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BACKGROUND: Aortic wall shear stress (WSS) is a known predictor of ascending aortic growth in patients with a bicuspid aortic valve (BAV). The aim of this study was to study regional WSS and changes over time in BAV patients. METHODS: BAV patients and age-matched healthy controls underwent 4D flow CMR. Regional, peak systolic ascending aortic WSS, aortic valve function, aortic stiffness measures and aortic dimensions were assessed. In BAV patients, 4D flow CMR was repeated after three years follow-up and both at baseline and follow-up computed tomography angiography (CTA) was acquired. Aortic growth (volume increase of ≥5%) was measured on CTA. Regional WSS differences within patients' aorta and WSS changes over time were analysed using linear mixed-effect models and were associated with clinical parameters. RESULTS: Thirty BAV patients (aged 34 years [IQR 25-41]) were included in the follow-up analysis. Additionally, another 16 BAV patients and 32 healthy controls (aged 33 years [IQR 28-48]) were included for other regional analyses. Magnitude, axial, and circumferential WSS increased over time (all p<0.001) irrespective of aortic growth. The percentage of regions exposed to a magnitude WSS >95th percentile of healthy controls increased from 21% (baseline 506/2400 regions) to 31% (follow-up 734/2400 regions) (p<0.001). WSS angle, a measure of helicity near the aortic wall, decreased during follow-up. Magnitude WSS changes over time were associated with systolic blood pressure, peak aortic valve velocity, aortic valve regurgitation fraction, aortic stiffness indexes, and normalized flow displacement (all p<0.05). CONCLUSIONS: An increase of regional WSS over time was observed in BAV patients, irrespective of aortic growth. The increasing WSSs comprising a larger area of the aorta warrants further research to investigate the possible predictive value for aortic dissection.
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BACKGROUND: Identifying bicuspid aortic valve (BAV) patients at risk for cardiac events remains challenging and the role of the electrocardiogram (ECG) has not yet been described. Therefore, this study aims to describe ECG parameters in BAV patients, and investigate their prognostic value. METHODS: In this single-center prospective study patients with BAV without a prior aortic valve replacement (AVR) were included. Transthoracic echocardiogram and 12lead resting-ECG were obtained. Associations between ECG parameters and the composite endpoint of all-cause mortality and AVR were assessed using Cox-proportional hazard analysis. RESULTS: 120 patients with BAV were included (median age 30 years, 61% male). Median aortic jet velocity was 2.4 m/s [IQR: 1.7-3.4] and 5 patients (4%) had severe aortic regurgitation. All patients were in sinus rhythm. Any ECG abnormality was present in 57 patients (48%). Median PR-interval was 156 [IQR: 138-170] msec. A deviating QRS axis was found in 17 patients (14%) and Cornell criteria for LVH were fulfilled in 20 patients (17%). Repolarization abnormalities were present in 12 patients (10%). Median follow-up duration was 7.0 [6.3-9.8] years, during which 23 patients underwent AVR and 2 patients died. After adjusting for age, a longer PR-interval was associated with worse intervention-free survival (HR 1.02, 95% CI: 1.01-1.04). CONCLUSION: Almost half of the patients with BAV had abnormalities on their ECG. Moreover, the PR-interval may be an interesting prognostic marker for intervention-free survival in BAV patients.
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Background: The adult congenital heart disease (ACHD) population is growing and risk prediction is important to predict adverse outcome and consult patients during their lifecourse. Objectives: This study aims to describe the long-term prognostic value of blood biomarkers in ACHD. Methods: In this prospective observational cohort study, 602 patients with moderate or complex ACHD were included (median age 32.5 years [IQR: 24.7-41.2], 42% female, 90% New York Heart Association I). N-terminal pro-brain natriuretic peptide (NT-proBNP), high-sensitive-troponin T, growth differentiation factor 15, high-sensitive-C-reactive protein, suppression of tumorigenicity-2 and galectin-3, as well as full blood count, renal function, LDL, and HDL were measured. Cox models were applied to relate the selected biomarkers with the primary end point of all-cause mortality and secondary end point of mortality or heart failure. Standardized HRs adjusted for relevant prognostic factors, including age, sex, and complexity of diagnosis, were reported. Results: Abnormal biomarker levels were present in 424 (70.4%) patients. During a median follow-up of 10.1 years, 41 (6.8%) patients died and 81 (13.5%) developed heart failure. Associations were observed between the primary and secondary end point and red cell distribution width, NT-proBNP, and growth differentiation factor 15. In a multibiomarker model, only NT-proBNP remained associated with mortality (HR: 2.74; 95% CI: 2.01-3.74). NT-proBNP significantly improved the C-statistic of the clinical prediction model (0.85-0.92). Based on NT-proBNP alone, low-risk patients could be identified. Patients with NT-proBNP <76 ng/L showed a 10-year heart failure-free survival of 98.5%. Conclusions: Blood biomarkers have prognostic value in ACHD. NT-proBNP improves risk prediction and is able to identify low-risk patients. Its routine use should be implemented in ACHD.
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Therapies for inoperable chronic thromboembolic pulmonary hypertension (CTEPH) include balloon pulmonary angioplasty (BPA) and PH-specific medical therapy. This study compares survival and its predictors before and after the introduction of BPA. BPA was independently associated with survival; however, there was no difference in overall survival between the two cohorts.
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Aims: By combining temporal changes in left ventricular (LV) global longitudinal strain (GLS) with LV volume, LV strain-volume loops can assess cardiac function across the cardiac cycle. This study compared LV strain-volume loops between bicuspid aortic valve (BAV) patients and controls, and investigated the loop's prognostic value for clinical events. Methods and results: From a prospective cohort of congenital heart disease patients, BAV patients were selected and compared with healthy volunteers, who were matched for age and sex at group level. GLS analysis from apical views was used to construct strain-volume loops. Associations with clinical events, i.e. a composite of all-cause mortality, heart failure, arrhythmias, and aortic valve replacement, were assessed by Cox regression. A total of 113 BAV patients were included (median age 32 years, 40% female). BAV patients demonstrated lower Sslope (0.21%/mL, [Q1-Q3: 0.17-0.28] vs. 0.27%/mL [0.24-0.34], P < 0.001) and ESslope (0.19%/mL [0.12-0.25] vs. 0.29%/mL [0.21-0.43], P < 0.001) compared with controls, but also greater uncoupling during early (0.48 ± 1.29 vs. 0.05 ± 1.21, P = 0.04) and late diastole (0.66 ± 1.02 vs. -0.07 ± 1.07, P < 0.001). Median follow-up duration was 9.9 [9.3-10.4] years. Peak aortic jet velocity (HR 1.22, P = 0.03), enlarged left atrium (HR 3.16, P = 0.003), E/e' ratio (HR 1.17, P = 0.002), GLS (HR 1.16, P = 0.008), and ESslope (HR 0.66, P = 0.04) were associated with the occurrence of clinical events. Conclusion: Greater uncoupling and lower systolic and diastolic slopes were observed in BAV patients compared with healthy controls, suggesting presence of altered LV cardiomechanics. Moreover, lower ESslope was associated with clinical events, highlighting the strain-volume loop's potential as prognostic marker.
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OBJECTIVE: This study aims to compare aortic morphology between repaired coarctation patients and controls, and to identify aortic morphological risk factors for hypertension and cardiovascular events (CVEs) in coarctation patients. METHODS: Repaired coarctation patients with computed tomography angiography (CTA) or magnetic resonance angiography (MRA) were included, followed-up and compared with sex-matched and age-matched controls. Three-dimensional aortic shape was reconstructed using patients' CTA or MRA, or four-dimensional flow cardiovascular magnetic resonance in controls, and advanced geometrical characteristics were calculated and visualised using statistical shape modelling. In patients, we examined the association of geometrical characteristics with (1) baseline hypertension, using multivariable logistic regression; and (2) cardiovascular events (CVE, composite of aortic complications, coronary artery disease, ventricular arrhythmias, heart failure hospitalisation, stroke, transient ischaemic attacks and cardiovascular death), using multivariable Cox regression. The least absolute shrinkage and selection operator (LASSO) method selected the most informative multivariable model. RESULTS: Sixty-five repaired coarctation patients (23 years (IQR 19-38)) were included, of which 44 (68%) patients were hypertensive at baseline. After a median follow-up of 8.7 years (IQR 4.8-15.4), 27 CVEs occurred in 20 patients. Aortic arch dimensions were smaller in patients compared with controls (diameter p<0.001, wall surface area p=0.026, volume p=0.007). Patients had more aortic arch torsion (p<0.001) and a higher curvature (p<0.001). No geometrical characteristics were associated with hypertension. LASSO selected left ventricular mass, male sex, tortuosity and age for the multivariable model. Left ventricular mass (p=0.014) was independently associated with CVE, and aortic tortuosity showed a trend towards significance (p=0.070). CONCLUSION: Repaired coarctation patients have a smaller aortic arch and a more tortuous course of the aorta compared with controls. Besides left ventricular mass index, geometrical features might be of importance in long-term risk assessment in coarctation patients.
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Coartación Aórtica , Angiografía por Tomografía Computarizada , Angiografía por Resonancia Magnética , Humanos , Coartación Aórtica/cirugía , Coartación Aórtica/complicaciones , Coartación Aórtica/diagnóstico por imagen , Masculino , Femenino , Angiografía por Tomografía Computarizada/métodos , Adulto , Factores de Riesgo , Adulto Joven , Estudios de Seguimiento , Factores de Tiempo , Aorta Torácica/diagnóstico por imagen , Aorta Torácica/cirugía , Estudios Retrospectivos , Imagen por Resonancia Cinemagnética/métodos , Medición de Riesgo/métodos , Resultado del Tratamiento , Hipertensión/complicaciones , Hipertensión/fisiopatología , AdolescenteRESUMEN
BACKGROUND: Adults with a systemic right ventricle (sRV) are at a high risk for heart failure (HF) hospitalization and mortality. Bioactive adrenomedullin (bio-ADM) has been proposed as a marker of congestion and prognosis in patients with cardiovascular disease. We aimed to evaluate the association between bio-ADM and mortality and HF events in sRV patients. METHODS: Plasma bio-ADM was measured by a novel immunoassay in plasma of 85 sRV patients. A composite endpoint of all-cause mortality and HF events was used as outcome. HF events were defined as onset or progression of HF signs or symptoms requiring hospitalization, initiation or intensification of therapy. Multivariable Cox regression analyses were performed to evaluate the association between bio-ADM and outcome. RESULTS: The mean age of the patients was 37 ± 9 years and 65% were male. Patients with higher plasma bio-ADM concentrations were more often treated with diuretics (p = 0.007), possibly because of signs and/or symptoms of congestion. During a median follow-up of 10.2 years, 33.7% of the patients reached the endpoint. After adjustment for age and N-terminal pro B-type natriuretic peptide (NT-pro BNP), higher bio-ADM levels were associated with a higher risk of the composite endpoint (hazard ratio: 2.09 [95%-confidence interval: 1.15-3.78]). Bio-ADM improved risk prediction when added to NT-proBNP and age (C-statistic improved from 0.748 to 0.776 [p = 0.03]). CONCLUSIONS: Bio-ADM can be considered as a marker of congestion and independent predictor of death and HF events in adult patients with a sRV. Moreover, in terms of risk prediction, it has added value to NT-proBNP.
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Adrenomedulina , Biomarcadores , Progresión de la Enfermedad , Insuficiencia Cardíaca , Humanos , Adrenomedulina/sangre , Masculino , Femenino , Biomarcadores/sangre , Adulto , Persona de Mediana Edad , Insuficiencia Cardíaca/sangre , Insuficiencia Cardíaca/diagnóstico , Estudios de Seguimiento , Disfunción Ventricular Derecha/sangre , Disfunción Ventricular Derecha/diagnóstico , Ventrículos Cardíacos/diagnóstico por imagen , Fragmentos de Péptidos/sangreRESUMEN
Aims: The prognostic value of functional tricuspid valve regurgitation (TR) in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension (CTEPH) remains undetermined. This study primarily aims to quantify the prognostic role of TR in relation to right ventricle (RV) dysfunction on clinical outcomes and secondarily the evolution of TR and RV dysfunction over time. Methods: Adult PAH or CTEPH patients diagnosed by right heart catheterization were included. Exclusion criteria were prevalent patients and age < 18 years.The primary endpoint was a composite of death or lung transplantation. Longitudinal evolution of TR and RV dysfunction were modelled with generalized mixed-effect models, which were inserted in a cox model under the joint-modelling framework in order to investigate the association of TR and RV dysfunction with the endpoint. Results: We included 76 PAH and 44 CTEPH patients (median age:59, females:62 %), with a mean follow-up of 3.2 ± 2.1 years. 31 patients reached the endpoint (2 transplant, 29 mortality). On average the probability of moderate-to-severe TR decreased during follow-up, whereas the probability of moderate-to-severe RV dysfunction remained stable. The cumulative effect of moderate-to-severe TR (HRper day 1.01 95 %CI[1.00-1.01],P < 0.001) and moderate-to-severe RV dysfunction (HRper day: 1.01 95 %CI[1.00-1.01],P < 0.001) was associated with the endpoint in univariable joint-models. In a multivariable joint-model with both the evolutions of TR and RV dysfunction only TR remained significant (HR per day: 1.01 95 %CI[1.00-1.01],P < 0.001). Conclusion: Persistent moderate-to-severe tricuspid valve regurgitation during follow-up predicts adverse outcomes and might be a better predictor of lung transplantation and mortality compared to right ventricle dysfunction.
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Background: Bicuspid aortic valve (BAV) is a common congenital heart defect. Patients with BAV are at risk for long-term complications such as valve stenosis and regurgitation. This study aimed to investigate sex differences in blood and imaging biomarkers and to describe the long-term prognostic value of blood and echocardiographic biomarkers. Methods: Patients were included from 2 prospective observational cohort studies; they underwent venous blood sampling and transthoracic echocardiography including speckle tracking. Analyzed blood biomarkers were red-cell distribution width (RDW), creatinine, C-reactive protein (CRP), troponin T, N-terminal pro B-type natriuretic peptide (NT-proBNP), and transforming growth factor-beta (TGF-ß). Sex differences were analyzed at baseline. Associations between biomarkers and arrhythmia-free and intervention-free survival were determined by Cox regression, adjusted for age and sex. Results: A total of 182 patients with BAV were included: median age 34; interquartile range [IQR]: 23-46 years; 55.5% male. CRP, NT-proBNP, and RDW were higher in women, whereas creatinine, troponin T and TGF-ß were higher among men. After a median follow-up time of 6.9 (IQR: 6.5-9.9) years, arrhythmia-free and intervention-free survival was, 81.0% and 73.1%, respectively. NT-proBNP was associated with both arrhythmia-free and intervention-free survival (hazard ratio [HR], 1.94, P = 0.005 and HR, 2.06, P = 0.002, respectively). On echocardiography higher left atrial (LA) size, left ventricular end-diastolic diameter (LVEDD), left ventricular (LV) mass index and E/e' ratio were associated with lower arrhythmia-free survival, whereas higher LA size, LV mass index, aortic valve peak velocity, and aortic regurgitation were associated with lower intervention-free survival. Conclusions: Differences were observed in blood biomarkers between men and women with BAV. Besides LV systolic parameters, diastolic LV function and NT-proBNP should have a more prominent role as prognostic markers in clinical care.
Contexte: La bicuspide valvulaire aortique (BVA) est une anomalie cardiaque congénitale fréquente. Les patients atteints d'une BVA présentent des risques de complications à long terme, comme la sténose valvulaire ou la régurgitation valvulaire. Cette étude visait 1) à évaluer les différences entre les sexes en ce qui concerne les biomarqueurs sanguins et les biomarqueurs à l'imagerie; et 2) à décrire la valeur pronostique à long terme des biomarqueurs sanguins et échocardiographiques. Méthodologie: Des patients de 2 études de cohortes observationnelles prospectives ont été inclus dans l'analyse. Des échantillons de sang veineux ont été prélevés, et des échocardiographies transthoraciques, y compris le suivi des marqueurs acoustiques, ont été effectuées. Les biomarqueurs sanguins analysés étaient les suivants : indice de distribution des globules rouges (IDR), créatinine, protéine C-réactive (CRP), troponine T, propeptide natriurétique de type B N-terminal (NT-proBNP) et facteur de croissance transformant ß (TGF-ß). Les différences entre les sexes ont été analysées au départ. Les liens entre les biomarqueurs et la survie sans arythmie et sans intervention ont été déterminés par la régression de Cox, avec correction en fonction de l'âge et du sexe. Résultats: Cent quatre-vingt-deux patients présentant une BVA étaient inclus (âge médian de 34 [écart interquartile : 23-46] ans, 55,5 % hommes). La CRP, la NT-proBNP et l'IDR étaient plus élevées chez les femmes, alors que la créatinine, la troponine T et le TGF-ß étaient plus élevés chez les hommes. Après une période de suivi médiane de 6,9 (écart interquartile : 6,5-9,9) ans, les taux de survie sans arythmie et sans intervention étaient respectivement de 81,0 % et de 73,1 %. La NT-proBNP a été associée à la survie sans arythmie (rapport des risques instantanés [RRI] : 1,94, p = 0,005) et à la survie sans intervention (RRI : 2,06, p = 0,002). À l'échocardiographie, des valeurs élevées pour la taille de l'oreillette gauche, le diamètre télédiastolique du ventricule gauche (VG), l'indice de masse du VG et le rapport E/e' étaient associées à un faible taux de survie sans arythmie, alors que des valeurs élevées pour la taille de l'oreillette gauche, l'indice de masse du VG, la vitesse maximale aortique et la régurgitation aortique étaient associées à un faible taux de survie sans intervention. Conclusions: Les biomarqueurs sanguins variaient en fonction du sexe des personnes présentant une BVA. Outre les paramètres systoliques du VG, la fonction VG diastolique et la NT-proBNP devraient être davantage utilisées comme marqueurs pronostiques en soins cliniques.
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OBJECTIVES: Congenital supravalvular aortic stenosis (SVAS) is a rare form of congenital outflow tract obstruction and long-term outcomes are scarcely reported. This study aims to provide an overview of outcomes after surgical repair for congenital SVAS. METHODS: A systematic review of published literature was conducted, including observational studies reporting long-term clinical outcome (>2 years) after SVAS repair in children or adults considering >20 patients. Early risks, late event rates and time-to-event data were pooled and entered into a microsimulation model to estimate 30-year outcomes. Life expectancy was compared to the age-, sex- and origin-matched general population. RESULTS: Twenty-three publications were included, encompassing a total of 1472 patients (13 125 patient-years; pooled mean follow-up: 9.0 (6.2) years; median follow-up: 6.3 years). Pooled mean age at surgical repair was 4.7 (5.8) years and the most commonly used surgical technique was the single-patch repair (43.6%). Pooled early mortality was 4.2% (95% confidence interval: 3.2-5.5%) and late mortality was 0.61% (95% CI: 0.45-0.83) per patient-year. Based on microsimulation, over a 30-year time horizon, it was estimated that an average patient with SVAS repair (mean age: 4.7 years) had an observed life expectancy that was 90.7% (95% credible interval: 90.0-91.6%) of expected life expectancy in the matched general population. The microsimulation-based 30-year risk of myocardial infarction was 8.1% (95% credible interval: 7.3-9.9%) and reintervention 31.3% (95% credible interval: 29.6-33.4%), of which 27.2% (95% credible interval: 25.8-29.1) due to repair dysfunction. CONCLUSIONS: After surgical repair for SVAS, 30-year survival is lower than the matched-general-population survival and the lifetime risk of reintervention is considerable. Therefore, lifelong monitoring of the cardiovascular system and in particular residual stenosis and coronary obstruction is recommended.
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Estenosis Aórtica Supravalvular , Estenosis de la Válvula Aórtica , Implantación de Prótesis de Válvulas Cardíacas , Niño , Adulto , Humanos , Preescolar , Estenosis Aórtica Supravalvular/cirugía , Implantación de Prótesis de Válvulas Cardíacas/efectos adversos , Reoperación , Constricción Patológica/etiología , Resultado del TratamientoRESUMEN
BACKGROUND: Higher resting heart rate has been described as a risk factor for adverse outcome in healthy individuals and cardiovascular patients. The aim of this study was to evaluate resting heart rate as risk factor in adult congenital heart disease (ACHD). METHODS: In this prospective observational cohort study, patients with moderate or complex ACHD were included at routine outpatient visit. Standard 12-lead ECGs were obtained in rest. Heart rate was obtained from the ECG automatically by the Modular ECG Analysis System (MEANS). The primary endpoint was all-cause mortality and the secondary endpoint was a composite of all-cause mortality and heart failure. Survival was derived using the Kaplan-Meier estimator. Subgroups based on heart rate tertiles were compared by the log-rank test. Cox proportional hazards models were adjusted for clinical factors including age, sex and diagnosis (moderate vs complex ACHD). RESULTS: A total of 556 patients were included (median age 32 years (IQR 24-41), 57.6% male). Mean heart rate was 69±13 bpm. Negative chronotropic medication was used by 74 (13.3%) patients. During a median follow-up of 10.1 (IQR 9.6-10.5) years, 36 patients (6.5%) died and 83 (14.9%) reached the secondary endpoint. Patients with higher heart rates had significantly lower survival and heart failure-free survival. After adjusting for clinical factors, heart rate remained associated with mortality (HR 1.57 per 10 bpm, 95% CI 1.26 to 1.96) and mortality or heart failure (HR 1.33 per 10 bpm, 95% CI 1.13 to 1.57). CONCLUSION: Higher heart rate is associated with lower survival and heart failure-free survival in ACHD.
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Cardiopatías Congénitas , Insuficiencia Cardíaca , Humanos , Adulto , Masculino , Femenino , Frecuencia Cardíaca , Estudios Prospectivos , Factores de RiesgoRESUMEN
OBJECTIVES: To describe the clinical outcome and quality of life up to 50 years after surgical atrial septal defect (ASD) closure at young age. Primary outcome is defined as MACE (all-cause mortality, cardiac re-interventions, ischemic stroke, endocarditis, heart failure and symptomatic arrhythmia). METHODS: Single-center, longitudinal cohort-study evaluating 135 consecutive patients who underwent ASD-closure before the age of 15 years between 1968 and 1980. Participants were invited for extensive cardiac evaluation and assessment of quality-of-life every 10 years. RESULTS: Eighty patients (86%) of 93 eligible survivors were included in this study (mean age 52 ± 5 years (range 41-63), 40% male). Median follow-up since surgery was 45 years (range 40-51). Cumulative survival after 50 years was 86% and comparable to the normal Dutch population. Cumulative event-free survival after 45 and 50-years was 59% and 46% respectively (re-intervention in 6, symptomatic arrhythmia in 25, and pacemaker implantation in 10 patients). Right ventricular ejection fraction on CMR was diminished in 6%. Exercise capacity was normal in 77%. There was no pulmonary hypertension. NT-proBNP was elevated in 61%. Quality of life was comparable with the general population. No predictors for late events were identified. CONCLUSION: Long-term survival after surgical ASD-closure in childhood is good and not statistically different at 50 years compared to the normal Dutch population. Re-intervention rate is low, there is no pulmonary hypertension. Right ventricular function was diminished in 6%, exercise capacity was good and stable over time with quality of life comparable to the general population. However, supraventricular tachycardia is common.
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Defectos del Tabique Interatrial , Hipertensión , Humanos , Masculino , Adulto , Persona de Mediana Edad , Adolescente , Femenino , Estudios de Seguimiento , Calidad de Vida , Resultado del Tratamiento , Volumen Sistólico , Función Ventricular Derecha , Defectos del Tabique Interatrial/diagnóstico , Defectos del Tabique Interatrial/cirugía , Arritmias Cardíacas/etiología , Hipertensión/complicaciones , Cateterismo Cardíaco/efectos adversosRESUMEN
To demonstrate prognostic utility of left atrial strain (LAS) in adult patients with repaired Tetralogy of Fallot (rTOF). Adults patients with rTOF were prospectively enrolled in this study between years 2011 and 2015. Left atrium (LA) phasic functions were assessed using 2D speckle tracking echocardiography. Association of LA strain (LAS) with primary (any cardiovascular event) and secondary (death, heart failure or arrhythmia) composite endpoints was assessed using Cox regression analysis. Hundred-and-twelve rTOF patients, in whom LAS was feasible and were in sinus rhythm, were included in the final analysis (age 33 ± 10 years, 68[61%] male). Median duration of follow-up was 8.6 [4.2-9.7] years in the study group. Primary composite endpoint was reached in 48 patients (mean event-free survival time: 7.2 [6.6-7.9] years), and secondary composite endpoint was reached in 22 patients (mean event-free survival time: 8.7 [8.1-9.2] years). LA reservoir strain (LAS-r) was defined as tertile groups (1st tertile < 33%, 2nd tertile = 33-44%, 3rd tertile > 44%). Decreasing tertiles of LAS-r was associated with primary and secondary composite endpoints in Kaplan-Meier analysis (p = 0.02 and 0.002, respectively). In univariable Cox-regression, both decreasing LAS-r and LAS-r tertiles were associated with primary and secondary composite endpoints. Adjusted by initial repair age and NT-proBNP quartiles, increased LAS-r was associated with significantly decreased occurrence of experiencing any events (HR = 0.97, CI 0.93-0.99, p < 0.001). Decreasing LAS-r was still associated with primary endpoint when adjusted by left atrium volume index (LAVImax) (HR = 0.96, CI 0.92-0.99, p = 0.01), left ventricle global longitudinal strain (HR = 0.96, CI 0.93-0.99, p < 0.001) or right ventricle free wall longitudinal strain (HR = 0.96, CI 0.93-0.99, p = 0.03). Assessment of LA mechanics with the use of STE has incremental utility in determination of mortality and morbidity in rTOF, and may be implemented in clinical practice.
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Tetralogía de Fallot , Adulto , Humanos , Masculino , Adulto Joven , Femenino , Tetralogía de Fallot/diagnóstico por imagen , Tetralogía de Fallot/cirugía , Pronóstico , Valor Predictivo de las Pruebas , Atrios Cardíacos/diagnóstico por imagen , EcocardiografíaRESUMEN
Aims: Although survival of patients with congenital heart disease (CHD) improved significantly over time, life expectancy is still not normal. We aimed to investigate how adult patients, their partners, and treating cardiologists estimated the individual life expectancy of CHD patients. Furthermore, preferences regarding end-of-life (EOL) communication were investigated. Methods and results: In this study, we included 202 patients (age: 50 ± 5) who were operated in childhood (<15 years old) between 1968 and 1980 for one of the following diagnoses: atrial septal defect, ventricular septal defect, pulmonary stenosis, tetralogy of Fallot, or transposition of the great arteries. A specific questionnaire was administered to both the patients and their partners, exploring their perceived life expectancy and EOL wishes. Two cardiologists independently assessed the life expectancy of each patient. Most adults with CHD believed their life expectancy to be normal. However, significant differences were found between estimated life expectancy by the cardiologist and patients (female: P = 0.001, male: P = 0.002) with moderate/severe defects, as well as for males with mild defects (P = 0.011). Regarding EOL communication, 85.1% of the patients reported that they never discussed EOL with a healthcare professional. Compared with patients with mild CHD, significantly more patients with moderate/severe defect discussed EOL with a physician (P = 0.011). The wish to discuss EOL with the cardiologist was reported by 49.3% of the patients and 41.7% of their partners. Conclusion: Adult patients, especially with moderate/severe CHD, perceived their life expectancy as normal, whereas cardiologists had a more pessimistic view than their patients. Increased attention is warranted for discussions on life expectancy and EOL to improve patient-tailored care.
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BACKGROUND: Sexual health related quality of life (SHRQoL) is an important pillar of health related quality of life (HRQoL). The aim of this study was to investigate sexual functioning in men and women with pulmonary hypertension (PH). METHODS AND RESULTS: In this cross-sectional study, a total of 78 patients were included, 49 were diagnosed with pulmonary arterial hypertension and 29 with chronic thromboembolic pulmonary hypertension (median age 53 [IQR: 46-67 years], 66.7% female). All patients completed SHRQoL questionnaires; for women: ASEX, FSFI, and FSDS and for men: ASEX and IIEF. A PH-specific SHRQoL questionnaire was created based on 4 semi-structured interviews to investigate PH-specific barriers in sexuality. More than half of the patients experienced symptoms during sexual activity, mainly dyspnea (52.6%) and palpitations (32.1%). Sexual dysfunction was present, according to the FSFI-questionnaire, in 63.0% of women. All of the men experienced at least mild dysfunction in one of the domains of the IIEF and erectile dysfunction was present in 48.0%. Sexual dysfunction occurred more often in both men and women with PH than in the general population. PAH-specific medication was not associated with sexual dysfunction, nor was subcutaneous or intravenous pump therapy (OR 1.14, 95%-CI: 0.75-1.73). Diuretics were associated with sexual dysfunction in women (OR 4.01, 95%-CI: 1.04-15.41). Of all patients committed in a relationship, 69.0% would like to discuss sexuality with their healthcare provider. CONCLUSION: This study showed a high prevalence of sexual dysfunction in men and women with PH. It is important for healthcare providers to discuss sexuality with patients.
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Disfunción Eréctil , Hipertensión Pulmonar , Disfunciones Sexuales Fisiológicas , Masculino , Humanos , Femenino , Persona de Mediana Edad , Calidad de Vida , Hipertensión Pulmonar/diagnóstico , Hipertensión Pulmonar/epidemiología , Estudios Transversales , Disfunción Eréctil/tratamiento farmacológico , Disfunción Eréctil/epidemiología , Disfunciones Sexuales Fisiológicas/epidemiología , Disfunciones Sexuales Fisiológicas/etiología , Encuestas y Cuestionarios , Conducta SexualRESUMEN
INTRODUCTION: The prognosis of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) remains dismal. Better risk prediction is needed. This study investigated the prognostic value of ECG characteristics. METHODS: In this single-centre prospective study, consecutive treatment-naïve patients with PAH or CTEPH were included at time of diagnosis. From the 12-lead ECG, obtained at baseline, the following parameters were collected: heart rate (HR), rhythm, QRS axis, conduction times, P-top amplitudes in II, R-top and S-wave amplitudes in V1 and V5 and repolarisation disorders. Associations between the ECG and transplant-free survival was assessed by Kaplan-Meier curves and Cox-proportional hazard regressions. RESULTS: In total, 140 patients were included (median age: 60.7 years, 63.6% female). The ECG was abnormal in 86.2%: sinus rhythm was not present in 9.3%, right QRS axis was observed in 47.8%, mean QRS duration was 101±17 ms. Only 42.5% of the patients had normal repolarisation, 34.5% had right ventricular strain and 14.4% non-specific repolarisation disorders. Over a median follow-up time of 3.49 (IQR: 1.37-6.42) years, 45 patients (32.5%) died or underwent lung transplantation. Transplant-free survival was worse in patients presenting with an abnormal ECG (64.0% vs 86.0%; p=0.037). The following ECG characteristics were associated with all-cause mortality or lung transplantation: heart rate (HR 1.02, 95% CI: 1.00 to 1.05), QRS duration >120 ms (HR 2.61, 95% CI: 1.01 to 6.71) and S-wave amplitude in V5 (HR 1.10, 95% CI: 1.04 to 1.17). CONCLUSION: Only 13.8% of patients with PAH and CTEPH presented with a normal ECG, which is associated with favourable outcome. The ECG provides additional prognostic value to current clinical parameters and should be considered in risk prediction.
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Hipertensión Pulmonar , Hipertensión Arterial Pulmonar , Humanos , Femenino , Persona de Mediana Edad , Masculino , Hipertensión Pulmonar/diagnóstico , Hipertensión Pulmonar/etiología , Estudios Prospectivos , Electrocardiografía , Hipertensión Pulmonar Primaria Familiar , Pronóstico , Medición de RiesgoRESUMEN
Aims: Advanced transthoracic echocardiography (TTE) using volumetric and deformational indices provides detailed quantification of right ventricular (RV) function in adults with congenital heart disease (ACHD). Two-dimensional multi-plane echocardiography (2D-MPE) has demonstrated regional wall differences in RV longitudinal strain (LS). This study aims to evaluate the association of these parameters with cardiovascular magnetic resonance (CMR). Methods and results: One-hundred stable ACHD patients with primarily affected RVs were included (age 50 ± 5 years; 53% male). Conventional and advanced echocardiographic RV functional parameters were compared with CMR-derived RV function. Advanced echocardiographic RV functional parameters were measurable in approximately one-half of the study cohort, while multi-wall LS assessment feasibility was lower. CMR RV ejection fraction (CMR-RVEF) was moderately correlated with deformational, area, and volumetric parameters [RV global LS (lateral wall and septum), n = 55: r = -0.62, P < 0.001; RV wall average LS, n = 34: r = -0.49, P = 0.002; RV lateral wall LS, n = 56: r = -0.45, P < 0.001; fractional area change, n = 67: r = 0.48, P < 0.001; 3D-RVEF, n = 48: r = 0.40, P = 0.005]. Conventional measurements such as TAPSE and RV S' correlated poorly. RV global LS best identified CMR-RVEF < 45% (area under the curve: 0.84, P < 0.001: cut-off value -19%: sensitivity 100%, specificity 57%). RVEF and LS values were significantly higher when measured by CMR compared with TTE (mean difference RVEF: 5 [-9 to 18] %; lateral (free) wall LS: -7 [7 to -21] %; RV global LS: -6 [5 to -16] %) while there was no association between respective LS values. Conclusion: In ACHD patients, advanced echocardiographic RV functional parameters are moderately correlated with CMR-RVEF, although significant differences exist between indices measurable by both modalities.
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The prognosis of pulmonary arterial hypertension (PAH) remains dismal. Over the years, multiple therapeutic advances have been introduced. This study evaluates the evolution of PAH survival over the past 15 years. We included 293 consecutive adult patients diagnosed with PAH between 2005 and 2019 (median age: 61.8 years, 70.3% female). Patients were divided into three cohorts based on the time of diagnosis: 2005-2009, 2010-2014, and 2015-2019 (2005-2009: n = 56; 2010-2014: n = 111; 2015-2019: n = 126). Transplant-free survival was measured from the date of right heart catheterization until patients reached the composite endpoint of lung transplant or death. Multivariable cox-pulmonary hypertension regression was used to study the effect of the time of diagnosis. The final cox model was fitted in both younger and older patients to evaluate the difference between these groups. During a median follow-up time of 4.1 (interquartile range: 2.2-7.3) years, 9 patients underwent lung transplantation and 151 patients died. The median overall transplant-free survival was 6.2 (5.5-8.0) years. Patients older than 56 years at baseline who were diagnosed in 2005-2009 showed better survival compared to patients diagnosed in 2010-2014 and 2015-2019 with an adjusted hazard ratio of, respectively, 2.12 (1.11-4.03) and 2.83 (1.41-5.69). Patients younger than 56 years showed neither an improved nor deteriorated survival over time. In conclusion, survival in patients with PAH did not improve over time, despite more available therapeutic options. This might be partly due to the changed demographic characteristics of the PAH patients and a still important diagnostic delay.
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Aim: To explore whether left atrial (LA) strain with speckle tracking echocardiography (STE) can contribute to prognostication in patients with congenital aortic stenosis (CAS). Methods and results: In this prospective study, consecutive outpatients with stable CAS and healthy adults were enrolled between 2011 and 2015. Left atrial function was analysed with STE using Tomtec software. Associations between LA strain (LAS) measurements and primary composite outcome (any adverse cardiovascular event, hospitalization, or re-intervention) and secondary outcome (re-interventions) were assessed with Cox regression analysis. In total, 98 patients with CAS (mean age: 35.0 ± 11.9 year, female: 59.2%) and 121 controls (age: 43.9 ± 13.8 year, female: 55.4%) were included. The majority of patients were in NYHA class I: 97 (99%) at baseline. At baseline, LA conduit strain (LAS-cd) and strain rate (LASR-cd) were significantly lower in patients than in controls when corrected for age and sex (-18.1 ± 8.7 vs. -23.5 ± 9.9%, P = 0.001 and -0.73 ± 0.31 vs. -1.02 ± 0.43/s, P < 0.001). During a median follow-up of 6.4 years (5.7-7.1), the primary composite outcome occurred in 48 (39.6%) patients. Kaplan-Meier analysis showed that decreased LAS-cd (<21%) was associated with a higher occurrence of the primary outcome (log-rank: P = 0.008). Depressed LAS-cd and LASR-cd were both associated with the primary composite outcome [univariable hazard ratio (HR) = 0.64(0.46-0.88), P = 0.005 and HR = 0.68(0.55-0.83), P < 0.001, respectively]; adjusted HR (for LAS-cd and LASR-cd, respectively): 0.31(0.09-1.04), P = 0.06 and 0.49(0.26-0.89), P = 0.02. Conclusion: Impairment in LA conduit function assessed with STE carries prognostic value in patients with CAS and can be implemented in clinical management.