Research of predictive factors for cardiac resynchronization therapy: a prospective study comparing data from phase-analysis of gated myocardial perfusion single-photon computed tomography and echocardiography : Trying to anticipate response to CRT.

BACKGROUND: Cardiac resynchronization therapy (CRT) reduces morbidity and mortality in chronic systolic heart failure. About 20% of implanted patients are considered as "non-responders". This study aimed to evaluate gated myocardial perfusion single-photon emission computed tomography (GMPS) phase parameters as compared to echocardiography in the assessment of predictors for response to CRT before and after CRT activation. METHODS: Forty-two patients were prospectively included during 15 months. A single injection of 99mTc-tetrofosmin was used to acquire GMPS phase pre- and post-CRT activation. Indicators of positive CRT response were improvement of functional status and 15% reduction in left ventricular end-systolic volume at 3 months. RESULTS: Phase parameters at baseline were similar in the two groups with no influence of perfusion data. Phase parameters after CRT activation were significantly improved in the responders' group (Δ Bandwidth -19° ± 24° vs. 13° ± 31°, p = 0.001; Δ SD -20° ± 30° vs. 26° ± 46°, p = 0.001; Δ Entropy -11 ± 12 vs. 2 ± 6%, p = 0.001). Feasibility and reproducibility were higher for GMPS. CONCLUSION: Acute phase modifications after CRT activation may predict response to CRT immediately after implantation, but not at baseline, even when adjusted to perfusion data.

[Multiple familial trichoepithelioma: a new CYLD gene mutation]. / Trichoépithéliomes multiples familiaux : nouvelle mutation du gène CYLD.

BACKGROUND: Multiple familial trichoepithelioma (MFT) is an autosomal dominant disease characterized by the development of numerous skin-coloured papules on the central area of the face. It is associated with various CYLD gene mutations that are also responsible for familial cylindromatosis and Brooke-Spiegler syndrome. PATIENTS AND METHODS: We report a novel mutation in the CYLD gene in a family with MFT and discuss new developments in therapeutic options. DISCUSSION: Recent studies indicate that CYLD is a tumour-suppressor gene.

Prevalence, characteristics, and prognosis role of type 1 ST elevation in the peripheral ECG leads in patients with Brugada syndrome.

BACKGROUND: Despite isolated reports of Brugada syndrome (BrS) in the inferior or lateral leads, the prevalence and prognostic value of ST elevation in the peripheral electrocardiographic (ECG) leads in patients with BrS remain poorly known. OBJECTIVE: To study the prevalence, characteristics, and prognostic value of type 1 ST elevation and ST depression in the peripheral ECG leads in a large cohort of patients with BrS. METHODS: ECGs from 323 patients with BrS (age 47 ± 13 years; 257 men) with spontaneous (n = 141) or drug-induced (n = 182) type 1 ECG were retrospectively reviewed. Two hundred twenty-five (70%) patients were asymptomatic, 72 (22%) patients presented with unexplained syncope, and 26 (8%) patients presented with sudden death (12 patients) or appropriated implantable cardioverter-defibrillator therapies (14 patients) at diagnosis or over a mean follow-up of 48 ± 34 months. RESULTS: Thirty (9%) patients presented with type 1 ST elevation in at least 1 peripheral lead (22 patients in the aVR leads, 2 in the inferior leads, 5 in both aVR and inferior leads, and 1 in the aVR and VL leads). Patients with type 1 ST elevation in the peripheral leads more often had mutations in the SCN5A gene, were more often inducible, had slower heart rate, and higher J-wave amplitude in the right precordial leads. Twenty-seven percent (8 of 30) of the patients with type 1 ST elevation in the peripheral leads experimented sudden death/appropriate implantable cardioverter-defibrillator therapy, whereas it occurred in only 6% (18 of 293) of other patients (P < .0001). In multivariate analysis, type 1 ECG in the peripheral leads was independently associated with malignant arrhythmic events (odds ratio 4.58; 95% confidence interval 1.7-12.32; P = .0025). CONCLUSIONS: Type 1 ST elevation in the peripheral ECG leads can be seen in 10% of the patients with BrS and is an independent predictor for a malignant arrhythmic event.

Primary electrical diseases diagnosis, genetic and management.

Primary electrical diseases or channelopathies are inherited genetic alterations of the cell ionic and electrical behavior leading to various cardiac arrhythmias carrying the risk of sudden death. A descriptive review of the successively described channelopathies is made in this article, with emphasis on the clinical manifestations, the genetic background and the currently accepted therapeutic options.

[A pseudotumoral cutaneous form of sarcoidosis associated with non-Hodgkin lymphoma]. / Sarcoïdose cutanée pseudotumorale associée à un lymphome malin non hodgkinien.

BACKGROUND: Sarcoidosis may be revealed by a variety of cutaneous signs, occasionally atypical. It may also be associated with a lymphoma. Herein we report an original case of pseudotumoral cutaneous sarcoidosis associated with malignant non-Hodgkin's lymphoma (MNHL). PATIENTS AND METHODS: A 64-year-old man was hospitalised for follicular and diffuse large B-cell MNHL localised in the cervical, subclavicular and inguinal lymph nodes. The skin examination revealed a bulky, erythematous, purple, infiltrated mass in the right lumbar region and two similar but smaller plaques in the dorsal region. The lesions were asymptomatic, present for six years, and stable. Histological examination of a skin biopsy sample revealed an epithelioid giant-cell granuloma without caseous necrosis. Bacteriological and mycobacteriological samples were sterile. Mediastinal adenopathies and pulmonary micronodules were found on CAT scan. A diagnosis of cutaneous and pulmonary sarcoidosis associated with the lymphoma was made. Polychemotherapy using CHOP combined with rituximab resulted in remission of the lymphoma but was ineffective against the sarcoidosis. COMMENTS: Pseudotumoral forms of sarcoidosis are rare and have a misleading clinical aspect. In our case, the cutaneous mass was evocative of a secondary lymphoma site. The diagnosis of sarcoidosis was based on clinical and laboratory findings and after elimination of other potential causes of granuloma. The appearance of a lymphoma in a patient with a history of sarcoidosis is rare but not fortuitous, since the notion of "sarcoidosis-lymphoma syndrome" exists. The incidence of lymphomas is 5.5-fold higher in patients presenting sarcoidosis than among the general population. Their time to onset is around 7 years after the discovery of sarcoidosis. The most common forms involve Hodgkin's disease. The efficacy of therapy on the blood disease underlying the sarcoidosis is variable.

[Radiotherapy for soft tissue sarcomas of extremities. Preliminary comparative dosimetric study of 3D conformal radiotherapy versus helical tomotherapy]. / Radiothérapie postopératoire des sarcomes des tissus mous des extrémités. Etude dosimétrique préliminaire comparative de la radiothérapie conformationnelle avec la tomothérapie hélicoïdale.

PURPOSE: To evaluate dosimetry of helicoidal tomotherapy versus three-dimensional conformal radiotherapy (3D-CRT) with and without IMRT for the treatment of soft tissue sarcoma (STS) of the thigh. METHODS AND PATIENTS: A retrospective study was performed for three patients who received 3D-CRT as adjuvant radiation therapy for STS of the thigh. These three patients had a tumor in posterior, adductor or anterior compartment of the thigh. In each case, three treatments plans were optimised in tomotherapy, without bloc, with directional bloc and complete bloc of controlateral limb, to adequately treat the planning target volume and spare organ at risk. For each patient, we compare the three modalities of tomotherapy or "classical" IMRT from a clinac with the 3D-CRT actually performed for the treatment. RESULTS: Tomotherapy provides improved PTV coverage and dose homogeneity. This benefit was comparable in the three tomotherapy plans. The average D95% for tomotherapy and 3D-CRT were 97.6% and 94.8% respectively and the standard deviation is, at least, divided by two with conformal and is always better than performed with a clinac. The volume of the surrounding soft tissues receiving at least full prescription and hot spots, as evaluated by D2%, were significantly reduced in tomotherapy. Nevertheless, the results concerning the skin, the femur and the gonads were dependent on the tumor site in the thigh and not always improved with tomotherapy dosimetric studies. CONCLUSIONS: For this preliminary study, tomotherapy can provide better coverage and dose uniformity in PTV and minimize the volume of surrounding muscular tissues receiving high doses. However, in this study, there is no benefit for others OAR (skin, femur and gonads) except in particular constraint (for instance for a precise sparing of cutaneous or bony area). Other dosimetric studies, followed by prospective evaluations with long-term follow-up are needed to determine whether tomotherapy can improve outcome for patients with STS of the thigh.

[Epithelioid sarcoma: a retrospective study of conservative treatment with initial surgery and radiotherapy]. / Sarcomes épithélioïdes: étude rétrospective après chirurgie conservatrice et radiothérapie.

INTRODUCTION: Epithelioid sarcoma is a rare type of soft tissue sarcomas with a high risk of recurrence both local and distant. The place of surgical conservative treatment and the role of radiation therapy remain controversial. PATIENTS AND METHODS: A serie of 9 consecutive patients treated with initial conservative surgery and postoperative radiotherapy (median dose of 52.8 Gy) from 1987 to 2006 in the same institution was analyzed. RESULTS: With a median follow-up of 40 months (range 15-153 months), the rate of local, nodal and distant relapse is respectively 56%, 11% and 33%. The rate of death is 44.5%. No imputation has been performed. CONCLUSION: Even with a high rate of local relapse observed, a conservative treatment doesn't seem to influence badly the overall survival (55.5% alive at 40 months). Indeed the rate of distant relapse and death are comparable with those found in the literature. Moreover relapse occurred almost within the irradiated volumes. An improvement of dose could be also discussed.

Optimal pacing in congenital complete atrioventricular block of immunological origin: interest of multisite stimulation.

An infant with a congenital auriculoventricular block (CAVB) of immunological origin was diagnosed prenatally. The mother had Gougerot-Sjögren disease with positive anti-Sjogren's Syndrome A (SSA) and Sjogren's Syndrome B (SSB) serologies. Cardiac pacing was necessary and the epicardial route was chosen. Considering the left ventricular (LV) dilatation, bi-ventricular (BiV) stimulation was preferred to the usual DDD mode, presumed to have a deleterious long-term effect. Echographic parameters were better with BiV stimulation: the asynchronism induced by mono-RV stimulation was corrected and the QRS complexes were narrower. BiV pacing of a CAVB with LV dilation looks clinically and echographically attractive but needs to be validated in the long term.

Epicardial catheter ablation of ventricular tachycardia using surgical subxyphoid approach.

We report the case of a patient presenting with a previous inferior myocardial infarction complicated by incessant monomorphic ventricular tachycardia resistant to antiarrhythmic drugs. Because endocardial catheter ablation failed and because of focal endocardial activation arising from the left ventricular inferior wall, an epicardial location of the reentry circuit was suspected. Catheter mapping of the pericardial space through a surgical subxyphoid approach performed in the electrophysiological laboratory confirmed the epicardial location of the arrhythmogenic substrate and allowed us successfully to ablate and cure the patient. Surgical subxyphoid approach can be performed in the electrophysiological laboratory when epicardial ablation is needed in case of inadvisable, difficult, or failed non-surgical percutaneous access.

[Conservative and functional management for soft tissue sarcomas of the extremities: role of radiotherapy]. / Approche conservatrice et fonctionnelle dans les sarcomes des tissus mous des membres : place de la radiothérapie.

Recent publications have permitted to quantify the benefit of radiotherapy in the conservative treatment of soft tissue sarcoma of the limbs. The aim of this review is to focus on aspects of radiotherapy witch influence local control and functional outcome for early and late normal tissue damage. The evaluation of late effects is performed according to Soma-Lent (Subjective-Objective-Management-Analytic-Late Effects of Normal Tissues) classification. About complications, neurological complications are probably under estimated and are related to total dose of radiation therapy.

[Aseptic meningitis during Sweet syndrome]. / Méningite aseptique au cours d'un syndrome de Sweet.

BACKGROUND: Sweet syndrome is a neutrophilic skin disease that can involve extracutaneous signs. Here we describe a case of aseptic meningitis, a rare potential extracutaneous sign of Sweet syndrome. CASE REPORT: A 42-year-old man was hospitalized for non-pruritic maculoerythematous skin lesions of the legs and back with subsequent myalgia. A histology specimen taken from a skin lesion revealed an acute neutrophilic disease consistent with Sweet syndrome. Marked inflammation and cholestasis were observed. Systemic corticosteroid therapy was given and resulted in good clinical and laboratory response. Two weeks later, in a setting of gradual dosage reduction, the patient was hospitalized for intense headaches associated with meningeal irritation in an inflammatory context. Liver function tests were again abnormal. We concluded on a diagnosis of Sweet syndrome complicated by aseptic meningitis and hepatic sites. Investigation for underlying disease, particularly digestive or hematologic, was negative. A favorable outcome was achieved following administration of higher doses of systemic corticosteroids. DISCUSSION: Aseptic meningitis constitutes an extracutaneous localization of Sweet syndrome. A multidisciplinary approach and exclusion of infectious origin are required in order to institute systemic corticosteroid therapy.

[Ablation of ventricular extra-systole in the left Valsalva sinus. A case report]. / Ablation d'une extrasystolie ventriculaire dans le sinus de Valsalva gauche. A propos d'un cas.

We report the case of a patient presenting with unrelenting isolated or repetitive monomorphic ventricular extra-systoles, with left block and right axis deviation, which appeared to arise from the right ventricular chamber, but for which ablation was finally performed in the left Valsalva sinus. The ECG and endocavity electro-physiological features which led us to suspect this atypical, although not exceptional, situation are reported, as well as the techniques for ablation available in this case.

[Pars plana phacoemulsification. Technics, indications and results]. / Phacoémulsification par la pars plana. Techniques, indications, résultats.

Pars plana phacoemulsification allows cataract extraction through a 3 mm posterior sclerotomy. The indications of these technics are the combined cataract-vitrectomy, the cataracts on vitrectomised eyes and cataracts on eyes filled with silicone oil. The alteration of corneal endothelium is very reduced with the endocapsular pars plana phacoemulsification. After the emulsification, the capsular bag is extracted with a microforceps through the pars plana. It is also possible to save the anterior capsule to reduce corneal alteration during endocular surgery. We present the indications and the advantages of these technics.