Long-term outcome of percutaneous intervention for pulmonary vein stenosis after pulmonary vein isolation procedure.

OBJECTIVES: Report long-term outcomes of percutaneous intervention in patients with pulmonary vein stenosis (PVS) after pulmonary vein isolation (PVI) from a single center over 16 years. BACKGROUND: Outcome reports of percutaneous intervention for PVS resulting from PVI are limited. METHODS: Retrospective review of all patients with PVS after PVI who underwent percutaneous intervention at the Cleveland Clinic Foundation between January 2000 and December 2016. RESULTS: A total of 205 patients underwent cardiac catheterization for PVS during the study period. Completely occluded veins which could not be recanalized occurred in six patients. Of the remaining 199 patients, 27 (14%) were lost to follow-up, leaving 172 patients with 276 veins for analysis. Balloon angioplasty was performed in 62 veins and stent implantation in 250 (primary in 214, to treat postdilation restenosis in 36). Re-intervention occurred in 45/62 (73%) balloon-dilated veins and 45/250 (18%) stented veins. Freedom from re-intervention at 1 and 5 years was 90 and 73% following stenting versus 40 and 23% following balloon dilation (p < .001, Hazard ratio (HR) = 5.7). Veins with stent diameter ≥7 mm (n = 231) had greater freedom from re-intervention (95% at 1 year, 79% at 5 years) than veins with stents <7 mm (43% at 1 year, 9% at 5 years), p < .001. There was clear symptomatic improvement after intervention and no procedural mortality. CONCLUSIONS: Stent implantation at ≥7 mm for PVS after PVI is associated with low rates of re-intervention, in contrast to balloon dilation and stenting with small conventional stents.

Low Indeterminate Rates Associated With Use of the QuantiFERON-TB Gold In-Tube Test in Children With Inflammatory Bowel Disease on Long-term Infliximab.

Background: Tumor necrosis factor alpha (TNF-α) inhibitors are linked with increased risk of reactivation of active tuberculosis. The QuantiFERON-TB Gold In-Tube test is approved for screening latent tuberculosis infection in children and adults. There are limited data on the test performance in children on long-term treatment with TNF-α inhibitors. The objective of this study was to assess the proportion of indeterminate results for the QuantiFERON-TB Gold In-Tube in children with inflammatory bowel disease (IBD) on long-term infliximab treatment and to evaluate the range of interferon-γ responses to mitogen. Methods: A single-center prospective study of children 5 to 19 years of age with IBD on long-term infliximab treatment (>3 months). Each child was assessed for tuberculosis exposure risk and had blood drawn for the QuantiFERON-TB Gold In-Tube. Data on the range of interferon-γ responses and final QuantiFERON-TB Gold In-Tube test results were collected. Results: Ninety-three children were included, with a median age of 16 years. The median total duration of infliximab therapy was 34 months (range, 3-119 months). The QuantiFERON-TB Gold In-Tube was indeterminate in 1 patient (1.1%), positive in 2 patients, and negative in 90 patients. The maximum interferon-γ response to mitogen (10 IU/mL) was observed in 82 patients (88%), with only 1 patient having an inadequate response. The proportion of indeterminate results was significantly lower than the prospectively hypothesized rate of 8%, based on prior studies in nonimmunosuppressed patients (P = 0.004). Conclusions: Pediatric patients with IBD on long-term treatment with infliximab had an adequate interferon-γ response to mitogen and a low indeterminate rate when assessed with the QuantiFERON-TB Gold In-Tube test. This study demonstrates a robust interferon gamma response to phytohemagglutinin stimulation in a pediatric population on long-term therapy with infliximab. The QuantiFERON-TB Gold In-Tube test may therefore be useful as a periodic screening tactic for latent TB in children on long-term infliximab therapy.

Long-term (5- to 20-year) outcomes after transcatheter or surgical treatment of hemodynamically significant isolated secundum atrial septal defect.

Truly long-term follow-up data after transcatheter closure (TC) of atrial septal defects (ASDs) are scarce. We report the 5- to 20-year outcomes of TC and surgical closure (SC) for typical secundum ASD. We reviewed the records of patients with isolated secundum ASD and right ventricular volume overload who underwent TC or SC (January 1, 1986 to September 30, 2005). Follow-up was obtained through a combination of chart review, physician records, and telephone survey. We identified 375 patients (207 SC and 168 TC) and obtained follow-up data >5 years (median follow-up 10 years) for 300 (152 SC, 148 TC). Nine patients have died (3%). The New York Heart Association functional class was unchanged in 227 patients, improved in 25, and was worse in 15. Clinically significant arrhythmia was found in 28 patients (9.3%); 21% aged >40 years developed arrhythmia. On multivariate analysis, the odds of significant arrhythmia tended to be greater in the SC group, but this was statistically insignificant (95% confidence interval 0.68 to 3.9, p = 0.27). Age and preprocedure arrhythmia, but not TC or SC, were independent risk factors for late arrhythmia (p <0.001). No difference was found in the incidence of late, probably embolic, stroke in the TC (3%) versus SC (2%) groups. In conclusion, long-term outcomes after secundum ASD closure using modern methods are excellent. No significant differences were found between TC versus SC with regard to survival, functional capacity, atrial arrhythmias, or embolic neurologic events. Arrhythmia and neurologic events remain long-term risks after ASD closure, especially if the patient had pre-existing arrhythmia.

Survival rates of children with acute lymphoblastic leukemia presenting to a pediatric rheumatologist in the United States.

BACKGROUND: Approximately 30% of pediatric acute lymphoblastic leukemia patients present with musculoskeletal symptoms and are often referred first to a pediatric rheumatologist. We examined the survival and causes of death of these patients presenting to a pediatric rheumatologist and compared the rates with that reported in the hematology-oncology literature. PROCEDURE: We used the Pediatric Rheumatology Disease Registry, including 49,023 patients from 62 centers, newly diagnosed between 1992 and 2001. Identifiers were matched with the Social Security Death Index censored for March 2005. Deaths were confirmed by death certificates, referring physicians, and medical records. Causes of death were derived by chart review or from the death certificate. RESULTS: There were 7 deaths of 89 patients (7.9%, 95% confidence interval: 3.9%-15.4%) with acute lymphoblastic leukemia with a 5-year survival rate of 95.5% (88.3 to 98.3) and 10-year survival rate of 89.8% (79.0% to 95.2%). The causes of death were sepsis (bacterial and/or fungal) in 4 (57%) patients, the disease in 2 (29%) and post bone-marrow transplantation in 1 (14%). CONCLUSION: The overall survival of patients with acute lymphoblastic leukemia seen first by pediatric rheumatologists is higher than the range reported in the pediatric oncology literature for the same period of diagnosis.

The acceptability of human papillomavirus vaccine among parents and guardians of newborn to 10-year-old children.

STUDY OBJECTIVE: The purpose of our study was to evaluate HPV vaccine acceptance among parents and guardians of children aged 0-10 years. DESIGN: Prospective questionnaire study. SETTING: Cleveland Clinic Children's Hospital. PARTICIPANTS: Parents and guardians of children aged 0-10 years. INTERVENTIONS: Brief HPV vaccine educational intervention. OUTCOME MEASURE: Desire for child to get HPV vaccine. RESULTS: We enrolled 81 participants in the study; 70 (86%) were female, and 39 (49%) were Caucasian. Prior to receiving an educational fact sheet about HPV and the HPV vaccine, only 49% of participants reported that they wanted their young child to receive the HPV vaccine when it becomes available. After receiving the fact sheet, this number increased to 70%, suggesting that a simple educational intervention could significantly affect vaccine acceptance in this population (P = .001). Other significant results of this study included that HPV vaccination would receive greater acceptance if the participants believed that it can prevent HPV infection in their child (P = .0024), it was perceived to be safe (P = .0005), and if the vaccine were recommended by a physician (P < .0001). Participants' attitudes about HPV vaccination were not affected by concerns over whether receiving the vaccine might mean the child is more likely to have sex or to have multiple sexual partners. CONCLUSIONS: Our results suggest that if it were approved for children aged 0-10 years, the HPV vaccine would be accepted by the parents and guardians provided they received adequate educational information about it.

A clinical picture of Guillain-Barré syndrome in children in the United States.

The authors describe the demographics, clinical presentation, investigation, treatment, and outcomes of pediatric patients with Guillain-Barré syndrome. They identified 35 pediatric patients with Guillain-Barré syndrome presenting to a tertiary academic center over a 20-year period. The most common presenting symptoms were paresthesias (54%), weakness (49%), and myalgias (49%). Sensation was affected in 54% of patients, and hyporeflexia or areflexia was present in 94% of patients. Cranial nerve dysfunction (46%) and autonomic involvement (eg, changes in blood pressure, pulse, bowel/bladder control, or priapism; 46%) were also common. Autonomic dysfunction, cranial nerve involvement, and albuminocytological dissociation were significantly associated with a decreased time to nadir, the point when symptoms peaked (P = .015, .007, and .005, respectively). Although not statistically significant, treatment with plasmapheresis had a better success rate than intravenous immunoglobulin. The authors' results will help to further delineate the clinical picture of Guillain-Barré syndrome in children and refine treatment strategies.

Total pulmonary vein occlusion complicating pulmonary vein isolation: diagnosis and treatment.

BACKGROUND: Pulmonary vein stenosis (PVS) complicating pulmonary vein isolation (PVI) can progress to total pulmonary vein occlusion (PVO). Little is known about the accuracy of noninvasive diagnosis and treatment of PVO. OBJECTIVE: The purpose of this study was to study the diagnostic accuracy of noninvasive testing and the feasibility and outcome of percutaneous intervention for PVO. METHODS: Computed tomography (CT)-diagnosed and angiographically confirmed PVOs were identified from percutaneous interventions for PVS complicating PVI between December 2000 and December 2008. Diagnostic accuracy of CT combined with lung perfusion scan was studied. Outcome of percutaneous intervention was reviewed. RESULTS: CT diagnosed "PVO" in 53 PVs, with only 20 of 53 determined angiographically to be totally occluded. True PVO had lower perfusion (4.0%) compared with CT-diagnosed "PVO" (7.3%, P = .024). Recanalization was attempted in 9 and successful in 8. Of the 8 patients, 7 were dilated with 4.5- to 7-mm balloons and 1 was stented primarily (7 mm). At repeat catheterization 2.9 +/- 0.8 months later, 6 of 7 pulmonary veins (PVs) were stented to 5 to 10 mm. At follow-up of 11.3 +/- 8.7 months, all but 1 PV remained patent (mean diameter 6.9 +/- 1.7 mm). Flow to the lung quadrant increased from 5.6% before recanalization to 12.2% at last follow-up (P = .016). Symptoms improved in all but one patient. CONCLUSION: PVO is overestimated by CT. Quantification of lung perfusion improves diagnostic accuracy, but angiography remains the gold standard. Recanalization of PVO can be attempted when a remnant of the PV is visible. Good mid-term patency rates and improved perfusion were observed with a two-stage approach of initial dilation and subsequent stenting. Longer follow-up and larger numbers of patients are needed to better understand when to intervene for PVO.

Mortality outcomes in pediatric rheumatology in the US.

OBJECTIVE: To describe mortality rates, causes of death, and potential mortality risk factors in pediatric rheumatic diseases in the US. METHODS: We used the Indianapolis Pediatric Rheumatology Disease Registry, which includes 49,023 patients from 62 centers who were newly diagnosed between 1992 and 2001. Identifiers were matched with the Social Security Death Index censored for March 2005. Deaths were confirmed by death certificates, referring physicians, and medical records. Causes of death were derived by chart review or from the death certificate. Standardized mortality ratios (SMRs) and 95% confidence intervals (95% CIs) were determined. RESULTS: After excluding patients with malignancy, 110 deaths among 48,885 patients (0.23%) were confirmed. Patients had been followed up for a mean +/- SD of 7.9 +/- 2.7 years. The SMR of the entire cohort was significantly decreased (0.65 [95% CI 0.53-0.78]), with differences in patients followed up for > or =9 years. The SMR was significantly greater for systemic lupus erythematosus (3.06 [95% CI 1.78-4.90]) and dermatomyositis (2.64 [95% CI 0.86-6.17]) but not for systemic juvenile rheumatoid arthritis (1.8 [95% CI 0.66-3.92]). The SMR was significantly decreased in pain syndromes (0.41 [95% CI 0.21-0.72]). Causes of death were related to the rheumatic diagnosis (including complications) in 39 patients (35%), treatment complications in 11 (10%), non-natural causes in 25 (23%), background disease in 23 (21%), and were unknown in 12 patients (11%). Rheumatic diagnoses, age at diagnosis, sex, and early use of systemic steroids and methotrexate were significantly associated with the risk of death. CONCLUSION: Our findings indicate that the overall mortality rate for pediatric rheumatic diseases was not increased. Even for the diseases and conditions associated with increased mortality, mortality rates were significantly lower than those reported in previous studies.

Comparison of stent versus balloon angioplasty for pulmonary vein stenosis complicating pulmonary vein isolation.

INTRODUCTION: Pulmonary vein stenosis (PVS) is a rare but significant complication of pulmonary vein isolation (PVI). Dilation and stent angioplasty have been described but not compared. METHODS AND RESULTS: All percutaneous interventions for PVS complicating PVI between December 2000 and March 2007 were reviewed. Acute success, defined as post-intervention stenosis or=10 mm in diameter had lower restenosis than smaller stents. Risk factors for restenosis included small reference vessel diameter and longer time from PVI to intervention for PVS. All but two patients experienced improvement (n = 10) or resolution of symptoms (n = 22). The mean percent stenosis decreased from 82% to 21% for the entire cohort and mean flow to the lung quadrant increased from 10% to 17%. CONCLUSION: Stent angioplasty results in less restenosis than dilation, particularly for stents >or=10 mm. Early referral may improve long-term patency by minimizing reference vessel atrophy. Most patients with PVS post-PVI can be improved symptomatically with catheter intervention.

Differential growth of human embryos in vitro: role of reactive oxygen species.

OBJECTIVE: To examine the relationship of early human embryonic development with the level of reactive oxygen species (ROS) in the culture media on the first day (day 1 ROS) after insemination. DESIGN: A prospective study. SETTING: Patients undergoing assisted reproduction in a teaching hospital. PATIENT(S): Patients undergoing conventional IVF (n = 104; 115 cycles) and intracytoplasmic sperm injection (ICSI) (n = 91; 96 cycles) were included. Both fertilization and early cultures were performed in human tubal fluid with 5% serum substitute supplement. INTERVENTION(S): Day 1 ROS levels in the central well (sample) and the outer well (control) of each embryo culture dish were measured after overnight incubation by chemiluminescence assay using luminol as the probe. MAIN OUTCOME MEASURE(S): Fertilization rate and embryo quality at day 3 and 5 were recorded for each cycle. Age, parity, and demographic features were also compared. RESULT(S): High day 1 ROS levels in culture media were associated with low blastocyst rate, low fertilization rate, low cleavage rate, and high embryonic fragmentation with ICSI but not with conventional IVF. High day 1 ROS levels in culture media were associated with lower pregnancy rates in both IVF and ICSI cycles. CONCLUSION(S): Reactive oxygen species generated in culture media by day 1 may be an important biochemical marker for early embryonic growth. Increased embryonic fragmentation and slow cleavage rate may be partially attributed to early exposure of embryos to high ROS levels in ICSI cycles. Differential growth of ICSI embryos incubated under identical conditions may be in part due to differences in ROS levels of the culture medium surrounding these embryos.

Mechanical failure of the American Medical Systems Ultrex inflatable penile prosthesis: before and after 1993 structural modification.

PURPOSE: The 700 Ultrex (American Medical Systems, Minnetonka, Minnesota) is the only penile prosthesis capable of length and girth expansion. Early experience with the 700 Ultrex showed an increased mechanical failure rate compared with the 700CX, mostly secondary to cylinder failure. In 1993 the Ultrex cylinders were modified. We examined the performance of the Ultrex device before and after modification. MATERIALS AND METHODS: We compared our results with the Ultrex prosthesis before (group 1) and after (group 2) the 1993 modification. We implanted 239 devices from October 1989 to December 1999. A total of 26 patients have died. Followup was obtained on the results of 137 of the remaining 213 implants (64%), including 85 pre-modification devices in 85 patients and 52 post-modification devices in 51, via a mailed questionnaire, telephone survey or chart review. The questionnaire and survey included a 5-point satisfaction scale. Groups 1 and 2 were compared in regard to 3 end points, namely cylinder, mechanical and overall failure. RESULTS: Followup was less than 1 to 136 months (median 92, 25th to 75th percentiles 43 to 108) in group 1 and less than 1 to 92 months (median 46, 25th to 75th percentiles 21 to 75) in group 2. The 5-year Kaplan-Meier estimates of overall, mechanical and cylinder survival in groups 1 and 2 were 64.7%, 70.7% and 80.2%, and 77.7% (p = 0.23), 93.7% (p = 0.017) and 96.2% (p = 0.008), respectively. Overall satisfaction was similarly high in groups 1 and 2 (mean 3.9 and 4 points). CONCLUSIONS: On long-term followup the 1993 modification of the Ultrex cylinders appears to have significantly decreased the propensity of cylinder failure of the pre-modification device.