transcribed, coded, and labeled for themes. SUBJECTS: 8 senior family physicians whose parents had experienced a serious illness within the past 5 years. All of the subjects reflected on experiences stemming from their fathers' illness. RESULTS: These physicians faced competing expectations: at an internal level, those of their ideal role in their family and their ideal professional identity; and at an external level, those originating from other family members and from other physicians. Reconciling these conflicting expectations was made more difficult by what they deemed to be suboptimal circumstances of the modern health care system. CONCLUSIONS: Conflicting rules of appropriate conduct, compounded by the inadequacies of modern health care, make the role of physician-family member especially challenging. The medical profession needs a clearer, more trenchant understanding of this role.

Arrhythmias after pediatric lung transplantation.

Atrial arrhythmias have been reported after congenital heart surgery involving extensive atrial suture lines. Experimental studies involving bilateral lung transplantation (Tx) suggest that the left atrial suture lines predispose to atrial flutter. The overall incidence and type of arrhythmias after pediatric lung Tx have not previously been described and therefore the purpose of this study was to prospectively screen and describe arrhythmias in a subset of our lung transplant population. Over a 1-yr study period, all recipients of bilateral lung Tx were admitted to a full-disclosure telemetry unit. Single-lead electrocardiograms were recorded continuously and reviewed daily via a beat-by-beat analysis. A total of 314 patient days (range 9-93, median 43 days) were recorded from seven patients. The incidence of arrhythmias observed per total patient days included junctional escape rhythm (4.8%), non-sustained ventricular tachycardia (4.1%), accelerated junctional (2.5%), sinus bradycardia (2.2%), non-sustained supraventricular tachycardia (1.3%), ectopic atrial tachycardia (1.0%), sustained ventricular tachycardia (0.3%), junctional ectopic tachycardia (0.3%), and second degree heart block (0.3%). No patient had sustained supraventricular tachycardia, atrial flutter, atrial fibrillation, or complete heart block. Arrhythmias were treated in two patients. During the follow-up period, one patient received amiodarone for ventricular tachycardia (which was also noted and treated prior to transplant). We conclude that among pediatric lung transplant recipients admitted for their transplant surgery, arrhythmia is uncommon and rarely requires therapy.

Atrioventricular node reentry tachycardia in pediatric patients.

Atrioventricular node reentry tachycardia (AVNRT) is a significant cause of paroxysmal supraventricular tachycardia (SVT) in the pediatric population. Symptoms can include palpitations, chest pain, fatigue, light-headedness and syncope. AVNRT is a reentry tachycardia that is comprised of dual conduction pathways through the AV node. On electrocardiogram, AVNRT usually manifests as a regular tachycardia with a narrow QRS complex and P waves that are either absent or distort the terminal portion of the QRS complex. Electrophysiology study will reveal dual AV node pathways: a fast pathway with a short AH interval and a long effective refractory period (ERP); and a slow pathway with a longer AH interval and a shorter ERP. During tachycardia, electrophysiologic signals will reveal conduction up the midline. Introduction of premature ventricular contractions and measurement of the HA interval during SVT can help distinguish AVNRT from a SVT utilizing an accessory pathway. Radiofrequency catheter ablation (RFA) has been used increasingly in children as treatment for AVNRT. The initial approach to RFA of AVNRT was modification of AV fast pathway conduction by lesions placed near the anterosuperior aspect of the triangle of Koch, known as the anterior approach method. However, this technique was associated with a significant risk of complete AV block. Now, the posterior approach slow pathway modification is used more commonly, which positions the ablation catheter along the tricuspid annulus immediately anterior to the coronary sinus ostium. This has been associated with a lower risk of complete AV block. Using this technique, RFA should be considered the method of choice for curative therapy of AVNRT in pediatric patients.

Epicardial atrial pacemaker lead placement after multiple cardiac operations.

We describe a simple technique for the implantation of left atrial epicardial pacing leads in children with congenital heart disease who have undergone multiple operations. The pulmonary veins are exposed to reveal the pulmonary venous to atrial confluence using a left thoracotomy. A pacemaker lead is secured to the posterior left atrium inferior to the lower pulmonary vein. This approach provides a reliable site for atrial lead placement without the need for extensive dissection.

Permanent epicardial pacing in pediatric patients: seventeen years of experience and 1200 outpatient visits.

BACKGROUND: The purpose of this study was to evaluate the long-term outcome of all pediatric epicardial pacing leads. METHODS AND RESULTS: All epicardial leads and 1239 outpatient visits between January 1, 1983, and June 30, 2000, were retrospectively reviewed. Pacing and sensing thresholds were reviewed at implant, at 1 month, and at subsequent 6-month intervals. Lead failure was defined as the need for replacement or abandonment due to pacing or sensing problems, lead fracture, or phrenic/muscle stimulation. A total of 123 patients underwent 207 epicardial lead (60 atrial/147 ventricular, 40% steroid) implantations (median age at implant was 4.1 years [range 1 day to 21 years]). Congenital heart disease was present in 103 (84%) of the patients. Epicardial leads were followed for 29 months (range 1 to 207 months). The 1-, 2-, and 5-year lead survival was 96%, 90%, and 74%, respectively. Compared with conventional epicardial leads, both atrial and ventricular steroid leads had better stimulation thresholds 1 month after implantation; however, only ventricular steroid leads had improved chronic pacing thresholds (at 2 years: for steroid leads, 1.9 muJ [from 0.26 to 16 mu]; for nonsteroid leads, 4.7 muJ [from 0.6 to 25 muJ]; P<0.01). Ventricular sensing was significantly better in steroid leads 1 month after lead implantation (at 2 years: for steroid leads, 8 mV [from 4 to 31 mV]; for nonsteroid leads, 4 mV [from 0.7 to 10 mV]; P<0.01). Neither congenital heart disease, lead implantation with a concomitant cardiac operation, age or weight at implantation, nor the chamber paced was predictive of lead failure. CONCLUSIONS: Steroid epicardial leads demonstrated relatively stable acute and chronic pacing and sensing thresholds. In this evaluation of >200 epicardial leads, lead survival was good, with steroid-eluting leads demonstrating results similar to those found with historical conventional endocardial leads.

Physicians' responses to patients' requests for physician-assisted suicide.

BACKGROUND: Studies show that patient requests for physician-assisted suicide (PAS) are a relatively common clinical occurrence. The purpose of this study was to describe how experienced physicians assess and respond to requests for assisted suicide. METHODS: Focused ethnography in the offices of 11 acquired immunodeficiency syndrome physicians, 8 oncologists, and 1 hospice physician who had received requests for assisted suicide in their practice. Ten had facilitated PAS. RESULTS: Informants had a similar approach to evaluating patients who requested assisted suicide, often asking, "Why do you want to die now?" Reasons for requests fell into 3 broad categories: physical symptoms, psychological issues, and existential suffering. Physicians thought they competently addressed patients' physical symptoms, and this obviated most requests. They treated depression empirically and believed they did not assist depressed patients with assisted suicide. Physicians had difficulty addressing patients' existential suffering, which led to most facilitated requests. Informants rarely talked to colleagues about requests for assisted suicide, suggesting a "professional code of silence." CONCLUSIONS: Regardless of divergent attitudes about PAS, physicians respond similarly to requests for assisted suicide from their patients, creating a common ground for professional dialogue. Our sample addressed physical suffering aggressively, treated depression empirically, but struggled with requests arising from existential suffering. A professional code of silence regarding PAS creates professional isolation. Clinicians do not share knowledge or receive social support from peers about their decisions regarding assisted suicide. Educational strategies drawing on approaches used by experienced clinicians may create an atmosphere that enables physicians with divergent beliefs to discuss this difficult subject.

Fetal and neonatal arrhythmias.

Perinatal arrhythmias may occur either during fetal life or in the early neonatal period. These arrhythmias include both tachycardias and bradycardias. This article presents a brief overview of fetal and neonatal arrhythmias concentrating on their presentation, diagnosis, and treatment.

Epicardial pacemaker implantation and follow-up in patients with a single ventricle after the Fontan operation.

OBJECTIVES: There is an increasing incidence of sinus node dysfunction after the Fontan procedure. Inability to maintain atrioventricular synchrony after the Fontan operation has been associated with an adverse late outcome. Although pacing may be helpful as a primary or adjunct modality after the Fontan procedure, the effects of performing a late thoracotomy or sternotomy for epicardial pacemaker implantation are unknown. In addition, little is known about the long-term effectiveness of epicardial leads in patients with single ventricles. The purpose of this study was to compare the hospital course and follow-up of epicardial pacing lead implantation in patients with Fontan physiology and patients with 2-ventricle physiology. METHODS: We retrospectively reviewed all isolated epicardial pacemaker implantations and outpatient evaluations performed between January 1983 and June 2000. RESULTS: There was no difference in the perioperative course for the 31 Fontan patients (27 atrial and 41 ventricular leads [68 total]) compared with the 56 non-Fontan subjects (9 atrial and 61 ventricular leads [70 total]). The median length of stay in Fontan and non-Fontan patients was 3 and 4 days, respectively. There was no early mortality in either group. Pleural drainage for 5 days or longer was reported in 4% of the Fontan cohort and 3% of the non-Fontan group. Late pleural effusions were identified in only 2 patients in the Fontan group and 2 patients in the non-Fontan group. There was no significant difference in epicardial lead survival between the Fontan group and the non-Fontan group (1 year, 96%; 2 years, 90%; 5 years, 70%). The overall incidence of lead failure was 17% (24/138). CONCLUSIONS: Epicardial leads can be safely placed in Fontan patients at no additional risk compared to patients with biventricular physiology. Sensing and pacing qualities were relatively constant in both the Fontan and non-Fontan groups over the first 2 years after implantation.

Modifications to the cavopulmonary anastomosis do not eliminate early sinus node dysfunction.

OBJECTIVE: To determine whether operations that theoretically jeopardize the sinus node (hemi-Fontan and/or lateral tunnel Fontan procedures) are associated with a greater risk of sinus node dysfunction than those that theoretically spare the sinus node (bidirectional Glenn and/or extracardiac conduit). METHODS: Between January 1, 1996, and December 31, 1999, a prospective cohort study was conducted evaluating the incidence of sinus node dysfunction in patients undergoing a bidirectional Glenn or hemi-Fontan procedure and those in whom the Fontan repair was completed with either an extracardiac conduit or a lateral tunnel. Sinus node dysfunction was defined (1) as a heart rate more than 2 SD below age-adjusted norms or (2) as a predominant junctional rhythm and/or a sinus pause of more than 3 seconds as determined by the resting electrocardiogram and/or ambulatory monitoring at hospital discharge. RESULTS: Fifty-one patients had a bidirectional Glenn shunt (mean age 7.8 +/- 5.1 months) and 79 a hemi-Fontan procedure (mean age 6.9 +/- 2.8 months). The incidence of sinus node dysfunction on postoperative day 1 was significantly higher after the hemi-Fontan (36%) than after the bidirectional Glenn shunt (9.8%); however, by hospital discharge this difference was no longer apparent (hemi-Fontan [8%]; bidirectional Glenn [6%]; P = not significant). No difference in early sinus node dysfunction was discernible after the extracardiac conduit (4/30 [13%]) compared with the lateral tunnel Fontan procedure (6/46 [13%]) (P = not significant). No diagnostic or perioperative variables were predictive of sinus node dysfunction. CONCLUSIONS: Avoidance of surgery near the sinus node has no discernible effect on the development of early sinus node dysfunction. Thus, concerns about early sinus node dysfunction should not override patient anatomy or surgeon preference as determinants of which cavopulmonary anastomosis to perform.