Incidence of Bloodstream Infection in Patients with Pulmonary Hypertension under Intravenous Epoprostenol or Iloprost-A Multicentre, Retrospective Study.

Intravenous synthetic prostacyclin analogs (iPCAs), such as epoprostenol, treprostinil and iloprost have been widely used for the treatment of pulmonary arterial hypertension (PAH). Despite having good outcomes, continuous infusion of iPCAs has been associated with some adverse effects. Bloodstream infection (BSI) is one of the most severe complications, although poorly recognized, especially under iloprost administration, which few studies have addressed. This study aimed to compare the BSI incidence rates between intravenous iloprost and epoprostenol administration. Patients with pulmonary hypertension (PH) functional class III or IV receiving intravenous iloprost or epoprostenol through Hickman catheter, between 2004 and 2019, were retrospectively selected from two PH treatment centers. From a total of 36 patients (13 for iloprost and 23 for epoprostenol), 75% (n = 27) fulfilled the PAH criteria, mainly belonging to the idiopathic group. Overall BSI rate was 1.5/1000 days of treatment (3.38 and 0.09/1000 days for iloprost and epoprostenol, respectively). Patients receiving iloprost were at a higher risk of developing BSI than those receiving epoprostenol (HR: 12.5; 95% CI: 1.569-99.092). A higher mortality rate from BSI was also identified in the iloprost group (p = 0.04). Twenty-seven patients developed BSI, with 92% of them requiring hospitalization. A total of 29 agents were found, 10 Gram-positive (mainly Staphylococcus aureus; n = 5) and 19 Gram-negative (mainly Pseudomonas aeruginosa; n = 6) bacteria. Iloprost administration was linked to a significantly higher incidence of BSI, worse prognosis, and more BSI-related deaths than epoprostenol. BSI due to Gram-negative, commensal, low-virulence bacteria was also higher in the iloprost group. In short, physicians should be aware when prescribing iPCA to guarantee their patients' safety and best medical care.

Chronic thromboembolic pulmonary hypertension: A 10-year analysis from a Portuguese referral center.

INTRODUCTION AND OBJECTIVES: Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive, but potentially curable, form of pulmonary hypertension. Pulmonary endarterectomy (PEA) is a complex surgery that frequently achieves hemodynamic normalization and symptom resolution, although not all patients are suitable for the procedure. We aimed to assess long-term outcomes of CTEPH, namely all-cause mortality and hospital admission for decompensated heart failure, according to treatment modalities in patients who underwent PEA or non-surgical therapy. METHODS: A 10-year retrospective study of patients with CTEPH at a referral center was conducted. Forty-five patients were included and median follow-up time was 57 (IQR 24-93) months. Survival analysis was performed and a multivariate Cox regression model was used to identify independent predictors of outcomes. RESULTS: Patients were mostly female (59%) and mean age was 63±16 years. Two-thirds were severely symptomatic at diagnosis, with 62.2% of patients presenting in WHO functional class (WHO FC) III or IV. One-, two- and three-year survival was 93.3%, 82.4% and 75.9%, respectively. Serum BNP (HR 1.003; 95% CI: 1.001-1.005; p=0.003) and creatinine (HR 12.092; 95% CI: 1.121-130.390; p=0.040) were predictors of death. Mortality was numerically lower in those who underwent PEA (p=0.135). PEA was associated with decreased risk of the combined endpoint of all-cause mortality and hospital admission for decompensated heart failure (HR 0.198; 95% CI: 0.040-0.982; p=0.047), as were lower serum BNP (HR 1.003; 95% CI: 1.001-1.005; p=0.008) and mPAP (HR 1.073; 95% CI: 1.022-1.128; p=0.005) at diagnosis. Most patients who underwent PEA presented improved WHO FC (92.9%) and post-surgical residual pulmonary hypertension was identified in only 21.4%. CONCLUSION: PEA provided a better overall prognosis than non-surgical therapy, improving symptoms and frequently achieving hemodynamic normalization, with a numerical trend for lower mortality. Higher serum BNP, creatinine and mPAP at diagnosis were independently associated with worse outcomes.

Atrial Mechanics in Hypertrophic Cardiomyopathy: Discriminating between Ventricular Hypertrophy and Fibrosis. / Mecânica Atrial na Cardiomiopatia Hipertrófica: Discriminando Hipertrofia de Fibrose Ventricular.

BACKGROUND: Hypertrophic cardiomyopathy (HCM) and left ventricular hypertrophy (LVH) secondary to systemic hypertension (HTN) may be associated with left atrial (LA) functional abnormalities. OBJECTIVES: We aimed to characterize LA mechanics in HCM and HTN and determine any correlation with the extent of left ventricular (LV) fibrosis measured by cardiac magnetic resonance (CMR) in HCM patients. METHODS: Two-dimensional speckle tracking-derived longitudinal LA function was acquired from apical views in 60 HCM patients, 60 HTN patients, and 34 age-matched controls. HCM patients also underwent CMR, with measurement of late gadolinium enhancement (LGE) extension. Association with LA strain parameters was analyzed. Statistical significance was set at p<0.05. RESULTS: Mean LV ejection fraction was not different between the groups. The E/e' ratio was impaired in the HCM group and preserved in the control group. LA mechanics was significantly reduced in HCM, compared to the HTN group. LA strain rate in reservoir (LASRr) and in contractile (LASRct) phases were the best discriminators of HCM, with an area under the curve (AUC) of 0.8, followed by LA strain in reservoir phase (LASr) (AUC 0.76). LASRr and LASR-ct had high specificity (89% and 91%, respectively) and LASr had sensitivity of 80%. A decrease in 2.79% of LA strain rate in conduit phase (LASRcd) predicted an increase of 1cm in LGE extension (r2=0.42, ß 2.79, p=0.027). CONCLUSIONS: LASRr and LASRct were the best discriminators for LVH secondary to HCM. LASRcd predicted the degree of LV fibrosis assessed by CMR. These findings suggest that LA mechanics is a potential predictor of disease severity in HCM.

FUNDAMENTO: A cardiomiopatia hipertrófica (CMH) e a hipertrofia ventricular esquerda (HVE) secundária à hipertensão arterial sistêmica (HAS) podem estar associadas a anormalidades funcionais do átrio esquerdo (AE). OBJETIVOS: Caracterizar a mecânica do AE na CMH e na HAS e avaliar qualquer correlação com a extensão da fibrose ventricular esquerda medida por ressonância magnética cardíaca (RMC) em pacientes com CMH. MÉTODOS: A função longitudinal do AE derivada do ecocardiograma bidimensional com speckle tracking foi adquirida a partir de cortes apicais de 60 pacientes com CMH e 34 indivíduos controles, pareados por idade. Pacientes com CMH também foram submetidos à RMC, com medida da extensão do realce tardio por gadolínio. A associação com parâmetros de strain do AE foi analisada. Valores p < 0,05 foram definidos como estatisticamente significativos. RESULTADOS: A média da fração de ejeção do ventrículo esquerdo não foi diferente entre os grupos. A razão E/e' estava comprometida no grupo CMH e preservada no grupo controle. A mecânica do AE estava significativamente reduzida na CMH em comparação aos pacientes com HAS. O strain rate do AE nas fases de reservatório (SRrAE) e na fase contrátil (SRctAE) foram os melhores parâmetros de discriminação de CMH com uma área sob a curva (AUC) de 0,8, seguido do strain do AE na fase de reservatório (SrAE) (AUC 0,76). O SRrAE e o SRctAE apresentaram elevada especificidade (89% e 91%, respectivamente), e o SrAE apresentou sensibilidade de 80%. Um decréscimo de 2,79% no strain rate do AE na fase de condução (SRcdAE) foi preditor de um aumento de 1 cm na extensão do RT pelo gadolínio (r2=0,42, ß 2,79, p=0,027). CONCLUSÕES: O SRrAE e o SRctAE foram os melhores fatores de discriminação de HVE secundária à CMH. O SRcdAE foi preditor do grau de fibrose ventricular esquerda avaliada por RMC. Esses achados sugerem que a mecânica do AE pode ser um potencial preditor de gravidade de doença na CMH.

Mecânica Atrial na Cardiomiopatia Hipertrófica: Discriminando Hipertrofia de Fibrose Ventricular / Atrial Mechanics in Hypertrophic Cardiomyopathy: Discriminating between Ventricular Hypertrophy and Fibrosis

Resumo Fundamento A cardiomiopatia hipertrófica (CMH) e a hipertrofia ventricular esquerda (HVE) secundária à hipertensão arterial sistêmica (HAS) podem estar associadas a anormalidades funcionais do átrio esquerdo (AE). Objetivos Caracterizar a mecânica do AE na CMH e na HAS e avaliar qualquer correlação com a extensão da fibrose ventricular esquerda medida por ressonância magnética cardíaca (RMC) em pacientes com CMH. Métodos A função longitudinal do AE derivada do ecocardiograma bidimensional com speckle tracking foi adquirida a partir de cortes apicais de 60 pacientes com CMH e 34 indivíduos controles, pareados por idade. Pacientes com CMH também foram submetidos à RMC, com medida da extensão do realce tardio por gadolínio. A associação com parâmetros de strain do AE foi analisada. Valores p < 0,05 foram definidos como estatisticamente significativos. Resultados A média da fração de ejeção do ventrículo esquerdo não foi diferente entre os grupos. A razão E/e' estava comprometida no grupo CMH e preservada no grupo controle. A mecânica do AE estava significativamente reduzida na CMH em comparação aos pacientes com HAS. O strain rate do AE nas fases de reservatório (SRrAE) e na fase contrátil (SRctAE) foram os melhores parâmetros de discriminação de CMH com uma área sob a curva (AUC) de 0,8, seguido do strain do AE na fase de reservatório (SrAE) (AUC 0,76). O SRrAE e o SRctAE apresentaram elevada especificidade (89% e 91%, respectivamente), e o SrAE apresentou sensibilidade de 80%. Um decréscimo de 2,79% no strain rate do AE na fase de condução (SRcdAE) foi preditor de um aumento de 1 cm na extensão do RT pelo gadolínio (r2=0,42, β 2,79, p=0,027). Conclusões O SRrAE e o SRctAE foram os melhores fatores de discriminação de HVE secundária à CMH. O SRcdAE foi preditor do grau de fibrose ventricular esquerda avaliada por RMC. Esses achados sugerem que a mecânica do AE pode ser um potencial preditor de gravidade de doença na CMH.

Abstract Background Hypertrophic cardiomyopathy (HCM) and left ventricular hypertrophy (LVH) secondary to systemic hypertension (HTN) may be associated with left atrial (LA) functional abnormalities. Objectives We aimed to characterize LA mechanics in HCM and HTN and determine any correlation with the extent of left ventricular (LV) fibrosis measured by cardiac magnetic resonance (CMR) in HCM patients. Methods Two-dimensional speckle tracking-derived longitudinal LA function was acquired from apical views in 60 HCM patients, 60 HTN patients, and 34 age-matched controls. HCM patients also underwent CMR, with measurement of late gadolinium enhancement (LGE) extension. Association with LA strain parameters was analyzed. Statistical significance was set at p<0.05. Results Mean LV ejection fraction was not different between the groups. The E/e' ratio was impaired in the HCM group and preserved in the control group. LA mechanics was significantly reduced in HCM, compared to the HTN group. LA strain rate in reservoir (LASRr) and in contractile (LASRct) phases were the best discriminators of HCM, with an area under the curve (AUC) of 0.8, followed by LA strain in reservoir phase (LASr) (AUC 0.76). LASRr and LASR-ct had high specificity (89% and 91%, respectively) and LASr had sensitivity of 80%. A decrease in 2.79% of LA strain rate in conduit phase (LASRcd) predicted an increase of 1cm in LGE extension (r2=0.42, β 2.79, p=0.027). Conclusions LASRr and LASRct were the best discriminators for LVH secondary to HCM. LASRcd predicted the degree of LV fibrosis assessed by CMR. These findings suggest that LA mechanics is a potential predictor of disease severity in HCM.

Left atrial functional assessment and mortality in patients with severe aortic stenosis with sinus rhythm.

BACKGROUND: Aortic valve stenosis (AS) is the most common primary valvular heart disease leading to surgical or percutaneous aortic valve replacement (AVR) in Europe and its prevalence keeps growing. While other risk factors in severe AS are well documented, little is known about the prognostic value of left atrial (LA) function in AS. Our aim is to clarify the relationship between LA function measured at severe AS diagnosis (evaluated by means of volumetric assessment) and all-cause mortality during follow-up. METHODS: We retrospectively evaluated patients diagnosed with severe AS for the first time at our echocardiography laboratory. We evaluated LA reservoir, conduit and pump function by measuring LA volumes at different timings of cardiac cycle. Treatment strategy was decided according to heart team consensus and patient decision. We divided patients into groups according to terciles of LA reservoir, conduit and pump function. Primary outcome was defined by the occurrence of all-cause mortality during follow-up. RESULTS: A total of 408 patients were included in the analysis, with a median follow-up time of 45 months (interquartile range 54 months). 57.9% of patients underwent AVR and 44.9% of patients registered the primary outcome during follow-up. Left atrial emptying fraction (LAEF) was the best LA functional parameter and the best overall parameter in discriminating primary outcome (AUC 0.845, 95%CI 0.81-0.88, P < 0.001). After adjustment for clinical, demographic and echocardiographic variables, cumulative survival of patients with LAEF < 37% and LAEF 37 to 53% relative to patients with LAEF ≥54% remained significantly lower (HR 13.91, 95%CI 6.20-31.19, P < 0.001 and HR 3.40, 95%CI 1.57-7.37, P = 0.002, respectively). After adjustment for AVR, excess risk of LAEF < 37% and LAEF 37 to 53% relative to LAEF ≥54% remained significant (HR 11.71, 95%CI 5.20-26.40, P < 0.001 and HR 3.59, 95%CI 1.65-7.78, P = 0.001, respectively). CONCLUSIONS: In patients with a first diagnosis of severe AS, LA function, evaluated by means of volumetric assessment, is an independent predictor of all-cause mortality and a more potent predictor of death compared to classical severity parameters. These data can be useful to identify high-risk patients who might benefit of AVR.

Real-world analysis of acute decompensated heart failure outcomes in Portugal.

AIMS: In Portugal, in the last 5 years, no study has published recent data regarding outcomes of patients with acute decompensated heart failure (ADHF). We aimed to determine the characteristics and outcomes of a large contemporaneous Portuguese cohort of ADHF patients admitted to our emergency department (ED). METHODS AND RESULTS: We conducted a retrospective, study of all 1024 patients admitted to our ED with a discharge diagnosis of ADHF from November 2016 to December 2017. Baseline clinical data and outcomes {in-hospital, 30 day, and follow-up all-cause mortality, and readmissions; median follow-up, 5 months; interquartile range [(IQR), 3-11 months]} were determined. Mean age was 78 ± 10 years, and 53% were male; of the 1024 patients, 554 (54%) were hospitalized. The median hospitalization length was 9 (IQR, 5-15) days, and in-hospital mortality was 12.7%. Hospitalized patients were predominantly men (56% vs. 47%; P < 0.001), younger (77 ± 9 vs. 79 ± 11 years; P = 0.002) and had higher creatinine values and B-type natriuretic peptide values (P < 0.001) than discharged patients. Patients with prior hospitalization had lower 30 day readmission rate (8% vs. 14%; P = 0.01), same overall readmission rate (30% vs. 32%), and higher 30 day (13% vs. 5%; P < 0.001) and overall mortality rates (28% vs. 15%; P < 0.001). CONCLUSIONS: Approximately half of the patients admitted to the ED were hospitalized. Of these, only 8% were readmitted in the ED within 30 days. The clinical and analytical status in the ED are important predictors of hospitalization.

Left atrial mechanics in moderate mitral valve disease: earlier markers of damage.

While the impairment of left atrial (LA) mechanics in mitral valve disease is well known, the exact onset of reservoir, conduit, and contractile dysfunction in mitral stenosis (MS) and mitral regurgitation (MR) remains unclear. We aimed to clarify the LA deformation mechanics in patients with moderate mitral valve disease. We conducted a prospective observational study of 80 patients with moderate isolated MR, 80 patients with moderate isolated MS, and 64 age-matched controls without mitral valve disease. Strain (ɛ) and strain rate (SR) on speckle tracking echocardiography were assessed as indicators of LA and right atrium (RA) reservoir (ɛsys, SRs), conduit (ɛe, SRe), and contractile (ɛa, SRa) functions. Conventional echocardiographic parameters of the left ventricle (LV) were also assessed. Comparisons were conducted according to mitral valve pathology (MR patients, MS patients, controls). The mean LV ejection fraction, end-diastolic diameter, and global longitudinal strain did not differ across the groups. The pulmonary artery systolic pressure, LA volume indexed to body surface area, and LA mechanics were significantly impaired in mitral valve disease (patients vs controls). While LA ɛ did not vary between MR and MS, MR patients had better LA SRs and SRe but worse SRa (p < 0.01). SRe > - 0.65% had higher specificity for MS, with an area under the curve of 0.85 (p < 0.01). RA mechanics were significantly impaired in mitral valve disease (patients vs controls) but did not vary significantly with disease pathology (MS vs MR). Patients with moderate mitral valve disease exhibit early and pathology-specific changes in the LA deformation mechanics, manifesting mainly as impaired contractile-phase SR in MR and impaired conduit-phase SR in MS. Our findings highlight SR as a potentially useful early marker of LA dysfunction in relation to mitral valve disease.

Going beyond classic echo in aortic stenosis: left atrial mechanics, a new marker of severity.

BACKGROUND: There is limited information regarding left atrial (LA) mechanics in aortic valve stenosis (AS). We assessed LA mechanics in AS through speckle-tracking echocardiography (STE) according to severity and prognosis. METHODS: We included 102 patients diagnosed with severe AS (sAS) and 80 patients with moderate AS (mAS), all with preserved ejection fraction and no coronary artery disease. LA mechanics and left ventricular global longitudinal strain (LV-GLS) were assessed by STE. The cohort was followed-up for a median of 30 (IQR 12.6-50) months, and outcomes were determined (combined outcome of HF, death, and aortic valve replacement). RESULTS: In our sample set, values of LV-GLS (- 18.5% vs - 17.1, p = 0.025), E/e' ratio (15.8 vs 18.4, p = 0.03), and global LA mechanics (LA ɛsys, 23% vs 13.8%, p < 0.001) were worse for sAS compared to those for mAS. However, LA ɛsys (AUC 0.85, 95% CI 0.78-0.90, p < 0.001), ɛe (AUC 0.83, 95% CI 0.75-0.88, p < 0.001), and ɛa (AUC 0.80, 95% CI 0.70-0.84, p < 0.001) were the best discriminators of sAS, with sensitivities higher than 85%. LA ɛsys showed a stronger correlation with both aortic valve area (r2 = 0.6, p < 0.001) and mean LV/aortic gradient (r2 = 0.55, p < 0.001) than LV-GLS (r2 = 0.3 and r2 = 0.25, p = 0.01). Either LV-GLS or LA ɛsys, but not the E/e' ratio, TAPSE, or RV/RA gradient, were a significant predictors of the combined outcome. CONCLUSIONS: LA global strain was the best discriminator of severity, surpassing E/e' ratio and LV-GLS, and a significant predictor of prognosis in AS.

Early manifestation of myocardial involvement in systemic sclerosis.

INTRODUCTION: Systemic sclerosis (SSc) is a systemic autoimmune disease involving multiple organs. We present a rare case of SSc in which clinical manifestations of cardiac fibrosis occurred early in the disease course. CASE REPORT: We report the case of a 40-year-old Caucasian man, previously diagnosed with SSc, who presented with decompensated heart failure. Transthoracic echocardiography was remarkable for severe right ventricular systolic dysfunction, abnormal ventricular septal motion, severe functional tricuspid regurgitation and normal pulmonary artery systolic pressure. Left ventricular ejection fraction was 45%. Right heart catheterization revealed no signs of pulmonary hypertension. Cardiac magnetic resonance (CMR) showed diffuse myocardial infiltration, later confirmed as myocardial fibrosis by endomyocardial biopsy. CONCLUSIONS: Myocardial fibrosis is an important cause of early heart failure in SSc patients and is associated with poor prognosis. Echocardiography and CMR help establish the diagnosis and enable an appropriate therapeutic strategy to be developed in such cases.

Two-dimensional speckle-tracking global longitudinal strain in high-sensitivity troponin-negative low-risk patients with unstable angina: a "resting ischemia test"?

With the introduction of high-sensitivity troponin (hsTNI) assays, the clinical significance unstable angina (UA) has become uncertain. We hypothesized that impaired left ventricular (LV) two-dimensional speckle-tracking echocardiography (2D-STE)-derived peak global longitudinal strain (GLS) was able to exclude the presence of significant coronary artery disease (CAD) in UA patients without prior cardiovascular (CV) events and with a normal LV ejection fraction (LVEF). From a cohort of 200 patients admitted with UA, we selected 61 patients without prior CV events and with a normal LVEF; of these, 48 (79%) underwent invasive coronary angiography (ICA) and 24 (39%) had at least ≥ 1 significant stenosis. 2D-STE LV GLS analysis was performed offline using a dedicated automated software program. The mean patient age was 61 ± 12 years and 66% were male. Among the 48 patients selected to undergo ICA, those without significant CAD had a better 2D-STE GLS (- 19.4 vs. - 15.9%, P < 0.001). A cutoff of - 17.5% had a sensitivity of 87% and a specificity of 82% for differentiating UA patients without significant stenosis (AUC 0.86, P < 0.001). Patients who did not undergo ICA had a better GLS (- 20.2 vs. - 17.7%, P = 0.017). 2D-STE GLS had a significantly better discriminative power compared with LVEF (P < 0.001) and the GRACE score (P < 0.001) for identifying patients with significant CAD. Impaired values of 2D-STE LV GLS were significantly associated with the presence of CAD in hsTnI-negative UA patients and demonstrated better discriminative power than LVEF and the GRACE score.