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1.
Lett Appl Microbiol ; 54(6): 530-6, 2012 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-22404299

RESUMO

AIMS: Larvae of the red palm weevil (RPW) Rhynchophorus ferrugineus Olivier (Coleoptera: Curculionidae) feed inside palm stem tissues, making galleries and producing a wet fermenting frass. We characterized the culturable bacteria associated with frass produced by tunnelling larvae inside the Canary island date palms and investigated the role of frass and gut bacteria in plant polymers breakdown. METHODS AND RESULTS: A culture-dependent method was used to isolate bacteria from frass and noninfested palm tissues. Bacterial isolates were grouped into operational taxonomic units based on polymorphisms in the ITS-PCR profiles, and representative isolates were identified by partial sequencing of the 16S rRNA gene. Frass bacteria were dominated by 2,3-butanediol fermenter Enterobacteriaceae. None of the bacterial isolates was able to degrade cellulose; however, cellulolytic and hemicellulolytic bacteria were isolated from the larval gut enrichment cultures. CONCLUSIONS: Frass bacteria are specifically associated with the RPW larvae and might play beneficial roles for RPW, other than nutritional, that deserve further investigations. Breakdown of plant polymers probably occurs inside the larvae digestive system. SIGNIFICANCE AND IMPACT OF THE STUDY: Frass and gut micro-organisms of R. ferrugineus should be included in studies of the interactions between RPW, its plant hosts, and its enemies.


Assuntos
Arecaceae , Bactérias/isolamento & purificação , Fezes/microbiologia , Gorgulhos/microbiologia , Animais , Bactérias/genética , Técnicas Bacteriológicas , DNA Bacteriano/genética , DNA Espaçador Ribossômico/genética , Sistema Digestório/microbiologia , Enterobacteriaceae/genética , Enterobacteriaceae/isolamento & purificação , Larva/microbiologia , Filogenia , RNA Ribossômico 16S/genética , Espanha
2.
Bull Soc Pathol Exot ; 104(1): 25-8, 2011 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-21287371

RESUMO

UNLABELLED: The aim of the study was to investigate the occurrence, pattern and clinical aspects of congenital heart diseases and their management in Cameroon. In this multicentred retrospective study from January 2006 till November 2009, out of 51,636 consulting in three referral centres, 505 were recruited. All the patients presented with the following symptoms: cyanosis, clubbing, frequent respiratory tract infections, failure to thrive, growth retardation, precordial murmur and dyspnoea. Patients were sent for the screening of congenital heart disease. After the comprehensive Doppler echocardiography, the recruited patients were diagnosed with congenital (67.2%) and in few, acquired heart disease. Heart murmur, dyspnoea and growth retardation was the triad mostly encountered. The occurrence of congenital heart diseases in Cameroon is 9.87%. In Douala, isolated ventricular septal defect, interatrial septal defect and isolated pulmonary valve stenosis were more diagnosed than in Shisong (P <0.05) and Yaoundé (P <0.05). In Yaoundé, there were more cases of common arterial trunk, transposition of great arteries with ventricular septal defect and Ebstein disease than in Shisong (P <0.05) and Douala (P < 0.05). At Shisong, tetralogy of Fallot, arterial duct, coarctation of the aorta, congenital mitral valve regurgitation, atresia of the tricuspid valve, double outlet right ventricle, anomalous pulmonary venous return and left isomerism were more diagnosed than in Yaoundé (P <0.05) and in Douala (P <0.05). Thirty percent of the patients were operated abroad; 9% in the cardiac centre. CONCLUSION: Our data show that congenital heart diseases are represented in Cameroon as in the literature; isolated ventricular septal defect is the predominant pathology.


Assuntos
Cardiopatias Congênitas/epidemiologia , Adulto , Camarões/epidemiologia , Criança , Pré-Escolar , Comorbidade , Dispneia/epidemiologia , Ecocardiografia Doppler , Feminino , Transtornos do Crescimento/epidemiologia , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Cardiopatias Congênitas/terapia , Sopros Cardíacos , Hospitais Gerais/estatística & dados numéricos , Hospitais Especializados/estatística & dados numéricos , Humanos , Lactente , Recém-Nascido , Masculino , Centros de Saúde Materno-Infantil/estatística & dados numéricos , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto Jovem
3.
Pediatr Med Chir ; 33(5-6): 236-40, 2011.
Artigo em Inglês | MEDLINE | ID: mdl-22428432

RESUMO

OBJECTIVE: Postoperative nutrition of newborns undergoing heart surgery (HS) is ill-defined. We compared the postoperative growth rates (grams/day) of neonates with congenital heart defects (CHDs) fed with their own mothers' milk (HU), a starting formula (SF; 67 kcal/100 mL), or a "preterm formula" (PF; 80 kcal/100 mL). PATIENTS AND METHODS: We studied 122 newborns undergoing HS: 81 underwent corrective surgery (group A), and 41 palliative surgery (group B). RESULTS: No statistically significant differences were found in the growth rate between group A and B. Moreover, in both groups, no differences in terms of growth rate between infants fed HU, SF, or PF were observed. This was also true when analyses were limited to infants undergoing HS either with or without cardiopulmonary bypass. CONCLUSIONS: In neonates undergoing HS, human milk allowed a growth rate similar to that observed with starting formulas and "preterm formulas". This effect may depend on the particular features of human milk and its protective properties for the intestinal mucosa.


Assuntos
Aleitamento Materno , Procedimentos Cirúrgicos Cardíacos , Cardiopatias Congênitas/cirurgia , Fórmulas Infantis , Cuidados Pós-Operatórios , Feminino , Humanos , Lactente , Recém-Nascido , Masculino
4.
Pediatr Med Chir ; 33(3): 124-8, 2011.
Artigo em Inglês | MEDLINE | ID: mdl-22145295

RESUMO

AIM: No study described reliably the changes in cerebral ultrasound (CUS) findings in neonatal heart surgery. We tried to define the modifications of CUS before and after heart surgery in neonates. PATIENTS: We studied 48 neonates with congenital heart defects were studied. Of these, 33 had correction of the malformation with cardiopulmonary bypass (CPB group); 15 underwent coarctation repair/shunting procedures (no-CPB group). Patients had CUS pre-operatively, 48-72 hours after surgery, and at discharge. RESULTS: Pre-operative studies did not show significant differences between the groups. In CPB group, preoperatively, 6/33 infants showed echogenicity of basal ganglia and 1/33 grade I IVH. In the early postoperative evaluation, 24/33 infants had abnormal scans: increased echogenicity of basal ganglia in 24 and grade I IVH in 14. At discharge, abnormal scans persisted in 20/33 (basal ganglia echogenicity in 20, grade I IVH in 12). In the no-CPB group, preoperatively, 1/15 infant showed echogenicity of basal ganglia. In the early postoperative evaluation, 6/15 had abnormal scans: increased echogenicity of basal ganglia in 6, grade I IVH in 4. At discharge, abnormal scans persisted in 3/15. CONCLUSIONS: CUS after neonatal heart surgery shows a definite pattern of increased echogenicity of the basal ganglia, more consistent after CPB. These changes may reflect disturbances of deep grey matter, and last 10-15 days at least.


Assuntos
Ecoencefalografia , Cardiopatias Congênitas/cirurgia , Procedimentos Cirúrgicos Cardíacos , Feminino , Humanos , Recém-Nascido , Masculino , Cuidados Pós-Operatórios , Cuidados Pré-Operatórios
5.
Pediatr Med Chir ; 32(6): 297-301, 2010.
Artigo em Italiano | MEDLINE | ID: mdl-21462454

RESUMO

Many paediatric cardiac patients now survive to adulthood following early surgery. This population of adult patients with a congenital heart disease offer distinct challenges such as unusual anatomy and demands such as pregnancy and exercise tolerability not found in conventional paediatric or traditional adult interventional patients. Starting with neonatal balloon atrial septostomy for transposition of the great arteries, improving and changing percutaneous interventional techniques have increasingly replaced surgery as a treatment option in several congenital heart diseases. There has been a rapid growth in interventional cardiology techniques to treat adults with congenital heart disease, mirroring the rise of interventional cardiology as a cardiology subspecialty and the increasing population of adults with congenital abnormalities. We review current indications for adult congenital heart disease intervention and best practice, reviewing the patient spectrum commonly treated, devices used, and emerging treatments.


Assuntos
Cardiopatias Congênitas/cirurgia , Cardiopatias/congênito , Cardiopatias/cirurgia , Adulto , Procedimentos Cirúrgicos Cardíacos/métodos , Humanos
6.
Pediatr Med Chir ; 32(6): 270-3, 2010.
Artigo em Italiano | MEDLINE | ID: mdl-21462448

RESUMO

The incidence of infective endocarditis (IE) in patients with congenital heart disease (CHD) is higher than in general population; this is a major problem considering the continuous expansion of such group of patients. Generally the more complex is the congenital heart disease the higher is the risk of IE. The aetiology, clinical features, complications, basis for diagnosis and treatment of IE in CHD patients don't differ from those in acquired cardiac disease; however, right-sided IE is more frequent in CHD patients. Due to the complex anatomy or presence of artificial material in many CHD, the transesophageal echocardiogram is extremely useful although echocardiographic assessment remains difficult. Prognosis is better than in other forms of IE with a mortality rate <10%. Primary prevention is crucial: a good oral-dental hygiene and regular dental review are as important as antibiotic prophylaxis; however this awareness in the CHD population is still not satisfactorily spread due to an educational problem. New IE guidelines from International Cardiology Societies emphasize the role of primary prevention and limit antibiotic prophylaxis to the highest risk patients undergoing the highest risk procedures. This article reviews the main reasons justifying the revision of previous IE guidelines, focuses on criteria to select CHD patients requiring antibiotic prophylaxis and gives information about antibiotic therapy to use.


Assuntos
Antibioticoprofilaxia , Endocardite Bacteriana/etiologia , Endocardite Bacteriana/prevenção & controle , Cardiopatias Congênitas , Cardiopatias/congênito , Adulto , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Criança , Cardiopatias Congênitas/cirurgia , Cardiopatias/cirurgia , Humanos , Guias de Prática Clínica como Assunto , Fatores de Risco
7.
Pediatr Med Chir ; 32(6): 256-9, 2010.
Artigo em Italiano | MEDLINE | ID: mdl-21462446

RESUMO

Cardiopulmonary exercise testing (CPET) gives important information about functional capacity by direct measurement of exercise respiratory gas exchange. It provides assessment of the integrative exercise responses involving the cardiovascular, respiratory and muscle-skeletal systems which are not adequately investigated through the measurement of individual organ system function. CPET involves measurements of oxygen uptake (VO2), carbon dioxide production (VCO2) and several ventilatory measures. CPET is increasingly being used as a clinical tool to determine functional capacity, prognosis and potential need for medical or surgical interventions in patients affected by congenital heart disease (CHD). Also in this population, peak VO2 and slope VE/VCO2 are the most predictive parameters in terms of mortality and need of hospitalization. Cyanotic patients with Esisenmenger syndrome show the worst functional limitation and consequently the worst prognosis. This article provides basic and practical information about CPET and focuses on its interpretation in patients with CHD.


Assuntos
Teste de Esforço , Cardiopatias Congênitas/diagnóstico , Cardiopatias/congênito , Cardiopatias/diagnóstico , Adulto , Humanos
8.
Bull Soc Pathol Exot ; 102(3): 155-8, 2009 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-19739409

RESUMO

UNLABELLED: Rheumatic heart disease (RHD) remains a major public health problem in developing countries. Whereas Africa has 10% of the world's population, broadly as many as half of the 2.4 million children affected by RHD live on the continent. We report on the occurrence and pattern of valve involvement in RHD using echocardiography in our centre and post surgical follow-up. METHODS: In this retrospective study, transthoracic echocardiography (TTE) data collected from the Shisong cardiac centre over a period of 24 months (August 2005 to August 2007) were reviewed. Patients with a precordial murmur were selected. A total of 262 echocardiographic examinations were done in the centre over the two-year study period. The screening allowed us to see two categories of patients: 169 (64.5%), 79 male and 90 female, out of the 262 patients with abnormal results had an echocardiographic diagnosis of RHD, 80 (30.5%) patients had congenital heart disease. The 13 (5%) patients left had innocent murmur. Mitral valve regurgitation was the commonest echocardiographic diagnosis present in 101 patients (59.7%). Thirty-six (13.7%) patients had mixed mitral valve disease, 40 (23.7%) had mixed aortic and mitral valve disease, 42 (25%) had pure mitral stenosis and 26 (15.3%) had pure aortic regurgitation. The complications of RHD being observed included secondary pulmonary hypertension in 20 patients (11.8%) and functional tricuspid regurgitation was seen in 39 (21.9%). The congenital heart disease were: tetralogy of Fallot 29.1%, isolated ventricular septal defect 62.5%, isolated atrial septal defect 3.2%, atrioventricular canal 1.1%, patent ductus arteriosus 2.2%, common arterial trunk 1.9%. Our data showed that in children above 10-years-old in rural zone of Cameroon presenting with a precordial murmur RHD has to be suspected. Acute rheumatic fever primary and secondary prevention as well as rheumatic fever registers are important for the disease eradication in our countries. More surgical centres for a better management of the RHD complications are needed in sub-Saharan Africa. Due to poverty and illiteracy of parents, the post surgical follow up of patients is challenging.


Assuntos
Sopros Cardíacos/etiologia , Doenças das Valvas Cardíacas/epidemiologia , Cardiopatia Reumática/epidemiologia , Adolescente , Camarões/epidemiologia , Criança , Pré-Escolar , Ecocardiografia Doppler , Feminino , Seguimentos , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/epidemiologia , Sopros Cardíacos/diagnóstico por imagem , Sopros Cardíacos/epidemiologia , Doenças das Valvas Cardíacas/complicações , Doenças das Valvas Cardíacas/diagnóstico por imagem , Doenças das Valvas Cardíacas/etiologia , Humanos , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/etiologia , Masculino , Prevalência , Estudos Retrospectivos , Cardiopatia Reumática/complicações , Cardiopatia Reumática/diagnóstico por imagem , População Rural/estatística & dados numéricos
9.
Pediatr Med Chir ; 30(1): 9-15, 2008.
Artigo em Italiano | MEDLINE | ID: mdl-18491673

RESUMO

OBJECTIVE: Pulmonary regurgitation may cause progressive right ventricular dilatation and dysfunction in adult patients previously repaired for tetralogy of Fallot. To assess the optimal surgical timing, the impact of the right ventricular restoration with a new surgical ventriculoplasty technique is evaluated following TFO repaired adult patients with severe pulmonary regurgitation and right ventricular dilatation. METHODS: Sixteen patients with severe pulmonary valve regurgitation (PVR) and right ventricular dilatation with RVOT aneurysm underwent right ventricular remodelling since January 2002. Each underwent preoperative evaluation by Doppler echocardiography, magnetic resonance imaging (MRI), and right ventricular myocardial acceleration during isovolumic contraction (IVC). The surgical procedure included pulmonary valve implantation and RVOT restoration achieved by removal of the aneurysm tissue, coupled with a ventriculoplasty to reduce volume, accomplished by creating a satisfactory RVOT dimension by placing with 2-0 Gortex suture to allow acceptance of a 26 Hegar dilator to avoid restriction. Nine patients had associated surgical procedures. RESULTS: All patients survived the operative procedure and underwent a 16-month follow-up interval. A reduction of cardio thoracic index and a clinical improvement occurred in each patient. Significant reduction of RVEDV and RVESV and increased right ventricular ejection fraction was observed. CONCLUSIONS: This preliminary database implies that the right ventricular restoration is a simple and effective procedure, and introduces a structural component that should be added in repaired TFO patients with right ventricular dilatation and underlying aneurysm or akinesia of the right ventricular outflow tract.


Assuntos
Aneurisma Cardíaco/cirurgia , Insuficiência da Valva Pulmonar/cirurgia , Tetralogia de Fallot/cirurgia , Disfunção Ventricular Direita/cirurgia , Adulto , Idoso , Ecocardiografia Doppler , Seguimentos , Aneurisma Cardíaco/diagnóstico , Implante de Prótese de Valva Cardíaca , Humanos , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Insuficiência da Valva Pulmonar/diagnóstico , Volume Sistólico , Fatores de Tempo , Resultado do Tratamento , Disfunção Ventricular Direita/diagnóstico , Remodelação Ventricular
10.
Arch Dis Child Fetal Neonatal Ed ; 91(6): F419-22, 2006 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-16820390

RESUMO

BACKGROUND: Frequent premature ventricular contractions (PVCs), couplets (CPLTs) and episodes of ventricular tachycardia are extremely rare in the neonatal population. Limited information is available with regard to clinical relevance and outcome. OBJECTIVES: To evaluate the clinical characteristics and outcomes of a group of newborns with ventricular arrhythmias without heart disease. PATIENTS AND DESIGN: Between January 2000 and January 2003, 16 newborns with ventricular arrhythmias in the absence of heart disease were studied. The newborns were divided into three groups: PVC group (n = 8), CPLT group (n = 4) and ventricular tachycardia group (n = 4). All patients underwent physical examination, electrocardiography, Holter monitoring and echocardiography at diagnosis and at follow-up (1, 3, 6 and 12 months, and yearly thereafter). RESULTS: Mean (standard deviation, SD) age of the patients was 3 (1.19) days in the PVC group, 3.25 (0.95) days in the CPLT group and 6.5 (9.1) days in the ventricular tachycardia group. Median follow-up was 36 months (range 24-48 months). PVCs disappeared during follow-up in all the neonates, in the PVC group, at a mean (SD) age of 2.1 (1.24) months; in the CPLT group, couplets disappeared at a mean (SD) age of 6.5 (1) months. All patients with ventricular tachycardia were treated; ventricular tachycardia disappeared at a mean (SD) age of 1.7 (0.9) months. Neither death nor complications occurred. CONCLUSIONS: Ventricular arrhythmias in newborns without heart disease have a good long-term prognosis. Frequent PVCs and CPLTs do not require treatment. Sustained ventricular tachycardia or high-rate ventricular tachycardia must be treated, but the prognosis is generally favourable.


Assuntos
Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/tratamento farmacológico , Ecocardiografia/métodos , Eletrocardiografia/métodos , Feminino , Seguimentos , Humanos , Recém-Nascido , Masculino , Exame Físico/métodos , Prognóstico
11.
Bull Soc Pathol Exot ; 109(5): 340-344, 2016 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-27534757

RESUMO

The aim of the study was to investigate the pattern of valvular lesions, the mortality and the challenges in five years follow-up in children with post-rheumatic valvulopathies in natural history in St. Elizabeth Catholic General hospital Shisong, cardiac centre. This retrospective analysis included 270 patients aged between 5 and 16 years old suffering from post-rheumatic valvulopathies who consulted in the cardiac centre from July 2008 through July 2013. Postrheumatic valvulopathies were diagnosed according to the World heart federation criteria. Data from patients' records, two-dimensional echocardiographic studies, and electrocardiograms were reviewed. Patients and their family were contacted every six months. The duration of the follow-up was 60months. Patients aged between 5 and 16 years old with a mean age of 12.4±4.5 years. Female gender was representing 63% (n=170) of the population. Surgery was indicated in 256 cases. Lesions of the valves needing prophylaxis with penicillin was diagnosed in 14 cases. In 95 patients surgical correction could not be performed. Mitral regurgitation was the commonest echocardiographic diagnosis present in 61.5%, n=164 patients; 38.5%, n=103 patients had aortic regurgitation. Mitral stenosis and mitral disease were also represented in 6%, n=16, and 8%, n= 21 patients respectively. Pulmonary hypertension was the common echocardiographic complication of the disease in=234, 87% of cases. Clinically, complications of the disease included congestive heart failure (n=229, 85%), growth retardation (n=162, 60%), sudden death (n=27, 10%). On presentation, n=210, 78% of cases were admitted. Mortality in two years was 35%, (p≤0.05, 95% CI=2.5-6.5), in five years was 65% (p≤ 0.05, 93% CI= 2.7-7.21). The challenges faced are patients' negligence and poor discipline, wrong beliefs, poverty. Post-rheumatic mitral valve regurgitation is the pathology the most encountered. Pulmonary hypertension is the most common echocardiographic complication of the disease. Five years mortality is very high in our setting. Due to financial limitation and illiteracy of parents, the follow up of patients is difficult.


Assuntos
Doenças das Valvas Cardíacas/epidemiologia , Cardiopatia Reumática/epidemiologia , Adolescente , Camarões/epidemiologia , Criança , Maus-Tratos Infantis/estatística & dados numéricos , Mortalidade da Criança , Pré-Escolar , Cultura , Feminino , Seguimentos , Doenças das Valvas Cardíacas/complicações , Hospitais Gerais , Humanos , Masculino , Cooperação do Paciente/estatística & dados numéricos , Pobreza/estatística & dados numéricos , Estudos Retrospectivos , Cardiopatia Reumática/complicações
13.
Am J Med Genet ; 73(4): 484-8, 1997 Dec 31.
Artigo em Inglês | MEDLINE | ID: mdl-9415479

RESUMO

We describe a child with trigonocephaly, strabismus, upslanting palpebral fissures, nasal bridge hypoplasia, hypertrophic alveolar ridges and large gingivo-labial frenula, short neck, hip "dysplasia," equinovarus deformities, cryptorchidism, atrial septal defect ostium secundum, and severe mental retardation, findings consistent with C syndrome. The patient also had a Dandy-Walker malformation, complete callosal agenesis, and occipital meningocele. These structural defects are independent of the premature closure of the metopic suture, and confirm that midline brain anomalies are part of C syndrome. The hypothesis that the basic developmental defect in this syndrome primarily affects the midline field is supported by the concomitance of other anomalies, such as conotruncal heart defects, omphalocele, and genital anomalies.


Assuntos
Encéfalo/anormalidades , Encéfalo/patologia , Anormalidades Craniofaciais/genética , Agenesia do Corpo Caloso , Pré-Escolar , Criptorquidismo/genética , Síndrome de Dandy-Walker/patologia , Deficiências do Desenvolvimento/genética , Feminino , Dedos/anormalidades , Humanos , Recém-Nascido , Imageamento por Ressonância Magnética , Masculino , Gravidez , Síndrome
14.
Ann Thorac Surg ; 72(3): 951-2, 2001 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-11565701

RESUMO

We report a surgical treatment for neonatal aortic coarctatin associated with distal aortic arch hypoplasia. This technique offers the possibility for augmentation of the aortic arch without sacrificing the subclavian artery or using prosthetic patch material. The procedure was successfully performed in 5 patients.


Assuntos
Angioplastia/métodos , Aorta/cirurgia , Coartação Aórtica/cirurgia , Artéria Subclávia/cirurgia , Anastomose Cirúrgica/métodos , Aorta/anormalidades , Coartação Aórtica/complicações , Humanos , Recém-Nascido
15.
Heart ; 79(2): 186-90, 1998 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-9538314

RESUMO

OBJECTIVE: To compare the morphology of the pulmonary vessels in tetralogy of Fallot or pulmonary atresia-ventricular septal defect (PA-VSD) with (del22q) and without 22Q11 deletion (non-del22q). PATIENTS: 94 consecutive infants (54 with tetralogy of Fallot, 40 with PA-VSD) were studied using ultrasound and catheterisation. MOLECULAR INVESTIGATIONS: Identification of the 22q deletion was performed either by fluorescent in situ hybridisation or polymerisation chain reaction genotyping. RESULTS: 25 patients were del22q (16/40 (40%) PA-VSD v 9/54 (17%) tetralogy of Fallot; p < 0.02). Major aortopulmonary collateral arteries was more common in patients with PA-VSD-del22q (p < 0.03). Such collaterals were identified in 13 patients: 10 del22q and three non-del22q (p < 0.001). The size of the right and left pulmonary arteries expressed as a standard deviation (SD) difference of the normal range was -4.2 (quartiles -5.3 and -2.9) for PA-VSD del22q, and -2.6 (-3.1 and -1.8) for PA-VSD non-del22q (p = 0.02). The mean (SD) difference between the measured and theoretical Nakata index was -373 (94) for PA-VSD del22q v -245 (93) in PA-VSD non-del22q (p = 0.0002). In tetralogy of Fallot patients with and without del22q, the size of the pulmonary arteries was similar (p = 0.6). CONCLUSIONS: A "specific" phenotype could be defined in patients with deletion: PA-VSD, major aortopulmonary collateral arteries with complex loop morphology, and small central pulmonary arteries. Differences in the morphology of the pulmonary vessels may indicate a different timing of the faulty developmental pathway in patients with and without 22q11 deletion.


Assuntos
Aorta/patologia , Cromossomos Humanos Par 22 , Circulação Colateral , Deleção de Genes , Artéria Pulmonar/patologia , Veias Pulmonares/patologia , Tetralogia de Fallot/genética , Tetralogia de Fallot/patologia , Aorta/diagnóstico por imagem , Comunicação Interventricular/diagnóstico por imagem , Comunicação Interventricular/genética , Comunicação Interventricular/patologia , Humanos , Hibridização in Situ Fluorescente , Lactente , Estudos Prospectivos , Artéria Pulmonar/diagnóstico por imagem , Atresia Pulmonar/diagnóstico por imagem , Atresia Pulmonar/genética , Atresia Pulmonar/patologia , Veias Pulmonares/diagnóstico por imagem , Radiografia , Tetralogia de Fallot/diagnóstico por imagem , Ultrassonografia
16.
Arch Mal Coeur Vaiss ; 92(5): 591-6, 1999 May.
Artigo em Francês | MEDLINE | ID: mdl-10367075

RESUMO

Prosthetic conduits, with or without biological valves, are often inserted in surgical procedures to correct or palliate cardiac malformations. The principal problem is degeneration which causes variable degrees of obstruction requiring reoperation for their replacement. The aims of this study were to assess the feasibility, safety and efficacy of a non-surgical method of treating these obstructive prostheses by dilatation-implantation of a metallic vascular endoprosthesis (stenting). Thirteen patients were treated (age range 7.7 to 36 years; mean: 15 years). Eight had pulmonary atresia with a ventricular septal defect corrected by a valved conduit from the right ventricle to the pulmonary artery which became obstructive nearly 10 years later: the implantation of the stent reduced the transconduit pressure gradient in all cases except one who had not undergone closure of the septal defect in which the cyanosis was improved. There are two cases of obstruction of a modified Blalock anastomosis in which the stent revascularised the shunt with improvement in cyanosis. In the final 3 cases, the whole Fontan procedure was compromised by obstruction of a conduit incorporated in the system, and which dilatation with stenting considerably improved. The efficacy of the procedure was constant with no complications other than rupture of the balloon in 3 cases. The good results were maintained for an average of 7.3 months (range 1 to 25 months), but it was necessary to redilate one restenosed stent after 8 months. Dilatation followed by stenting in obstructive cardiovascular prostheses is a simple, safe and effective alternative to surgical reoperation.


Assuntos
Cardiopatias Congênitas/cirurgia , Comunicação Interventricular/cirurgia , Próteses Valvulares Cardíacas , Falha de Prótese , Atresia Pulmonar/cirurgia , Stents , Adolescente , Adulto , Cateterismo Cardíaco , Criança , Feminino , Humanos , Masculino , Resultado do Tratamento
17.
Arch Mal Coeur Vaiss ; 92(5): 583-90, 1999 May.
Artigo em Francês | MEDLINE | ID: mdl-10367074

RESUMO

Congenital valvular heart disease in childhood is often complex. Conventional echocardiography provides two-dimensional views which require mental reconstruction for three-dimensional assessment. This problem may be solved by the use of three-dimensional (3D) echocardiography which obtains images of valves comparable to those seen at surgery. This was confirmed by 4 cases of congenital valvular heart disease studied by 3D echocardiography: stenotic bicuspid aortic valve disease, parachute mitral valve and two cases of mitral regurgitation in patients with atrioventricular canal. The 3D views of the aortic valve showed the commissural opening after percutaneous balloon valvuloplasty of the bicuspid valve. The surface of the aortic orifice and the surface of the two mitral leaflets were measured from 3D reconstructions. The longitudinal 3D view analysed the extension of the single obstructive mitral papillary muscle of the parachute malformation. The 3D ventricular views allowed assessment of the extension of the cleft and the surface of the three mitral leaflets of the 2 cases of atrioventricular canal. In one of these cases, the results of surgical valvuloplasty were evaluated after 3D reconstruction of the valve. 3D echocardiography is not only a diagnostic tool for congenital heart disease but also a very useful complementary investigation for accurate evaluation of congenital valvular lesions to optimise possible valve repair.


Assuntos
Angioplastia Coronária com Balão , Cardiopatias Congênitas/diagnóstico por imagem , Doenças das Valvas Cardíacas/congênito , Adolescente , Insuficiência da Valva Aórtica/diagnóstico por imagem , Insuficiência da Valva Aórtica/cirurgia , Criança , Ecocardiografia Tridimensional , Ecocardiografia Transesofagiana , Feminino , Cardiopatias Congênitas/cirurgia , Doenças das Valvas Cardíacas/diagnóstico por imagem , Doenças das Valvas Cardíacas/cirurgia , Humanos , Masculino , Insuficiência da Valva Mitral/diagnóstico por imagem , Insuficiência da Valva Mitral/cirurgia , Resultado do Tratamento
18.
Arch Mal Coeur Vaiss ; 91(5): 623-9, 1998 May.
Artigo em Francês | MEDLINE | ID: mdl-9749214

RESUMO

Ventricular tachycardia is rare and poorly understood in the neonate. The authors undertook a retrospective study in 2 foetus and 8 neonates aged 1 to 20 days at the time of diagnosis. The tachycardia was permanent in 2 cases, observed in runs of variable variation in the other 8, incessant in 7 of these cases. Only two cases were symptomatic: cardiac failure with shock 16 hours after birth and hydramnios at 16 weeks gestation. The electrocardiographic criteria of ventricular tachycardia (wide QRS complexes of different morphology to the sinus QRS complexes, atrioventricular dissociation) were fulfilled in all patients. The arrhythmia was monomorphic 9 times out of 10 with a fixed (3 cases) or variable (7 cases) rate which was always > 150/min. Intravenous magnesium sulphate in the severe and permanent forms, oral betablockers in forms triggered by acceleration of the sinus rhythm, oral amiodarone alone or associated in one case with propranolol were prescribed but three neonates were not treated, either from the outset or after inefficacy of amiodarone: nine of the patients were cured and are treatment-free 12 to 24 months later: the other patient has a slow, well tolerated ventricular tachycardia. No aetiology was detected in 9 cases; the other had a metabolic disease of B-oxidation of long chain fatty acids. The authors conclude that isolated, idiopathic ventricular tachycardia of the neonate usually carry a good prognosis which is not dependent on the tachycardia of the permanence of the arrhythmia. Simple treatment (betablocker or amiodarone) is usually associate with restoration of sinus rhythm and definitive cure during the first year of life.


Assuntos
Taquicardia Ventricular/diagnóstico , Antagonistas Adrenérgicos beta/uso terapêutico , Amiodarona/uso terapêutico , Antiarrítmicos/uso terapêutico , Eletrocardiografia , Feminino , Coração Fetal/diagnóstico por imagem , Coração Fetal/fisiopatologia , Humanos , Recém-Nascido , Gravidez , Estudos Retrospectivos , Taquicardia Ventricular/tratamento farmacológico , Taquicardia Ventricular/embriologia , Ultrassonografia Pré-Natal
19.
Arch Mal Coeur Vaiss ; 94(5): 487-93, 2001 May.
Artigo em Francês | MEDLINE | ID: mdl-11434017

RESUMO

This study sought to evaluate the quality of life of surviving adults with univentricular heart and to determine the impact of sociodemographic and clinical characteristics on quality of life. The health records of 89 survivors with univentricular heart living in France at the time of the study were reviewed. They were 17 to 49 years old (median: 21). Sixty-seven patients answered the Duke questionnaire. Sociodemographic and clinical variables were similar in the responders and non-responders. The scores of patients who answered the questionnaire were compared with national norms. The impact of sociodemographic and clinical variables on individual Duke's measures was assessed. The Duke scores of adults with univentricular heart were similar to those of the normal population. Cyanosis predicted a worse score for physical (p = .05) and perceived health measures (p = .02). The higher educational level predicted a better score for physical (p .004), mental (p = .01), and general health measures (p = .02). Orthopaedic problems worsened social score (p = .05). Psychosocial problems worsened pain score (p = .04). Mitral atresia, in comparison with the other anatomical types, worsened perceived health score (p = .02). Finally, patients younger than 23 years scored better for almost all of health and dysfunction measures. In conclusion, despite repeated interventions and other disease-related everyday stresses, a selected group of adults with univentricular heart had a satisfying quality of life. These patients probably developed copying mechanisms, easing the psychological stress, and oriented themselves towards a different set of values in everyday life so that to accept disabilities and to recalibrate personal expectations.


Assuntos
Pessoas com Deficiência , Ventrículos do Coração/anormalidades , Qualidade de Vida , Adolescente , Adulto , Estudos Transversais , Cianose/complicações , Cianose/etiologia , Demografia , Educação , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Dor , Prognóstico , Índice de Gravidade de Doença , Classe Social
20.
Arch Mal Coeur Vaiss ; 92(5): 637-40, 1999 May.
Artigo em Francês | MEDLINE | ID: mdl-10367081

RESUMO

Transposition of the great arteries (TGA) is a common malformation which sometimes has a dramatic presentation at birth but which is completely curable with early and appropriate initial management. Antenatal diagnosis of this condition may change the neonatal prognosis. The authors compared morbidity and mortality in the pre- and postoperative periods of 68 neonates with an antenatal diagnosis of TGA (foetal diagnosis) with that of 250 neonates in whom the diagnosis was made after birth (neonatal diagnosis). The delay before admission to the department was 2 +/- 2.8 hours in the foetal group and 73 +/- 210 hours in the neonatal group (p < 0.01). Severe haemodynamic distress (metabolic acidosis, multi-organ failure) were more common in the neonatal group (p < 0.01). Management on admission was identical in the two groups (p > 0.05). The preoperative mortality was 15/250 in the neonatal group (6%, 95% CI = 3-9%) compared with 0/68 in the foetal group (p < 0.05). The postoperative morbidity was comparable in the two groups (25/235 and 6/68) but the hospital stay was longer in the neonatal group (30 +/- 17 versus 24 +/- 11 days, p < 0.01). Finally, postoperative mortality was significantly higher in the neonatal group (20/235 compared with 0/68, p < 0.01) although the risk factors of death at arterial switch surgery were identical in the two groups. Therefore, antenatal diagnosis of TGA reduces neonatal morbidity and mortality in this condition. Antenatal diagnosis must be developed by the education of obstetricians. The transfer of mothers with a foetus affected by TGA to centres capable of assuming the initial management, sometimes during labour, is essential.


Assuntos
Transposição dos Grandes Vasos/diagnóstico por imagem , Feminino , Humanos , Mortalidade Infantil , Recém-Nascido , Gravidez , Transposição dos Grandes Vasos/mortalidade , Transposição dos Grandes Vasos/cirurgia , Ultrassonografia Pré-Natal
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