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1.
BMJ Open Respir Res ; 11(1)2024 Apr 11.
Artigo em Inglês | MEDLINE | ID: mdl-38604738

RESUMO

INTRODUCTION: People living with HIV (PLHIV) have a higher risk of developing pulmonary hypertension (PH) with subsequent poorer prognosis. This review aimed to determine the (1) survival outcomes and (2) proportion of emergency department (ED) visits and hospitalisations of PLHIV and PH. METHODS: We conducted a systematic review and meta-analysis of observational studies reporting survival outcomes for PLHIV and PH. Electronic databases (Medline, EMBASE, PubMed, Web of Science, Global Index Medicus and Cochrane Library), trial registries and conference proceedings were searched until 22 July 2023. We pooled similar measures of effect, assessed apriori subgroups and used meta-regression to determine mortality and associated variables. RESULTS: 5248 studies were identified; 28 studies were included with a total of 5459 PLHIV and PH. The mean survival (95% CI) of PLHIV and PH was 37.4 months (29.9 to 44.8). Participants alive at 1, 2 and 3 years were 85.8% (74.1% to 95.0%), 75.2% (61.9% to 86.7%) and 61.9% (51.8% to 71.6%), respectively. ED visits and hospitalisation rates were 73.3% (32.5% to 99.9%) and 71.2% (42.4% to 94.2%), respectively. More severe disease, measured by echocardiogram, was associated with poorer prognosis (ß -0.01, 95% CI -0.02 to 0.00, p=0.009). Survival was higher in high-income countries compared with lower-income countries (ß 0.50, 95% CI 0.28 to 0.73, p<0.001) and in Europe compared with the America (ß 0.56, 95% CI 0.37 to 0.75, p<0.001). CONCLUSION: Our study confirms poor prognosis and high healthcare utilisation for PLHIV and PH. Prognosis is associated with country income level, geographic region and PH severity. This highlights the importance of screening in this population. PROSPERO REGISTRATION NUMBER: CRD42023395023.


Assuntos
Infecções por HIV , Hipertensão Pulmonar , Humanos , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/terapia , Hospitalização , Infecções por HIV/complicações , Infecções por HIV/epidemiologia
2.
Methodist Debakey Cardiovasc J ; 20(2): 70-80, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38495664

RESUMO

Pulmonary arterial hypertension (PAH) is a rare devastating disease characterized by elevated pulmonary artery pressure and increased pulmonary vascular resistance. Females have a higher incidence of PAH, which is reflected globally across registries in the United States, Europe, and Asia. However, despite female predominance, women had better outcomes compared with male patients, a finding that has been labeled the "estrogen paradox." Special considerations should be given to women with PAH regarding sexual health, contraception, family planning, and treatment before, during, and after pregnancy. Pregnant women with PAH should be referred to a pulmonary hypertension care center; a multidisciplinary team approach is recommended, and Cesarean section is the preferred mode of delivery. While pregnancy outcomes have improved over the years with PAH-specific therapy, pregnancy portends a high-risk for those with PAH. Continued research is needed to tailor PAH treatment for women.


Assuntos
Hipertensão Pulmonar , Hipertensão , Feminino , Gravidez , Humanos , Masculino , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/terapia , Cesárea/efeitos adversos , Resultado da Gravidez , Hipertensão Pulmonar Primária Familiar/complicações
3.
Chest ; 165(3): 682-691, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38461018

RESUMO

Pulmonary arterial hypertension (PAH) and pulmonary hypertension associated with left-sided heart and lung diseases are most commonly easily discriminated and treated accordingly. With the changing epidemiology of PAH, however, a growing proportion of patients at the time of diagnosis present with comorbidities of varying severity. In addition to classical PAH, two distinct phenotypes have emerged: a heart failure with preserved ejection fraction-like phenotype and a lung phenotype. Importantly, the evidence supporting the currently proposed treatment algorithm for PAH has been generated mainly from PAH trials in which patients with cardiopulmonary comorbidities have been underrepresented or excluded. As a consequence, the best therapeutic approach for patients with common PAH with cardiopulmonary comorbidities remains largely unknown and requires further investigation. The present article reviews the relevant literature on the topic and describes the authors' views on the current therapeutic approach for these patients.


Assuntos
Cardiopatias , Insuficiência Cardíaca , Hipertensão Pulmonar , Hipertensão Arterial Pulmonar , Humanos , Hipertensão Arterial Pulmonar/epidemiologia , Hipertensão Arterial Pulmonar/terapia , Hipertensão Arterial Pulmonar/complicações , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/terapia , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar Primária Familiar/complicações , Cardiopatias/complicações , Insuficiência Cardíaca/complicações , Insuficiência Cardíaca/epidemiologia , Insuficiência Cardíaca/terapia
4.
Int J Cardiol ; 403: 131900, 2024 May 15.
Artigo em Inglês | MEDLINE | ID: mdl-38403203

RESUMO

BACKGROUND: Pulmonary hypertension (PH) is a known complication of HIV infection. Outcomes of HIV-infected patients with PH (HIV-PH) have not been well established. We aim to assess various in-hospital outcomes such as mortality, resource utilization, and health care burden associated with HIV patients with concurrent PH. MATERIALS AND METHODS: We used National Inpatient Sample (NIS) 2015 Quarter 4 through 2019 for this study. We identified patients using International Classification of Diseases, 10th Revision, Clinical Modification (ICD-10-CM) diagnostic codes with both HIV and pulmonary hypertension. Cohorts were weighted by NIS-provided algorithm which allows for national estimates. Univariate and multivariate regression analyses were used to determine odds ratios. RESULTS: A total of 910,120 patients were identified with HIV, among which 28,175 (3.19%) were identified to have concurrent PH. When compared to HIV alone, HIV-PH patients were older (54.53(±11.61) vs. 49.44(±13.11), predominantly black (64.45% vs. 51.8%%), more often male (57.2%), all p < 0.001. HIV-PH cohort had higher comorbidities with higher Charlson Comorbidity Index (CCI) (7.07(±3.53) vs. 5.17(±3.65), had slightly longer LOS [adjusted mean difference (aMD) 0.79], higher healthcare burden corrected for inflation (aMD $17,065); all p < 0.001. In univariate regression analysis, patients with HIV-PH had significantly higher rates of developing heart failure (aOR 10.44), cardiogenic shock (aOR 5.67), cardiomyopathy (aOR 4.97), in-hospital cardiac arrest (aOR 1.94), respiratory failure (aOR 3.29), invasive mechanical ventilation (aOR 1.71), aspiration pneumonia (aOR 1.29), acute kidney injury (aOR 2.14). Lastly, patients with HIV-PH had higher in-hospital mortality within 30 days of admission (aOR 1.28) & overall in-hospital mortality (aOR 1.23); p < 0.005). CONCLUSION: In patients with concomitant HIV and PH, there is a higher burden of comorbidities, and is associated with worse outcomes including mortality. Through this study, we highlight outcomes that will better risk stratifying patients with concurrent HIV and PH.


Assuntos
Infecções por HIV , Hipertensão Pulmonar , Humanos , Masculino , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/epidemiologia , Infecções por HIV/complicações , Infecções por HIV/diagnóstico , Infecções por HIV/epidemiologia , Estudos de Coortes , Hospitalização , Hospitais
5.
Eur Respir J ; 63(3)2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38302154

RESUMO

BACKGROUND: Diagnostic rates and risk factors for the subsequent development of chronic thromboembolic pulmonary hypertension (CTEPH) following pulmonary embolism (PE) are not well defined. METHODS: Over a 10-year period (2010-2020), consecutive patients attending a PE follow-up clinic in Sheffield, UK (population 554 600) and all patients diagnosed with CTEPH at a pulmonary hypertension (PH) referral centre in Sheffield (referral population estimated 15-20 million) were included. RESULTS: Of 1956 patients attending the Sheffield PE clinic 3 months following a diagnosis of acute PE, 41 were diagnosed with CTEPH with a cumulative incidence of 2.10%, with 1.89% diagnosed within 2 years. Of 809 patients presenting with pulmonary hypertension (PH) and diagnosed with CTEPH, 32 were Sheffield residents and 777 were non-Sheffield residents. Patients diagnosed with CTEPH at the PE follow-up clinic had shorter symptom duration (p<0.01), better exercise capacity (p<0.05) and less severe pulmonary haemodynamics (p<0.01) compared with patients referred with suspected PH. Patients with no major transient risk factors present at the time of acute PE had a significantly higher risk of CTEPH compared with patients with major transient risk factors (OR 3.6, 95% CI 1.11-11.91; p=0.03). The presence of three computed tomography (CT) features of PH in combination with two or more out of four features of chronic thromboembolic pulmonary disease at the index PE was found in 19% of patients who developed CTEPH and in 0% of patients who did not. Diagnostic rates and pulmonary endarterectomy (PEA) rates were higher at 13.2 and 3.6 per million per year, respectively, for Sheffield residents compared with 3.9-5.2 and 1.7-2.3 per million per year, respectively, for non-Sheffield residents. CONCLUSIONS: In the real-world setting a dedicated PE follow-up pathway identifies patients with less severe CTEPH and increases population-based CTEPH diagnostic and PEA rates. At the time of acute PE diagnosis the absence of major transient risk factors, CT features of PH and chronic thromboembolism are risk factors for a subsequent diagnosis of CTEPH.


Assuntos
Hipertensão Pulmonar , Embolia Pulmonar , Tromboembolia , Humanos , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/epidemiologia , Seguimentos , Embolia Pulmonar/complicações , Embolia Pulmonar/diagnóstico , Embolia Pulmonar/epidemiologia , Fatores de Risco , Tromboembolia/complicações , Tromboembolia/diagnóstico , Sistema de Registros , Doença Crônica
6.
Clin Perinatol ; 51(1): 171-193, 2024 03.
Artigo em Inglês | MEDLINE | ID: mdl-38325940

RESUMO

Pulmonary hypertension (PH) in preterm neonates has multifactorial pathogenesis with unique characteristics. Premature surfactant-deficient lungs are injured following exposure to positive pressure ventilation and high oxygen concentrations resulting in variable phenotypes of PH. The prevalence of early PH is variable and reported to be between 8% and 55% of extremely preterm infants. Disruption of the lung development and vascular signaling pathway could lead to abnormal pulmonary vascular transition. The management of early PH and the off-label use of selective pulmonary vasodilators continue to be controversial.


Assuntos
Hipertensão Pulmonar , Surfactantes Pulmonares , Lactente , Recém-Nascido , Humanos , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/terapia , Pulmão , Lactente Extremamente Prematuro , Respiração com Pressão Positiva , Surfactantes Pulmonares/uso terapêutico
7.
Clin Perinatol ; 51(1): 271-289, 2024 03.
Artigo em Inglês | MEDLINE | ID: mdl-38325946

RESUMO

Long-term outcomes of persistent pulmonary hypertension of newborn (PPHN) depend on disease severity, duration of ventilation, and associated anomalies. Congenital diaphragmatic hernia survivors may have respiratory morbidities and developmental delay. The presence of PPHN is associated with increased mortality in hypoxic-ischemic encephalopathy, though the effects on neurodevelopment are less clear. Preterm infants can develop pulmonary hypertension (PH) early in the postnatal course or later in the setting of bronchopulmonary dysplasia (BPD). BPD-PH is associated with higher mortality, particularly within the first year. Evidence suggests that both early and late PH in preterm infants are associated with neurodevelopmental impairment.


Assuntos
Displasia Broncopulmonar , Hérnias Diafragmáticas Congênitas , Hipertensão Pulmonar , Lactente , Recém-Nascido , Humanos , Óxido Nítrico , Recém-Nascido Prematuro , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/terapia , Displasia Broncopulmonar/epidemiologia , Displasia Broncopulmonar/terapia , Hérnias Diafragmáticas Congênitas/complicações , Hérnias Diafragmáticas Congênitas/epidemiologia , Hérnias Diafragmáticas Congênitas/terapia
8.
Curr Med Res Opin ; 40(4): 555-565, 2024 04.
Artigo em Inglês | MEDLINE | ID: mdl-38420663

RESUMO

BACKGROUND: Although pulmonary hypertension (PH) and Eisenmenger's syndrome (ES) are common complications in adult congenital heart disease (ACHD), the frequency of diagnostic tests and the incidence of PH/ES in patients with ACHD in Japanese clinical practice are unclear. Therefore, we sought to clarify the frequency of diagnostic tests and incidence of PH/ES in patients with ACHD using the Medical Data Vision (MDV) database, the largest anonymized database of diagnosis procedure combination hospitals in Japan. METHODS: We conducted a retrospective cohort study using the MDV database (April 2008 to December 2021) of patients with ACHD (International Classificaiton of Diseases, 10th revision codes: Q203-204, Q210-213, Q250) aged ≥15 years. The frequency of laboratory/clinical tests and the incidence of PH/ES were calculated. Subgroup analyses were performed for the periods 2008-2015 and 2016-2021. RESULTS: Overall, 28219 ACHD patients were extracted from the MDV database (females 56.3%, males 43.7%; mean ± standard deviation age 44.7 ± 23.5 years). The mean ± standard deviation follow-up period was 2.5 ± 2.7 years. The frequencies of electrocardiography, ultrasonography, brain natriuretic peptide (BNP), N-terminal pro-BNP (NT-proBNP), right heart catheterization, and pulmonary function tests (DLCO) were 2149.8, 1054, 1233, 340, 40.0, and 6.0 per 1000 person-years, respectively. The incidence rate of PH/ES was 32.8 per 1000 person-years. The incidence rate of PH/ES increased from 24.6 to 46.7 per 1000 person-years from 2008-2015 to 2016-2021. CONCLUSION: We have clarified the frequency of diagnostic tests related to PH/ES and the incidence of PH/ES in patients with ACHD in clinical practice in Japan, including non-specialist institutions for PH.


Assuntos
Cardiopatias Congênitas , Hipertensão Pulmonar , Masculino , Feminino , Adulto , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/etiologia , Incidência , Japão/epidemiologia , Estudos Retrospectivos , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/epidemiologia
9.
Respiration ; 103(2): 79-87, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38325355

RESUMO

INTRODUCTION: Chronic thromboembolic pulmonary disease (CTEPD) consists of persistent pulmonary vascular obstruction on imaging and involves long-term functional limitations, with or without chronic thromboembolic pulmonary hypertension (CTEPH). The aim of this study was to evaluate the incidence and risk factors of both persistent pulmonary vascular defects and CTEPH after hospitalization in patients with COVID-19 and PE during a 2-year follow-up. METHODS: A prospective observational study was carried out in a tertiary hospital center. Patients were hospitalized between March 2020 and December 2021 with a diagnosis of PE during SARS-CoV-2 infection. Patients received anticoagulant treatment for at least 3 months and were followed up for 2 years. Between the third and fourth months after discharge, all patients were evaluated for the presence of residual thrombotic defects by CTPA and/or perfusion pulmonary scintigraphy. Clinical findings, lung function tests with DLCO, exercise capacity, and echocardiograms were also assessed. RESULTS: Of the 133 patients included, 18% had persistent thrombotic defects on lung imaging at follow-up. The incidence of CTEPD was 0.75% at 2 years of follow-up. Patients with persistent defects were significantly older, had a higher prevalence of systemic arterial hypertension, higher D-dimer and NT-proBNP levels, and more severe PE at diagnosis. Furthermore, there was a higher prevalence of right ventricular dysfunction on echocardiogram at diagnosis of PE (25.0% vs. 2.7%, p = 0.006). This was the only variable independently related to persistent defects in multivariate analyses (OR: 8.13 [95% CI: 1.82-36.32], p = 0.006). CONCLUSION: The persistence of thrombotic defects after PE is a common finding after SARS-CoV-2 infection, affecting 18% of the population. However, the incidence of CTEPH appears to be lower (0.75%) in COVID-19-related PE compared to that previously observed in PE unrelated to COVID-19.


Assuntos
COVID-19 , Hipertensão Pulmonar , Embolia Pulmonar , Tromboembolia , Humanos , Doença Crônica , COVID-19/complicações , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/diagnóstico , Pulmão , Embolia Pulmonar/diagnóstico por imagem , Embolia Pulmonar/epidemiologia , Embolia Pulmonar/etiologia , SARS-CoV-2 , Tromboembolia/complicações , Estudos Prospectivos
10.
Curr Probl Cardiol ; 49(4): 102406, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38237814

RESUMO

BACKGROUND: Pulmonary Hypertension (PH) is a sequela of arteriovenous (AV) fistulas (AVF) or AV grafts (AVG) in patients with end-stage kidney disease (ESKD) due to the creation of shunt physiology and increased pulmonary blood flow. PH has been consistently associated with increased mortality but there is a paucity of data regarding management. RESEARCH QUESTION: The objective of this study was to identify risk factors and outcomes in patients who develop PH after AVF or AVG creation for hemodialysis access. METHODS: Using the United States Renal Data System, we identified all patients over age 18 initiated on dialysis from 2012-2019 who did not receive renal transplant. We identified a) the predictors of PH in patients with ESKD on hemodialysis; b) the independent mortality risk associated with development of PH. RESULTS: We identified 478,896 patients initiated on dialysis from 2012-2019 of whom 27,787 (5.8 %) had a diagnosis of PH. The median age was 65 (IQR: 55-74) years and 59.1 % were male. Reduced ejection fraction, any congestive heart failure, obstructive sleep apnea, and female sex were the strongest predictors of PH diagnosis. Both AVG and AVF were also associated with an increased rate of PH diagnosis compared to catheter-based dialysis (p < 0.001). PH portended a poor prognosis and was associated with significantly increased mortality (p < 0.001). CONCLUSIONS: Patients with AVF or AVG should be screened using echocardiography prior to creation and monitored with serial echocardiography for the development of PH, and if present, considered for revision of the AVA. This is also the first study to identify that AVG are a risk factor for PH in dialysis patients.


Assuntos
Hipertensão Pulmonar , Falência Renal Crônica , Humanos , Feminino , Masculino , Idoso , Adolescente , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/etiologia , Falência Renal Crônica/complicações , Falência Renal Crônica/terapia , Diálise Renal/efeitos adversos , Rim , Progressão da Doença
11.
Eur Respir Rev ; 33(171)2024 Jan 31.
Artigo em Inglês | MEDLINE | ID: mdl-38232991

RESUMO

BACKGROUND: The prevalence of tuberculosis (TB)-associated pulmonary hypertension (PH) has not previously been quantified, resulting in an underappreciated burden of disease. We aimed to estimate the prevalence of PH in post-TB and active TB populations. METHODS: In this systematic review and meta-analysis, we searched PubMed/Medline, Cochrane Library, EBSCOhost, Scopus, African Journals Online and Google Scholar, with no language restriction, for available literature published after 1950. Eligible studies described adult participants (≥16 years), with documented evidence of active or prior TB, diagnosed with PH. Study quality was assessed using a risk of bias tool specifically developed for prevalence studies. Aggregate prevalence estimates with 95% confidence intervals were synthesised using a random-effects meta-analysis model, incorporating the Freeman-Tukey transformation. Subgroup analysis was conducted to ascertain prevalence estimates in specific patient populations. RESULTS: We identified 1452 unique records, of which 34 met our inclusion criteria. 23 studies, with an acceptable risk of bias and where PH was diagnosed at right heart catheterisation or echocardiography, were included in the meta-analysis. In post-TB studies (14/23), the prevalence of PH was 67.0% (95% CI 50.8-81.4) in patients with chronic respiratory failure, 42.4% (95% CI 31.3-54.0) in hospitalised or symptomatic patients and 6.3% (95% CI 2.3-11.8) in nonhealthcare-seeking outpatients (I2=96%). There was a lower estimated prevalence of PH in studies of populations with active TB (9.4%, 95% CI 6.3-13.0), I2=84%). CONCLUSION: Our results highlight the significant burden of PH in post-TB and active TB populations. We emphasise the need for increased recognition of TB-associated PH and additional high-quality prevalence data.


Assuntos
Hipertensão Pulmonar , Hipertensão Arterial Pulmonar , Tuberculose , Adulto , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/epidemiologia , Prevalência , Tuberculose/diagnóstico
12.
Nat Rev Dis Primers ; 10(1): 1, 2024 Jan 04.
Artigo em Inglês | MEDLINE | ID: mdl-38177157

RESUMO

Pulmonary hypertension encompasses a range of conditions directly or indirectly leading to elevated pressures within the pulmonary arteries. Five main groups of pulmonary hypertension are recognized, all defined by a mean pulmonary artery pressure of >20 mmHg: pulmonary arterial hypertension (rare), pulmonary hypertension associated with left-sided heart disease (very common), pulmonary hypertension associated with lung disease (common), pulmonary hypertension associated with pulmonary artery obstructions, usually related to thromboembolic disease (rare), and pulmonary hypertension with unclear and/or multifactorial mechanisms (rare). At least 1% of the world's population is affected, with a greater burden more likely in low-income and middle-income countries. Across all its forms, pulmonary hypertension is associated with adverse vascular remodelling with obstruction, stiffening and vasoconstriction of the pulmonary vasculature. Without proactive management this leads to hypertrophy and ultimately failure of the right ventricle, the main cause of death. In older individuals, dyspnoea is the most common symptom. Stepwise investigation precedes definitive diagnosis with right heart catheterization. Medical and surgical treatments are approved for pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. There are emerging treatments for other forms of pulmonary hypertension; but current therapy primarily targets the underlying cause. There are still major gaps in basic, clinical and translational knowledge; thus, further research, with a focus on vulnerable populations, is needed to better characterize, detect and effectively treat all forms of pulmonary hypertension.


Assuntos
Hipertensão Pulmonar , Hipertensão Arterial Pulmonar , Humanos , Idoso , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/etiologia , Hipertensão Arterial Pulmonar/complicações , Artéria Pulmonar , Pulmão
14.
RMD Open ; 10(1)2024 Jan 08.
Artigo em Inglês | MEDLINE | ID: mdl-38191213

RESUMO

BACKGROUND: Systemic lupus erythematosus (SLE) significantly affects the lungs and heart, and pulmonary hypertension (PH) is a severe manifestation that leads to considerable morbidity and mortality. OBJECTIVES: We aimed to determine the prevalence and risk factors of probable SLE-PH, assess the main echocardiographic predictors and develop a potential screening strategy. METHODS: A prospective single-centre study was conducted on 201 patients with SLE who underwent transthoracic echocardiography. Patients meeting PH criteria were referred for right heart catheterisation (RHC). RESULTS: Among patients, 88.56% were women, 85.57% were of Spanish origin and 43.78% had structural heart disease. Out of these, 16 (7.96%) had intermediate or high probability criteria for PH according to European Society of Cardiology (ESC) 2022. Six RHCs confirmed PH with a prevalence of 2.99% for SLE-PH and 1.99% for SLE-pulmonary arterial hypertension (PAH). KEY RISK FACTORS: Key risk factors included age, cardiorespiratory symptoms, serositis, anti-Ro, cardiac biomarkers and altered pulmonary function tests (PFTs). PH was linked to a higher Systemic Lupus International Collaborative Clinics/American College of Rheumatology Damage Index (SDI) (mean SDI 4.75 vs 2.05, p<0.001) and increased mortality risk in a 2-year follow-up (12.50% vs 1.08%, p=0.002). CONCLUSION: In our cohort, 7.96% of patients with SLE had an intermediate or high PH probability. By RHC, six patients (2.99%) met the ESC/European Respiratory Society criteria for PH and four (1.99%) for PAH. The main risk factors were older age, cardiorespiratory symptoms, serositis, anti-Ro, cardiac biomarkers and altered PFTs. PH was a severe SLE complication, suggesting the need for earlier diagnosis through data-driven screening to reduce associated morbidity and mortality.


Assuntos
Hipertensão Pulmonar , Lúpus Eritematoso Sistêmico , Serosite , Humanos , Feminino , Masculino , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/etiologia , Prevalência , Estudos Prospectivos , Ecocardiografia , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/epidemiologia , Biomarcadores
15.
Rev Med Interne ; 45(1): 26-40, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37925256

RESUMO

Pulmonary hypertension (PH) is a possible complication of connective tissue diseases (CTDs), especially systemic sclerosis (SSc), systemic lupus erythematosus (SLE) and mixed connective tissue disease (MCTD). It is defined by an elevation of the mean pulmonary arterial pressure above 20mmHg documented during a right heart catheterization (RHC). Due to their multiorgan involvement, CTDs can induce PH by several mechanisms, that are sometimes intricated: pulmonary vasculopathy (group 1) affecting arterioles (pulmonary arterial hypertension, PAH) and possibly venules (pulmonary veno-occlusive-like disease), left-heart disease (group 2), chronic lung disease (group 3) and/or chronic thromboembolic PH (group 4). PH suspicion is often raised by clinical manifestations (dyspnea, fatigue), echocardiographic data (increased peak tricuspid regurgitation velocity), isolated decrease in DLCO in pulmonary function tests, and/or unexplained elevation of BNP/NT-proBNP. Its formal diagnosis always requires a hemodynamic confirmation by RHC. Strategies for PH screening and RHC referral have been extensively investigated for SSc-PAH but data are lacking in other CTDs. Therapeutic management of PH depends of the underlying mechanism(s): PAH-approved therapies in group 1 PH (with possible use of immunosuppressants, especially in case of SLE or MCTD); management of an underlying left-heart disease in group 2 PH; management of an underlying chronic lung disease in group 3 PH; anticoagulation, pulmonary endartectomy, PAH-approved therapies and/or balloon pulmonary angioplasty in group 4 PH. Regular follow-up is mandatory in all CTD-PH patients.


Assuntos
Doenças do Tecido Conjuntivo , Cardiopatias , Hipertensão Pulmonar , Lúpus Eritematoso Sistêmico , Doença Mista do Tecido Conjuntivo , Escleroderma Sistêmico , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/etiologia , Doença Mista do Tecido Conjuntivo/complicações , Doenças do Tecido Conjuntivo/complicações , Doenças do Tecido Conjuntivo/diagnóstico , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/diagnóstico , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico
17.
Chest ; 165(1): 132-145, 2024 01.
Artigo em Inglês | MEDLINE | ID: mdl-37582424

RESUMO

BACKGROUND: Pulmonary involvement is the leading cause of death in systemic sclerosis (SSc) and may manifest as interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), or in combination of both (ILD with pulmonary hypertension [ILD-PH]). The aim of this analysis was to determine prevalence, clinical characteristics, and survival of these different forms within the registry of the German Network for Systemic Sclerosis. RESEARCH QUESTION: Does SSc-associated ILD-PH or ILD without PH affect survival differently, and are there any risk factors that have an additional impact? STUDY DESIGN AND METHODS: Clinical data of 5,831 patients with SSc were collected in the German Network for Systemic Sclerosis registry. Kaplan-Meier estimates were used to compare overall survival in patients with SSc-associated ILD-PH and ILD without PH with patients without pulmonary involvement and those with PAH. The Cox proportional hazard model was used to analyze the influence of pulmonary involvement and other potential predictors on patient survival. RESULTS: Clinical data of 3,257 patients with a mean follow-up time of 3.45 ± 1.63 years have been included in our analysis. At baseline, ILD was present in 34.5%, whereas PH without ILD had a lower prevalence with 4.5%. At the end of follow-up, 47.6% of patients with SSc had ILD, 15.2% had ILD-PH, and 6.5% had PAH. ILD was more frequent in the diffuse cutaneous form (57.3%), whereas PAH did not differ significantly between SSc subtypes. Significant differences in baseline characteristics between PAH vs ILD-PH vs ILD without PH were found for age at diagnosis, sex, SSc subsets, antibody status, FVC, diffusing capacity of the lung for carbon monoxide, and therapy. Overall survival at 5 years was 96.4% for patients without pulmonary involvement and differed significantly between patients with ILD without PH, PAH, and being worst in patients with ILD-PH. Female sex (hazard ratio [HR], 0.3), higher BMI (HR, 0.9), and higher diffusing capacity of the lung for carbon monoxide values (HR, 0.98) were associated with a lower mortality risk. INTERPRETATION: ILD is the most prevalent pulmonary involvement in SSc, whereas the combination of ILD and PH is associated with the most detrimental survival.


Assuntos
Hipertensão Pulmonar , Doenças Pulmonares Intersticiais , Hipertensão Arterial Pulmonar , Escleroderma Sistêmico , Humanos , Feminino , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/tratamento farmacológico , Estudos de Coortes , Monóxido de Carbono , Escleroderma Sistêmico/diagnóstico , Doenças Pulmonares Intersticiais/etiologia , Doenças Pulmonares Intersticiais/complicações , Hipertensão Pulmonar Primária Familiar/complicações , Hipertensão Arterial Pulmonar/complicações
18.
Curr Probl Cardiol ; 49(2): 102136, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37858849

RESUMO

Right heart catheterization (RHC) represents the gold standard diagnostic approach for pulmonary hypertension (PH). Historically, the complication rates of RHC are known to be low. The study aimed to evaluate the indications for performing RHC and the occurrence of adverse events related to the procedure in patients > over 70 years of age in a Mexican Tertiary Care Center. We conducted a retrospective single-center registry from July 2017 to July 2022. A total of 517 patients with suspected PH underwent RHC. The cohort included patients <70 (n = 427) and ≥70 years of age (n = 90). Adverse events were classified as major (eg, death, pneumothorax, and carotid artery puncture) and minor (eg, atrial arrhythmia, superior vena cava dissection, incidental arterial puncture, and local hematoma). Appropriate hemodynamic parameters were recorded. No report of major adverse events in the entire cohort. In the <70 years age group, 9 minor events, and 3 minor events were in the ≥70-year-old patients (P < 0.0001). There was a significant difference in the measurement of mean pulmonary artery pressure (mPAP) between the <70 years old vs ≥70 years old (P < 0.001); there was a significant difference in right atrial pressures: 4.71 ± 3.14 mmHg in the <70-year-old vs 4.07 ± 1.94 mmHg for the ≥ 70-year-old group (P = 0.014). Our findings suggest that RHC can be safely performed in patients aged ≥70 years using different vascular access routes without significant major complications.


Assuntos
Hipertensão Pulmonar , Humanos , Idoso , Idoso de 80 Anos ou mais , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/etiologia , Artéria Pulmonar , Estudos Retrospectivos , Centros de Atenção Terciária , Veia Cava Superior , Cateterismo Cardíaco/efeitos adversos , Cateterismo Cardíaco/métodos
19.
Am J Cardiol ; 212: 67-72, 2024 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-38040280

RESUMO

In this study, using a large database, we examined the association between atrial fibrillation (AF) in hospitalized patients with pulmonary hypertension (PH) and in-hospital mortality and other adverse hospital outcomes. This study was a retrospective analysis of the United States National (Nationwide) Inpatient Sample from 2005 to 2014. All hospitalizations for patients diagnosed with primary PH and over the age of 65 years were included and then grouped based on the presence AF. The outcomes were in-hospital mortality rate, hospital length of stay, and hospitalization costs. Weighted regression analyses were performed to find the association between AF and outcomes. Of the 5,428,332 hospitalizations with PH, 2,531,075 (46.6%) had concomitant AF. The Cox proportional regression analysis showed that in patients with PE, all-cause mortality (hazard ratio 1.35, confidence interval [CI] 1.15 to 1.55) was significantly higher in patients with AF than those without AF. In addition, PH hospitalizations with AF had a longer hospital length of stay (ß coefficient 1.74, 95% CI 1.58 to 1.83) and higher hospitalization cost (ß coefficient 1.33, 95% CI 1.12 to 1.42). In patients aged over 65 years admitted for PH, the presence of AF was very frequent and worsened the prognosis. In conclusion, to improve patient outcomes and decrease hospital burden, it is important to consider AF during risk stratification for patients with PH to provide timely and prompt interventions. An interdisciplinary approach to treatment should be used to account for the burden of co-morbidities in this population.


Assuntos
Fibrilação Atrial , Hipertensão Pulmonar , Humanos , Estados Unidos/epidemiologia , Idoso , Fibrilação Atrial/complicações , Fibrilação Atrial/epidemiologia , Fibrilação Atrial/terapia , Estudos Retrospectivos , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/complicações , Hospitalização , Mortalidade Hospitalar , Hospitais
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