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1.
Physiol Int ; 107(1): 145-154, 2020 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-32598330

RESUMEN

INTRODUCTION: Left ventricular (LV) twist is considered an essential part of LV function due to oppositely directed LV basal and apical rotations. Several factors could play a role in determining LV rotational mechanics in normal circumstances. This study aimed to investigate the relationship between LV rotational mechanics and mitral annular (MA) size and function in healthy subjects. METHODS: The study comprised 118 healthy adult volunteers (mean age: 31.5 ± 11.8 years, 50 males). All subjects had undergone complete two-dimensional (2D) Doppler echocardiography and three-dimensional speckle-tracking echocardiography (3DSTE) at the same time by the same echocardiography equipment. RESULTS: The normal mean LV apical and basal rotations proved to be 9.57 ± 3.33 and -3.75 ± 1.98°, respectively. LV apical rotation correlated with end-systolic MA diameter, area, perimeter, fractional area change, and fractional shortening, but did not correlate with any end-diastolic mitral annular morphologic parameters. The logistic regression model identified MA fractional area change as an independent predictor of ≤6° left ventricular apical rotation (P < 0.003). CONCLUSIONS: Correlations could be detected between apical LV rotation and end-systolic MA size and function, suggesting relationships between MA dimensions and function and LV rotational mechanics.


Asunto(s)
Ecocardiografía Doppler/métodos , Ecocardiografía Tridimensional/métodos , Ventrículos Cardíacos/fisiopatología , Válvula Mitral/fisiología , Adulto , Femenino , Ventrículos Cardíacos/diagnóstico por imagen , Humanos , Masculino , Válvula Mitral/diagnóstico por imagen , Curva ROC , Rotación , Función Ventricular Izquierda
2.
Herz ; 44(5): 405-411, 2019 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-29234843

RESUMEN

BACKGROUND: Idiopathic hypereosinophilic syndrome is characterized by a persistent eosinophil blood count of >1.5 × 109 cells/l and organ damage, independent of the primary and secondary causes of eosinophilia. The purpose of the present study was to assess the three-dimensional speckle tracking echocardiography-derived right atrial volumetric and functional properties between hypereosinophilic syndrome patients and matched controls. METHODS: A total of 11 patients with idiopathic hypereosinophilic syndrome and 22 age- and gender-matched healthy controls were enrolled in the study. Three-dimensional speckle tracking echocardiography was used for calculation of right atrial volumes, volume-based functional properties, and strain parameters. RESULTS: Significantly increased right atrial maximum (68.7 ± 33.1 ml vs. 40.3 ± 12.1 ml, respectively; p = 0.001) and minimum volumes (48.3 ± 31.0 ml vs. 28.3 ± 9.4 ml, respectively; p = 0.009), as well as right atrial volume before atrial contraction (58.6 ± 27.3 ml vs. 34.5 ± 11.8 ml, respectively; p = 0.001), were found in hypereosinophilic syndrome patients compared with controls. Total and passive right atrial stroke volumes proved to be significantly increased in hypereosinophilic syndrome patients. However, global and mean segmental strain parameters did not differ significantly between the groups. CONCLUSION: Increased cyclic right atrial volumes and mild alterations in right atrial functional properties could be demonstrated in idiopathic hypereosinophilic syndrome patients.


Asunto(s)
Ecocardiografía Tridimensional , Atrios Cardíacos , Síndrome Hipereosinofílico , Adulto , Anciano , Estudios de Casos y Controles , Ecocardiografía , Atrios Cardíacos/diagnóstico por imagen , Atrios Cardíacos/patología , Humanos , Síndrome Hipereosinofílico/complicaciones , Síndrome Hipereosinofílico/diagnóstico por imagen , Masculino , Persona de Mediana Edad
3.
Lymphology ; 51(3): 102-108, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-30422432

RESUMEN

The present study aimed to assess LV rotational mechanics by three-dimensional speckle-tracking echocardiography (3DSTE) in lipedema (n=25), lymphedema (n=26) patient groups with age- and gender-matched healthy controls (n=54). 3 lipedema and 4 lymphedema patients were excluded due to insufficient image quality for 3DSTE analysis. LV apical rotation (9.61 ± 4.25 degree vs. 6.40 ± 2.63 degree, p <0.05) and LV twist (13.83 ± 4.89 degree vs. 10.04 ± 3.56 degree, p <0.05) are impaired in lipedema patients as compared to matched controls; similar alterations in lymphedema were not found. Moreover, in some lipedema and lymphedema patients severe LV rotational abnormalities could be detected. Our results suggest that lipedema-associated impaired LV apical rotation and twist assessed by 3DSTE could be a novel differential diagnostic point between lipedema and lymphedema.


Asunto(s)
Ecocardiografía Tridimensional/métodos , Ventrículos Cardíacos/patología , Lipedema/patología , Linfedema/patología , Adulto , Estudios de Casos y Controles , Femenino , Ventrículos Cardíacos/diagnóstico por imagen , Humanos , Lipedema/diagnóstico por imagen , Linfedema/diagnóstico por imagen , Masculino , Persona de Mediana Edad , Pronóstico , Torsión Mecánica
4.
Physiol Int ; 104(2): 206-215, 2017 Jun 01.
Artículo en Inglés | MEDLINE | ID: mdl-28648119

RESUMEN

Introduction Myocardial contractility of the left ventricle (LV) is related to arterial distensibility. Sport activity is frequently associated with changes in both LV and arterial functions. This study aimed to find correlations between three-dimensional speckle-tracking echocardiography-derived segmental LV deformation parameters and echocardiographically assessed aortic stiffness index (ASI) in athletes. This study comprised 26 young elite athletes (mean age: 26.7 ± 8.4 years, nine men). Results Among segmental circumferential strains (CSs), only that of apical anterior (r = 0.40, p = 0.05), septal (r = 0.47, p = 0.01), inferior (r = 0.59, p = 0.001), lateral (r = 0.44, p < 0.05), and midventricular anteroseptal (r = 0.44, p < 0.05) segments correlated with ASI, whereas LV-CS of the midventricular anterior segment showed a correlation tendency. Only longitudinal strain of basal anteroseptal (r = -0.46, p < 0.05) and inferoseptal (r = -0.57, p < 0.01) segments showed correlations with ASI, whereas that of the basal anterior segment had only a tendency to correlate. Some segmental multidirectional strains also correlated with ASI. Conclusions Correlations could be demonstrated between increased aortic stiffness and circular function of the apical and midventricular LV fibers and longitudinal motion of the basal septum and LV anterior wall (part of LV outflow tract) in maintaining circulation in the elite athletes.


Asunto(s)
Aorta/fisiología , Contracción Miocárdica/fisiología , Deportes/fisiología , Volumen Sistólico/fisiología , Rigidez Vascular/fisiología , Función Ventricular Izquierda/fisiología , Adulto , Aorta/diagnóstico por imagen , Ecocardiografía/métodos , Módulo de Elasticidad/fisiología , Diagnóstico por Imagen de Elasticidad/métodos , Femenino , Ventrículos Cardíacos/diagnóstico por imagen , Humanos , Interpretación de Imagen Asistida por Computador/métodos , Masculino , Estadística como Asunto
5.
Eur J Soil Sci ; 66(1): 226-238, 2015 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-25866465

RESUMEN

A range of continental-scale soil datasets exists in Europe with different spatial representation and based on different principles. We developed comprehensive pedotransfer functions (PTFs) for applications principally on spatial datasets with continental coverage. The PTF development included the prediction of soil water retention at various matric potentials and prediction of parameters to characterize soil moisture retention and the hydraulic conductivity curve (MRC and HCC) of European soils. We developed PTFs with a hierarchical approach, determined by the input requirements. The PTFs were derived by using three statistical methods: (i) linear regression where there were quantitative input variables, (ii) a regression tree for qualitative, quantitative and mixed types of information and (iii) mean statistics of developer-defined soil groups (class PTF) when only qualitative input parameters were available. Data of the recently established European Hydropedological Data Inventory (EU-HYDI), which holds the most comprehensive geographical and thematic coverage of hydro-pedological data in Europe, were used to train and test the PTFs. The applied modelling techniques and the EU-HYDI allowed the development of hydraulic PTFs that are more reliable and applicable for a greater variety of input parameters than those previously available for Europe. Therefore the new set of PTFs offers tailored advanced tools for a wide range of applications in the continent.

6.
Neth Heart J ; 23(2): 143-4, 2015 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-23184598

RESUMEN

A recently developed computerized method for estimation of myocardial perfusion, based on the analysis of the time-density curves, is demonstrated to assess myocardial blush over a selected myocardial region of interest in a patient with obstructive hypertrophic cardiomyopathy before and after alcohol septal ablation.

9.
Herz ; 39(6): 770-3, 2014 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-23880949

RESUMEN

Aneurysm of the left sinus of Valsalva is an extremely rare entity. It may be asymptomatic and incidentally discovered, or may be symptomatic and manifest acutely with compression of adjacent cardiac structures. Encasement of the left main coronary artery by such an aneurysm is a recognized but infrequent complication that can lead to severe coronary insufficiency. Surgical decompression of the left main coronary artery is the standard treatment for such conditions. We describe a patient presenting with extrinsic compression of the left main coronary artery by a large unruptured aneurysm of the left sinus of Valsalva occurring 4 months after unsuccessful surgical repair. Since reoperation was considered high-risk for the patient, successful fractional flow reserve- and intravascular ultrasound-guided percutaneous treatment of the left main coronary artery was performed with implantation of one bare-metal stent.


Asunto(s)
Aneurisma de la Aorta/complicaciones , Aneurisma de la Aorta/cirugía , Estenosis Coronaria/etiología , Estenosis Coronaria/cirugía , Seno Aórtico/cirugía , Aneurisma de la Aorta/diagnóstico por imagen , Rotura de la Aorta/diagnóstico por imagen , Estenosis Coronaria/diagnóstico por imagen , Humanos , Masculino , Persona de Mediana Edad , Intervención Coronaria Percutánea/métodos , Radiografía , Seno Aórtico/diagnóstico por imagen , Resultado del Tratamiento
11.
Lymphology ; 45(2): 71-9, 2012 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-23057152

RESUMEN

Lipedema is a disproportional obesity due to unknown pathomechanism. Its major hallmark is frequent hematoma formation related to increased capillary fragility and reduced venoarterial reflex. Beyond microangiopathy, both venous and lymphatic dysfunction have also been documented. However, arterial circulation in lipedema has not been examined, and therefore we explored aortic elastic properties by echocardiography. Fourteen women with and 14 without lipedema were included in the study. Each subject consented to blood pressure measurement, physical examination, and transthoracic echocardiography. Aortic stiffness index (beta), distensibility, and strain were evaluated from aortic diameter and blood pressure data. Mean systolic (30.0 +/- 3.2 vs. 25.5 +/- 3.6, P < 0.05) and diastolic (27.8 +/- 3.3 vs. 22.3 +/- 3.1) aortic diameters (in mm) and aortic stiffness index (9.05 +/- 7.45 vs. 3.76 +/- 1.22, P < 0.05) were significantly higher, while aortic strain (0.082 +/- 0.04 vs. 0.143 +/- 0.038, P < 0.05) and distensibility (2.24 +/- 1.07 vs. 4.38 +/- 1.61, P < 0.05) were significantly lower in lipedematous patients compared to controls. Thus, lipedema is characterized with increased aortic stiffness.


Asunto(s)
Tejido Adiposo/fisiopatología , Aorta/fisiopatología , Enfermedades del Tejido Conjuntivo/fisiopatología , Edema/fisiopatología , Rigidez Vascular/fisiología , Adulto , Presión Sanguínea/fisiología , Estudios de Casos y Controles , Ecocardiografía , Elasticidad , Femenino , Humanos
12.
Acta Physiol Hung ; 99(3): 271-8, 2012 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-22982715

RESUMEN

PURPOSE: In recent studies, reduction in coronary flow velocity reserve (CFR) has been demonstrated in patients with increased aortic stiffness. Stress transoesophageal echocardiography (TEE) is a suitable method for the simultaneous evaluation of CFR and aortic stiffness parameters. The present study was designed to test whether increased echocardiography-derived aortic elastic modulus [E(p)] predicts impaired CFR in patients with suspected coronary artery disease (CAD). RESULTS: The present study comprised 158 patients with suspected CAD. A CFR value < 2 was considered abnormal. Both men grade of aortic atherosclerosis (AA) (as a morphologic characteristic) (1.31 ± 0.68 vs. 1.02 ± 0.89, p < 0.05) and aortic distensibility (E(p) as a functional characteristic) (892 ± 594 mmHg vs. 723 ± 495 mmHg, P < 0.05) were increased in subjects with CFR < 2. In ROC analysis, the cut-off value for E(p) to predict impaired CFR was ≥ 670 mmHg, with 61% sensitivity and 61% specificity (ROC area 0.60, p = 0.026). The logistic regression model identified higher AA grade (hazard ratio (HR) 2.01, p < 0.05) and increased E(p) as independent predictors of reduced CFR (HE 1.10, p < 0.05). CONCLUSION: Increased aortic stiffness predicts impaired CFR in patients with suspected CAD.


Asunto(s)
Enfermedades de la Aorta/diagnóstico por imagen , Enfermedad de la Arteria Coronaria/diagnóstico por imagen , Circulación Coronaria/fisiología , Ecocardiografía Transesofágica/métodos , Rigidez Vascular/fisiología , Adulto , Anciano , Aorta/fisiopatología , Enfermedades de la Aorta/epidemiología , Enfermedades de la Aorta/fisiopatología , Velocidad del Flujo Sanguíneo/fisiología , Enfermedad de la Arteria Coronaria/epidemiología , Enfermedad de la Arteria Coronaria/fisiopatología , Ecocardiografía Transesofágica/normas , Femenino , Humanos , Masculino , Persona de Mediana Edad , Análisis Multivariante , Valor Predictivo de las Pruebas , Pronóstico , Curva ROC , Factores de Riesgo , Sensibilidad y Especificidad
13.
J Cardiovasc Surg (Torino) ; 52(2): 169-76, 2011 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-21464818

RESUMEN

AIM: The aim of the study was to evaluate the early and late results of aortic replacement using cryopreserved homografts and autologous deep veins for infected infrarenal prosthetic reconstructions and the influence of type of bacteria on the mortality. METHODS: Thirty-three patients were treated from 30 March 1994 to 01 September 2008 for aorto-femoral or iliaco-femoral graft infections with homografts (HG:19) or autologous deep veins (DV:14). The diagnosis was based on physical signs, bacteriological tests and computed tomography(CT) scans. We obtain cryopreserved homografts from our non-profit vessel bank, deep veins were harvested before the arterial reconstruction. Patients were followed by clinical examination and ultrasound. RESULTS: The infections occurred 47.78 ± 20 months after the primary operations; 45% of the infections were caused by Gram-negative bacteria. Treatment indications included seven aortoduodenal fistula (21.2%) and six septic para-anastomotic bleedings (18.2%). There were six in-hospital deaths (18.2%). All the deceased patients had Gram-negative bacteria in cultures and pluribacterial infections. No patient died with single staphylococcus, streptococcus or MRSA infection. At three years freedom from reinfection was 100% in DV group and 82% (CI:0.56-0.92) in the homograft group. Survival after three years was 71% (CI:0.48-0.88) in HG group and 79% (CI:0.49-0.94) in DV group. The difference is not significant. CONCLUSION: For infrarenal graft infection homograft replacement or deep vein implantation is durable method and eradicate late reinfection. The in-hospital mortality is significantly higher in patients with aortoduodenal fistulas with Gram-negative organisms regardless of the conduit used for aortic replacement.


Asunto(s)
Aorta/cirugía , Bioprótesis , Implantación de Prótesis Vascular/efectos adversos , Prótesis Vascular/efectos adversos , Criopreservación , Arteria Femoral/cirugía , Arteria Ilíaca/cirugía , Infecciones Relacionadas con Prótesis/cirugía , Venas/trasplante , Anciano , Aorta/fisiopatología , Implantación de Prótesis Vascular/instrumentación , Implantación de Prótesis Vascular/mortalidad , Femenino , Arteria Femoral/fisiopatología , Mortalidad Hospitalaria , Humanos , Hungría , Arteria Ilíaca/fisiopatología , Estimación de Kaplan-Meier , Recuperación del Miembro , Masculino , Persona de Mediana Edad , Diseño de Prótesis , Infecciones Relacionadas con Prótesis/diagnóstico , Infecciones Relacionadas con Prótesis/microbiología , Infecciones Relacionadas con Prótesis/mortalidad , Infecciones Relacionadas con Prótesis/fisiopatología , Recurrencia , Reoperación , Estudios Retrospectivos , Tasa de Supervivencia , Factores de Tiempo , Trasplante Autólogo , Trasplante Homólogo , Resultado del Tratamiento , Grado de Desobstrucción Vascular
14.
Eur J Vasc Endovasc Surg ; 40(1): 88-93, 2010 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-20171909

RESUMEN

OBJECTIVE: Improved maintenance of endothelial function and higher viability of saphenous vein grafts stored in a complex tissue culture medium (TCM) have been demonstrated. This article studies the biomechanical properties of saphenous vein segments. DESIGN: Biomechanical properties of 72 saphenous vein segments remaining from coronary bypass grafting of 32 patients have been studied after different storage procedures. MATERIALS: The materials studied included fresh segments, segments stored in a cooled conventional physiological salt solution (normal Krebs-Ringer (nKR)) for 1-2 weeks, segments stored in a cooled chemically defined TCM (X-Vivo) for 1,2,3 and 4 weeks and segments cryopreserved for a few weeks. METHODS: Specimens were cannulated at both ends and pressure-diameter curves were recorded in the 0-85-mmHg range in nKR with 10 microM norepinephrine added to induce maximum smooth muscle contraction, as well as in Ca(2+)-free medium to induce full relaxation. Tensile strength was checked at 300 mmHg. Distensibility, elastic modulus and active strain were computed. RESULTS: Segments stored in nKR dilated morphologically, their distensibility decreased and they lost their ability to contract (1.5+/-0.7% from 10.1+/-1.5% of control) in 1 week. The TCM-stored segments preserved their contractility until 1 week, and this parameter only slowly decreased afterwards (first week, 11.5+/-7.3%; fourth week, 3.9+/-0.6%). There was a slight decrease in wall thickness but the lumen diameter was not affected. The elastic parameters of these segments were practically identical to those of fresh segments. Cryopreserved segments narrowed morphologically, their wall thickened and contractility diminished. CONCLUSIONS: Storage in TCM helps preserve the passive and active biomechanical properties of human saphenous vein segments. Such properties can be expected to improve graft tissue viability.


Asunto(s)
Medios de Cultivo/farmacología , Soluciones Preservantes de Órganos/farmacología , Vena Safena/efectos de los fármacos , Conservación de Tejido/métodos , Vasoconstricción/efectos de los fármacos , Vasodilatación/efectos de los fármacos , Fenómenos Biomecánicos , Calcio/metabolismo , Criopreservación , Elasticidad , Humanos , Norepinefrina/farmacología , Presión , Vena Safena/anatomía & histología , Vena Safena/fisiología , Resistencia a la Tracción , Factores de Tiempo , Supervivencia Tisular/efectos de los fármacos , Vasoconstrictores/farmacología
15.
Neth Heart J ; 16(6): 217-8, 2008 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-18665207

RESUMEN

Dobutamine stress magnetic resonance imaging is considered the superior stress modality to detect wall motion abnormalities. In this report we demonstrate the strengths of a newly developed stress modality: dobutamine stress contrastenhanced real-time three-dimensional echocardiography. This stress modality may become a competitor of stress magnetic resonance imaging allowing fast acquisition and an unlimited number of left ventricular cross sections. Unfortunately, at the moment adequate imaging with stress realtime three-dimensional echocardiography is only possible in a minority of cardiac patients. (Neth Heart J 2008;16:217-8.).

16.
J Intern Med ; 264(4): 333-9, 2008 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-18397245

RESUMEN

BACKGROUND: Glycogen storage disease type II or Pompe disease is a neuromuscular disorder caused by deficiency of lysosomal acid alpha- glucosidase. Classic infantile Pompe disease results in massive left ventricular (LV) hypertrophy and failure. Although Pompe disease is often included in the differential diagnosis of LV hypertrophy the true frequency of cardiac involvement in adults with Pompe disease is not known. METHODS: Forty-six consecutive adult patients (mean age 48 +/- 12, 22 men) with Pompe disease were included. Each patient underwent a clinical examination, electrocardiography, and rest and low-dose dobutamine (in 20 patients) two-dimensional echocardiography including contrast and tissue Doppler imaging. RESULTS: All patients had limited exercise tolerance; a rollator walking aid was used in seven patients (15%), a wheelchair in 13 patients (28%), and assisted ventilation in 14 patients (30%). Prior to this study, one patient was known with permanent atrial fibrillation, His-bundle ablation and a VVI pacemaker and another patient was known with fluid retention. The first patient had increased LV end-diastolic diameter, impaired LV ejection fraction, low systolic mitral annular velocities and diastolic dysfunction grade II. The patient with fluid retention was wheelchair bound and dependent on 24-h assisted ventilation and showed right ventricular and LV hypertrophy (septum 16 mm, posterior wall 15 mm). LV hypertrophy was not seen in any of the other patients. One woman of advanced age had isolated low systolic mitral annular velocities. Mean global systolic LV function, including contractile reserve, was not decreased in patients with Pompe disease. Eight patients (17%) had mild diastolic dysfunction grade I, related to hypertension in four and advanced age in seven. CONCLUSIONS: In adult patients with Pompe disease without objective signs of cardiac affection by 12-leads electrocardiography or physical examination, echocardiographic screening for LV hypertrophy seems not effective.


Asunto(s)
Enfermedad del Almacenamiento de Glucógeno Tipo II/complicaciones , Cardiopatías/complicaciones , Adulto , Anciano , Estudios de Casos y Controles , Dobutamina , Ecocardiografía , Ecocardiografía Doppler de Pulso , Tolerancia al Ejercicio , Femenino , Enfermedad del Almacenamiento de Glucógeno Tipo II/diagnóstico por imagen , Cardiopatías/diagnóstico por imagen , Humanos , Hipertrofia Ventricular Izquierda/complicaciones , Hipertrofia Ventricular Izquierda/diagnóstico por imagen , Masculino , Persona de Mediana Edad , Estudios Prospectivos
17.
Eur Phys J E Soft Matter ; 25(4): 395-402, 2008 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-18425409

RESUMEN

We report a novel type of electro-optical switching in a tilted smectic phase of bent-shaped mesogens. The switching consists of a continuous stage and two bistable transitions. Detailed optical and electro-optical measurements using high-speed imaging are given and possible interpretations of the experimental results are discussed.


Asunto(s)
Compuestos Férricos/química , Cristales Líquidos/química , Óptica y Fotónica , Calorimetría , Electricidad , Electroquímica , Emulsiones , Procesamiento de Imagen Asistido por Computador , Cristales Líquidos/ultraestructura , Microscopía , Propiedades de Superficie , Temperatura , Factores de Tiempo , Difracción de Rayos X
18.
Eur J Ophthalmol ; 18(2): 309-12, 2008.
Artículo en Inglés | MEDLINE | ID: mdl-18320530

RESUMEN

PURPOSE: Leber hereditary optic neuropathy (LHON) is recognized as the most common cause of isolated blindness in young men. The current study was designed to test whether LHON as a mitochondrial disease is associated with vascular functional alterations characterized by aortic elastic properties during echocardiography. METHODS: A total of 19 patients with typical features of LHON aged 42+/-13 years (10 males) were included. Their results were compared to 19 age- and gender-matched healthy controls. Aortic stiffness index was calculated from the echocardiographically derived aortic diameters and the clinical blood pressure data. RESULTS: In this patient population, the point mutation was present in 3460G>A position in five cases, in 11778G>A position in five cases, and in 14484T>C position in nine patients. Diastolic aortic diameter (26.0+/-2.5 mm vs 28.4+/-4.1 mm, p<0.05) and aortic stiffness index (5.1+/-2.6 vs 12.0+/-7.9, p<0.05) were significantly increased in LHON patients compared to controls. CONCLUSIONS: Aortic stiffness can be increased in LHON disease, but further studies are warranted to confirm these findings in a larger LHON patient population with a more reliable method focusing on the pathophysiologic background.


Asunto(s)
Aorta/fisiopatología , Atrofia Óptica Hereditaria de Leber/fisiopatología , Adulto , Velocidad del Flujo Sanguíneo , Presión Sanguínea , ADN Mitocondrial/genética , Ecocardiografía , Elasticidad , Femenino , Humanos , Masculino , Atrofia Óptica Hereditaria de Leber/genética , Mutación Puntual , Estudios Prospectivos , Ultrasonografía Doppler
19.
J Inherit Metab Dis ; 30(5): 750-7, 2007 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-17574537

RESUMEN

BACKGROUND: Cardiac involvement in mucopolysaccharidosis type I (MPS I) has been studied primarily in its most severe forms. Cardiac involvement, particularly left ventricular (LV) systolic and diastolic function, in the attenuated form of MPS I is less well known. METHODS: Cardiac function was prospectively investigated in 9 adult patients with the attenuated form of MPS I. All patients underwent 12-lead electrocardiography, 24 h Holter monitoring and two-dimensional echocardiography including tissue Doppler imaging (TDI). Eighteen age- and sex-matched healthy volunteers served as a control group. RESULTS: Aortic, mitral and tricuspid valve thickening was seen in, respectively, 5 (56%), 4 (44%) and 2 (22%) patients. Moderate mitral valve stenosis was seen in 1 patient and moderate aortic stenosis in 2 patients. All patients had mild-to-moderate aortic and mitral valve regurgitation and 6 patients (67%) had mild-to-moderate tricuspid valve regurgitation. Despite normal LV dimensions, ejection fraction and mass index, MPS patients had lower mean systolic mitral annular velocities (6.1 +/- 0.6 vs 9.1 +/- 1.4 cm/s, p < 0.01) compared to normal control subjects. Similarly, mean early diastolic mitral annular velocities were lower in MPS patients (7.8 +/- 0.9 vs 13.3 +/- 3.3 cm/s, p < 0.01). CONCLUSION: MPS I patients with the attenuated phenotype have not only valvular abnormalities but also LV diastolic and systolic abnormalities.


Asunto(s)
Enfermedades de las Válvulas Cardíacas/etiología , Mucopolisacaridosis I/complicaciones , Función Ventricular Izquierda , Adulto , Insuficiencia de la Válvula Aórtica/etiología , Estenosis de la Válvula Aórtica/etiología , Estudios de Casos y Controles , Diástole , Ecocardiografía Doppler , Electrocardiografía Ambulatoria , Femenino , Enfermedades de las Válvulas Cardíacas/diagnóstico por imagen , Enfermedades de las Válvulas Cardíacas/fisiopatología , Humanos , Masculino , Insuficiencia de la Válvula Mitral/etiología , Estenosis de la Válvula Mitral/etiología , Mucopolisacaridosis I/diagnóstico por imagen , Mucopolisacaridosis I/fisiopatología , Fenotipo , Estudios Prospectivos , Proyectos de Investigación , Índice de Severidad de la Enfermedad , Sístole , Insuficiencia de la Válvula Tricúspide/etiología
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