Heart failure in congenital heart disease: management options and clinical challenges

Introduction: The population of adults with congenital heart disease (ACHD) is rapidly expanding and one of the major complications is heart failure. Timely diagnosis and treatment are crucial, but strong evidence for effectiveness of heart failure treatment in ACHD is currently lacking. Components of the medical history, physical examination and further diagnostic tests including ECG, echocardiography, cardiac magnetic resonance imaging, exercise testing, and biomarkers can identify patients at risk for early mortality or heart failure. Areas covered: Although the number of studies guiding evidence-based treatment are expanding, many clinical questions have not been completely answered yet. Therefore, in this review we provide an overview of current available insights in epidemiology, diagnosis, risk stratification and treatment options in ACHD patients, including non-medical therapies and advanced care planning. Expert Opinion: We strongly advocate expanding current use of biomarkers in the diagnostic process and timely initiation of discussing advanced treatment options and advanced care planning with patients and their loved ones. More research in multi-center collaborations is needed to study all aspects of care of adult congenital heart disease patients

Early detection of ventricular arrhythmias in adults with congenital heart disease using an insertable cardiac monitor (EDVA-CHD study)

Background: Sudden cardiac death (SCD) due to ventricular arrhythmias (VA) is an important mode of death in adults with congenital heart disease (CHD). Risk stratification is difficult in this heterogeneous population. Insertable cardiac monitors (ICM) may be useful for risk stratification. The purpose of the present study was to evaluate the use of ICM for the detection of VA in adults with CHD. Methods: In this prospective single-center observational study we included consecutive adults with CHD deemed at risk of VA who received an ICM between March 2013 and February 2019. The decision to implant an ICM was made in a Heart Team consisting of a cardiac electrophysiologist and a cardiologist specialized in CHD. Results: A total of 30 patients (mean age, 38 ± 15 years; 50% male) received an ICM. During a median follow-up of 16 months, 8 patients (27%) had documented nonsustained VA. Of these 8 patients, 3 (10%) received a prophylactic ICD. Furthermore, ICM-detected arrhythmias were present in 22 patients (73%) leading to a change in clinical management in 16 patients (53%). Besides the patients receiving an ICD, 10 patients (33%) had a change in their antiarrhythmic drugs, 6 patients (20%) underwent an electrophysiology study, and 1 patient (3%) received a pacemaker. Conclusions: The detection of VA by the ICM contributed to the clinical decision to implant a prophylactic ICD. Furthermore, ICM-detected arrhythmias led to important changes in the clinical management. Therefore, long-term arrhythmia monitoring by an ICM seems valuable for risk stratification in adults with CHD

Influence of age and sex on left ventricular diastolic strain analysis

Speckle tracking analysis (STE) of the left ventricle offers a new method to assess left ventricular (LV) diastolic function. LV diastolic strain measurements offer a non-invasive, global and direct measure of LV diastolic function. However, there is little data on normal values and the influence of anthropomorphic factors which is crucial in clinical practice for new techniques. The aims of this study were to formulate reference values for LV diastolic strain rate, elucidate effects of age and sex on LV diastolic strain analysis and compare STE measurements with conventional LV diastolic measurements. One-hundred-forty-seven healthy subjects aged 20–72 years (≥ 28 subjects per age decade) were prospectively included (Mean age 44 ± 13.7 years, 50% female) and examined with electrocardiography and 2D-echocardiography, including speckle tracking. Left ventricular peak early diastolic strain rate (Sre) was measured in the apical windows, using STE. Men had significantly lower LV Sre values than women (1.02 ± 0.22 vs. 1.18 ± 0.23, p value < 0.001). Left ventricular Sre was inversely associated with age, with values decreasing with ageing. An inverse relation was also found with blood pressure and body surface area. Linear regression analysis showed that LV Sre was independently associated with both age and sex. A multivariable linear regression analysis for LV Sre with conventional LV diastolic variables accounted for 70.9% of the variation of LV Sre, showing good model performance. Reference values for LV Sre are reported and found to be both age- and sex-dependent. Therefore we recommend age- and sex-specific references values to be used in daily clinical practice

Screening for thoracic aortic pathology: Clinical practice in a single tertiary center

Objective: The aim is to present current clinical practice of thoracic aorta screening in a tertiary referral center. We identified how often imaging techniques were used for screening and established the value of transthoracic echocardiography (TTE) in comparison with computed tomography (CT) to detect aortic dilation. We also investigated which additional abnormalities of the heart, aorta or smaller arteries were discovered. Design: All patients ≥15 years who visited our tertiary center in 2012-2016 for first thoracic aortic screening were retrospectively included. Diameters of the sinus of Valsalva (SoV) and maximum ascending aorta (AA) were compared between TTE and CT. The sensitivity and specificity of TTE to detect aortic dilation (≥40 mm) was assessed with CT as reference standard. Intracardiac abnormalities found with TTE and arterial abnormalities found with CT were identified. Results: In total 349 patients (155 men, age 41 ± 15 years, 10% genetic mutation) were included. Screening wa

Prognostic Value of Serial High-Sensitivity Troponin T Measurements in Adults With Congenital Heart Disease

Background: Single high-sensitivity troponin T (hs-TnT) measurement is predictive of cardiac events in adults with congenital heart disease (ACHD). We aimed to study the prognostic value of serial hs-TnT measurements in stable patients with ACHD. Methods: In total, 602 consecutive patients with ACHD were enrolled in this prospective study (2011-2013). Blood sampling was performed at enrollment and thereafter yearly during scheduled visits, up to 4 years. Hs-TnT, N-terminal pro B-type natriuretic peptide (NT-proBNP), and estimated glomerular filtration rate (eGFR) were measured. The composite primary endpoint was defined as all-cause mortality, heart failure, arrhythmia, hospitalization, cardiac (re)interventions, or thromboembolic events. The relationship between changes in serial hs-TnT and the primary endpoint was studied by joint models with adjustment for repeated NT-proBNP and eGFR. Results: In 601 patients (median age, 33 [interquartile range, 25-41] years, 42% women, 90% NYHA I), at least 1 hs-TnT measurement was performed; a mean of 4.3 hs-TnT measurements per patient were collected. After a median follow-up of 5.8 [interquartile range, 5.3-6.3] years, 229 (38.1%) patients reached the primary endpoint. On average, hs-TnT levels increased over time, and more in patients who reached the primary endpoint (P < 0.001). A 2-fold higher hs-TnT was associated with the primary endpoint (unadjusted hazard ratio, 1.62; 95% confidence interval, 1.44-1.82; P < 0.001). The association remained after adjustment for repeated eGFR but not when adjusted for repeated NT-proBNP; repeated NT-proBNP remained associated with the primary endpoint. Conclusion: In stable patients with ACHD, hs-TnT levels increased before the occurrence of an event and repeated hs-TnT was associated with the risk of adverse cardiac events. However, repeated hs-TnT was not superior to repeated NT-proBNP.Contexte : Le dosage unique de la troponine T hypersensible (hs-TnT) est predictif d ’ev enements cardiaques chez les adultes atteints de cardiopathie congenitale. Notre objectif etait d ’etudier la valeur pro- nostique du dosage seriel de la hs-TnT chez des patients adultes atteints de cardiopathie congenitale qui pr esentaient un etat stable. Methodologie : Au total, 602 patients adultes atteints de cardiopathie congenitale ont et e inscrits cons ecutivement à cette etude prospective (2011-2013). Les prelèvements sanguins ont et e effectu es au moment de l’inscription et chaque annee par la suite au cours des visites prevues, jusqu ’à la quatrième annee. La hs-TnT, le propeptide natriuretique de type B N-terminal (NT-proBNP) et le taux de filtration glomerulaire estim e (TFGe) ont et e mesur es. Le paramètre d’evaluation principal regroupait les d ecès toutes causes confondues, l’insuffisance cardiaque, l’arythmie cardiaque, les hospitalisations, les (re)interventions cardiaques et les ev enements thromboemboliques. La relation entre les variations des taux seriels de hs-TnT et le paramètre d’evaluation principal a et e etudi ee à l ’aide de modèles conjoints corriges pour tenir compte de la mesure r ep et ee du taux de NT-proBNP et du TFGe. Resultats : Chez 601 patients (âge median : 33 ans [intervalle inter- quartile : 25-41 ans], 42 % de sexe feminin, 90 % pr esentant une maladie de classe I de la NYHA), au moins un dosage de la hs-TnT a et e effectu e; les investigateurs ont effectu e, en moyenne, 4,3 dosages de la hs-TnT par patient. Au terme d’un suivi median de 5,8 ans [intervalle interquartile : 5,3-6,3 ans], le paramètre d’evaluation prin- cipal a et e atteint chez 229 (38,1 %) patients. E

Psychological well-being in patients with aneurysms-osteoarthritis syndrome

Aneurysms-osteoarthritis syndrome (AOS) is characterized by arterial aneurysms and dissection in combination with early-onset osteoarthritis, which can impact quality of life. We describe the subjective quality of life and investigate anxiety and depression in 28 AOS patients aged 15–73 years. Three questionnaires were used: 36-Item Short Form Survey (SF-36), hospital anxiety and depression scale (HADS) and Rotterdam disease specific questionnaire. Results of the SF-36 and HADS were compared to a reference Dutch cohort and the SF-36 questionnaire also to patients with Marfan syndrome. Compared to the general population, AOS patients scored significantly lower on the following SF-36 domains: physical functioning, vitality, social functioning, bodily pain, and general health. Physical functioning was also lower than in Marfan patients. Patients with AOS scored higher on the HADS depression scale, while anxiety did not show a significant difference compared to the general population. No difference in SF-36 and HADS domain scores were found between patient with and without orthopaedic symptoms and patients with or without previous aortic surgery. Additionally, we found that patients' worries for their future and heredity o

Incidencia del sistema de detracciones en la situación económica y financiera de la empresa de transportes HW E.I.R.L. de la ciudad de Juliaca, periodo 2014 - 2015

TesisEl presente trabajo de investigación titulado “INCIDENCIA DEL SISTEMA DE DETRACCIONES EN LA SITUACIÓN ECONÓMICA Y FINANCIERA DE LA EMPRESA DE TRANSPORTES HW E.I.R.L. DE LA CIUDAD DE JULIACA PERIODO 2014-2015”, se desarrolló con la finalidad de analizar y evaluar la aplicación del Sistema de Detracciones utilizando como instrumento de información a la empresa de Transportes HW E.I.R.L. durante los periodos 2014 – 2015, los resultados han sido obtenidos gracias a los datos recolectados del análisis de los documentos contables, realización de cuadros y gráficos para el debido procesamiento de datos. Las fuentes de información del presente trabajo de investigación fueron el Estado de Situación Financiera, El Estado de Resultados, los libros auxiliares de la empresa en estudio. En la recopilación de información a la empresa de Transportes HW E.I.R.L.se encontró que el Sistema de Detracciones a pesar de que el porcentaje de detracción es minina (4%) ocasiona una disminución en la liquidez, el nivel de liquidez de la empresa en estudio en los periodos 2014 – 2015, fueron de 1.76 y 1.21 para cubrir cada nuevo sol del pasivo corriente, la razón acida muestra una disminución en la capacidad de pago de 1.24 y 0.97 para pagar las deudas a corto plazo y finalmente al evaluar la liquidez absoluta disminuyo a 0.77 y 0.76. En relación al capital de trabajo se obtuvo una disminución con la aplicación del sistema de detracciones a 50,378.64 y 21,876.36 en los dos últimos periodos. Y como segundo análisis realizado a la empresa se tomó el indicador de rentabilidad económica, ya que se observó dinero inmovilizado en la cuenta de detracciones del Banco de la Nación ya que los montos detraídos ascienden a 11,346.39 y 11,728.10 los mismos que representan el 2.98% y 2.92% del total de los activos, la realidad de la empresa respecto a su rentabilidad es de 3.14% y 5.22% para los periodos 2014 y 2015, finalmente para el tercer análisis se observó que las detracciones cubren el total de los tributos a pagar e inclusive existe un saldo para el siguiente pago de tributos para el año 2014 tuvo un saldo de 2,012.27 y para el 2015 se incrementó a 2956.5