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Lipoprotein apheresis is an extracorporeal procedure for the treatment of patients with homozygous familial hypercholesterolemia, patients with severe treatment-resistant hypercholesterolemia and patients with lipoprotein(a) hypercholesterolemia, who show progressive atherosclerotic cardiovascular disease despite optimal treatment. This article reports on the historical developments of the procedures, the most frequently used methods for apheresis as well as the data situation on efficacy and tolerability. Randomized prospective studies on clinical outcomes are not available. Furthermore, the article reports on a patient with homozygous familial hypercholesterolemia and 34 years of treatment with heparin-induced extracorporeal low-density lipoprotein (LDL) precipitation (HELP) apheresis, the longest treatment of this kind worldwide. A second patient with combined heterozygous familial hypercholesterolemia and 31 years of liposorber and HELP apheresis is also described. The observational studies and the case reports demonstrate the safety and long-term tolerability of the procedure.
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Eliminación de Componentes Sanguíneos , Hipercolesterolemia , Hiperlipoproteinemia Tipo II , Eliminación de Componentes Sanguíneos/métodos , Humanos , Hipercolesterolemia/terapia , Hiperlipoproteinemia Tipo II/terapia , Lipoproteínas , Estudios ProspectivosRESUMEN
AIMS: The aim of this study was to analyze the impact of the tracking area width on myocardial wall motion and deformation parameters in 2D speckle tracking. METHODS AND RESULTS: Standardized apical views were acquired in 30 healthy subjects and 15 patients with left ventricular systolic dysfunction using 2D echocardiography. Longitudinal peak systolic strain (PSS), longitudinal peak systolic strain rate (PSSR), postsystolic index (PSI), peak longitudinal (PLD), and peak transverse displacement (PTD) were determined by 2D speckle tracking to analyze the impact of the tracking area width on global and regional myocardial wall motion and deformation parameters. The dimension of the tracking area has a significant impact on all parameters. With increasing width of the tracking area higher values of PSS, PSSR, PSI, and lower values of PLD and PTD were determined. With increasing width of the tracking area a significant number of segments were not tracked. In summary, especially global PSS is significantly influenced by the width of the tracking area. CONCLUSIONS: The strain values determined by 2D speckle tracking are significantly influenced by the tracking area width. The tracking of the subendocardial layers only results in lower global strain values than tracking the complete ventricular wall using the medium or wide tracking area widths. The tracking quality in the far field is worse if the tracking area is too wide. The present data show that standard and reference values of deformation imaging should include detailed information about the position and the width of the tracking area.
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Diagnóstico por Imagen/métodos , Ecocardiografía/métodos , Ventrículos Cardíacos/diagnóstico por imagen , Disfunción Ventricular Izquierda/diagnóstico por imagen , Función Ventricular Izquierda/fisiología , Femenino , Estudios de Seguimiento , Ventrículos Cardíacos/fisiopatología , Humanos , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Reproducibilidad de los Resultados , Volumen Sistólico , Sístole , Disfunción Ventricular Izquierda/fisiopatologíaRESUMEN
INTRODUCTION: Current 2019 ESC/EAS guidelines for the management of dyslipidemia recommend LDL cholesterol (LDL-C) goals according to the patients' cardiovascular (CV) risk. SANTORINI is the first large European observational study since the 2019 guidelines to assess whether lipid management in patients at high and very high CV risk has improved. METHODS: SANTORINI is a multinational, prospective, non-interventional, observational study in 9602 patients ≥â18 years at high and very high CV risk requiring lipid-lowering therapy. Individual CV risk was assessed by the investigator. The primary study objective is to document, in a real-world setting, the effectiveness of current lipid management regarding LDL-C levels. RESULTS: For this analysis, complete recruitment data was available for 2086 patients in Germany and 6958 patients Europe. Investigators used the 2019 ESC/EAS guidelines as a basis for CV risk classification in >â50â% of the patients and classified 15.6â% (173/1112) of patients in Germany as high and 84.4â% (939/1112) as very high-risk (Europe: 20.7â% [743/3594] high, 79.3â% [2851/3594] very high CV risk). An independent re-calculation of the CV risk based on these guidelines classified 4.1â% (46/1112) of patients in Germany as high and 94.5â% (1051/1112) as very high-risk. Also in Europe, CV risk was underestimated in around 10â% of patients.At baseline, 59.5â% (1241/2086) patients in Germany and 52.6â% (3661/6958) patients in Europe received lipid-lowering monotherapy; 19.9â% (416/2086) and 25.2â% (1753/6958) of patients in Germany and Europe received combination therapy. 78.6â% (1640/2086) of patients in Germany missed the 2019 ESC/EAS guideline recommended LDL-C treatment goals (Europe: 71.1â% [4989/6958]). DISCUSSION: The 2019 ESC/EAS guideline recommendations are only implemented in a minority of patients. The study identifies opportunities for improvements in the prevention of CV diseases in Germany.
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Enfermedades Cardiovasculares , Dislipidemias , Inhibidores de Hidroximetilglutaril-CoA Reductasas , Humanos , LDL-Colesterol , Enfermedades Cardiovasculares/prevención & control , Estudios Prospectivos , Factores de Riesgo , Europa (Continente) , Alemania , Factores de Riesgo de Enfermedad CardiacaRESUMEN
AIMS: Surgical and FFP2 masks are recommended to reduce transmission of SARS-CoV-2. The cardiopulmonary effects of facemasks in patients with chronic heart failure are unknown. This prospective, cross-over study quantified the effects of wearing no mask (nm), surgical mask (sm), and FFP2 mask (ffpm) in patients with stable heart failure. METHODS: 12 patients with clinically stable chronic heart failure (HF) (age 63.8±12 years, left ventricular ejection fraction (LVEF) 43.8±11%, NTProBNP 573±567 pg/ml) underwent spiroergometry with and without masks in a randomized sequence. Comfort/discomfort was assessed using a standardized questionnaire. RESULTS: Maximum power was reduced with both types of masks (nm: 108.3 W vs. sm: 101.2 W vs. ffpm: 95.6 W, p<0.01). Maximum respiratory oxygen uptake (1499ml/min vs. 1481 ml/min vs. 1300 ml/min, p = 0.95 and <0.01), peak ventilation (62.1 l/min vs. 56.4 l/min vs. 50.3 l/min, p = 0.15 and p<0.05) and O2-pulse (11.6 ml/beat vs. 11.8 ml/beat vs. 10.6 ml/beat, p = 0.87 and p<0.01) were significantly changed with ffpm but not sm. Discomfort was moderately but significantly increased (nm: 1.6 vs. sm: 3.4 vs. ffpm: 4.4, p<0.05). CONCLUSION: Both surgical and FFP masks reduce exercise capacity in heart failure patients, while FFP2 masks reduce oxygen uptake and peak ventilation. This reduction in cardiopulmonary performance should be considered in heart failure patients whose daily life activities are often just as challenging as exercise is for healthy adults.
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COVID-19 , Insuficiencia Cardíaca , Adulto , Anciano , COVID-19/prevención & control , Estudios Cruzados , Prueba de Esfuerzo , Tolerancia al Ejercicio , Insuficiencia Cardíaca/terapia , Humanos , Persona de Mediana Edad , Oxígeno , Estudios Prospectivos , SARS-CoV-2 , Volumen Sistólico , Función Ventricular IzquierdaRESUMEN
BACKGROUND: The diagnosis of acute myocarditis (AM) is often challenging and mainly performed by cardiac magnetic resonance (CMR). CASE SUMMARY: The present case describes echocardiographic findings of a 38-year-old male patient with exertional dyspnoea probably due to myocardial involvement of previously undiagnosed acute human immunodeficiency virus (HIV) infection. Myocardial deformation imaging might be helpful to detect early stages of myocardial dysfunction in patients with AM and/or systemic infectious diseases by documentation of patchy abnormalities of longitudinal, circumferential and rotational left ventricular (LV) deformation. DISCUSSION: CMR still represents the gold standard to diagnose AM, which has been confirmed by myocardial oedema and hyperaemia in the present case. However, speckle tracking echocardiography seems to be useful to detect myocardial involvement in HIV infection by dynamic alterations of different components of LV deformation. This was documented by comparing echocardiographic findings at the acute stage of HIV infection to findings at follow-ups during antiviral treatment. The diagnostic option to detect myocardial involvement by deformation imaging in a patient with HIV infection is described for the first time.
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RATIONALE: Familial chylomicronemia syndrome is a congenital, severe form of hypertriglyceridemia associated with increased risk of acute pancreatitis. Treatment options are limited. PATIENT CONCERNS: A 52-year-old woman was referred with recurrent pancreatitis and severe hypertriglyceridemia to our lipid clinic. DIAGNOSIS: Laboratory examination showed elevated serum triglyceride concentrations of 8090âmg/dL (90âmmol/L). Lipid electrophoresis showed a type V phenotype with positive chylomicrons. Genetic investigation revealed a novel heterozygous large deletion of the lipoprotein lipase gene on chromosome 8. A familial chylomicronemia syndrome was diagnosed. Other causes of hypertriglyceridemia were excluded. INTERVENTIONS: Fibrates and diet did not lower triglyceride levels. Therefore, treatment with the apolipoprotein CIII (apoCIII) inhibitor volanesorsen was initiated. OUTCOMES: After 3 months of treatment, a 90% reduction of triglycerides was observed. ApoCIII concentrations were reduced by 90% in the total and by 61% in the chylomicron-free serum. Treatment was well tolerated with only minor local reaction after the first application. The platelet count was monitored weekly and did not decrease <150âcells/µL. LESSONS: This case report shows that inhibition of apoCIII potently reduces serum triglycerides in patients with heterozygous monogenetic deletion of the lipoprotein lipase gene. Follow-up will show the effect on recurrent episodes of pancreatitis.
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Apolipoproteína C-III/antagonistas & inhibidores , Cromosomas Humanos Par 8/genética , Hiperlipoproteinemia Tipo I/tratamiento farmacológico , Hiperlipoproteinemia Tipo I/genética , Oligonucleótidos/uso terapéutico , Femenino , Humanos , Lipoproteína Lipasa/genética , Persona de Mediana Edad , Triglicéridos/sangreRESUMEN
BACKGROUND AND AIMS: Familial hypercholesterolaemia (FH) is associated with high cardiovascular risk and underdiagnosed. Cutaneous manifestations are traditionally used as a major criterion of FH. They are included in the Dutch Lipid Clinic Network or Simon Broome registry criteria. The objective of this study was to evaluate cutaneous manifestations in contemporary FH patients. METHODS: We prospectively analysed the clinical presentation of FH patients referred to a University lipid clinic and validated these data in the German FH registry CaRe High. RESULTS: Physical examination revealed that only 14.4% of the FH patients in the lipid clinic cohort (n = 223) showed cutaneous manifestations. An arcus cornealis was present in 0.9%, xanthomata in 1.8%, and xanthelasmata in 12.1%. Xanthelasmata are not part of the clinical scores, but represented 84.4% of all cutaneous manifestations. In 42.6% (n = 95) of the patients, genetic analysis was available. A causal FH mutation was detected in 50.5%. Among carriers, 66.7% had no cutaneous manifestation, 8.3% exhibited an arcus cornealis or xanthomata, and 25.0% had xanthelasmata. In the CaRe High FH registry, data on cutaneous manifestations were available in n = 1274 patients. 3.5% had xanthomata, 5.7% an arcus cornealis, and 7.7% at least one of both; xanthelasmata were present in 10.3%. CONCLUSIONS: Cutaneous manifestations are only present in a minority of contemporary patients with FH including the subgroup with monogenic FH mutations. Although rare, the cutaneous signs have value in terms of specificity. However, the clinical characteristics shared by the majority of FH patients may be better suited for screening purposes.
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Hiperlipoproteinemia Tipo II , Enfermedades de la Piel , Xantomatosis , LDL-Colesterol/genética , Pruebas Genéticas , Humanos , Hiperlipoproteinemia Tipo II/diagnóstico , Hiperlipoproteinemia Tipo II/epidemiología , Hiperlipoproteinemia Tipo II/genética , Mutación , Enfermedades de la Piel/diagnóstico , Enfermedades de la Piel/epidemiología , Enfermedades de la Piel/genética , Xantomatosis/diagnóstico , Xantomatosis/epidemiología , Xantomatosis/etiologíaRESUMEN
The present case of a patient with acute myocarditis with preserved left ventricular (LV) ejection fraction at the acute stage illustrates the obvious impairment of circumferential and rotational deformation, which can be documented by speckle tracking echocardiography. Thus, qualitative patterns of LV twist, radial strain, and circumferential layer strain, might be a new approach to detect acute myocarditis. The early diagnosis of acute myocarditis by echocardiography is important because of the considerable risk of cardiovascular morbidity as documented by the occurrence of an acute myocardial infarction presumably induced by inflammatory process in this case.
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Endocarditis Bacteriana/etiología , Válvula Mitral/cirugía , Complicaciones Posoperatorias , Ecocardiografía , Ecocardiografía Transesofágica , Endocarditis Bacteriana/diagnóstico , Endocarditis Bacteriana/diagnóstico por imagen , Prótesis Valvulares Cardíacas/efectos adversos , Implantación de Prótesis de Válvulas Cardíacas/efectos adversos , Humanos , Masculino , Persona de Mediana Edad , Infecciones Relacionadas con Prótesis/diagnóstico , Recurrencia , Reoperación , Infecciones Estafilocócicas/diagnósticoRESUMEN
The aim of the present study was to find out whether early cardiac changes in patients receiving chemotherapy can be detected by the conventional and deformation parameters of 2D and 3D echocardiography. Twenty-five healthy subjects with normal regional left ventricular function (group 1) and 25 patients receiving chemotherapy (group 2) underwent 2D and 3D transthoracic echocardiography (Toshiba Artida Medical System). All patients (group 2) were examined before and during cardiotoxic chemotherapy at a 3-month follow-up. Left ventricular volumes, ejection fraction, muscle mass, global longitudinal, global radial, global circumferential strain, and rotation were analyzed with 2D and 3D echocardiography, while twist and time-to-peak-intervals were analyzed with 3D echocardiography. For left ventricular volumes and muscle mass, no significant differences were seen between the two study groups (P<0.05). According to our results, myocardial dysfunction induced by cardiotoxic chemotherapy can be detected by 2D global radial strain. Detecting myocardial dysfunction by global longitudinal and circumferential strain requires more than 3 months follow-up. Changes in rotation, twist or time-to-peak intervals could not be verified at the 3-month follow-up in the present study. 2D global radial strain seems to be the most sensitive and robust parameter to detect early myocardial damage during chemotherapy. 3D echocardiography is not yet an established method to detect myocardial damage in clinical practice due to lower spatial and temporal resolution.
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Comparison of 3D and 2D speckle tracking performed on standard 2D and triplane 2D datasets of normal and pathological left ventricular (LV) wall-motion patterns with a focus on the effect that 3D volume rate (3DVR), image quality and tracking artifacts have on the agreement between 2D and 3D speckle tracking. 37 patients with normal LV function and 18 patients with ischaemic wall-motion abnormalities underwent 2D and 3D echocardiography, followed by offline speckle tracking measurements. The values of 3D global, regional and segmental strain were compared with the standard 2D and triplane 2D strain values. Correlation analysis with the LV ejection fraction (LVEF) was also performed. The 3D and 2D global strain values correlated good in both normally and abnormally contracting hearts, though systematic differences between the two methods were observed. Of the 3D strain parameters, the area strain showed the best correlation with the LVEF. The numerical agreement of 3D and 2D analyses varied significantly with the volume rate and image quality of the 3D datasets. The highest correlation between 2D and 3D peak systolic strain values was found between 3D area and standard 2D longitudinal strain. Regional wall-motion abnormalities were similarly detected by 2D and 3D speckle tracking. 2DST of triplane datasets showed similar results to those of conventional 2D datasets. 2D and 3D speckle tracking similarly detect normal and pathological wall-motion patterns. Limited image quality has a significant impact on the agreement between 3D and 2D numerical strain values.