Resimmune, an anti-CD3ε recombinant immunotoxin, induces durable remissions in patients with cutaneous T-cell lymphoma.

Resimmune is a second-generation recombinant immunotoxin composed of the catalytic and translocation domains of diphtheria toxin fused to two single chain antibody fragments reactive with the extracellular domain of CD3ε. We gave intravenous infusions of Resimmune 2.5 - 11.25 µg/kg over 15 minutes to 30 patients (25 with cutaneous T-cell lymphoma, 3 with peripheral T-cell lymphoma, 1 with T-cell large granular lymphocytic leukemia and 1 with T-cell prolymphocytic leukemia) in an inter-patient dose escalation trial. The most common adverse events were fever, chills, hypotension, edema, hypoalbuminemia, hypophosphatemia, and transaminasemia. Among the 25 patients with cutaneous T-cell lymphoma, there were nine responses for a response rate of 36% (95% CI, 18%-57%) including four complete remissions (16%, 95% CI, 5%-36%). The durations of the complete remissions were 72+, 72+, 60+ and 38+ months. There were five partial remissions lasting 3, 3, 3+, 6+ and 14 months. Of 17 patients with a modified skin weighted assessment tool score <50, 17 patients with stage IB/IIB, and 11 patients with both a score <50 and stage IB/IIB, nine (53%), eight (47%), and eight (73%) had responses, respectively. Further studies of Resimmune in patients with low tumor burden, stage IB-IIB cutaneous T-cell lymphoma are warranted. This trial is registered at clinicaltrials.gov as #NCT00611208.

Eversion for Stabilization: A Standardized Technique for Pulmonary Autograft Inclusion.

We have developed a new technique avoiding autograft distortion and narrowing at inclusion during Ross procedure, in order to preserve the functional anatomy and the process of adaptation.

Modeling the distribution of the endangered Jemez Mountains salamander (Plethodon neomexicanus) in relation to geology, topography, and climate.

The Jemez Mountains salamander (Plethodon neomexicanus; hereafter JMS) is an endangered salamander restricted to the Jemez Mountains in north-central New Mexico, United States. This strictly terrestrial and lungless species requires moist surface conditions for activities such as mating and foraging. Threats to its current habitat include fire suppression and ensuing severe fires, changes in forest composition, habitat fragmentation, and climate change. Forest composition changes resulting from reduced fire frequency and increased tree density suggest that its current aboveground habitat does not mirror its historically successful habitat regime. However, because of its limited habitat area and underground behavior, we hypothesized that geology and topography might play a significant role in the current distribution of the salamander. We modeled the distribution of the JMS using a machine learning algorithm to assess how geology, topography, and climate variables influence its distribution. The best habitat suitability model indicates that geology type and maximum winter temperature (November to March) were most important in predicting the distribution of the salamander (23.5% and 50.3% permutation importance, respectively). Minimum winter temperature was also an important variable (21.4%), suggesting this also plays a role in salamander habitat. Our habitat suitability map reveals low uncertainty in model predictions, and we found slight discrepancies between the designated critical habitat and the most suitable areas for the JMS. Because geological features are important to its distribution, we recommend that geological and topographical data are considered, both during survey design and in the description of localities of JMS records once detected.

Risk factors for valve-related complications after mechanical heart valve replacement in 505 patients with long-term follow up.

BACKGROUND AND AIM OF THE STUDY: Currently, valve thrombosis, thromboembolic events and bleeding events account for 75% of all complications that occur after mechanical heart valve replacement. The study aim was to determine the main risk factors for valve-related complications in patients undergoing mechanical heart valve replacement. METHODS: Data were available from the systematic follow up of patients who had received a CarboMedics bileaflet mechanical heart valve replacement at the authors' institution. Follow up examinations were conducted prospectively at two-year intervals, via questionnaires sent to the patients' general practitioners, or by telephone calls. RESULTS: Between January 1988 and December 2005, a total of 505 consecutive patients (300 males, 205 females; mean age 52 years; range: 5 to 77 years) underwent heart valve replacement using a CarboMedics mechanical prosthesis. Aortic valve replacement (AVR) was performed in 308 patients, mitral valve replacement (MVR) in 134 patients, and double-valve replacement (DVR) in 62 patients. The follow up was 95.4% complete; the mean follow up was 7.5 years, and the total follow up 3,718 patient-years. Thromboembolic and bleeding complications represented the leading cause of valve-related events (104/195), of valve-related mortality (15/25), and of the need for repeat surgery (9/16). Valve thrombosis occurred in 12 patients. Implantation in the mitral position was identified as a risk factor (HR = 15.07; CI: 8.41-23.07; p 0.0001). Thromboembolism occurred in 32 patients; the use of antiplatelet agents was found to be a protective factor (HR = 0.23; CI: 0.08-0.70; p = 0.01). Bleeding events occurred in 52 patients; risk factors for bleeding events included a history of thromboembolic or bleeding complications (HR = 2.70; CI: 1.33-5.26; p = 0.006) and an unstable International Normalized Ratio (INR) (HR = 2.86; CI: 1.01-8.08; p = 0.05). CONCLUSION: After mechanical heart valve replacement, the only risk factors for bleeding complications were an unstable INR and a history of thromboembolic or bleeding events. The use of antiplatelet agents proved to be a protective factor against thromboembolic events.

Reconstruction of the aortic valve leaflet with autologous pulmonary artery wall.

Leaflet reconstruction outcomes in young patients can be compromised by treated autologous pericardium utilization. We present a new and simple unicuspid/unicommissural aortic valve repair technique with an autologous pulmonary artery wall graft. With comparative and longitudinal follow-up studies, this technique could constitute a living reconstruction of the aortic valve that does not preclude a future Ross procedure.

Outcome analysis of a conservative approach to diaphragmatic paralysis following congenital cardiac surgery in neonates and infants: a bicentric retrospective study.

OBJECTIVES: Diaphragmatic paralysis following congenital cardiac surgery is associated with significant morbidity and mortality. Spontaneous recovery of diaphragmatic function has been described, contrasting with centres providing early diaphragmatic plication. We aimed to describe the outcomes of a conservative approach, as well as to identify factors associated with a failure of the strategy. METHODS: This is a retrospective study of patients admitted after cardiac surgery and suffering unilateral diaphragmatic paralysis within 2 French Paediatric Cardiac Surgery Centers. The conservative approach, defined by the prolonged use of ventilation until successful weaning from respiratory support, was the primary strategy adopted in both centres. In case of unsuccessful evolution, a diaphragmatic plication was scheduled. Total ventilation time included invasive and non-invasive ventilation. Diaphragm asymmetry was defined by the number of posterior rib segments counted between the 2 hemi-diaphragms on the chest X-ray after cardiac surgery. RESULTS: Fifty-one neonates and infants were included in the analysis. Patients' median age was 12.0 days at cardiac surgery (5.0-82.0), and median weight was 3.5 kg (2.8-4.9). The conservative approach was successful for 32/51 patients (63%), whereas 19/51 patients (37%) needed diaphragm plication. There was no difference in patients' characteristics between groups. Respiratory support prolonged for 21 days or more and diaphragm asymmetry more than 2 rib segments were independently associated with the failure of the conservative strategy [odds ratio (OR) 6.9 (1.29-37.3); P = 0.024 and OR 6.0 (1.4-24.7); P = 0.013, respectively]. CONCLUSIONS: The conservative approach was successful for 63% of the patients. We identified risk factors associated with the strategy's failure.

Long-term evolution of stents implanted in branch pulmonary arteries.

BACKGROUND: Branch pulmonary artery stenosis complicates the management of congenital heart diseases. Surgical branch pulmonary artery angioplasty is associated with a high reintervention rate. As an alternative, percutaneous or intraoperative branch pulmonary artery stents have been implanted to improve efficiency, but long-term evaluations are limited. AIM: To describe the long-term evolution of branch pulmonary artery stents. METHODS: We conducted a retrospective cohort study at Tours University Hospital. All stents implanted by surgery or catheterization in branch pulmonary arteries with a minimum follow-up of 12 months and at least one catheterization control were included. The primary endpoint combined cardiovascular mortality, surgical or percutaneous reintervention for stent complication or new stent implantation. RESULTS: Between 2007 and 2017, 76 stents in 51 patients were included (62 stents implanted by surgery, 14 by catheterization). At implantation, the patients' mean age and weight were 4.7years (interquartile range 4.2years) and 17.3kg (interquartile range 11.0kg), respectively. Mean branch pulmonary artery minimum diameter was 4.1±2.1mm (mean Z-score-4.9±2.9), and mean initial stent diameter was 9.1±3.1mm. During a follow-up of 5.3years (range 0-11.2 years), freedom from primary endpoint was 86.8% (95% confidence interval 79.6-94.8%) at 1 year, 71.5% (95% confidence interval 61.9-82.7%) at 5years and 69.6% (95% confidence interval 59.6-81.2%) at 10 years. We did not identify any factors associated with major adverse cardiovascular events. Among stents without major adverse cardiovascular events, the mean branch pulmonary artery diameter Z-score at last evaluation had increased by +4.8±3.2 compared with the initial diameter (P<0.001). After stent implantation, a median of 2 re-expansions were performed for each stent (range 0-7). CONCLUSIONS: Stent implantation should offer a good long-term solution for branch pulmonary artery stenosis, although iterative re-expansions are required.

Immediate and midterm results of balloon angioplasty for recurrent aortic coarctation in children aged<1 year.

BACKGROUND: Several publications have considered results of percutaneous angioplasty for aortic recoarctation, but none focused on procedures performed in children aged<1 year. AIMS: To describe the immediate and midterm results of balloon angioplasty for recoarctation before the age of 1 year, and to define the factors that might influence outcome. METHODS: We retrospectively reviewed data from 20 consecutive children undergoing percutaneous dilatation for aortic recoarctation before the age of 1 year in the University Hospitals of Tours and Nantes. RESULTS: In all patients except one, dilatation improved the median recoarctation diameter Z-score from -5.5 (range -10.6 to -2.5) to -2.8 (range -4.3 to 0.7) (P<0.001), and reduced the median peak systolic gradient from 33mmHg (range 20 to 60mmHg) to 21mmHg (range 6 to 50mmHg) (P<0.001). There was no procedure-induced mortality and no acute intimal flap or long-term aneurysm. Three patients experienced a transient femoral artery thrombosis, one of whom had a transient ischemic stroke. Eight children (40%) needed reintervention for further recoarctation (new surgery [n=4] or new dilatation [n=4]). A smaller balloon size was significantly associated with the risk of reintervention: balloon to recoarctation diameter ratio 2.0 (range 1.3 to 3.3) vs. 2.7 (range 2.1 to 4.5) (P=0.05); balloon to descending aorta ratio 0.8 (range 0.7 to 1.2) vs. 1.0 (range 0.9 to 1.3) (P<0.05). CONCLUSIONS: In this study, percutaneous balloon angioplasty for recoarctation in young infants aged<1 year improved aortic isthmus diameter with a low incidence of adverse event. However, the rate of further intervention is high, and is associated with a smaller balloon size.

Intraoperative Stenting of Pulmonary Artery Stenosis in Children With Congenital Heart Disease.

BACKGROUND: Branch pulmonary artery (BPA) stenosis is frequently associated with congenital heart disease. Management of BPA stenosis is challenging for surgeons due to a high rate of recurrence. The purpose of this study was to assess the results of intraoperative pulmonary artery stenting associated with or without surgical angioplasty. METHODS: We included 33 children from our center between January 2008 and July 2014. Patients had pulmonary atresia with ventricular septal defect (13), tetralogy of Fallot (10), troncus arteriosus (4), double outlet right ventricle (2), and single left or right ventricle (4). A total of 44 balloon-expandable stents (mean diameter, 9.5 mm; range, 4 to 16 mm) were deployed in left or right PA under direct visualization, without the use of fluoroscopy, after branch angioplasty for 28 of them (64%). The mean age at surgery was 4.3 ± 4.3 years (range, 6 days to 15 years) and the mean weight was 14.3 ± 11.9 kg (range, 2.8 to 63 kg). RESULTS: Postoperative mortality was 9% (3 patients), but only 1 death was related to the stenting procedure. Twenty-five patients underwent angiographic control after a mean follow-up of 22 months after surgery. All stents were well positioned. The mean stented BPA Z-score increased from -2.6 ± 1.8 to -0.4 ± 1.6 (p < 0.0001). Eleven patients experienced intrastent proliferation (44%). Among them, 2 patients required a reoperation for severe intrastent stenosis, whereas the 9 others had mild intrastent neointimal proliferation, which was successfully managed by balloon expansion. CONCLUSIONS: Intraoperative stenting of BPA is a safe and effective option to treat BPA stenosis and prevent recurrence.

Perimount pericardial bioprosthesis for aortic calcified stenosis: 18-year experience with 1133 patients.

BACKGROUND AND AIM OF THE STUDY: Aortic stenosis (AS) is the most important cause of aortic valve disease, its prevalence increasing with patient age. The present study formed part of a long-term evaluation on use of the Perimount pericardial valve for aortic calcified stenosis. METHODS: A total of 1133 consecutive patients who underwent aortic valve replacement (AVR) with a Perimount pericardial bioprosthesis for degenerative AS between July 1984 and December 2003 at the authors' institution, was followed up in 2004. Among the patients (716 males, 417 females; mean age 72.6 years), 997 were in sinus rhythm, and the mean NYHA functional class was 2.3. Preoperative echocardiography indicated a mean gradient of 56 mmHg, a peak gradient of 89 mmHg, and an effective orifice area of 0.6 cm2. Associated procedures were required in 336 patients. RESULTS: All patients but 18 (1.5%) were followed up for an average of 5.5 years postoperatively; thus, the total follow up was 6,180 patient-years. Operative mortality was 2.8% (n=32), and there were 330 late deaths. At 18 years the actuarial survival rate was 22 +/- 4%. Among the 725 patients followed, 80% were in sinus rhythm and 98% in NYHA classes I or II. Valve-related complications included 39 thromboembolic episodes, 24 endocarditis, 22 anticoagulant-related hemorrhage, 28 reoperations, and 19 structural valve failures. A total of 54 patients died from valve-related causes (13 embolic events, two endocarditis, two hemorrhage, one structural failure, 36 unknown causes), and 57 died from cardiac failure. Neither thrombosis nor hemolysis was observed. At 18 years, freedom from embolism was 92 +/- 2%, from endocarditis 93 +/- 4%, from hemorrhage 95 +/- 2%, from reoperation 62 +/- 11%, from valve failure 68 +/- 12%, and from all complications 47 +/- 8%. Among patients aged >60 years, the 18-year actuarial freedom from reoperation was 76 +/- 14%, and from valve failure 85 +/- 8%. CONCLUSION: With a low rate of valve-related events at 18 years, and an especially low rate of structural failure, the Perimount pericardial prosthesis is a reliable choice for patients with aortic calcified stenosis.

GIS habitat analysis for lesser prairie-chickens in southeastern New Mexico.

BACKGROUND: We conducted Geographic Information System (GIS) habitat analyses for lesser prairie-chicken (LPCH, Tympanuchus pallidicinctus) conservation planning. The 876,799 ha study area included most of the occupied habitat for the LPCH in New Mexico. The objectives were to identify and quantify: 1. suitable LPCH habitat in New Mexico, 2. conversion of native habitats, 3. potential for habitat restoration, and 4. unsuitable habitat available for oil and gas activities. RESULTS: We found 16% of suitable habitat (6% of the study area) distributed in 13 patches of at least 3,200 ha and 11% of suitable habitat (4% of the study area) distributed in four patches over 7,238 ha. The area converted from native vegetation types comprised 17% of the study area. Ninety-five percent of agricultural conversion occurred on private lands in the northeastern corner of the study area. Most known herbicide-related conversions (82%) occurred in rangelands in the western part of the study area, on lands managed primarily by the US Bureau of Land Management (BLM). We identified 88,190 ha (10% of the study area) of habitats with reasonable restoration potential. Sixty-two percent of the primary population area (PPA) contained occupied, suitable, or potentially suitable habitat, leaving 38% that could be considered for oil and gas development. CONCLUSION: Although suitable LPCH habitat appears at first glance to be abundant in southeastern New Mexico, only a fraction of apparently suitable vegetation types constitute quality habitat. However, we identified habitat patches that could be restored through mesquite control or shin-oak reintroduction. The analysis also identified areas of unsuitable habitat with low restoration potential that could be targeted for oil and gas exploration, in lieu of occupied, high-quality habitats. Used in combination with GIS analysis and current LPCH population data, the habitat map represents a powerful conservation and management tool.

Unusual Outcome of a Right Ventricular Rhabdomyoma in an Infant.

Rhabdomyomas are the most common benign cardiac tumors. They may often be associated with tuberous sclerosis. In many cases, cardiac rhabdomyomas undergo spontaneous regression. Here, we report the unusual case of an infant with a large nonobstructive right ventricular rhabdomyoma at birth and at four months of age, which subsequently caused severe right ventricle outflow tract obstruction at six months of age, prompting surgery to remove the tumor. Close monitoring should be done in infants with large nonobstructive cardiac rhabdomyomas.

Is Seprafilm valuable in infant cardiac redo procedures?

BACKGROUND: Morbidity and mortality are higher for cardiac reoperations than first operation due to the presence of post-operative adhesions. We retrospectively evaluated the efficacy of the bioresorbable membrane Seprafilm to prevent pericardial adhesions after cardiac surgery in a paediatric congenital heart disease population. METHODS: Seventy-one children undergoing reoperations with sternotomy redo and cardiopulmonary bypass for congenital malformations were included. Twenty-nine of these patients were reoperated after previous application of Seprafilm (treatment group). The duration of dissection, aortic cross clamping and total surgery were recorded. A tenacity score was established for each intervention from the surgeon's description in the operating report. RESULTS: In multivariate analysis, the duration of dissection and the tenacity score were lower in the treatment than control group (p < 0.01), independent of age and interval since preceding surgery. CONCLUSION: Our results suggest that Seprafilm is effective in reducing the post-operative adhesions associated with infant cardiac surgery. We recommend the use of Seprafilm in paediatric cardiac surgery when staged surgical interventions are necessary.

Left retropectoral axillary implantation of defibrillators in young women.

We propose a complete surgical approach by left retropectoral transaxillary implantation with no vein puncture to improve the aesthetic and psychological tolerance of the implantable cardioverter defibrillator and avoid the pneumothorax and the subclavian crush syndrome.

A new technique for interrupted aortic arch repair: the Neville tube.

We have developed a new technique for interrupted aortic arch repair in which the pulmonary artery anterior wall is cut off and tailored so as to re-establish aortic continuity with an autologous tube. We are describing this method herein, with an 8-year follow-up of the first patient.

Outcome of coronary artery bypass grafting performed in young children.

OBJECTIVES: The long-term patency rate of coronary artery bypass grafting for which arterial grafts are used is known to be high in the pediatric population. However, this issue remains uncertain in children under 3 years of age. Here, we report the outcome in this specific population. METHODS: From July 1988 to July 2007, 18 children less than 3 years of age (age at operation, 0.1-35 months; median, 4 months) underwent 20 coronary artery bypass graft operations using an arterial graft. Indications for bypass grafting were coronary artery complications related to the arterial switch operation for transposition of the great arteries in 12 patients (coronary obstruction in 8 patients, peroperative coronary anomalies precluding coronary transfer in 4 patients), congenital anomalies of the coronary arteries in 4 patients, and Kawasaki disease in 2 patients. RESULTS: After a mean follow-up of 55 months (range, 1-176 months; median, 41 months), patency of 19 bypass grafts was assessed. One was occluded and 2 have necessitated a percutaneous procedure. Two patients died suddenly (1 with an occluded graft and 1 with a patent graft and hypertrophic myocardiopathy) 3.5 and 4.6 months, respectively, after bypass grafting. CONCLUSIONS: Coronary artery bypass grafting should be considered as a possible alternative for coronary revascularization in young children. Although our series shows quite a good patency rate, this procedure remains a technical challenge and requires careful follow-up.

15-year comparison of supra-annular porcine and PERIMOUNT aortic bioprostheses.

The second-generation Carpentier-Edwards bioprostheses, the supra-annular porcine valve and the PERIMOUNT pericardial valve, have been evaluated longitudinally for several years. This study compared clinical performance over 15 years. Aortic valve replacement was performed with a supra-annular porcine valve in 1,823 patients (group 1) aged 19-89 years (mean, 68.9 +/- 10.9 years) and with a PERIMOUNT pericardial bioprosthesis in 1,430 patients (group 2) aged 16-90 years (mean, 69.5 +/- 10.4 years). The groups were similar except for concomitant coronary artery bypass in 43% of group 1 and 18% of group 2 ( p < 0.001). Overall survival at 15 years was 29.3% +/- 1.5% for group 1 and 35.2% +/- 3.1% for group 2 ( p = 0.0009). The actual freedom from valve-related mortality was 88.5% +/- 0.9% for group 1 and 84.9% +/- 1.7% for group 2. The actual freedom from structural valve deterioration at 15 years was similar overall, and for patients aged > 60 years, between the groups, but was dissimilar (group 2 > group 1) for age group 2), sex (male > female), age, and concomitant coronary artery bypass. Both bioprostheses provided satisfactory clinical performance at 15 years after aortic valve replacement.