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1.
J Vasc Surg ; 72(6): 1873-1882, 2020 12.
Artículo en Inglés | MEDLINE | ID: mdl-32665182

RESUMEN

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.


Asunto(s)
Aorta Torácica/cirugía , Implantación de Prótesis Vascular , Anomalías Cardiovasculares/cirugía , Procedimientos Endovasculares , Arteria Subclavia/anomalías , Anciano , Aorta Torácica/anomalías , Aorta Torácica/diagnóstico por imagen , Implantación de Prótesis Vascular/efectos adversos , Implantación de Prótesis Vascular/mortalidad , Anomalías Cardiovasculares/diagnóstico por imagen , Anomalías Cardiovasculares/mortalidad , Procedimientos Endovasculares/efectos adversos , Procedimientos Endovasculares/mortalidad , Femenino , Humanos , Masculino , Persona de Mediana Edad , Complicaciones Posoperatorias/etiología , Sistema de Registros , Estudios Retrospectivos , Medición de Riesgo , Factores de Riesgo , Arteria Subclavia/diagnóstico por imagen , Arteria Subclavia/cirugía , Factores de Tiempo , Resultado del Tratamiento
2.
J Vasc Interv Radiol ; 30(9): 1378-1385, 2019 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-31133449

RESUMEN

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.


Asunto(s)
Aneurisma de la Aorta/cirugía , Disección Aórtica/cirugía , Implantación de Prótesis Vascular , Anomalías Cardiovasculares/cirugía , Procedimientos Endovasculares , Arteria Subclavia/anomalías , Adulto , Anciano , Disección Aórtica/diagnóstico por imagen , Disección Aórtica/mortalidad , Disección Aórtica/fisiopatología , Aneurisma de la Aorta/diagnóstico por imagen , Aneurisma de la Aorta/mortalidad , Aneurisma de la Aorta/fisiopatología , Prótesis Vascular , Implantación de Prótesis Vascular/efectos adversos , Implantación de Prótesis Vascular/instrumentación , Implantación de Prótesis Vascular/mortalidad , Anomalías Cardiovasculares/diagnóstico por imagen , Anomalías Cardiovasculares/mortalidad , Anomalías Cardiovasculares/fisiopatología , Procedimientos Endovasculares/efectos adversos , Procedimientos Endovasculares/instrumentación , Procedimientos Endovasculares/mortalidad , Femenino , Humanos , Masculino , Persona de Mediana Edad , Tempo Operativo , Complicaciones Posoperatorias/etiología , Diseño de Prótesis , Estudios Retrospectivos , Factores de Riesgo , Stents , Arteria Subclavia/diagnóstico por imagen , Arteria Subclavia/fisiopatología , Arteria Subclavia/cirugía , Factores de Tiempo , Resultado del Tratamiento
3.
Rev Saude Publica ; 53: 31, 2019 Apr 01.
Artículo en Inglés | MEDLINE | ID: mdl-30942273

RESUMEN

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.


Asunto(s)
Enfermedades Cardiovasculares/etiología , Enfermedades Cardiovasculares/mortalidad , Mortalidad Infantil , Adolescente , Certificado de Nacimiento , Brasil , Anomalías Cardiovasculares/mortalidad , Causas de Muerte , Niño , Preescolar , Femenino , Cardiopatías Congénitas/mortalidad , Humanos , Lactante , Recién Nacido de Bajo Peso , Recién Nacido , Estudios Longitudinales , Masculino , Edad Materna , Factores de Riesgo
4.
Rev. saúde pública (Online) ; 53: 31, jan. 2019. tab, graf
Artículo en Inglés | LILACS | ID: biblio-991636

RESUMEN

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.


Asunto(s)
Humanos , Masculino , Femenino , Recién Nacido , Lactante , Preescolar , Niño , Adolescente , Recién Nacido de Bajo Peso , Enfermedades Cardiovasculares/etiología , Enfermedades Cardiovasculares/mortalidad , Brasil , Certificado de Nacimiento , Mortalidad Infantil , Factores de Riesgo , Estudios Longitudinales , Causas de Muerte , Edad Materna , Anomalías Cardiovasculares/mortalidad , Cardiopatías Congénitas/mortalidad
5.
BMC Pediatr ; 18(1): 393, 2018 12 22.
Artículo en Inglés | MEDLINE | ID: mdl-30579351

RESUMEN

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.


Asunto(s)
Anomalías Congénitas/mortalidad , Anomalías Cardiovasculares/mortalidad , Trastornos de los Cromosomas/mortalidad , Femenino , Francia/epidemiología , Guyana Francesa/epidemiología , Humanos , Incidencia , Lactante , Recién Nacido , Masculino , Malformaciones del Sistema Nervioso/mortalidad
6.
J Endovasc Ther ; 23(5): 783-90, 2016 10.
Artículo en Inglés | MEDLINE | ID: mdl-27324591

RESUMEN

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.


Asunto(s)
Aneurisma/cirugía , Aorta Torácica/cirugía , Aneurisma de la Aorta Torácica/cirugía , Disección Aórtica/cirugía , Implantación de Prótesis Vascular , Anomalías Cardiovasculares/cirugía , Procedimientos Endovasculares , Arteria Subclavia/anomalías , Enfermedad Aguda , Adulto , Anciano , Anciano de 80 o más Años , Aneurisma/diagnóstico por imagen , Aneurisma/mortalidad , Disección Aórtica/diagnóstico por imagen , Disección Aórtica/mortalidad , Aorta Torácica/diagnóstico por imagen , Aneurisma de la Aorta Torácica/diagnóstico por imagen , Aneurisma de la Aorta Torácica/mortalidad , Aortografía/métodos , Prótesis Vascular , Implantación de Prótesis Vascular/efectos adversos , Implantación de Prótesis Vascular/instrumentación , Implantación de Prótesis Vascular/mortalidad , Anomalías Cardiovasculares/diagnóstico por imagen , Anomalías Cardiovasculares/mortalidad , Angiografía por Tomografía Computarizada , Bases de Datos Factuales , Procedimientos Quirúrgicos Electivos , Urgencias Médicas , Procedimientos Endovasculares/efectos adversos , Procedimientos Endovasculares/instrumentación , Procedimientos Endovasculares/mortalidad , Estudios de Factibilidad , Femenino , Mortalidad Hospitalaria , Humanos , Estimación de Kaplan-Meier , Londres , Masculino , Persona de Mediana Edad , Complicaciones Posoperatorias/mortalidad , Estudios Retrospectivos , Factores de Riesgo , Stents , Arteria Subclavia/diagnóstico por imagen , Arteria Subclavia/cirugía , Centros de Atención Terciaria , Factores de Tiempo , Resultado del Tratamiento
7.
Arq. bras. cardiol ; 106(6): 464-473, tab, graf
Artículo en Inglés | LILACS | ID: lil-787318

RESUMEN

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.


Asunto(s)
Humanos , Masculino , Femenino , Lactante , Preescolar , Niño , Adolescente , Enfermedades Cardiovasculares/mortalidad , Brasil/epidemiología , Causas de Muerte , Distribución por Sexo , Distribución por Edad , Anomalías Cardiovasculares/mortalidad , Cardiopatías Congénitas/mortalidad
8.
Cardiovasc J Afr ; 27(2): 84-8, 2016.
Artículo en Inglés | MEDLINE | ID: mdl-27213855

RESUMEN

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.


Asunto(s)
Anomalías Cardiovasculares/mortalidad , Sistema Cardiovascular/patología , Causas de Muerte/tendencias , Mortalidad Materna , Atención Prenatal , Anomalías Cardiovasculares/patología , Países en Desarrollo , Femenino , Humanos , Embarazo
9.
Arq Bras Cardiol ; 106(6): 464-73, 2016 Jun.
Artículo en Inglés, Portugués | MEDLINE | ID: mdl-27192384

RESUMEN

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.


Asunto(s)
Enfermedades Cardiovasculares/mortalidad , Adolescente , Distribución por Edad , Brasil/epidemiología , Anomalías Cardiovasculares/mortalidad , Causas de Muerte , Niño , Preescolar , Femenino , Cardiopatías Congénitas/mortalidad , Humanos , Lactante , Masculino , Distribución por Sexo
10.
Am J Med Genet A ; 170(6): 1520-4, 2016 06.
Artículo en Inglés | MEDLINE | ID: mdl-27028275

RESUMEN

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.


Asunto(s)
Anomalías Cardiovasculares/diagnóstico , Anomalías Cardiovasculares/genética , Anomalías del Sistema Digestivo/diagnóstico , Anomalías del Sistema Digestivo/genética , Anemia de Fanconi/diagnóstico , Anemia de Fanconi/genética , Estudios de Asociación Genética , Enfermedades Genéticas Ligadas al Cromosoma X/diagnóstico , Enfermedades Genéticas Ligadas al Cromosoma X/genética , Hidrocefalia/diagnóstico , Hidrocefalia/genética , Anomalías Musculoesqueléticas/diagnóstico , Anomalías Musculoesqueléticas/genética , Fenotipo , Adolescente , Adulto , Anomalías Cardiovasculares/mortalidad , Niño , Preescolar , Estudios de Cohortes , Diagnóstico Diferencial , Anomalías del Sistema Digestivo/mortalidad , Anemia de Fanconi/mortalidad , Proteínas del Grupo de Complementación de la Anemia de Fanconi/genética , Femenino , Enfermedades Genéticas Ligadas al Cromosoma X/mortalidad , Genotipo , Humanos , Hidrocefalia/mortalidad , Masculino , Persona de Mediana Edad , Anomalías Musculoesqueléticas/mortalidad , Mutación , Síndrome , Adulto Joven
12.
J Pediatr Surg ; 50(11): 1817-22, 2015 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-26259558

RESUMEN

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.


Asunto(s)
Anomalías Cardiovasculares/mortalidad , Hernias Diafragmáticas Congénitas/mortalidad , Pulmón/anomalías , Peso al Nacer , Dióxido de Carbono/sangre , Anomalías Cardiovasculares/complicaciones , Femenino , Hernias Diafragmáticas Congénitas/sangre , Hernias Diafragmáticas Congénitas/complicaciones , Humanos , Recién Nacido , Modelos Logísticos , Masculino , Probabilidad , Estudios Retrospectivos , Tasa de Supervivencia
13.
Ann Thorac Surg ; 99(5): 1639-46; discussion 1646-7, 2015 May.
Artículo en Inglés | MEDLINE | ID: mdl-25818570

RESUMEN

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.


Asunto(s)
Procedimiento de Blalock-Taussing/métodos , Anomalías Cardiovasculares/mortalidad , Anomalías Cardiovasculares/cirugía , Ventrículos Cardíacos/anomalías , Cuidados Paliativos , Anomalías Cardiovasculares/complicaciones , Estudios de Cohortes , Femenino , Procedimiento de Fontan , Trasplante de Corazón , Humanos , Recién Nacido , Masculino , Circulación Pulmonar , Factores de Riesgo , Tasa de Supervivencia , Resultado del Tratamiento
14.
Cardiol Young ; 25(3): 539-43, 2015 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-24666999

RESUMEN

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.


Asunto(s)
Anomalías Cardiovasculares/mortalidad , Anomalías Cardiovasculares/cirugía , Síndrome de Down/complicaciones , Procedimiento de Fontan/métodos , Ventrículos Cardíacos/anomalías , Cuidados Paliativos/métodos , Anomalías Cardiovasculares/diagnóstico por imagen , Anomalías Cardiovasculares/etiología , Síndrome de Down/mortalidad , Síndrome de Down/patología , Síndrome de Down/fisiopatología , Ecocardiografía , Femenino , Estudios de Seguimiento , Cardiopatías Congénitas/mortalidad , Cardiopatías Congénitas/cirugía , Hemodinámica , Humanos , Lactante , Recién Nacido de Bajo Peso , Recién Nacido , Masculino , Circulación Pulmonar , Estudios Retrospectivos , Factores de Riesgo , Resultado del Tratamiento , Resistencia Vascular
15.
Int J Occup Med Environ Health ; 27(3): 474-86, 2014 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-24847732

RESUMEN

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.


Asunto(s)
Anomalías Cardiovasculares/mortalidad , Sistema Nervioso Central/anomalías , Exposición a Riesgos Ambientales/efectos adversos , Mortalidad Infantil/tendencias , Plaguicidas/toxicidad , Brasil/epidemiología , Anomalías Cardiovasculares/inducido químicamente , Humanos , Lactante , Recién Nacido , Oportunidad Relativa , Plaguicidas/economía , Población Rural/estadística & datos numéricos , Población Urbana/estadística & datos numéricos
16.
Rev. Soc. Cardiol. Estado de Säo Paulo ; 24(1): 52-55, jan.-mar. 2014. tab, graf
Artículo en Portugués | LILACS | ID: lil-729293

RESUMEN

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.


Asunto(s)
Humanos , Masculino , Femenino , Niño , Anomalías Cardiovasculares/mortalidad , Aptitud Física/fisiología , Enfermedades Cardiovasculares/mortalidad , Ejercicio Físico/fisiología , Análisis Costo-Beneficio , Atletas , Evaluación en Salud/métodos , Examen Físico/métodos , Guías como Asunto/normas , Muerte Súbita/prevención & control
17.
Arq Neuropsiquiatr ; 71(10): 807-10, 2013 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-24212520

RESUMEN

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.


Asunto(s)
Contaminación del Aire/efectos adversos , Muerte Súbita/etiología , Epilepsia/mortalidad , Anomalías Cardiovasculares/mortalidad , Exposición a Riesgos Ambientales/efectos adversos , Epilepsia/prevención & control , Ácidos Grasos Omega-3/administración & dosificación , Humanos , Factores de Riesgo
18.
Biomed Res Int ; 2013: 796586, 2013.
Artículo en Inglés | MEDLINE | ID: mdl-24102059

RESUMEN

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.


Asunto(s)
Anomalías Cardiovasculares/mortalidad , Ghrelina/sangre , Diálisis Renal/mortalidad , Factor de Necrosis Tumoral alfa/sangre , Biomarcadores/sangre , Anomalías Cardiovasculares/sangre , Anomalías Cardiovasculares/complicaciones , Femenino , Humanos , Inflamación/sangre , Inflamación/complicaciones , Interleucina-6/sangre , Fallo Renal Crónico/sangre , Fallo Renal Crónico/complicaciones , Fallo Renal Crónico/mortalidad , Leptina/sangre , Masculino , Persona de Mediana Edad
19.
Arq. neuropsiquiatr ; 71(10): 807-810, out. 2013.
Artículo en Inglés | LILACS | ID: lil-689785

RESUMEN

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.


Asunto(s)
Humanos , Contaminación del Aire/efectos adversos , Muerte Súbita/etiología , Epilepsia/mortalidad , Anomalías Cardiovasculares/mortalidad , Exposición a Riesgos Ambientales/efectos adversos , Epilepsia/prevención & control , /administración & dosificación , Factores de Riesgo
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