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
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.
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J. Heart Lung Transplant. · Mar 2015
Multicenter StudyPredicting outcomes in pulmonary arterial hypertension based on the 6-minute walk distance.
Clinical studies of pulmonary arterial hypertension have used the change in the 6-minute walk distance (6MWD) as a clinical end point; however, its association with survival outcomes has not been well established. In this analysis, we examined the prognostic value of the baseline 6MWD, absolute thresholds of the 6MWD, and change in the 6MWD. ⋯ No 6MWD improvement threshold carries particular prognostic value. Improvement in the 6MWD was not associated with survival, but worsening of the 6MWD was strongly and significantly associated with poor prognosis.
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J. Heart Lung Transplant. · Mar 2015
Pulmonary endarterectomy in severe chronic thromboembolic pulmonary hypertension.
The outcome of patients with chronic thromboembolic pulmonary hypertension (CTEPH) undergoing pulmonary endarterectomy (PEA) after urgent hospitalization for decompensated right heart failure (DRHF) remains unclear. ⋯ DRHF occurs more frequently in patients with TPR > 1,200 dynes · sec · cm(-5), increasing the operative risk in these patients. The outcome of patients with high TPR in the absence of DRHF is excellent. However, patients with residual mean pulmonary artery pressure ≥35 mm Hg frequently receive pulmonary hypertension therapy after PEA.