The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation
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J. Heart Lung Transplant. · Mar 2015
Right ventricular dyssynchrony in idiopathic pulmonary arterial hypertension: determinants and impact on pump function.
Right ventricular (RV) dyssynchrony has been described in pulmonary arterial hypertension (PAH), but no evidence is available on its morphologic determinants and its effect on systolic function. The aim of this study was to evaluate the morphologic determinants of RV dyssynchrony by echocardiographic and cardiac magnetic resonance imaging and its effect on systolic function. ⋯ In IPAH with narrow QRS, RV dyssynchrony is associated with RV dilation and eccentric hypertrophy pattern, suggesting a role of segmental wall stress heterogeneity as the major determinant of mechanical delay. Post-systolic shortening, as inefficient contraction, contributes to pump dysfunction.
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J. Heart Lung Transplant. · Mar 2015
Endothelin-Bone morphogenetic protein type 2 receptor interaction induces pulmonary artery smooth muscle cell hyperplasia in pulmonary arterial hypertension.
Endothelin receptor antagonists improve pulmonary arterial hypertension (PAH). Mutations in the bone morphogenetic protein (BMP) type 2 receptor (BMPR2) predispose to PAH. Here, we sought to determine whether there might exist interactions between these 2 signaling pathways and their effect on the acquisition of the altered phenotype of pulmonary artery smooth muscle cells (PA-SMCs) observed in PAH. ⋯ Endothelin-1 downregulates canonical BMPR2 signaling. This is related to decreased BMPR2 and increased anti-BMP gremlin expression associated with increased activation of p38(MAPK) and results in PA-SMC proliferation.
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J. Heart Lung Transplant. · Mar 2015
Multicenter Study Observational StudyPrognostic implications of serial risk score assessments in patients with pulmonary arterial hypertension: a Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL) analysis.
Data from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL) were used previously to develop a risk score calculator to predict 1-year survival. We evaluated prognostic implications of changes in the risk score and individual risk-score parameters over 12 months. ⋯ Changes in REVEAL risk scores occur in most patients with pulmonary arterial hypertension over a 12-month period and are predictive of survival. Thus, serial risk score assessments can identify changes in disease trajectory that may warrant treatment modifications.
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J. Heart Lung Transplant. · Mar 2015
Multicenter StudyEffect of pulmonary hypertension on survival in patients with idiopathic pulmonary fibrosis after lung transplantation: an analysis of the United Network of Organ Sharing registry.
Pulmonary hypertension (PH) is a comorbidity associated with idiopathic pulmonary fibrosis (IPF). There is limited research regarding the effect on survival after lung transplantation (LTx). ⋯ On the basis of our models and despite PH being prevalent, there is no strong evidence suggesting that PH significantly alters the risk of death in IPF patients after LTx.
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J. Heart Lung Transplant. · Mar 2015
ReviewPulmonary hypertension in heart failure with preserved ejection fraction.
In heart failure with preserved ejection fraction (HFpEF), an entity that remains challenging and difficult to treat, the development of pulmonary hypertension (PH), via an increase in left atrial pressure, is the direct consequence of reduced relaxation and enhanced stiffness of the left ventricle and is now viewed as an important contributor to clinical worsening and increased mortality. PH becomes a relevant clinical phenotype in approximately 50% of patients with HFpEF and represents a true challenge in the clinical follow-up and management of these patients. Along with these epidemiologic insights, there has been increasing recognition of the pathophysiology of PH and its consequences on the right ventricle in patients with HFpEF. ⋯ Many theoretical rationales as well as progressively accumulating evidence support the usefulness of nitric oxide pathway-potentiating compounds in targeting the lung vasculature through phosphodiesterase 5 inhibitors or guanylate cyclase stimulators to produce vasodilation and potentially a biologic effect. These pharmacologic strategies may be clinically effective options for the treatment of PH in patients with HFpEF; however, large controlled trials are necessary to address definitively the safety, tolerability, and potential impact on morbidity and mortality. This review details the pathophysiologic process, prevalence, and consequences of HFpEF-associated PH and discusses current and emerging treatment strategies to prevent or treat this deleterious sequela when present.