Pulmonary Arterial Hypertension

McLaughlin, Vallerie V.; Davis, Melinda B.; Cornwell, William K.

doi:10.1016/j.cpcardiol.2011.08.002

Cited by 94 publications

(103 citation statements)

References 196 publications

(83 reference statements)

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“…WHO functional class, 6-min walking distance (6MWD), peak VO 2 , right ventricular function, hemodynamic parameters and plasma levels of brain natriuretic peptide (BNP) have all been found to influence prognosis [27]. Recently, it has been proposed that multivariable risk models might better address a patient's overall clinical status and outcome.…”

Section: Outcome and Outcome Predictorsmentioning

confidence: 99%

The challenge of managing pulmonary arterial hypertension in adults with congenital heart disease

Radke

Diller

Baumgartner

2013

Expert Review of Cardiovascular Therapy

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Section: Outcome and Outcome Predictorsmentioning

confidence: 99%

The challenge of managing pulmonary arterial hypertension in adults with congenital heart disease

Radke

Diller

Baumgartner

2013

Expert Review of Cardiovascular Therapy

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“…1 Current therapies for PAH aim to reverse the endothelial dysfunction and vasoconstriction observed. 2 However, despite these therapies, PAH mortality rates remain high, and the 3-year survival of patients is only 54.9%. 3 Changes in the pulmonary vasculature are the primary cause of PAH; however, right ventricle (RV) function is a major determinant of the severity of symptoms and prognosis of pulmonary hypertension.…”

mentioning

confidence: 99%

Regulation and Function of miR‐214 in Pulmonary Arterial Hypertension

et al. 2016

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Dysregulation of microRNAs (miRNAs) can contribute to the etiology of diseases, including pulmonary arterial hypertension (PAH). Here we investigated a potential role for the miR-214 stem loop miRNA and the closely linked miR-199a miRNAs in PAH. All 4 miRNAs were upregulated in the lung and right ventricle (RV) in mice and rats exposed to the Sugen (SU) 5416 hypoxia model of PAH. Further, expression of the miRNAs was increased in pulmonary artery smooth muscle cells exposed to transforming growth factor β1 but not BMP4. We then examined miR-214 −/− mice exposed to the SU 5416 hypoxia model of PAH or normoxic conditions and littermate controls. There were no changes in RV systolic pressure or remodeling observed between the miR-214 −/− and wild-type hypoxic groups. However, we observed a significant increase in RV hypertrophy (RVH) in hypoxic miR-214 −/− male mice compared with controls. Further, we identified that the validated miR-214 target phosphatase and tensin homolog was upregulated in miR-214 −/− mice. Thus, miR-214 stem loop loss leads to elevated RVH and may contribute to the heart failure associated with PAH. Pulmonary arterial hypertension (PAH) is a disease characterized by narrowing of the small pulmonary arteries, leading to vascular remodeling, an elevation in pulmonary artery pressure, right ventricular hypertrophy (RVH), and heart failure. 1 Current therapies for PAH aim to reverse the endothelial dysfunction and vasoconstriction observed. 2 However, despite these therapies, PAH mortality rates remain high, and the 3-year survival of patients is only 54.9%. 3 Changes in the pulmonary vasculature are the primary cause of PAH; however, right ventricle (RV) function is a major determinant of the severity of symptoms and prognosis of pulmonary hypertension. Many therapies in development for PAH are focused on targeting the RV since heart failure is the ultimate cause of mortality in PAH. 4 PAH is predominant in females, with female ∶ male ratios of 1.4-4.1 ∶ 1. 5 Sexual dimorphism has also been observed in RV failure. Female PAH patients exhibit improved RV ejection fraction and survival compared with men. 6 This could be due at least in part to the protective effect of estrogen on RV function. 7,8 MicroRNAs (miRNAs) are involved in multiple cellular responses during normal development and disease; they act as posttranscriptional regulators to fine-tune protein synthesis. Evidence has emerged for a key role for miRNA in regulation of the cellular processes involved in PAH. We previously demonstrated that a range of miRNAs are dysregulated in rats exposed to models of PAH. 9 Later studies have shown that miR-21, the miR-143/145 cluster, miR-27a, the miR-17-92 cluster, miR-124, miR-150, miR-138, miR-190, miR-204, miR-206, miR-210, and miR-328 play a role in the development of PAH. 10 Multiple miRNAs could potentially be targeted in concert as therapeutics in PAH. 11 MicroRNA miR-214 is transcribed as a bicistronic primary transcript, which is processed to generate 4 separate mature miRNAs...

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“…10 Previously lost to follow-up, she presented to the Department of Cardiology with a 3-week history of worsening dyspnea and exercise intolerance. She had not established prenatal care and was evaluated at 23 weeks gestation by ultrasound.…”

Section: Case Presentationmentioning

confidence: 99%

Epoprostenol treatment for idiopathic pulmonary arterial hypertension in pregnancy

et al. 2010

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Idiopathic pulmonary arterial hypertension is a rare condition associated with significant maternal mortality. We report the management of a 37-year-old multigravida with severe disease using epoprostenol, a multidisciplinary approach, and a planned delivery. Although the patient survived the pregnancy, her pulmonary function significantly worsened. Epoprostenol, a pulmonary vasodilator, should be considered when indicated during pregnancy. Neither fetal nor neonatal harm was identified.

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Pulmonary Arterial Hypertension

Cited by 94 publications

References 196 publications

The challenge of managing pulmonary arterial hypertension in adults with congenital heart disease

The challenge of managing pulmonary arterial hypertension in adults with congenital heart disease

Regulation and Function of miR‐214 in Pulmonary Arterial Hypertension

Epoprostenol treatment for idiopathic pulmonary arterial hypertension in pregnancy

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