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1.
Front Plant Sci ; 15: 1363153, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38745929

RESUMO

Artocarpus altilis, commonly known as breadfruit, is a potential crop adapted to a wide variety of climates and widely spread, including in Indonesia. However, information on how this species can adapt to climate change, in particular in Central Java, is still limited. In Indonesia, Central Java is the center for cultivation areas for many crop species to support the 145 million people living on Java Island. One of the potential crops being developed in Central Java is breadfruit. To assess the suitable cultivation areas for breadfruit, species distribution modeling (SDM) was used to predict the current and future (2050-2070) distribution of breadfruit. Two climate change scenarios, including optimistic RCP2.6 and pessimistic RCP8.5 models, were considered to represent future climate change impacts. Based on the results for both optimistic and pessimistic scenarios, the breadfruit's suitable cultivation areas will expand eastward. Implementing a mitigation climate change scenario and limiting the temperature increase to only 1°C under RCP2.6 will provide 270.967 km2 more of suitable cultivation areas for breadfruit in 2050 and 133.296 km2 in 2070. To conclude, this study provides important information on the status and potential cultivation areas for breadfruit, mainly in the Southeast Asia region. The identification of suitable areas will guide land conservation for breadfruit to support food security in this region.

2.
Pulm Circ ; 13(1): e12204, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-36883189

RESUMO

Evaluation for right ventricular (RV) dysfunction is an important part of risk assessment in care of patients with pulmonary hypertension (PH) as it is associated with morbidity and mortality. Echocardiography provides a widely available and acceptable method to assess RV function. RV global longitudinal strain (RVGLS), a measure of longitudinal shortening of RV deep muscle fibers obtained by two-dimensional echocardiography, was previously shown to predict short-term mortality in patients with PH. The purpose of the current study was to assess the performance of RVGLS in predicting 1-year outcomes in PH. We retrospectively identified 83 subjects with precapillary PH and then enrolled 50 consecutive prevalent pulmonary arterial hypertension (PAH) subjects into a prospective validation cohort. Death as well as combined morbidity and mortality events at 1 year were assessed as outcomes. In the retrospective cohort, 84% of patients had PAH and the overall 1-year mortality rate was 16%. Less negative RVGLS was marginally better than tricuspid annular plane systolic excursion (TAPSE) as a predictor for death. However, in the prospective cohort, 1-year mortality was only 2%, and RVGLS was not predictive of death or a combined morbidity and mortality outcome. This study supports that RV strain and TAPSE have similar 1-year outcome predictions but highlights that low TAPSE or less negative RV strain measures are often false-positive in a cohort with low baseline mortality risk. While RV failure is considered the final common pathway for disease progression in PAH, echocardiographic measures of RV function may be less informative of risk in serial follow-up of treated PAH patients.

3.
Pulm Circ ; 12(2): e12063, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-35514770

RESUMO

Inhaled treprostinil is an approved therapy for pulmonary arterial hypertension (PAH) and pulmonary hypertension associated with interstitial lung disease in the United States. Studies have confirmed the robust benefits and safety of nebulized inhaled treprostinil, but it requires a time investment for nebulizer preparation, maintenance, and treatment. A small, portable treprostinil dry powder inhaler has been developed for the treatment of PAH. The primary objective of this study was to evaluate the safety and tolerability of treprostinil inhalation powder (TreT) in patients currently treated with treprostinil inhalation solution. Fifty-one patients on a stable dose of treprostinil inhalation solution enrolled and transitioned to TreT at a corresponding dose. Six-minute walk distance (6MWD), device preference and satisfaction (Preference Questionnaire for Inhaled Treprostinil Devices [PQ-ITD]), PAH Symptoms and Impact (PAH-SYMPACT®) questionnaire, and systemic exposure and pharmacokinetics for up to 5 h were assessed at baseline for treprostinil inhalation solution and at Week 3 for TreT. Adverse events (AEs) were consistent with studies of inhaled treprostinil in patients with PAH, and there were no study drug-related serious AEs. Statistically significant improvements occurred in 6MWD, PQ-ITD, and PAH-SYMPACT. Forty-nine patients completed the 3-week treatment phase and all elected to participate in an optional extension phase. These results demonstrate that, in patients with PAH, transition from treprostinil inhalation solution to TreT is safe, well-tolerated, and accompanied by statistically significant improvements in key clinical assessments and patient-reported outcomes with comparable systemic exposure between the two formulations at evaluated doses (trial registration: clinicaltrials.gov identifier: NCT03950739).

4.
Pulm Circ ; 12(1): e12010, 2022 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-35506090

RESUMO

Pulmonary hypertension affects about one in four patients with advanced chronic kidney disease and significantly increases the risk of death. Kidney transplantation is the recommended management option for patients with progressive or end-stage kidney disease. However, the resource-limited nature of kidney transplantation and its intensive peri-operative and posttransplantation management motivates careful consideration of potential candidates' medical conditions to optimally utilize available graft organs. Since pulmonary hypertension is known to increase peri-operative morbidity and mortality among patients living with chronic kidney disease, we performed a retrospective cohort study to assess the impact of pretransplantation pulmonary hypertension on posttransplantation outcome. All patients who underwent single-organ kidney transplantation at our center in calendar years 2010 and 2011 were identified and the presence of pulmonary hypertension was determined from pretransplantation echocardiography. Outcome was assessed at 5 years following kidney transplantation. Of 350 patients who were included, 117 (33%) had evidence of pulmonary hypertension. The risk of death, graft dysfunction, or graft failure at 5 years after kidney transplantation was higher among those with pulmonary hypertension, primarily owing to an increased risk of graft dysfunction. Importantly, in this institutional cohort of kidney transplant recipients, pretransplant pulmonary hypertension was not associated with a difference in posttransplant survival at 5 years. While institutional and regional differences in outcome can be expected, this report suggests that carefully selected patients with pulmonary hypertension receive similar long-term benefits from kidney transplantation.

5.
MethodsX ; 7: 101032, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32874944

RESUMO

This paper outlines a land and power framework for assessing whether a new voluntary conservation area policy is a return to the classical bureaucratic status quo or anticipates the opportunity to establish new bureaucratic norms. The application of this conceptual framework produces two possibilities. The first possibility is that outcomes are tied to the conventional bureaucratic models of conservation with management regimes that remain unchanged. The second possibility is the anticipation of new management forms, in which goals are not to fulfill the bureaucratic process, but rather, produce adaptive outcomes reflective of the interests of diverse actors engaged in site-specific voluntary conservation initiatives.•The land and power framework methodology is rooted in an interest-based power framework.•The framework analyses the land and power inputs for both conservation bureaucracies or actors participating in multi-stakeholder arrangements struggling to achieve their interests and establish their agendas.•The framework proposes a conceptual framework to assess two possible process outcomes, namely that management regimes will either be tied to the conventional bureaucracy or that actors anticipate new bureaucratic norms that achieve outcomes accommodating their broader interests.

6.
MethodsX ; 7: 100917, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32477895

RESUMO

We extend the Actor-Centred Power framework to consider dimensions beyond the life of community natural resource management partnership initiatives by examining social forestry partnership projects in Indonesia. We do this by examining how power constellations realign across the temporal phases that operationalize project partnerships. We propose a sequential power analysis framework that examines power in three parts. The framework first proposes a method for historicizing actors into their power background. Second, we present mode for examining the arrival of a partnership scheme, which we call the power delivery phase. Third, we highlight approaches for examining the way power relations are adjusted, whether reinforced or reconfigured, by introducing an approach for examining programmatic outcomes of social forestry partnership schemes. This article thus provides broadly applicable but targeted guide for the researchers collecting data and seeking to make sense of power relations on community forest partnership schemes in various contexts. This framework is particularly useful for analysing equity and justice dimensions by highlighting who benefits and who loses.•Sequential Power Analysis (SPA) methodology is rooted in interest based and historical power framework.•SPA is consisted of three parts: power background, power delivery, and power adjustment•SPA framing provides a protocol for researchers to collect data.

7.
MethodsX ; 6: 2536-2543, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31763186

RESUMO

Recent land management policies around the world have experienced a broader political push to resolve forest and land tenure conflict through agrarian reform policy. As a result, conservation bureaucracies are responding with both formal and informal interventions to acknowledge the role of people in forests. In this methods paper, we provide a closer examination of the ways that conservation bureaucracies apply their political capacity in negotiating forest and land tenure conflicts. Our proposed method measures both the capacity and actions of conservation bureaucracies, combining formal dimensions (such as of legal status, budget availability, and the type of organization unit) with informal dimensions (including ways of gaining authority, donors and funding, and trust). The framing is rooted in theories of bureaucratic politics, and while culled from rich empirical experiences from Indonesia, the proposed method is also applicable in examining bureaucratic politics in other natural resource governance contexts. •We develop a method rooted in bureaucratic politics to measure the capacities of conservation agencies to manage forest land tenure conflict•The proposed typology guides forest and land use policy researchers to incorporate emergent governance issues such as land tenure reform into their assessments of changing conservation bureaucracies•The can be adapted for examination of bureaucratic capacities and actions in other contested natural resource contexts.

9.
Ann Thorac Surg ; 103(6): e539-e540, 2017 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-28528062

RESUMO

Plastic bronchitis is a rare and potentially life-threatening disease characterized by the development of obstructive fibrinous tracheobronchial casts and hypoxic respiratory failure. With its poorly understood cause and rare occurrence in the adult population, few treatment strategies have been described in adults with this condition. In this report, we present a case of successful treatment of an adult with plastic bronchitis, using thoracic duct ligation and resulting in full resolution of airway cast development.


Assuntos
Bronquite/cirurgia , Ducto Torácico , Cirurgia Torácica Vídeoassistida , Adulto , Bronquite/diagnóstico , Bronquite/etiologia , Feminino , Humanos , Ligadura
10.
PLoS Biol ; 13(6): e1002167, 2015 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-26061091

RESUMO

Ongoing climate change can alter conditions for plant growth, in turn affecting ecological and social systems. While there have been considerable advances in understanding the physical aspects of climate change, comprehensive analyses integrating climate, biological, and social sciences are less common. Here we use climate projections under alternative mitigation scenarios to show how changes in environmental variables that limit plant growth could impact ecosystems and people. We show that although the global mean number of days above freezing will increase by up to 7% by 2100 under "business as usual" (representative concentration pathway [RCP] 8.5), suitable growing days will actually decrease globally by up to 11% when other climatic variables that limit plant growth are considered (i.e., temperature, water availability, and solar radiation). Areas in Russia, China, and Canada are projected to gain suitable plant growing days, but the rest of the world will experience losses. Notably, tropical areas could lose up to 200 suitable plant growing days per year. These changes will impact most of the world's terrestrial ecosystems, potentially triggering climate feedbacks. Human populations will also be affected, with up to ~2,100 million of the poorest people in the world (~30% of the world's population) highly vulnerable to changes in the supply of plant-related goods and services. These impacts will be spatially variable, indicating regions where adaptations will be necessary. Changes in suitable plant growing days are projected to be less severe under strong and moderate mitigation scenarios (i.e., RCP 2.6 and RCP 4.5), underscoring the importance of reducing emissions to avoid such disproportionate impacts on ecosystems and people.


Assuntos
Mudança Climática , Ecossistema , Desenvolvimento Vegetal , Agricultura , Humanos
11.
J Rheumatol ; 38(11): 2410-8, 2011 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-21965638

RESUMO

OBJECTIVE: The tricuspid annular plane systolic excursion (TAPSE) strongly reflects right ventricular (RV) function and predicts survival in idiopathic pulmonary arterial hypertension (PAH). But its role in systemic sclerosis (SSc)-associated PAH has not been established. Our objective was to validate the TAPSE in the assessment of RV function and prediction of survival in SSc-PAH. METHODS: Fifty consecutive patients with SSc-PAH who underwent echocardiography with TAPSE measurement within 1 h of clinically indicated right heart catheterization were followed prospectively. The relationship between TAPSE and measures of RV function and measures of survival was assessed. RESULTS: The majority of the cohort were women in New York Heart Association class III/IV with severe PAH (mean cardiac index 2.4 ± 0.8 l/min/m(2)). RV function was significantly impaired (mean cardiac index 2.1 ± 0.7 vs 2.9 ± 0.8 l/min/m(2); p < 0.01) and RV afterload was significantly greater (mean pulmonary vascular resistance 11.1 ± 5.1 vs 5.8 ± 2.5 Wood units; p < 0.01) in subjects with a TAPSE ≤ 1.7 cm. The proportion surviving in the low TAPSE group was significantly lower [0.56 (95% CI 0.37-0.71) and 0.46 (95% CI 0.28-0.62) vs 0.87 (95% CI 0.55-0.96) and 0.79 (95% CI 0.49-0.93), 1- and 2-year survival, respectively]. TAPSE ≤ 1.7 cm conferred a nearly 4-fold increased risk of death (HR 3.81, 95% CI 1.31-11.1, p < 0.01). CONCLUSION: TAPSE is a robust measure of RV function and strongly predicts survival in patients with PAH-SSc. Future studies are needed to identify the responsiveness of TAPSE to PAH-specific therapy and to assess its diagnostic utility in PAH-SSc.


Assuntos
Ventrículos do Coração/diagnóstico por imagem , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/fisiopatologia , Avaliação de Resultados em Cuidados de Saúde , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/fisiopatologia , Valva Tricúspide/diagnóstico por imagem , Função Ventricular Direita/fisiologia , Idoso , Pressão Sanguínea/fisiologia , Estudos de Coortes , Feminino , Seguimentos , Humanos , Hipertensão Pulmonar/mortalidade , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Estudos Prospectivos , Fluxo Sanguíneo Regional/fisiologia , Reprodutibilidade dos Testes , Escleroderma Sistêmico/mortalidade , Sensibilidade e Especificidade , Taxa de Sobrevida , Sístole/fisiologia , Valva Tricúspide/fisiologia , Ultrassonografia , Resistência Vascular/fisiologia
13.
Am J Respir Crit Care Med ; 179(7): 615-21, 2009 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-19164700

RESUMO

RATIONALE: Transthoracic Doppler echocardiography is recommended for screening for the presence of pulmonary hypertension (PH). However, some recent studies have suggested that Doppler echocardiographic pulmonary artery pressure estimates may frequently be inaccurate. OBJECTIVES: Evaluate the accuracy of Doppler echocardiography for estimating pulmonary artery pressure and cardiac output. METHODS: We conducted a prospective study on patients with various forms of PH who underwent comprehensive Doppler echocardiography within 1 hour of a clinically indicated right-heart catheterization to compare noninvasive hemodynamic estimates with invasively measured values. MEASUREMENTS AND MAIN RESULTS: A total of 65 patients completed the study protocol. Using Bland-Altman analytic methods, the bias for the echocardiographic estimates of the pulmonary artery systolic pressure was -0.6 mm Hg with 95% limits of agreement ranging from +38.8 to -40.0 mm Hg. Doppler echocardiography was inaccurate (defined as being greater than +/-10 mm Hg of the invasive measurement) in 48% of cases. Overestimation and underestimation of pulmonary artery systolic pressure by Doppler echocardiography occurred with a similar frequency (16 vs. 15 instances, respectively). The magnitude of pressure underestimation was greater than overestimation (-30 +/- 16 vs. +19 +/- 11 mm Hg; P = 0.03); underestimates by Doppler also led more often to misclassification of the severity of the PH. For cardiac output measurement, the bias was -0.1 L/min with 95% limits of agreement ranging from +2.2 to -2.4 L/min. CONCLUSIONS: Doppler echocardiography may frequently be inaccurate in estimating pulmonary artery pressure and cardiac output in patients being evaluated for PH.


Assuntos
Ecocardiografia Doppler/efeitos adversos , Hipertensão Pulmonar/diagnóstico por imagem , Adulto , Idoso , Reações Falso-Negativas , Reações Falso-Positivas , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Sensibilidade e Especificidade
14.
J Am Soc Echocardiogr ; 21(9): 1028-34, 2008 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-18558476

RESUMO

BACKGROUND: Right ventricular (RV) function has major prognostic implications for patients with pulmonary arterial hypertension (PAH). Intraventricular dyssynchrony might play an important role in RV dysfunction in these patients. METHODS: Thirty-six patients with PAH without right bundle branch block (mean age 44 +/- 14 yr, 24 women) and 39 controls (mean age 43 +/- 18 yr, 26 women) were evaluated. Global and segmental RV longitudinal deformation parameters were recorded by 2-dimensional strain echocardiography from apical 4-chamber views using a 6-segment RV model. The standard deviation of the heart rate-corrected intervals from QRS onset to peak strain for the 6 segments (RV-SD(6)) was used to quantify right intraventricular dyssynchrony. RESULTS: RV-SD(6) was significantly higher in patients with PAH compared with controls (63 +/- 21 vs 25 +/- 15ms, P < .001). Dyssynchrony in patients with PAH was found to derive mainly from delayed contraction of the basal and mid RV free wall. In patients with PAH, RV-SD(6) was strongly correlated with RV fractional area change (beta = -.519, P = .002), RV myocardial performance index (beta = .427, P = .009), and RV global strain (beta = .512, P = .002); in models controlling for RV systolic pressure, RV size, and QRS duration, RV-SD(6) was still an independent predictor of RV fractional area change (beta = -.426, P = .005) and RV global strain (beta = .358, P = .031). RV function was significantly worse in the subgroup of patients with PAH (n = 25) with RV-SD(6) > 55 ms (the upper 95% limit in controls). CONCLUSION: Right intraventricular dyssynchrony, as quantified by 2-dimensional strain echocardiography, is prevalent in PAH and is associated with more pronounced RV dysfunction. The clinical implications of these findings remain to be determined in follow-up studies.


Assuntos
Ecocardiografia/métodos , Técnicas de Imagem por Elasticidade/métodos , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/diagnóstico por imagem , Disfunção Ventricular Direita/complicações , Disfunção Ventricular Direita/diagnóstico por imagem , Adulto , Feminino , Humanos , Masculino , Reprodutibilidade dos Testes , Sensibilidade e Especificidade
15.
Transl Res ; 151(4): 197-207, 2008 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-18355767

RESUMO

We hypothesize that pulmonary arterial hypertension (PAH)-associated genes identified by expression profiling of peripheral blood mononuclear cells (PBMCs) from patients with idiopathic pulmonary arterial hypertension (IPAH) can also be identified in PBMCs from scleroderma patients with PAH (PAH-SSc). Gene expression profiles of PBMCs collected from IPAH (n = 9), PAH-SSc (n = 10) patients, and healthy controls (n = 5) were generated using HG_U133A_2.0 GeneChips and were processed by the RMA/GCOS_1.4/SAM_1.21 data analysis pipeline. Disease severity in consecutive patients was assessed by functional status and hemodynamic measurements. The expression profiles were analyzed using PAH severity-stratification, and identified candidate genes were validated with real-time polymerase chain reaction (PCR). Transcriptomics of PBMCs from IPAH patients was highly comparable with that of PMBCs from PAH-SSc patients. The PBMC gene expression patterns significantly correlate with right atrium pressure (RA) and cardiac index (CI), which are known predictors of survival in PAH. Array stratification by RA and CI identified 364 PAH-associated candidate genes. Gene ontology (GO) analysis revealed significant (Z(score) > 1.96) alterations in angiogenesis genes according to PAH severity: matrix metalloproteinase 9 (MMP9) and vascular endothelial growth factor (VEGF) were significantly upregulated in mild as compared with severe PAH and healthy controls, as confirmed by real-time PCR. These data demonstrate that PBMCs from patients with PAH-SSc carry distinct transcriptional expression. Furthermore, our findings suggest an association between angiogenesis-related gene expression and severity of PAH in PAH-SSc patients. Deciphering the role of genes involved in vascular remodeling and PAH development may reveal new treatment targets for this devastating disorder.


Assuntos
Predisposição Genética para Doença , Hipertensão Pulmonar/genética , Metaloproteinase 9 da Matriz/genética , Artéria Pulmonar , Escleroderma Sistêmico/genética , Fator A de Crescimento do Endotélio Vascular/genética , Idoso , Feminino , Perfilação da Expressão Gênica , Humanos , Leucócitos Mononucleares/metabolismo , Masculino , Pessoa de Meia-Idade , Projetos Piloto , Artéria Pulmonar/fisiopatologia , Escleroderma Sistêmico/fisiopatologia , Índice de Gravidade de Doença , Regulação para Cima/genética
16.
Am J Respir Crit Care Med ; 177(12): 1364-9, 2008 Jun 15.
Artigo em Inglês | MEDLINE | ID: mdl-18356560

RESUMO

RATIONALE: Hyponatremia is associated with decompensated heart failure and poor prognosis in patients with left ventricular systolic dysfunction. OBJECTIVES: We sought to determine if hyponatremia is associated with right heart failure and worse prognosis in patients with pulmonary arterial hypertension (PAH). METHODS: We prospectively followed 40 patients with PAH and examined the relationship between serum sodium and right heart function as well as survival. MEASUREMENTS AND MAIN RESULTS: Subjects with hyponatremia (Na < or = 136 mEq/L) were more symptomatic (11/13 World Health Organization [WHO] class III/IV vs. 12/27 WHO class III/IV; P = 0.02), had more peripheral edema (69 vs. 26%; P = 0.009), and had higher hospitalization rates (85 vs. 41%; P = 0.009) than normonatremic subjects. Hyponatremic subjects had higher right atrial pressure (14 +/- 6 vs. 9 +/- 3 mm Hg; P < 0.001), lower stroke volume index (21 +/- 7 vs. 32 +/- 10 ml/m(2); P < 0.01), larger right ventricular:left ventricular area ratio (1.8 +/- 0.4 vs. 1.3 +/- 0.4; P < 0.001), and lower tricuspid annular plane systolic excursion (1.4 +/- 0.3 vs. 2.0 +/- 0.6 cm; P = 0.001), despite similar mean pulmonary artery pressure (49 +/- 10 vs. 47 +/- 12 mm Hg; P = 0.60). The 1- and 2-year survival estimates were 93% (95% confidence interval [CI], 73-98%) and 85% (95% CI, 65-94%), and 38% (95% CI, 14-63%) and 15% (95% CI, 2-39%) for normonatremic and hyponatremic subjects, respectively (log-rank chi(2) = 25.19, P < 0.001). The unadjusted risk of death (hazard ratio) in hyponatremic compared with normonatremic subjects was 10.16 (95% CI, 3.42-30.10, P < 0.001). Hyponatremia predicted outcome after adjusting for WHO class, diuretic use, as well as right atrial pressure and cardiac index. CONCLUSIONS: Hyponatremia is strongly associated with right heart failure and poor survival in PAH.


Assuntos
Insuficiência Cardíaca/sangue , Hipernatremia/sangue , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/mortalidade , Índice de Gravidade de Doença , Estudos de Casos e Controles , Feminino , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/mortalidade , Humanos , Hipernatremia/etiologia , Hipernatremia/mortalidade , Hipertensão Pulmonar/complicações , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Prognóstico , Modelos de Riscos Proporcionais , Estudos Prospectivos , Taxa de Sobrevida
17.
Arthritis Rheum ; 54(9): 3043-50, 2006 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-16947776

RESUMO

OBJECTIVE: Pulmonary arterial hypertension related to scleroderma (PAH-Scl) is associated with high morbidity and mortality as well as poorer response to therapy and worse outcomes compared with the idiopathic form of PAH (IPAH). Scleroderma is an autoimmune disease that can affect left and right heart function directly through inflammation and fibrosis and indirectly through systemic and pulmonary hypertension. This study tested the hypothesis that an increased prevalence of left heart disease might explain the higher mortality in patients with PAH-Scl compared with patients with IPAH. METHODS: The study was designed as a retrospective cohort study comparing the baseline clinical data from 91 consecutive patients (41 with IPAH and 50 with PAH-Scl). Cox proportional hazards models were used to predict the effect of clinical covariates on patient survival. RESULTS: Patients with PAH-Scl had a lower mean pulmonary artery pressure (46.6 mm Hg versus 54.4 mm Hg in patients with IPAH; P = 0.002) despite similar levels of cardiac dysfunction (cardiac index 2.2 and 2.1 liters/minute/m(2), respectively; P = 0.19). Echocardiography revealed similar degrees of right ventricular dysfunction in the 2 groups, whereas a predominance of left heart dysfunction was observed in patients with PAH-Scl. One- and three-year survival estimates were 87.8% and 48.9%, respectively, in patients with PAH-Scl and 95.1% and 83.6%, respectively, in those with IPAH. Patients with PAH-Scl were 3.06 times more likely to die than were patients with IPAH, after controlling for the presence of pericardial effusion; there was no significant change in increased risk of death in PAH-Scl after controlling for left heart disease. CONCLUSION: The results confirm that there are significant clinical and survival differences between IPAH and PAH-Scl. The presence of left heart disease, although more common in PAH-Scl, was not predictive of the higher mortality in these patients.


Assuntos
Hipertensão/complicações , Hipertensão/fisiopatologia , Esclerodermia Limitada/complicações , Esclerodermia Limitada/fisiopatologia , Adulto , Índice de Massa Corporal , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Artéria Pulmonar/fisiopatologia , Testes de Função Respiratória , Esclerodermia Limitada/mortalidade , Análise de Sobrevida
18.
Am J Respir Crit Care Med ; 174(9): 1034-41, 2006 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-16888289

RESUMO

RATIONALE: Right ventricular (RV) function is an important determinant of prognosis in pulmonary hypertension. However, noninvasive assessment of the RV function is often limited by complex geometry and poor endocardial definition. OBJECTIVES: To test whether the degree of tricuspid annular displacement (tricuspid annular plane systolic excursion [TAPSE]) is a useful echo-derived measure of RV function with prognostic significance in pulmonary hypertension. METHODS: We prospectively studied 63 consecutive patients with pulmonary hypertension who were referred for a clinically indicated right heart catheterization. Patients underwent right heart catheterization immediately followed by transthoracic echocardiogram and TAPSE measurement. RESULTS: In the overall cohort, a TAPSE of less than 1.8 cm was associated with greater RV systolic dysfunction (cardiac index, 1.9 vs. 2.7 L/min/m2; RV % area change, 24 vs. 33%), right heart remodeling (right atrial area index, 17.0 vs. 12.1 cm(2)/m), and RV-left ventricular (LV) disproportion (RV/LV diastolic area, 1.7 vs. 1.2; all p < 0.001), versus a TAPSE of 1.8 cm or greater. In patients with pulmonary arterial hypertension (PAH; n = 47), survival estimates at 1 and 2 yr were 94 and 88%, respectively, in those with a TAPSE of 1.8 cm or greater versus 60 and 50%, respectively, in subjects with a TAPSE less than 1.8 cm. The unadjusted risk of death (hazard ratio) in patients with a TAPSE less than 1.8 versus 1.8 cm or greater was 5.7 (95% confidence interval, 1.3-24.9; p = 0.02) for the PAH cohort. For every 1-mm decrease in TAPSE, the unadjusted risk of death increased by 17% (hazard ratio, 1.17; 95% confidence interval, 1.05-1.30; p = 0.006), which persisted after adjusting for other echocardiographic and hemodynamic variables and baseline treatment status. CONCLUSIONS: TAPSE powerfully reflects RV function and prognosis in PAH.


Assuntos
Hipertensão Pulmonar/mortalidade , Hipertensão Pulmonar/fisiopatologia , Função Ventricular Direita , Adulto , Idoso , Cateterismo Cardíaco , Ecocardiografia Doppler , Feminino , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Prospectivos , Curva ROC
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