Experiência inicial com o implante percutâneo da valva Venus-P valve® no trato de saída nativo pulmonar / Initial experience with percutaneous Venus-P Valve® valve implantation in the native pulmonary outflow tract

INTRODUÇÃO: O restabelecimento da função valvar pulmonar com implante percutâneo de próteses atualmente é realizado somente nos pacientes portadores de biopróteses, condutos ou homoenxertos posicionados na via de saída do VD. Próteses dedicadas para tratos de saída nativo estão sendo estudadas e implantadas com sucesso em diversos centros mundiais. OBJETIVOS: Apresentar a experiência inicial do implante percutâneo da VENUS-P Valve® em trato de saída nativo pulmonar. Serão demonstrados critérios de escolha dos pacientes, aspectos técnicos relacionados aos procedimentos e resultados de curto prazo. MATERIAIS E MÉTODOS: A prótese VENUS-P Valve® (Venus Medtech, Shanghai) é um stent de nitinol autoexpansível com uma valva de pericárdio porcino montada no seu interior. Possui aspecto de ampulheta e na extremidade distal o stent é de células abertas permitindo o fluxo para ambas artérias pulmonares. O seu sistema de entrega é 22 ou 24F. Os pacientes candidatos são avaliados com ecocardiografia transtorácica, angiotomografia, ressonância magnética e cateterismo cardíaco. São critérios de inclusão a presença de regurgitação pulmonar importante, com volumes diastólicos do VD>130ml/m2, sem estenoses na VSVD ou ramos pulmonares e trato de saída com no máximo 35mm de diâmetro após insuflação de cateter-balão medidor. O ecocardiograma transtorácico é o método padrão de escolha do diâmetro e comprimento da prótese. Todos os procedimentos foram realizados em sala híbrida, com anestesia geral endovenosa, profilaxia para endocardite bacteriana e heparina (TCA>250 seg). Os pacientes realizaram ecocardiografia transtorácica e avaliação clínica 24 horas e 30 dias após os procedimentos. RESULTADOS: Quatro pacientes (3 homens) foram submetidos ao implante percutâneo das valvas pulmonares em 2 dias consecutivos. O peso e idade médio foi de 62,2kg e 25,8 anos. A via de saída teve 30mm em média e a prótese utilizada apresentou diâmetro médio de 32mm. Três próteses tinham 25 mm de comprimento e a última 30mm. Houve sucesso no implante de todos os dispositivos com restabelecimento imediato da função valvar pulmonar. Uma prótese foi considerada em posição mais baixa no trato de saída porém sem interferência em estruturas cardíacas. Ao ecocardiograma de controle todos os dispositivos encontravam-se sem refluxo significativo, com fluxo preservado para as artérias pulmonares bilaterais e sem complicações relacionadas. Não houve complicações ou óbitos relacionados aos procedimentos. CONCLUSÕES: O restabelecimento da função valvar pulmonar em pacientes com trato de saída nativo com o implante percutâneo da VENUS-P Valve® mostrou-se uma excelente alternativa nesta experiência inicial. Os procedimentos são factíveis e seguros quando realizados por grupos experientes e familiarizados com o implante de stents na via de saída pulmonar. Por tratar-se de experiência inicial, acreditamos que um maior número de implantes e análise dos resultados tardios devem ser realizadas para incorporação definitiva destes dispositivos neste grupo selecionado de pacientes. (AU)

[Pellagra and panniculitis induced by chronic bacterial colonisation of the small intestine]. / Colonisation bactérienne chronique de l'intestin grêle révélée par un érythème pellagroïde et une panniculite.

INTRODUCTION: Pellagra is an exceptional disorder in France. The classical description of pellagra associates a photoinduced rash with neurological impairment and intestinal dysfunction. Without adapted treatment, the progression is fatal. CASE REPORT: A 62 year-old women developed a photoinduced rash, composed of circular and erythematous elements with pustular edges. She also had panniculitis, peripheral neuropathy, depressive mood and diarrhea. Her medical past was marked by epilepsy treated with sodium valproate and hydantoin. Biological exams revealed lowered plasma levels of vitamins PP, B1, B6 and zinc, secondary to intestinal impairment induced by bacteria proliferating in the small intestine. The rash resolved with vitamin PP and zinc supplementation. The bacterial colonisation was improved by long-term, sequential antibiotics. DISCUSSION: We report a rare clinical form of pellagroid rash. The rash was induced by chronic malabsorption resulting from excessive bacterial proliferation in the diverticules of the small intestine. The antiepileptic treatment could have facilitated vitamin PP et zinc deficiency. Panniculitis was related to the bacterial proliferation. We discuss in this paper the relationship between some vitamin deficiencies, their clinical manifestations and the direct role of intestinal bacterial proliferation in the cutaneous manifestations.

Use of covered stents in the management of coarctation of the aorta.

We report our experience with the use of covered stents for the management of coarctation of the aorta. From December 2001 to March 2004, nine patients (seven males; median age, 31 years; mean weight, 65 +/- 15 kg) underwent implantation. Indications included critical or atretic native coarctation (n = 4), patients >50 years of age (n = 2), associated patent ductus arteriosus (n = 1) or adjacent aneurysm (n = 1), and the presence of a circumferential fracture within a previously implanted stent (n = 1). The covered balloon-expandable Cheatham-Platinum stent and the self-expandable stent graft Braile were employed. Adequate implantation was observed in all patients. Gradients were reduced from 54 +/- 14 to 3 +/- 8 mmHg and the coarctation site increased from 2.4 +/- 2.9 to 15.9 +/- 4.3 mm. The patent ductus arteriosus was immediately closed and the aneurysm excluded. Two patients >35 years with aneurysmal ascending aorta and metallic aortic prosthesis had aneurysm formation at follow-up, with one undergoing aneurysm exclusion using a Braile stent. Although covered stents are useful in the management of selected patients with coarctation, aneurysm formation may still occur in patients with markers of aortic wall weakness. Refinements in the deployment technique and/or the stent design are needed to eliminate this risk.

[Knowledge and problems regarding the topical treatment of leg ulcers: survey among general practitioners in the Indre-et-Loire area]. / Connaissances et difficultés des médecins dans le traitement local des ulcères de jambe. Enquête auprès de médecins généralistes en Indre-et-Loire.

INTRODUCTION: The quantity of topical treatments for leg ulcers has increased over the last 15 years. OBJECTIVE: To determine the prescriptions for topical treatments and the problems in the management of leg ulcers using a questionnaire sent to general practitioners. MATERIAL AND METHODS: A questionnaire in 3 parts was sent to 95 general practitioners in the area working in 36 different districts with an explanatory letter. The first part was composed of 29 closed questions regarding prescription of topical treatment for leg ulcers. The second part, also closed, included 3 pictures showing a budding, a necrotic and a fibrinous ulcer. The physicians had to choose which treatment they would have prescribed and the frequency of dressings changes. The third part was composed of two open questions regarding the problems encountered. The survey started on May 1st and was closed on June 30th 2002. No reminders were sent. RESULTS: We received 52 replies, 49 of which were exploitable. Forty-five percent (43/95) did not reply. Occlusive dressings were widely prescribed (mainly hydrocolloid 38/49 and hydrocellular 28/49). Vaseline impregnated gauze were less prescribed (14/49). Dressings with balsam of Peru were prescribed often by 10 and occasionally by 27 general practitioners. Alginate or charcoal dressings were not always used appropriately. Antiseptics were prescribed by 10 physicians. Mechanical debridement of fibrinous or necrotic wounds was rarely used even for necrotic ulcers. Topical anesthetics were prescribed in 21 out of 33 cases of mechanical debridement. Some physicians referred care (n=4), others complained about lack of compliance (n=15), cost (n=14), local intolerance (n=10) and the excessively wide variety of dressings (n=10). We drew-up a synopsis with guidelines for treatment, which was sent to all the general practitioners who had participated in the survey. CONCLUSION: Forty-three of the 95 physicians approached did not reply. This poor response rate, which is common in this type of survey, may include physicians who are not at ease in the management of ulcers and this may bias the results. The physicians who replied to the survey knew the subject well but complained of the costs and variety of dressings. They were all eager to receive further information and guidelines.

[Atopic dermatitis: which are the diagnostic criteria used in medical literature?]. / Dermatite atopique: quels sont les critères diagnostiques utilisés dans les articles scientifiques?

INTRODUCTION: Diagnosis of atopic dermatitis currently relies on diagnostic criteria scales developed by Hanifin and Rafka in 1980 and by the "United Kingdom Working party" in 1994. Some authors have proposed: "AEDS" [sM1] and "Atopiform Dermatitis", which has led to the distinction between different sub-populations and the exclusion of certain diseases from the diagnosis of atopic dermatitis. The aim of our study was to collect the criteria retained in the scientific medical literature during the year 2002 for the definition of atopic dermatitis and to try to understand not only the interest but also the questions that the various definitions lead to. METHOD: A PubMed research was launched with the key word "atopic dermatitis" from January to September of 2002. All the scientific articles either in French or in English were studied. RESULTS: Hanifin and Rafka's criteria were selected in 44 p. 100 of the scientific articles, and the "United Kingdom Working Party" criteria in 12 p. 100. Personal definitions were used in 21 p. 100 of the articles; these were based on the level of total and specific IgE or on personal clinical criteria. For twenty-three p. 100 of the authors, the definition of atopic dermatitis was not specified. DISCUSSION: There was not just one definition of atopic dermatitis. This may affect the interpretation of diagnostic or therapeutic papers concerning the disease, because there has been no proof that these definitions cover the same population of patients.

[Clinical manifestations of the mouth revealing Vitamin B12 deficiency before the onset of anemia]. / Signes buccaux révélateurs d'une carence en vitamine B12 avant l'apparition d'une anémie.

INTRODUCTION: The association of manifestations in the mouth and Vitamin B12 deficiency is already known. The signs are not specific to Vitamin B12 deficiency, however they may reveal the deficiency and this is often ignored and leads to delays in diagnosis. We report two cases of Vitamin B12 deficiency revealed by stomatodynia, glossitis and erosions in the mouth. OBSERVATIONS: Two women aged 51 and 76 years consulted, one for atrophic glossitis and the other for stomatodynia that had progressed over the past few months. No other symptoms were present. The biological examinations revealed isolated macrocytosis without anemia. Vitamin B12 could not be measured in the serum. The symptoms regressed in less than one month following replacement therapy. DISCUSSION: As with our two patients, the majority of cases of Vitamin B12 deficiency reported in the literature were only discovered several months or years after their onset, or were treated for other causes. Vitamin B12 deficiency leads to severe neuropathies. The neurological damage is reversible when replacement therapy is initiated early. Since the oral signs appear before the modification in the systemic markers of deficiency, it is crucial that these signs be recognized and diagnosed before the occurrence of severe after effects.

[Cutaneous vasculitis, myelodysplasia and relapsing polychondritis]. / Vascularite cutanée et myélodysplasie au cours d'une polychondrite chronique atrophiante.

BACKGROUND: Relapsing polychondritis is a rare autoimmune disease, characterized by recurrent inflammation of cartilaginous tissues. In some cases, many other tissues can be involved. CASE REPORT: We describe the case of a 64 year-old man with relapsing polychondritis, whose first symptoms were papulonodular and mucosal aphthous lesions. The skin biopsy revealed vasculitis without leukocytoclasic features. The diagnostic of relapsing polychondritis was made only two years later, thanks to recurrent auricular chondritis. Since the beginning, the disease was associated with a myelodysplasia. DISCUSSION: Dermatological manifestations are noticed in less than 50 p. 100 of cases during the evolution of relapsing polychondritis. An association with a myelodysplasia has already been reported, and it could be a particular form of relapsing polychondritis. Myelodysplasic syndromes are more frequently associated with relapsing polychondritis than with other vasculitis. It must be sought especially when cutaneous and mucosal manifestations exist.

[Recurrent aphthous stomatitis: treatment with colchicine. An open trial of 54 cases]. / Aphtose buccale récidivante: traitement par colchicine. Etude ouverte de 54 cas.

INTRODUCTION: Colchicine is a treatment used for aphthous stomatitis but its efficacy has never been proved. We report the results of an open trial of 54 patients treated with colchicine for aphthous stomatitis. The aim of our study was to assess the long and short term efficacy and tolerance of this molecule in this disease. PATIENTS AND METHODS: The case reports of 64 patients presenting with severe, recurrent aphthous stomatitis treated with colchicine between 1986 and 2000 were analyzed retrospectively. Only immunocompetent patients exhibiting idiopathic aphthous stomatitis or Behçet's disease were included. Ten patients were excluded: 4 cases of Behçet's disease treated with colchicine and systemic corticosteroids, 4 cases of aphthous stomatitis secondary to a hematological cause and 2 patients who were lost to follow-up after 3 month's treatment. Colchicine was prescribed at a dose of 1 to 1.5 mg/d for at least 3 months. All the patients were assessed after 3 month's treatment and all were contacted by phone in May 2000. Our end-point criteria were: the frequency and duration of the episodes, intensity of pain and impact on the quality of life of the patients. RESULTS: Fifty-four patients were included: 39 women (mean age=44 +/- 16.8) and 15 men (mean age=49 +/- 13.5). The disease had progressed for a mean of 11.6 +/- 13.5 years. After 3 month's treatment, 12 patients (22 p. 100) no longer had aphta and were in complete remission, 22 patients (41 p. 100) were significantly improved, since the frequency and duration of the lesions had decreased by at least 50 p. 100 and the latter were no longer painful. Treatment failed or tolerance was poor in 20 patients (37 p. 100). Patients were monitored and assessed for a mean of 4.7 years (range: 6 months to 13 years). Six patients were lost to follow-up. Three patients (6 p. 100) were considered as cured, since they were still in complete remission after suspension of colchicine respectively 6, 24 and 72 months later. Fifteen patients (31 p. 100) had still improved. Among the latter, 10 continued colchicine for a mean of 27 months, and 5 patients stopped treatment, which they considered tedious, after a mean of 16 months. These 15 patients noted a significant reduction in the frequency and duration of the lesions. Moreover, the subjective end-point criteria had improved significantly for them not to wish to change treatment. Thirty patients (63 p. 100) were not improved by the treatment. Some benign adverse events were noted in 10 patients (18.5 p. 100) and led to suspension of treatment in 4 cases. DISCUSSION: This study, which is the first in size (n=54) and duration of follow-up (4.7 years), showed that colchicine is an efficient preventive treatment of severe aphthous stomatitis for 63 p. 100 of patients after 3 month's treatment and that this improvement was lasting in 37 p. 100 of them. Colchicine should therefore be proposed in first intention in severe recurrent aphthous stomatitis, since it is effective, well tolerated and easy to use.

New percutaneous techniques for perforating the pulmonary valve in pulmonary atresia with intact ventricular septum.

We report new percutaneous techniques for perforating the pulmonary valve in pulmonary atresia with intact ventricular septum, in 3 newborns who had this birth defect. There was mild to moderate hypoplastic right ventricle, a patent infundibulum, and no coronary-cavitary communications. We succeeded in all cases, and no complications related to the procedure occurred. The new coaxial radiofrequency system was easy to handle, which simplified the procedure. Two patients required an additional source of pulmonary flow (Blalock-Taussig shunt) in the first week after catheterization. All patients had a satisfactory short-term clinical evolution and will undergo recatheterization within 1 year to define the next therapeutic strategy. We conclude that this technique may be safely and efficiently performed, especially when the new coaxial radiofrequency system is used, and it may become the initial treatment of choice in select neonates with pulmonary atresia and intact ventricular septum.

Percutaneous closure of ductus arteriosus with the amplatzer prosthesis. The Brazilian experience.

OBJECTIVE - To report the results of percutaneous occlusion of persistent ductus arteriosus with the Amplatzer prosthesis in 2 Brazilian cardiological centers. METHODS - From May 1998 to July 2000, 33 patients with clinical and laboratory diagnosis of persistent ductus arteriosus underwent attempts at percutaneous implantation of the Amplatzer prosthesis. The median age was 36 months (from 6 months to 38 years), and the median weight was 14kg (from 6 to 92kg). Sixteen patients (48.5%) were under 2 years of age at the time of the procedure. All patients were followed up with periodical clinical and echocardiographic evaluations to assess the presence and degree of residual shunt and possible complications, such as pseudocoarctation of the aorta and left pulmonary artery stenosis. RESULTS - The minimum diameter of the arterial ducts ranged from 2.5 to 7.0mm (mean of 4.0+/-1.0, median of 3.9). The rate of success for implantation of the prosthesis was 100%. Femoral pulse was lost in 1 patient. The echocardiogram revealed total closure prior to hospital discharge in 30 patients, and in the follow-up visit 3 months later in the 3 remaining patients. The mean follow-up duration was 6.4+/-3.4 months. All patients were clinically well, asymptomatic, and did not need medication. No patient had narrowing of the left pulmonary artery or of the aorta. No early or late embolic events occurred, nor did infectious endarteritis. A new hospital admission was not required for any patient. CONCLUSION - The Amplatzer prosthesis for persistent ductus arteriosus is safe and highly effective for occlusion of ductus arteriosus of varied diameters, including large ones in small symptomatic infants.

Indications, technique, results and clinical impact of reocclusion procedures for residual shunts after transcatheter closure of the patent ductus arteriosus.

PURPOSE: To assess the results of reocclusion procedures for late residual shunts after transcatheter closure of the patent ductus arteriosus. MATERIAL AND METHODS: Nineteen patients underwent reocclusion procedures either with Rashkind technique (2 patients) or with coils. Mean age was 96.5 months and mean interval between the initial and the additional procedure was 15.7 months. In 5 patients, the duct was balloon-dilated prior to occlusion. RESULTS: In 3 patients the procedure was unsuccessful because it was impossible to cross the duct using either a guide wire or a catheter. Immediate or late complete occlusion occurred in 13 patients. Two patients required a third procedure for complete closure. One patient developed severe intravascular hemolysis after coil implantation and was sent to surgery. The rate of final late residual shunting decreased from 17.1% to 6.6% (5/76) in the umbrella group and from 15.8% to 1.8% (1/56) in the coil group. CONCLUSIONS: Reocclusion procedures are indicated after one year of persistent shunting following the primary procedure. They are generally effective and reduce significantly the rate of late residual shunting. In patients with tiny shunts, the duct may need to be dilated. Coils are preferred because of their low cost.

Transcatheter embolization of congenital coronary arterial fistulas in adults.

In this report, we describe our experience with transcatheter occlusion of congenital coronary arterial fistulas in adults. From November 1992 to November 1996, 5 symptomatic patients, aged from 47 to 70 years, underwent transcatheter occlusion of fistulas using a retrograde arterial approach. All had chest pain or dyspnea on exertion. Detachable balloons were used in 4 patients, and Gianturco coils in 1. Detachable balloons were implanted through a Debrun system, while the coils were implanted through a 5 French right coronary Judkins catheter. Both were passed through an 8 French guiding catheter (Amplatz II). Each patient had a single fistula. The fistulas originated from the right coronary artery in 3 patients, and from the circumflex artery in 2. They drained into the pulmonary trunk in 3 patients, into the right atrium in 1, and into a bronchial artery in the other. All fistulas were occluded completely in the catheterization laboratory, and the procedures were uncomplicated. At follow up, 3 patients underwent coronary angiography, and there was no evidence of recanalization. Transcatheter embolization in adults of single congenital coronary fistulas with detachable balloons and coils is safe and effective and can be regarded as an acceptable alternative to surgery.

Percutaneous closure of atrial septal defects. The role of transesophageal echocardiography.

PURPOSE: Evaluation of the role of transesophageal echocardiography in percutaneous closure of atrial septal defects (ASD) with the Amplatzer septal occluder. METHODS: Patients were selected for percutaneous closure of ASD by transesophageal echocardiography (TEE), which was also used to monitor the procedure, helping to select the appropriate size of the Amplatzer device, to verify its position, and to access the immediate results of the procedure. During the follow-up, TEE was used to evaluate the presence and magnitude of residual shunt (RS), device position, and right cardiac chamber diameters. RESULTS: Twenty-two (40%) of a total of 55 studied patients were selected. Thirteen underwent Amplatzer device implantation, eight are still waiting for it, and one preferred the conventional surgical treatment. All procedures were successful, which was mainly due to proper patient selection. Six (23%) patients acutely developed RS, which spontaneously disappeared at the three-month follow-up examination in three patients. There was a significant reduction in the right ventricle diastolic diameter, from 27 mm (average) to 24 mm and 20 mm, one and three months after the procedure, respectively (p < 0.0076). CONCLUSION: With the aid of TEE, percutaneous closure of ASD can be successfully, safely, and effectively performed.

Percutaneous mitral valvotomy in patients eighteen years old and younger. Immediate and late results.

OBJECTIVE - To analyze immediate and late results of percutaneous mitral valvotomy (PMV) in patients < or = 18 year. METHODS - Between August '87 and July '97, 48 procedures were performed on 40 patients. The mean age was 15.6 years; 68.7% were females four of whom were pregnant. RESULTS - Success was obtained in 91.7% of the procedures. Immediate complications were severe mitral regurgitation (6.3%) and cardiac tamponade (2.0%). Late follow-up was obtained in 88.8% of the patients (mean value=43.2+/-33.9 months). NYHA functional class (FC) I or II was observed in 96.2% of the patients and restenosis developed in five patients, at a mean follow-up of 29.7+/-11.9 months. Three patients presented with severe mitral insufficiency and underwent surgery. Two patients died. CONCLUSION - PMV represents a valid therapeutic option in young patients. In these patients, maybe because of subclinical rheumatic activity, restenosis may have a higher incidence and occur at an earlier stage than in others persons.