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3.
Congenit Heart Dis ; 12(6): 733-739, 2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-28703367

RESUMO

OBJECTIVE: We sought to identify a time during cardiac ejection when the instantaneous pressure gradient (IPG) correlated best, and near unity, with peak-to-peak systolic ejection gradient (PPSG) in patients with congenital aortic stenosis. Noninvasive echocardiographic measurement of IPG has limited correlation with cardiac catheterization measured PPSG across the spectrum of disease severity of congenital aortic stenosis. A major contributor is the observation that these measures are inherently different with a variable relationship dependent on the degree of stenosis. DESIGN: Hemodynamic data from cardiac catheterizations utilizing simultaneous pressure measurements from the left ventricle (LV) and ascending aorta (AAo) in patients with congenital valvar aortic stenosis was retrospectively reviewed over the past 5 years. The cardiac cycle was standardized for all patients using the percentage of total LV ejection time (ET). Instantaneous gradient at 5% intervals of ET were compared to PPSG using linear regression and Bland-Altman analysis. RESULTS: A total of 22 patients underwent catheterization at a median age of 13.7 years (interquartile range [IQR] 10.3-18.0) and median weight of 51.1 kg (IQR 34.2-71.6). The PPSG was 46.5 ± 12.6 mm Hg (mean ± SD) and correlated suboptimally with the maximum and mean IPG. The midsystolic IPG (occurring at 50% of ET) had the strongest correlation with the PPSG ( PPSG = 0.97(IPG50%)-1.12, R2 = 0.88), while the IPG at 55% of ET was closest to unity ( PPSG = 0.997(IPG55%)-1.17, R2 = 0.87). CONCLUSIONS: The commonly measured maximum and mean IPG are suboptimal estimates of the PPSG in congenital aortic stenosis. Using catheter-based data, IPG at 50%-55% of ejection correlates well with PPSG. This may allow for a more accurate estimation of PPSG via noninvasive assessment of IPG.


Assuntos
Aorta/fisiopatologia , Estenose da Valva Aórtica/congênito , Pressão Sanguínea/fisiologia , Cateterismo Cardíaco/métodos , Ventrículos do Coração/fisiopatologia , Volume Sistólico/fisiologia , Função Ventricular Esquerda/fisiologia , Adolescente , Aorta/diagnóstico por imagem , Estenose da Valva Aórtica/diagnóstico , Estenose da Valva Aórtica/fisiopatologia , Criança , Ecocardiografia , Feminino , Seguimentos , Ventrículos do Coração/diagnóstico por imagem , Humanos , Masculino , Prognóstico , Estudos Retrospectivos , Sístole , Adulto Jovem
4.
Pediatrics ; 135(4): e939-44, 2015 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-25780068

RESUMO

BACKGROUND: Rheumatic heart disease (RHD) remains a major public health concern in developing countries, and routine screening has the potential to improve outcomes. Standard portable echocardiography (STAND) is far more sensitive than auscultation for the detection of RHD but remains cost-prohibitive in resource-limited settings. Handheld echocardiography (HAND) is a lower-cost alternative. The purpose of this study was to assess the incremental value of HAND over auscultation to identify RHD. METHODS: RHD screening was completed for schoolchildren in Gulu, Uganda, by using STAND performed by experienced echocardiographers. Any child with mitral or aortic regurgitation or stenosis plus a randomly selected group of children with normal STAND findings underwent HAND and auscultation. STAND and HAND studies were interpreted by 6 experienced cardiologists using the 2012 World Heart Federation criteria. Sensitivity and specificity of HAND and auscultation for the detection of RHD and pathologic mitral or aortic regurgitation were calculated by using STAND as the gold standard. RESULTS: Of 4773 children who underwent screening with STAND, a subgroup of 1317 children underwent HAND and auscultation. Auscultation had uniformly poor sensitivity for the detection of RHD or valve disease. Sensitivity was significantly improved by using HAND compared with auscultation for the detection of definite RHD (97.8% vs 22.2%), borderline or definite RHD (78.4% vs 16.4%), and pathologic aortic insufficiency (81.8% vs 13.6%). CONCLUSIONS: Auscultation alone is a poor screening test for RHD. HAND significantly improves detection of RHD and may be a cost-effective screening strategy for RHD in resource-limited settings.


Assuntos
Países em Desenvolvimento , Ecocardiografia/instrumentação , Auscultação Cardíaca , Sistemas Automatizados de Assistência Junto ao Leito , Cardiopatia Reumática/diagnóstico , Adolescente , Criança , Pré-Escolar , Análise Custo-Benefício , Ecocardiografia/economia , Ecocardiografia Doppler em Cores/instrumentação , Feminino , Auscultação Cardíaca/economia , Doenças das Valvas Cardíacas/diagnóstico , Doenças das Valvas Cardíacas/economia , Humanos , Masculino , Programas de Rastreamento/economia , Sistemas Automatizados de Assistência Junto ao Leito/economia , Cardiopatia Reumática/economia , Sensibilidade e Especificidade , Uganda
5.
J Am Soc Echocardiogr ; 28(4): 463-9, 2015 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-25660669

RESUMO

BACKGROUND: Using 2012 World Heart Federation criteria, standard portable echocardiography (STAND) reveals a high burden of rheumatic heart disease (RHD) in resource-poor settings, but widespread screening is limited by cost and physician availability. Handheld echocardiography (HAND) may decrease costs, but World Heart Federation criteria are complicated for rapid field screening, particularly for nonphysician screeners. The aim of this study was to determine the best simplified screening strategy for RHD detection using HAND. METHODS: In this prospective study, STAND (GE Vivid q or i or Philips CX-50) was performed in five schools in Gulu, Uganda; a random subset plus all children with detectable mitral regurgitation or aortic insufficiency also underwent HAND (GE Vscan). Borderline or definite RHD cases were defined by 2012 World Heart Federation criteria on STAND images, by two experienced readers. HAND studies were reviewed by cardiologists blinded to STAND results. Single and combined HAND parameters were evaluated to determine the simplified screening strategy that maximized sensitivity and specificity for case detection. RESULTS: In 1,439 children (mean age, 10.8 ± 2.6 years; 47% male) with HAND and STAND studies, morphologic criteria and the presence of any mitral regurgitation by HAND had poor specificity. The presence of aortic insufficiency was specific but not sensitive. Combined criteria of mitral regurgitation jet length ≥ 1.5 cm or any aortic insufficiency best balanced sensitivity (73.3%) and specificity (82.4%), with excellent sensitivity for definite RHD (97.9%). With a prevalence of 4% and subsequent STAND screening of positive HAND studies, this would reduce STAND studies by 80% from a STAND-based screening strategy. CONCLUSIONS: In resource-limited settings, HAND with simplified criteria can detect RHD with good sensitivity and specificity and decrease the need for standard echocardiography. Further study is needed to validate screening by local practitioners and long-term outcomes.


Assuntos
Algoritmos , Ecocardiografia/métodos , Doenças das Valvas Cardíacas/diagnóstico por imagem , Programas de Rastreamento/métodos , Posicionamento do Paciente/métodos , Cardiopatia Reumática/diagnóstico por imagem , Criança , Ecocardiografia/instrumentação , Feminino , Humanos , Masculino , Miniaturização , Sistemas Automatizados de Assistência Junto ao Leito , Reprodutibilidade dos Testes , Sensibilidade e Especificidade , Uganda
7.
Paediatr Respir Rev ; 15(1): 4-12, 2014 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-24361300

RESUMO

Sickle cell disease (SCD), caused by a mutation in the ß-globin gene HBB, is widely distributed in malaria endemic regions. Cardiopulmonary complications are major causes of morbidity and mortality. Hemoglobin SS (Hb SS) represents a large proportion of SCD in the Americas, United Kingdom, and certain regions of Africa while higher proportions of hemoglobin SC are observed in Burkina Faso and hemoglobin Sß-thalassemia in Greece and India. Coinheritance of α-thalassemia and persistence of hemoglobin F production are observed in highest frequency in certain regions of India and the Middle East. As confirmed in the PUSH and Walk-PHaSST studies, Hb SS, absence of co-inheriting alpha-thalassemia, and low hemoglobin F levels tend to be associated with more hemolysis, lower hemoglobin oxygen saturations, greater proportions of elevated tricuspid regurgitant jet velocity and brain natriuretic peptide, and increased left ventricular mass index. Identification of additional genetic modifiers will improve prediction of cardiopulmonary complications in SCD.


Assuntos
Anemia Falciforme , Hemoglobina Fetal/genética , Anemia Falciforme/etnologia , Anemia Falciforme/genética , Anemia Falciforme/metabolismo , Grupos Étnicos , Hemoglobina Fetal/metabolismo , Genótipo , Saúde Global , Humanos , Incidência , Mutação
9.
Pediatr Cardiol ; 34(2): 231-9, 2013 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-22797483

RESUMO

In patients with repaired tetralogy of Fallot (rTOF), left-ventricular ejection fraction (LVEF) predicts adverse outcomes. Two-dimensional echocardiographic (2DE) methods of measuring LVEF require geometric assumptions and may be limited in this population due to altered ventricular geometry. This study evaluated the performance of the 5/6 area × length (AL) method in this population as well as which factors limit agreement with the results of cardiovascular magnetic resonance (CMR). In 20 patients with rTOF (28.5 ± 14.7 years old) and CMR and 2DE within 3 months, two investigators blinded to CMR measured LVEF from 2DE by the AL method, biplane Simpson's (BiS) method, and visual estimate. Two investigators blinded to 2DE measured LVEF from CMR by Simpson's and AL methods. The AL method on 2DE more closely approximated LVEF by CMR (r = 0.73, p = 0.0003) than BiS method (r = 0.53, p = 0.02). AL method was not limited by geometric assumptions, as AL method on CMR closely approximated Simpson's method on CMR (r = 0.90, p < 0.0001) despite median left-ventricular diastolic eccentricity index of 1.24. AL method on 2DE was primarily limited by short-axis area measurement rather than foreshortening of the ventricle. In conclusion, in adults with rTOF, AL method on 2DE moderately approximates LVEF by CMR, even in the context of altered left-ventricular geometry. Although the AL method may be the most appropriate 2DE method in this population, significant limitations remain for LVEF assessment by 2DE, and strategies to optimize image position and border detection are essential.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Ecocardiografia/métodos , Ventrículos do Coração/fisiopatologia , Imagem Cinética por Ressonância Magnética/métodos , Volume Sistólico/fisiologia , Tetralogia de Fallot/diagnóstico , Função Ventricular Esquerda/fisiologia , Adulto , Feminino , Seguimentos , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/patologia , Humanos , Masculino , Período Pós-Operatório , Curva ROC , Reprodutibilidade dos Testes , Estudos Retrospectivos , Tetralogia de Fallot/fisiopatologia , Tetralogia de Fallot/cirurgia
12.
Pediatr Cardiol ; 30(8): 1176-9, 2009 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-19787389

RESUMO

Hypertrophic cardiomyopathy (HCM) is a heterogeneous disorder characterized by thickening of the heart and an increased incidence of sudden death. This study is aimed to determine the genetic cause of severe cardiac hypertrophy in an infant. An infant was assigned a diagnosis of ventricular preexcitation and severe biventricular HCM requiring septal myectomy. Genetic testing showed a novel heterozygous E506Q mutation of the adenosine monophosphate (AMP)-activated protein kinase (PRKAG2) gene. Endomyocardial biopsy samples did not demonstrate significant glycogen accumulation. Hypertrophic cardiomyopathy due to PRKAG2 mutations may have a degree of cardiac hypertrophy exceeding that expected from observed amounts of glycogen deposition.


Assuntos
Proteínas Quinases Ativadas por AMP/genética , Cardiomiopatia Hipertrófica/genética , Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/cirurgia , Eletrocardiografia , Glicogênio/análise , Humanos , Lactente , Masculino , Miocárdio/química , Miocárdio/ultraestrutura , Fatores de Risco
13.
Congenit Heart Dis ; 4(3): 193-5, 2009.
Artigo em Inglês | MEDLINE | ID: mdl-19489950

RESUMO

INTRODUCTION: Pectus excavatum is commonly viewed as a benign condition. Associated alterations in hemodynamics are rare. We present an unusual case of right ventricular inflow obstruction and hemodynamic compromise as a consequence of pectus excavatum encountered during surgical intervention. CASE: a 15-year-old male with pectus excavatum and thoracolumbar scoliosis developed severe hypotension after induction of general anesthesia and placement in the prone position for elective spinal fusion. A transesophageal echocardiogram revealed anterior compression of the right heart by the sternum with peak and mean right ventricular inflow gradients of 7 and 4 mm Hg, respectively. The gradient resolved with supine positioning and was reproduced with direct compression of the sternum. CONCLUSIONS: Although pectus excavatum is generally a benign condition, the cardiologist should be aware of the potential for serious hemodynamic compromise related to positioning in these patients.


Assuntos
Ecocardiografia Doppler em Cores , Ecocardiografia Transesofagiana , Tórax em Funil/cirurgia , Hipotensão/diagnóstico por imagem , Decúbito Ventral , Fusão Vertebral , Decúbito Dorsal , Obstrução do Fluxo Ventricular Externo/diagnóstico por imagem , Adolescente , Tórax em Funil/complicações , Tórax em Funil/fisiopatologia , Hemodinâmica , Humanos , Hipotensão/etiologia , Hipotensão/fisiopatologia , Vértebras Lombares , Masculino , Escoliose/complicações , Esterno/diagnóstico por imagem , Vértebras Torácicas , Resultado do Tratamento , Obstrução do Fluxo Ventricular Externo/etiologia , Obstrução do Fluxo Ventricular Externo/fisiopatologia
14.
Echocardiography ; 25(7): 727-31, 2008 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-18445061

RESUMO

There has traditionally been less concern regarding coronary anomalies with left-sided congenital heart lesions such as hypoplastic left heart syndrome (HLHS)or Shone complex than with other lesions. However, coronary anomalies in this setting can profoundly affect surgical intervention, particularly when surgical repair involves the ascending aorta. We describe four patients with congenital left-sided heart lesions in which left coronary artery (LCA) anomalies substantially affected intervention and outcome. In the first two cases, the coronary anomalies were not identified prospectively and resulted in surgical injury directly to the coronary or to its surrounding region. In the latter two cases, successful identification of the coronary anomaly preoperatively allowed for modification of surgical technique and/or intervention. We conclude that detailed coronary artery assessment should be part of the routine echocardiographic evaluation of congenital left-sided heart lesions that require surgery.


Assuntos
Anormalidades Múltiplas/cirurgia , Procedimentos Cirúrgicos Cardíacos/métodos , Anomalias dos Vasos Coronários/cirurgia , Ecocardiografia Doppler em Cores , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Interpretação de Imagem Assistida por Computador , Artéria Pulmonar/anormalidades , Anormalidades Múltiplas/diagnóstico por imagem , Anormalidades Múltiplas/mortalidade , Procedimentos Cirúrgicos Cardíacos/mortalidade , Anomalias dos Vasos Coronários/diagnóstico por imagem , Feminino , Seguimentos , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/mortalidade , Recém-Nascido , Masculino , Gravidez , Artéria Pulmonar/diagnóstico por imagem , Medição de Risco , Amostragem , Taxa de Sobrevida , Resultado do Tratamento , Ultrassonografia Pré-Natal/métodos
15.
Pediatr Radiol ; 38(3): 292-6, 2008 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-18172637

RESUMO

BACKGROUND: Endocardial fibroelastosis (EFE) is characterized by a diffuse white fibrous tissue lining the endocardium. The diagnosis is difficult to establish because clinical symptoms and electrocardiographic findings are nonspecific. Surgical resection of EFE requires the establishment of the diagnosis and delineation of the extent of the fibrotic changes. OBJECTIVE: To describe the use of MRI in the assessment of EFE in children. MATERIALS AND METHODS: Three children after surgery for aortic stenosis who were suspected of having EFE were evaluated by echocardiography and MRI. The MR evaluation consisted of black-blood, triple IR, bright-blood, perfusion and myocardial delayed-enhancement sequences. EFE was confirmed at surgery in all patients. RESULTS: Echocardiograms demonstrated vigorous systolic function but substantial diastolic dysfunction of the left ventricle in all. Mild endocardial brightening of the anterior septum, anterior wall, or papillary muscles was present in two. No study was thought to be diagnostic of endocardial fibrosis. On MRI EFE manifested at the endocardial surface as a rim of hypointense signal in the perfusion sequences and as a rim of hyperintense signal in the myocardial delayed-enhancement sequences. The black-blood, triple IR, and bright-blood sequences were not diagnostic. CONCLUSION: The diagnosis of EFE is difficult to establish by echocardiography. MRI using perfusion and myocardial delayed enhancement can be useful in establishing the diagnosis.


Assuntos
Fibroelastose Endocárdica/diagnóstico , Imagem por Ressonância Magnética/métodos , Estenose da Valva Aórtica/cirurgia , Pré-Escolar , Ecocardiografia Doppler , Fibroelastose Endocárdica/diagnóstico por imagem , Fibroelastose Endocárdica/cirurgia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino
19.
Am J Cardiol ; 96(6): 857-60, 2005 Sep 15.
Artigo em Inglês | MEDLINE | ID: mdl-16169377

RESUMO

To investigate the association of catheter-derived and newer echocardiographically derived measures of diastolic function with rejection in heart transplant recipients, 48 transplant recipients had Doppler and Doppler tissue imaging assessment along with catheter-measured pulmonary capillary wedge pressure (PCWP) at the time of endomyocardial biopsy. Of echocardiographic measures, propagation velocity (Vp), mitral E-wave velocity (E)/Vp, and E/annular mitral E-wave velocity (Em) were significantly associated with rejection (p <0.02), and an elevated PCWP was associated with rejection (p = 0.023) but with poor sensitivity. Weak but insignificant correlations with PCWP were found for E/Vp (r = 0.28) and E/Em (r = 0.37).


Assuntos
Ecocardiografia Doppler em Cores , Rejeição de Enxerto/diagnóstico por imagem , Rejeição de Enxerto/fisiopatologia , Transplante de Coração/diagnóstico por imagem , Pressão Propulsora Pulmonar , Função Ventricular Esquerda , Adolescente , Adulto , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Masculino , Valor Preditivo dos Testes , Estudos Prospectivos , Sensibilidade e Especificidade
20.
Am J Cardiol ; 95(5): 686-8, 2005 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-15721123

RESUMO

Echocardiographic indexes of left ventricular (LV) diastolic function were assessed in 18 patients before and after device closure of secundum atrial septal defects. The tissue Doppler early diastolic peak annular velocity (Ea) and color M-mode velocity of propagation (Vp), indexes of relaxation, seemed to be load independent and were not affected by the change in LV filling hemodynamics, whereas the mitral inflow peak E-wave velocity and E/Ea ratio were more load dependent, with a resultant increase after the closure of large atrial shunts.


Assuntos
Diástole/fisiologia , Comunicação Interatrial/cirurgia , Função Ventricular Esquerda/fisiologia , Adolescente , Adulto , Criança , Pré-Escolar , Ecocardiografia Doppler , Feminino , Comunicação Interatrial/diagnóstico por imagem , Humanos , Lactente , Masculino , Resultado do Tratamento
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