Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 36
Filtrar
1.
J Vasc Surg ; 72(6): 1873-1882, 2020 12.
Artigo em Inglês | MEDLINE | ID: mdl-32665182

RESUMO

OBJECTIVE: Aberrant subclavian artery (ASA), a well-described aortic arch anomaly, is frequently associated with dysphagia and development of Kommerell diverticulum (KD) with aneurysmal degeneration. Historically, open repair has been performed, which can be associated with significant morbidity. More recently, hybrid approaches using different arch vessel revascularization techniques in combination with thoracic endovascular aortic repair (hybrid TEVAR) have been described, but there is a paucity of literature describing outcomes. The objective of this analysis was to describe our experience with management of complicated ASAs using hybrid TEVAR, further adding to the literature describing approaches to and outcomes of hybrid ASA repair. METHODS: A retrospective, single-institution review was performed of all patients treated for ASA complications using hybrid TEVAR (2002-2018). The primary end point was technical success, defined as absence of type I or type III endoleak intraoperatively and within 30 days postoperatively. Secondary end points included complications, reintervention, and survival. Centerline measurement of KD diameters (maximum diameter = opposing aortic outer wall to diverticulum apex) was employed. Kaplan-Meier methodology was used to estimate secondary end points. RESULTS: Eighteen patients (1.4% of 1240 total TEVAR procedures; male, 67%; age, 59 ± 13 years) were identified (left-sided arch and right ASA, 94% [n = 17]; right-sided arch and left ASA, n = 1 [6%]; retroesophageal location and associated KD, 100%); median preoperative KD diameter was 60 mm (interquartile range [IQR], 37-108 mm). Operative indications included diverticulum diameter (61%), dysphagia (17%), rupture (11%), rapid expansion (6%), and endoleak after TEVAR (6%). All procedures used some combination of supraclavicular revascularization and TEVAR (staged, 50% [n = 9]), whereas partial open arch reconstruction was used in 17% (n = 3). There were no perioperative deaths or spinal cord ischemic events. Major complications occurred in 22% (n = 4): nondisabling stroke, one; arm ischemia, one; upper extremity neuropathy, one; and iatrogenic descending thoracic aortic dissection, one. Technical success was 83%, but 44% (n = 8) had an endoleak (type I, n = 3; type II, n = 5 [intercostal, n = 2; aneurysmal subclavian artery origin, n = 3]) during follow-up (median, 4 months; IQR, 1-15 months). Two endoleaks resolved spontaneously, three were treated, and three were observed (1-year freedom from reintervention, 75% ± 10%). Median KD diameter decreased by 7 mm (IQR, 1-12 mm), and 78% (n = 14) experienced diameter reduction or stability in follow-up. The 1- and 3-year survival was 93% ± 6% and 84% ± 10%, respectively. CONCLUSIONS: Hybrid open brachiocephalic artery revascularization with TEVAR appears to be safe and reasonably effective in management of ASA complications as evidenced by a low perioperative complication risk and reasonable positive aortic remodeling. However, endoleak rates raise significant concerns about durability. Therefore, if this technique is employed, the mandatory need for surveillance and high rate of reintervention should be emphasized preoperatively. This analysis represents a relatively large series of a hybrid TEVAR technique to treat ASA complications, but greater patient numbers and longer follow-up are needed to further establish the role of this procedure.


Assuntos
Aorta Torácica/cirurgia , Implante de Prótese Vascular , Anormalidades Cardiovasculares/cirurgia , Procedimentos Endovasculares , Artéria Subclávia/anormalidades , Idoso , Aorta Torácica/anormalidades , Aorta Torácica/diagnóstico por imagem , Implante de Prótese Vascular/efeitos adversos , Implante de Prótese Vascular/mortalidade , Anormalidades Cardiovasculares/diagnóstico por imagem , Anormalidades Cardiovasculares/mortalidade , Procedimentos Endovasculares/efeitos adversos , Procedimentos Endovasculares/mortalidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/etiologia , Sistema de Registros , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Artéria Subclávia/diagnóstico por imagem , Artéria Subclávia/cirurgia , Fatores de Tempo , Resultado do Tratamento
2.
J Vasc Interv Radiol ; 30(9): 1378-1385, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31133449

RESUMO

PURPOSE: To evaluate the early and midterm outcomes of endovascular repair of Stanford type B aortic dissection (TBAD) with an aberrant right subclavian artery (ARSA). MATERIALS AND METHODS: From November 2012 to July 2018, 15 patients (13 male, 2 female) who had TBAD with ARSA underwent total endovascular repair, including thoracic endovascular aortic repair (TEVAR), left subclavian artery (LSA) chimney technique, LSA fenestration technique, and double chimney technique (LSA chimney and ARSA periscope). The mean age was 55 years. Kommerell diverticulum (KD) occurred in 2 patients, which were covered by means of oversized aortic stents. All patients had preoperative imaging examinations for assessing cerebral blood flow and the dominant vertebral arteries. RESULTS: All procedures were completed successfully. There was no in-hospital mortality. The mean procedural time was 98 ± 40 minutes (range 50-190). The mean preoperative diameters of the maximum descending aorta, false lumen, and true lumen in the descending aorta versus postoperative were 37 ± 10 mm (range 28-67), 18 ± 9 mm (range 9-41), and 19 ± 6 mm (range 10-35) versus 34 ± 9 mm (range 25-64), 5 ± 8 mm (range 0-28), and 28 ± 5 mm (range 19-37), respectively. Right upper extremity weakness was observed in 2 patients, which recovered gradually during follow-up. Immediate type Ia endoleak was detected in 1 patient. The mean follow-up time was 33 ± 20 months. During follow-up, no ARSA steal syndrome, spinal cord ischemia, or strokes were not observed. CONCLUSIONS: Endovascular treatment for TBAD with ARSA was feasible and safe with a satisfactory midterm follow-up outcome.


Assuntos
Aneurisma Aórtico/cirurgia , Dissecção Aórtica/cirurgia , Implante de Prótese Vascular , Anormalidades Cardiovasculares/cirurgia , Procedimentos Endovasculares , Artéria Subclávia/anormalidades , Adulto , Idoso , Dissecção Aórtica/diagnóstico por imagem , Dissecção Aórtica/mortalidade , Dissecção Aórtica/fisiopatologia , Aneurisma Aórtico/diagnóstico por imagem , Aneurisma Aórtico/mortalidade , Aneurisma Aórtico/fisiopatologia , Prótese Vascular , Implante de Prótese Vascular/efeitos adversos , Implante de Prótese Vascular/instrumentação , Implante de Prótese Vascular/mortalidade , Anormalidades Cardiovasculares/diagnóstico por imagem , Anormalidades Cardiovasculares/mortalidade , Anormalidades Cardiovasculares/fisiopatologia , Procedimentos Endovasculares/efeitos adversos , Procedimentos Endovasculares/instrumentação , Procedimentos Endovasculares/mortalidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Duração da Cirurgia , Complicações Pós-Operatórias/etiologia , Desenho de Prótese , Estudos Retrospectivos , Fatores de Risco , Stents , Artéria Subclávia/diagnóstico por imagem , Artéria Subclávia/fisiopatologia , Artéria Subclávia/cirurgia , Fatores de Tempo , Resultado do Tratamento
3.
Rev Saude Publica ; 53: 31, 2019 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-30942273

RESUMO

OBJECTIVE: To analyze the association of characteristics recorded at the time of birth, including weight, occurrence of asphyxia, gestation duration, maternal age and education level, with death from diseases or malformations of the circulatory system in children under 18 years of age. METHODS: The Brazilian Information System on Live Births and Information System on Mortality databases were linked and evaluated following a longitudinal cohort analysis strategy. The following independent variables were evaluated: characteristics recorded at the time of birth, including weight, occurrence of asphyxia, gestation duration, maternal age and education level. Dependent variables were death from diseases or malformations of the circulatory system in children under 18 years of age. Crude relative risks were estimated and relative risks were adjusted for the variables. RESULTS: 6,380 deaths were linked to 4,282,260 birth records, yielding 5,062 pairs considered as true. Low birth weight (RR = 2.26), asphyxia at 1 (RR = 1.72) and 5 minutes (RR = 1.51), prematurity (RR = 1.50), maternal age ≥ 40 years (RR = 2.06), and low maternal education level (RR = 1.45) increased the probability of death caused by circulatory system diseases. In the association with death by malformations of the circulatory system, the predictive variables showed the same association profile, but with greater intensity. CONCLUSIONS: Fetal and maternal factors are associated with increased mortality due to diseases and malformations of the circulatory system. Measures to control these factors and improve access to their diagnosis and treatment would contribute to reducing the number of deaths caused by diseases and malformations of the circulatory system. However, the identification of environmental influences during gestation and birth on the risk of death should be carefully considered due to being influenced by genetic factors.


Assuntos
Doenças Cardiovasculares/etiologia , Doenças Cardiovasculares/mortalidade , Mortalidade Infantil , Adolescente , Declaração de Nascimento , Brasil , Anormalidades Cardiovasculares/mortalidade , Causas de Morte , Criança , Pré-Escolar , Feminino , Cardiopatias Congênitas/mortalidade , Humanos , Lactente , Recém-Nascido de Baixo Peso , Recém-Nascido , Estudos Longitudinais , Masculino , Idade Materna , Fatores de Risco
4.
Rev. saúde pública (Online) ; 53: 31, jan. 2019. tab, graf
Artigo em Inglês | LILACS | ID: biblio-991636

RESUMO

ABSTRACT OBJECTIVE To analyze the association of characteristics recorded at the time of birth, including weight, occurrence of asphyxia, gestation duration, maternal age and education level, with death from diseases or malformations of the circulatory system in children under 18 years of age. METHODS The Brazilian Information System on Live Births and Information System on Mortality databases were linked and evaluated following a longitudinal cohort analysis strategy. The following independent variables were evaluated: characteristics recorded at the time of birth, including weight, occurrence of asphyxia, gestation duration, maternal age and education level. Dependent variables were death from diseases or malformations of the circulatory system in children under 18 years of age. Crude relative risks were estimated and relative risks were adjusted for the variables. RESULTS 6,380 deaths were linked to 4,282,260 birth records, yielding 5,062 pairs considered as true. Low birth weight (RR = 2.26), asphyxia at 1 (RR = 1.72) and 5 minutes (RR = 1.51), prematurity (RR = 1.50), maternal age ≥ 40 years (RR = 2.06), and low maternal education level (RR = 1.45) increased the probability of death caused by circulatory system diseases. In the association with death by malformations of the circulatory system, the predictive variables showed the same association profile, but with greater intensity. CONCLUSIONS Fetal and maternal factors are associated with increased mortality due to diseases and malformations of the circulatory system. Measures to control these factors and improve access to their diagnosis and treatment would contribute to reducing the number of deaths caused by diseases and malformations of the circulatory system. However, the identification of environmental influences during gestation and birth on the risk of death should be carefully considered due to being influenced by genetic factors.


Assuntos
Humanos , Masculino , Feminino , Recém-Nascido , Lactente , Pré-Escolar , Criança , Adolescente , Recém-Nascido de Baixo Peso , Doenças Cardiovasculares/etiologia , Doenças Cardiovasculares/mortalidade , Brasil , Declaração de Nascimento , Mortalidade Infantil , Fatores de Risco , Estudos Longitudinais , Causas de Morte , Idade Materna , Anormalidades Cardiovasculares/mortalidade , Cardiopatias Congênitas/mortalidade
5.
BMC Pediatr ; 18(1): 393, 2018 12 22.
Artigo em Inglês | MEDLINE | ID: mdl-30579351

RESUMO

BACKGROUND: In French Guiana, pregnant women may be exposed to infectious, environmental, and social risks leading to congenital malformation. The objective of the study was to study mortality rates from congenital malformations among infants < 1 year and to compare them with those in mainland France. METHODS: We used the CEPI DC (INSERM) database, which compiles annual data from death certificates in all French territories using the International Classification of Diseases. Annual deaths for French Guiana and mainland France between 2005 and 2015 were compiled. The age category studied was children less than 1 year and deaths from congenital malformations, deformations and chromosomal abnormalities were compiled. Crude risk ratios and 95% confidence intervals were calculated to quantify the excess risk of disease in French Guiana. RESULTS: In French Guiana between 2005 and 2015 there were 666 deaths of children aged < 1 year, among which, 132 (19.8%) were due to congenital malformations and chromosomal anomalies. Overall the risk ratio of death from congenital malformations and chromosomal anomalies between French Guiana and mainland France was 2.7 (1.5-4.7), P < 0.001 for neurological congenital malformations it was 4.8 (1.2-19.7), P = 0.01 and for congenital malformations of the circulatory system it was 3.3 (1.5-6.9), P = 0.001. CONCLUSIONS: The incidence of death from congenital malformations or chromosomal anomalies in French Guiana was significantly higher than in mainland France. Explanations for this may be infections, genetic causes, nutritional causes, and toxic causes that are prevalent. There is a need to identify factors that predispose children born in French Guiana to having a higher risk of congenital malformations and chromosomal anomalies.


Assuntos
Anormalidades Congênitas/mortalidade , Anormalidades Cardiovasculares/mortalidade , Transtornos Cromossômicos/mortalidade , Feminino , França/epidemiologia , Guiana Francesa/epidemiologia , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Malformações do Sistema Nervoso/mortalidade
6.
J Endovasc Ther ; 23(5): 783-90, 2016 10.
Artigo em Inglês | MEDLINE | ID: mdl-27324591

RESUMO

PURPOSE: To evaluate the short- and long-term outcomes of hybrid repair of the arch and proximal descending aorta in a single tertiary center for aortic disease. METHODS: A retrospective analysis was performed of 55 patients (median age 67 years; 36 men) who underwent hybrid repair of thoracic aortic pathology with involvement of the arch between January 2005 and May 2015 at a single tertiary center. The pathologies included 40 (73%) with aneurysmal disease, 10 (18%) acute type B aortic dissections, 2 with acute aortic syndrome, an acute type A dissection, and left and aberrant right subclavian artery aneurysms. Seven (13%) procedures were performed as an emergency. Demographics and procedure characteristics were collected for analysis of survival and reinterventions. RESULTS: Complete aortic debranching was performed in 14 (25%) to facilitate endograft placement in zone 0; debranching was partial in 20 (36%) patients for zone 1 deployments and 21 (38%) for zone 2. Primary technical success was achieved in 51 (93%) cases. One patient died in-hospital from aneurysm rupture following aortic debranching prior to stent-graft repair. In another, the stent-graft procedure proved infeasible and was abandoned. The other 2 technical failures were due to type Ia endoleaks. Five (9%) patients died in-hospital (4 of 48 elective and 1 of 7 emergency cases); 2 of these patients died within 30 days (4%). Eight (14%) patients had a stroke, 6 of 48 elective and 2 of the 7 emergency patients. Spinal cord ischemia was reported in 3 (6%) patients. Mean follow-up was 74.6 months. Overall cumulative survival was 70% at 1 year, 68% at 2 years, and 57% at 5 years. Reintervention to the proximal landing zone for type Ia endoleak was required in 6% of cases. The overall rate of aortic reintervention was 18% at 1 year, 21% at 2 years, and 36% at 5 years. Overall extra-anatomic graft patency was 99%. CONCLUSION: Hybrid repair of the aortic arch and proximal descending thoracic aorta is technically feasible, with acceptable short-term mortality. There is a low rate of proximal landing zone reintervention when hybrid techniques are used to create an adequate proximal landing zone. Extra-anatomic bypass grafts have good long-term patency. Ongoing disease progression means that further distal aortic interventions are often necessary in patients with extensive disease.


Assuntos
Aneurisma/cirurgia , Aorta Torácica/cirurgia , Aneurisma da Aorta Torácica/cirurgia , Dissecção Aórtica/cirurgia , Implante de Prótese Vascular , Anormalidades Cardiovasculares/cirurgia , Procedimentos Endovasculares , Artéria Subclávia/anormalidades , Doença Aguda , Adulto , Idoso , Idoso de 80 Anos ou mais , Aneurisma/diagnóstico por imagem , Aneurisma/mortalidade , Dissecção Aórtica/diagnóstico por imagem , Dissecção Aórtica/mortalidade , Aorta Torácica/diagnóstico por imagem , Aneurisma da Aorta Torácica/diagnóstico por imagem , Aneurisma da Aorta Torácica/mortalidade , Aortografia/métodos , Prótese Vascular , Implante de Prótese Vascular/efeitos adversos , Implante de Prótese Vascular/instrumentação , Implante de Prótese Vascular/mortalidade , Anormalidades Cardiovasculares/diagnóstico por imagem , Anormalidades Cardiovasculares/mortalidade , Angiografia por Tomografia Computadorizada , Bases de Dados Factuais , Procedimentos Cirúrgicos Eletivos , Emergências , Procedimentos Endovasculares/efeitos adversos , Procedimentos Endovasculares/instrumentação , Procedimentos Endovasculares/mortalidade , Estudos de Viabilidade , Feminino , Mortalidade Hospitalar , Humanos , Estimativa de Kaplan-Meier , Londres , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/mortalidade , Estudos Retrospectivos , Fatores de Risco , Stents , Artéria Subclávia/diagnóstico por imagem , Artéria Subclávia/cirurgia , Centros de Atenção Terciária , Fatores de Tempo , Resultado do Tratamento
7.
Arq. bras. cardiol ; 106(6): 464-473, tab, graf
Artigo em Inglês | LILACS | ID: lil-787318

RESUMO

Abstract Background: The epidemiological profile of mortality in a population is important for the institution of measures to improve health care and reduce mortality Objective: To estimate mortality rates and the proportional mortality from cardiovascular diseases and malformations of the circulatory system in children and adolescents. Methods: This is a descriptive study of mortality from cardiovascular diseases, malformations of the circulatory system, from all causes, ill-defined causes and external causes in children and adolescents in the state of Rio de Janeiro from 1996 to 2012. Populations were obtained from the Brazilian Institute of Geography and Statistics (Instituto Brasileiro de Geografia e Estatística - IBGE) and deaths obtained from the Department of Informatics of the Unified Health System (DATASUS)/Ministry of Health. Results: There were 115,728 deaths from all causes, 69,757 in males. The annual mortality from cardiovascular diseases was 2.7/100,000 in men and 2.6/100,000 in women. The annual mortality from malformations of the circulatory system was 7.5/100,000 in men and 6.6/100,000 in women. Among the specific causes of circulatory diseases, cardiomyopathies had the highest rates of annual proportional mortality, and from malformations of the circulatory system, it occurred due to unspecified malformations of the circulatory system, at all ages and in both genders. Conclusion: Mortality from malformations of the circulatory system was most striking in the first years of life, while cardiovascular diseases were more relevant in adolescents. Low access to prenatal diagnosis or at birth probably prevented the proper treatment of malformations of the circulatory system.


Resumo Fundamento: Conhecer o perfil epidemiológico de mortalidade de uma população é importante para instituir medidas de melhoria da assistência em saúde e redução da mortalidade. Objetivo: Estimar as taxas de mortalidade e a mortalidade proporcional por doenças e malformações do aparelho circulatório em crianças e adolescentes. Métodos: Estudo descritivo das mortalidades por doenças e malformações do aparelho circulatório, todas as causas, causas mal definidas e causas externas em crianças e adolescentes no Estado do Rio de Janeiro, de 1996 a 2012. As populações foram obtidas no Instituto Brasileiro de Geografia e Estatística (IBGE), e os óbitos no Departamento de Informática do Sistema Único de Saúde (DATASUS)/Ministério da Saúde. Resultados: Ocorreram 115.728 óbitos por todas as causas, sendo 69.757 no sexo masculino. A mortalidade anual por doenças do aparelho circulatório foi 2,7/100mil no sexo masculino e 2,6/100 mil no feminino. A mortalidade anual por malformações do aparelho circulatório foi 7,5/100 mil no sexo masculino e 6,6/100 mil no feminino. Dentre as causas específicas de doenças do aparelho circulatório, as cardiomiopatias apresentaram as maiores taxas de mortalidade proporcional anual e, por malformações do aparelho circulatório, ocorreu por malformações não especificadas do aparelho circulatório, em todas as idades e sexos. Conclusão: A mortalidade por malformações do aparelho circulatório foi mais marcante nos primeiros anos de vida, enquanto as doenças do aparelho circulatório foram mais relevantes nos adolescentes. O baixo acesso ao diagnóstico pré-natal e/ou ao nascimento provavelmente impossibilitou o adequado tratamento das doenças e malformações do aparelho circulatório.


Assuntos
Humanos , Masculino , Feminino , Lactente , Pré-Escolar , Criança , Adolescente , Doenças Cardiovasculares/mortalidade , Brasil/epidemiologia , Causas de Morte , Distribuição por Sexo , Distribuição por Idade , Anormalidades Cardiovasculares/mortalidade , Cardiopatias Congênitas/mortalidade
8.
Cardiovasc J Afr ; 27(2): 84-8, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27213855

RESUMO

Maternal mortality ratio in low- to middle-income countries (LMIC) is 14 times higher than in high-income countries. This is partially due to lack of antenatal care, unmet needs for family planning and education, as well as low rates of birth managed by skilled attendants. While direct causes of maternal death such as complications of hypertension, obstetric haemorrhage and sepsis remain the largest cause of maternal death in LMICs, cardiovascular disease emerges as an important contributor to maternal mortality in both developing countries and the developed world, hampering the achievement of the millennium development goal 5, which aimed at reducing by three-quarters the maternal mortality ratio until the end of 2015. Systematic search for cardiac disease is usually not performed during pregnancy in LMICs despite hypertensive disease, rheumatic heart disease and cardiomyopathies being recognised as major health problems in these settings. New concern has been rising due to both the HIV/AIDS epidemic and the introduction of highly active antiretroviral therapy. Undetected or untreated congenital heart defects, undiagnosed pulmonary hypertension, uncontrolled heart failure and complications of sickle cell disease may also be important challenges. This article discusses issues related to the role of cardiovascular disease in determining a substantial portion of maternal morbidity and mortality. It also presents an algorhitm to be used for suspected and previously known cardiac disease in pregnancy in the context of LIMCs.


Assuntos
Anormalidades Cardiovasculares/mortalidade , Sistema Cardiovascular/patologia , Causas de Morte/tendências , Mortalidade Materna , Cuidado Pré-Natal , Anormalidades Cardiovasculares/patologia , Países em Desenvolvimento , Feminino , Humanos , Gravidez
9.
Arq Bras Cardiol ; 106(6): 464-73, 2016 Jun.
Artigo em Inglês, Português | MEDLINE | ID: mdl-27192384

RESUMO

BACKGROUND: The epidemiological profile of mortality in a population is important for the institution of measures to improve health care and reduce mortality Objective: To estimate mortality rates and the proportional mortality from cardiovascular diseases and malformations of the circulatory system in children and adolescents. METHODS: This is a descriptive study of mortality from cardiovascular diseases, malformations of the circulatory system, from all causes, ill-defined causes and external causes in children and adolescents in the state of Rio de Janeiro from 1996 to 2012. Populations were obtained from the Brazilian Institute of Geography and Statistics (Instituto Brasileiro de Geografia e Estatística - IBGE) and deaths obtained from the Department of Informatics of the Unified Health System (DATASUS)/Ministry of Health. RESULTS: There were 115,728 deaths from all causes, 69,757 in males. The annual mortality from cardiovascular diseases was 2.7/100,000 in men and 2.6/100,000 in women. The annual mortality from malformations of the circulatory system was 7.5/100,000 in men and 6.6/100,000 in women. Among the specific causes of circulatory diseases, cardiomyopathies had the highest rates of annual proportional mortality, and from malformations of the circulatory system, it occurred due to unspecified malformations of the circulatory system, at all ages and in both genders. CONCLUSION: Mortality from malformations of the circulatory system was most striking in the first years of life, while cardiovascular diseases were more relevant in adolescents. Low access to prenatal diagnosis or at birth probably prevented the proper treatment of malformations of the circulatory system.


Assuntos
Doenças Cardiovasculares/mortalidade , Adolescente , Distribuição por Idade , Brasil/epidemiologia , Anormalidades Cardiovasculares/mortalidade , Causas de Morte , Criança , Pré-Escolar , Feminino , Cardiopatias Congênitas/mortalidade , Humanos , Lactente , Masculino , Distribuição por Sexo
10.
Am J Med Genet A ; 170(6): 1520-4, 2016 06.
Artigo em Inglês | MEDLINE | ID: mdl-27028275

RESUMO

VACTERL-H association includes three of eight features: vertebral anomalies, anal atresia, congenital heart disease, tracheo-esophageal fistula, esophageal atresia, renal, limb anomalies, and hydrocephalus. The VACTERL-H phenotype among cases with FA is considered to be about 5%; the frequency of FA among patients with VACTERL-H is unknown. We examined 54 patients with FA in the National Cancer Institute Inherited Bone Marrow Failure Syndrome Cohort for features of VACTERL-H, including imaging studies (radiology and ultrasound). Eighteen of the fifty-four patients had three or more VACTERL-H features. The presence of VACTERL-H association in 33% of those with FA is much higher than the previous estimate of 5% (P < 0.0001). We created the acronym PHENOS (Pigmentation, small Head, small Eyes, central Nervous system (not hydrocephalus), Otology, and Short stature) which includes all major phenotypic features of FA that are not in VACTERL-H; these findings were more frequent in the patients with FA who had VACTERL-H. Identification of any components of the VACTERL-H association should lead to imaging studies, and to consideration of the diagnosis of FA, particularly if the patient has radial ray and renal anomalies, as well as many features of PHENOS. There was no association of the presence or absence of VACTERL-H with development of cancer, stem cell transplant, or survival. Early diagnosis will lead to genetic counseling and early surveillance and management of complications of FA. © 2016 Wiley Periodicals, Inc.


Assuntos
Anormalidades Cardiovasculares/diagnóstico , Anormalidades Cardiovasculares/genética , Anormalidades do Sistema Digestório/diagnóstico , Anormalidades do Sistema Digestório/genética , Anemia de Fanconi/diagnóstico , Anemia de Fanconi/genética , Estudos de Associação Genética , Doenças Genéticas Ligadas ao Cromossomo X/diagnóstico , Doenças Genéticas Ligadas ao Cromossomo X/genética , Hidrocefalia/diagnóstico , Hidrocefalia/genética , Anormalidades Musculoesqueléticas/diagnóstico , Anormalidades Musculoesqueléticas/genética , Fenótipo , Adolescente , Adulto , Anormalidades Cardiovasculares/mortalidade , Criança , Pré-Escolar , Estudos de Coortes , Diagnóstico Diferencial , Anormalidades do Sistema Digestório/mortalidade , Anemia de Fanconi/mortalidade , Proteínas de Grupos de Complementação da Anemia de Fanconi/genética , Feminino , Doenças Genéticas Ligadas ao Cromossomo X/mortalidade , Genótipo , Humanos , Hidrocefalia/mortalidade , Masculino , Pessoa de Meia-Idade , Anormalidades Musculoesqueléticas/mortalidade , Mutação , Síndrome , Adulto Jovem
12.
J Pediatr Surg ; 50(11): 1817-22, 2015 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-26259558

RESUMO

BACKGROUND/PURPOSE: Congenital diaphragmatic hernia (CDH) is associated with high mortality. Survival is influenced by the extent of pulmonary hypoplasia and additional congenital defects. The purpose of this study was to assess the association of congenital anomalies and admission capillary carbon dioxide levels (PcCO2), as a measure of extent of pulmonary hypoplasia, on survival in neonates with CDH. METHODS: This is a retrospective review of neonates with CDH admitted to a tertiary neonatal intensive care unit between 1990 and 2014. Logistic regression was used to assess whether hospital survival was associated with admission PcCO2 or associated anomalies (isolated CDH, CDH with cardiovascular anomalies, and CDH with noncardiac anomalies). The probabilities of survival (POS) score, based on birth weight and 5-min Apgar as defined by the Congenital Diaphragmatic Hernia Study Group were included as a covariate. RESULTS: Of 97 patients, 55 had additional malformations (cardiovascular n=12, noncardiac anomalies n=43). POS was lower in CDH with other anomalies compared to isolated CDH. Survival rate was 61.9%, 53.5% and 41.7% in isolated CDH, CDH with noncardiac anomalies and CDH with cardiovascular anomalies, respectively. After adjusting for POS score the likelihood of survival in CDH groups with additional anomalies was similar to isolated CDH (OR 0.95, 95% CI 0.22-4.15, and 1.10, 0.39-3.08, for CDH with and without cardiovascular anomalies, respectively). After adjusting for POS score, lower PcCO2 levels (OR=1.25 per 5mmHg decrease, P=0.003) were associated with better survival. CONCLUSIONS: Neonates with CDH have a high prevalence of congenital malformations. However, after adjusting for POS score the presence of additional anomalies was not associated with survival. The POS score and admission PcCO2 were important prognosticating factors for survival.


Assuntos
Anormalidades Cardiovasculares/mortalidade , Hérnias Diafragmáticas Congênitas/mortalidade , Pulmão/anormalidades , Peso ao Nascer , Dióxido de Carbono/sangue , Anormalidades Cardiovasculares/complicações , Feminino , Hérnias Diafragmáticas Congênitas/sangue , Hérnias Diafragmáticas Congênitas/complicações , Humanos , Recém-Nascido , Modelos Logísticos , Masculino , Probabilidade , Estudos Retrospectivos , Taxa de Sobrevida
13.
Ann Thorac Surg ; 99(5): 1639-46; discussion 1646-7, 2015 May.
Artigo em Inglês | MEDLINE | ID: mdl-25818570

RESUMO

BACKGROUND: We report our current-era results after placement of a modified Blalock-Taussig shunt (mBTS) as the first surgery in the multistage palliation strategy of neonates born with single ventricle (SV) anomalies associated with restrictive pulmonary blood flow. METHODS: Between 2002 and 2012, 174 neonates with SV underwent mBTS. Competing risks analysis modeled events after mBTS (death or transplantation, transition to Glenn) and subsequently after Glenn (death or transplantation, transition to Fontan) and examined risk factors affecting outcomes. RESULTS: Competing risks analysis showed that 2 years after mBTS 27% of patients had died or received transplantation and 73% had undergone Glenn. Five years after Glenn, 6% had died, 73% had undergone Fontan, and 21% were alive awaiting Fontan. Overall survival 8 years after mBTS was 68%. On multivariable analysis, risk factors for mortality were increased shunt size to weight ratio (hazard ratio [HR]:1.2 per 0.1 ratio increase [1.0 to 1.4], p = 0.015), postoperative extracorporeal membrane oxygenation (HR: 4.0 [2.2 to 7.4], p < 0.001), chromosomal and extracardiac malformations (HR: 2.5 [1.3 to 5.0], p = 0.008), cardiopulmonary bypass use (HR: 2.5 [1.3 to 4.5], p = 0.004), and underlying pulmonary atresia with intact ventricular septum (PAIVS) or atrial isomerism (HR: 2.1 [1.1 to 4.2], p = 0.035). CONCLUSIONS: Palliation outcomes with mBTS in SV patients trail behind the generally improved results of congenital heart surgery. Several anatomic and patient-related factors continue to affect survival. Mortality is high in low weight neonates in whom a balance between overcirculation due to large shunt and low pulmonary blood flow and risk of occlusion due to small shunt is difficult to attain. Patients with chromosomal and extracardiac malformations, atrial isomerism, and PAIVS continue to be the most challenging and are associated with higher hospital and interstage mortality.


Assuntos
Procedimento de Blalock-Taussig/métodos , Anormalidades Cardiovasculares/mortalidade , Anormalidades Cardiovasculares/cirurgia , Ventrículos do Coração/anormalidades , Cuidados Paliativos , Anormalidades Cardiovasculares/complicações , Estudos de Coortes , Feminino , Técnica de Fontan , Transplante de Coração , Humanos , Recém-Nascido , Masculino , Circulação Pulmonar , Fatores de Risco , Taxa de Sobrevida , Resultado do Tratamento
14.
Cardiol Young ; 25(3): 539-43, 2015 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-24666999

RESUMO

BACKGROUND: Of the children with Down syndrome 40-50% have cardiac defects and the majority of these cardiac defects are amenable to biventricular repair. The outcome of single ventricle palliation is improving; nonetheless, there are limited data on Down syndrome patients with associated high-risk factors undergoing single ventricle palliation. Our aim was to study the outcomes of children with Down syndrome and high-risk factors on the single ventricle palliation pathway. METHODS: A retrospective study on all patients with Down syndrome on the single ventricle palliation pathway from 2005 until 2011 was conducted. Operative, clinical, echocardiographic, haemodynamic data, and follow-up data were reviewed. RESULTS: A total of 310 patients underwent at least one single ventricle surgical intervention. Of those, eight patients had Down syndrome, five of which had associated risk factors - low birth weight, high pulmonary vascular resistance, pulmonary vein stenosis, significant atrioventricular valve regurgitation, and extracardiac anomalies. Mortality in the high-risk group was 80% (4/5), compared with 33% (1/3) in the non-high-risk patients. Overall, after a median follow-up period of 138 days (8-576 days), only 37.5% (3/8) of patients were alive. CONCLUSION: Despite many improvements in the care of single ventricle patients, the fate of those with Down syndrome and associated high-risk factors remains poor. Further multicentre longer-term studies are needed to validate and quantify the cumulative effects of negative prognostic factors in this complex group of patients.


Assuntos
Anormalidades Cardiovasculares/mortalidade , Anormalidades Cardiovasculares/cirurgia , Síndrome de Down/complicações , Técnica de Fontan/métodos , Ventrículos do Coração/anormalidades , Cuidados Paliativos/métodos , Anormalidades Cardiovasculares/diagnóstico por imagem , Anormalidades Cardiovasculares/etiologia , Síndrome de Down/mortalidade , Síndrome de Down/patologia , Síndrome de Down/fisiopatologia , Ecocardiografia , Feminino , Seguimentos , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/cirurgia , Hemodinâmica , Humanos , Lactente , Recém-Nascido de Baixo Peso , Recém-Nascido , Masculino , Circulação Pulmonar , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento , Resistência Vascular
15.
Int J Occup Med Environ Health ; 27(3): 474-86, 2014 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-24847732

RESUMO

OBJECTIVES: To investigate the association between per capita pesticide consumption and infant mortality rates from CNS and CVS congenital malformations in microregions in the South and Southeast Region of Brazil. MATERIAL AND METHODS: An ecological study was conducted using data on pesticide expenditure in 1985 and 1996, and deaths caused by CNS and CVS malformations in infants under 1 year old in 1986-1990 and 1997-2001, respectively. Per capita pesticide consumption and infant mortality rates were calculated for each microregion. Microregions were grouped according to quintiles of pesticide consumption, taking the first quintile as reference. The association between pesticide consumption and infant mortality was examined by calculating Spearman correlation coefficients (r) and mortality rate ratios (RR), stratifying by gender and type of microregion (urban or rural). RESULTS: Significant and positive correlations between per capita pesticide consumption and rates of mortality due to CNS and CVS defects were observed in rural but not urban microregions. In general, mortality RRs for the 2 types of malformations in rural microregions were significantly higher in each quintile of pesticide consumption compared to the lowest quintile in the 2 study periods, with elevations ranging between 10% and 30%. Likewise, mortality RRs in these microregions showed significant trends of increase across quintiles of pesticide consumption in both study periods. In urban areas, however, mortality RRs from both CNS and CVS malformations were weak and not statistically significant, and a trend of increase of mortality with increasing pesticide usage was not observed. CONCLUSIONS: The results show the relevance of pesticide exposure in rural areas with intense agricultural activity, suggesting that such prenatal exposures may be related with the occurrence of certain congenital defects.


Assuntos
Anormalidades Cardiovasculares/mortalidade , Sistema Nervoso Central/anormalidades , Exposição Ambiental/efeitos adversos , Mortalidade Infantil/tendências , Praguicidas/toxicidade , Brasil/epidemiologia , Anormalidades Cardiovasculares/induzido quimicamente , Humanos , Lactente , Recém-Nascido , Razão de Chances , Praguicidas/economia , População Rural/estatística & dados numéricos , População Urbana/estatística & dados numéricos
16.
Rev. Soc. Cardiol. Estado de Säo Paulo ; 24(1): 52-55, jan.-mar. 2014. tab, graf
Artigo em Português | LILACS | ID: lil-729293

RESUMO

O objetivo principal da avaliação pré-participação em atividades esportivas (APP) é o de identificar doenças cardiovasculares que sejam compatíveis com a prática de esportes competitivos, tanto por profissionais como amadores, minimizando, assim, a progressão de anormalidades cardiovasculares e a ocorrência de morte súbita relacionada ao exercício. A maioria das sociedades de Cardiologia e Medicina do Esporte são concordantes em recomendar a APP para todos os atletas profissionais, apesar das divergências sobre o tema persistirem entre os maiores grupos de especialistas no assunto. Esse artigo se propõe avaliar a viabilidade da realização da APP no contexto brasileiro, embasada nas evidências científicas disponíveis atualmente e considerando a logística necessária para sua implementação.


The main objective of the sports pre-participation evaluation (PPE) is to identify cardiovascular diseases that are inconsistente with the practice of competitive sports, both professionally and non-professionally, aiming at preventing the progress of cardiovascular diseases (CVDs) and sudden death (SD). Most cardiology and sports medicine societes agree in recommending the PPE for all professional athletes, although there are disagreements among the biggest groups of experts in this matter. The purpose of this article is to evaluate the feasibility of the PPE in the Brazilian context, establishing a connection with the current scientific evidence that supports this practice.


Assuntos
Humanos , Masculino , Feminino , Criança , Anormalidades Cardiovasculares/mortalidade , Aptidão Física/fisiologia , Doenças Cardiovasculares/mortalidade , Exercício Físico/fisiologia , Análise Custo-Benefício , Atletas , Avaliação em Saúde/métodos , Exame Físico/métodos , Guias como Assunto/normas , Morte Súbita/prevenção & controle
17.
Arq Neuropsiquiatr ; 71(10): 807-10, 2013 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-24212520

RESUMO

It is extremely difficult to estimate the occurrence of sudden unexpected death in epilepsy (SUDEP). On the other hand, discovering and carefully evaluating new risk factors that may contribute to the onset of cardiovascular abnormalities in people with refractory epilepsy may prevent fatal events in these individuals. In this context, we should not ignore that urban air pollution is a leading problem for environmental health and is able to cause serious cardiovascular dysfunctions that culminate in sudden death. In this regard, we aimed to determine whether environmental exposure to air pollution is an aggravating event for SUDEP.


Assuntos
Poluição do Ar/efeitos adversos , Morte Súbita/etiologia , Epilepsia/mortalidade , Anormalidades Cardiovasculares/mortalidade , Exposição Ambiental/efeitos adversos , Epilepsia/prevenção & controle , Ácidos Graxos Ômega-3/administração & dosagem , Humanos , Fatores de Risco
18.
Biomed Res Int ; 2013: 796586, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-24102059

RESUMO

Obestatin, a proposed anorexigenic gut hormone, has been shown to have a number of beneficial cardiotropic effects in experimental studies. We hypothesized that obestatin alteration in hemodialysis patients may link to clinical outcomes. This cross-sectional study with prospective followup for almost 4 years was performed on 94 prevalent hemodialysis patients. Obestatin, leptin, proinflammatory cytokines (tumor necrosis factor-α [TNF-α], interleukin-6, and various nutritional markers were measured. Patients with low obestatin levels, defined as a level less than median, had a worse all-cause mortality and cardiovascular mortality. The crude all-cause (HR 2.23, 95% CI 1.17 to 4.24) and cardiovascular mortality hazard ratios (HR 4.03, 95% CI 1.27 to 12.76) in these patients continued to be significant after adjustment for various confounders for all-cause mortality. Across the four obestatin-TNF-α categories, the group with low obestatin and high TNF-α (above median level) exhibited a worse outcome in both all-cause mortality and cardiovascular mortality. Clinical characteristics of patients in low obestatin high TNF-α group did not differ from other obestatin-TNF-α categorized groups. In summary, low serum obestatin concentration is an independent predictor of mortality in prevalent hemodialysis patients. Novel interactions were observed between obestatin and TNF-α, which were associated with mortality risk, especially those due to cardiovascular causes.


Assuntos
Anormalidades Cardiovasculares/mortalidade , Grelina/sangue , Diálise Renal/mortalidade , Fator de Necrose Tumoral alfa/sangue , Biomarcadores/sangue , Anormalidades Cardiovasculares/sangue , Anormalidades Cardiovasculares/complicações , Feminino , Humanos , Inflamação/sangue , Inflamação/complicações , Interleucina-6/sangue , Falência Renal Crônica/sangue , Falência Renal Crônica/complicações , Falência Renal Crônica/mortalidade , Leptina/sangue , Masculino , Pessoa de Meia-Idade
19.
Arq. neuropsiquiatr ; 71(10): 807-810, out. 2013.
Artigo em Inglês | LILACS | ID: lil-689785

RESUMO

It is extremely difficult to estimate the occurrence of sudden unexpected death in epilepsy (SUDEP). On the other hand, discovering and carefully evaluating new risk factors that may contribute to the onset of cardiovascular abnormalities in people with refractory epilepsy may prevent fatal events in these individuals. In this context, we should not ignore that urban air pollution is a leading problem for environmental health and is able to cause serious cardiovascular dysfunctions that culminate in sudden death. In this regard, we aimed to determine whether environmental exposure to air pollution is an aggravating event for SUDEP.


É extremamente difícil estimar a ocorrência de morte súbita em epilepsia (SUDEP). Por outro lado, detectar e avaliar cuidadosamente novos factores de risco que podem contribuir para o aparecimento de alterações cardiovasculares em pessoas com epilepsia refratária poderá ser capaz de impedir a ocorrência de eventos fatais nestes indivíduos. Neste contexto, não devemos negligenciar hoje que a poluição do ar nas grandes cidades é um problema para a saúde ambiental, podendo causar graves disfunções cardiovasculares, que culminam em morte súbita. Neste sentido, propusemos nesse trabalho que a exposição ambiental a poluição do ar é um evento agravante para a ocorrência de SUDEP.


Assuntos
Humanos , Poluição do Ar/efeitos adversos , Morte Súbita/etiologia , Epilepsia/mortalidade , Anormalidades Cardiovasculares/mortalidade , Exposição Ambiental/efeitos adversos , Epilepsia/prevenção & controle , /administração & dosagem , Fatores de Risco
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA