A decade of achievement in pulmonary hypertension

Humbert, Marc; Simonneau, Gérald; Rubin, Leona J.

doi:10.1183/09059180.00007311

Cited by 14 publications

(12 citation statements)

References 30 publications

(42 reference statements)

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“…PAH symptoms include dyspnoea, fatigue, chest pain, syncope and peripheral oedema [2]. Although significant advances have been made regarding the understanding of the disease and its treatment, it remains a devastating, progressively debilitating disease [1,[3][4][5][6].…”

Section: Introductionmentioning

confidence: 99%

Understanding the impact of pulmonary arterial hypertension on patients' and carers' lives

Guillevin¹,

Armstrong²,

Aldrighetti³

et al. 2013

European Respiratory Review

129

215

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Pulmonary arterial hypertension (PAH) is a rare, debilitating and rapidly progressive disease. Although there have been important medical advances in PAH management, the search for a cure continues. Despite an increased understanding of the disease, data on the wider effect of PAH on patients and carers, beyond the clinical symptoms, are still limited. In order to explore this, a large-scale international survey investigated four key areas affected by PAH (physical and practical, emotional, social, and information needs) and provides new insight into patients' and carers' experiences of living with the disease. The results from the survey highlight not only the limited ability of patients to carry out everyday tasks, but also the financial impact and social isolation experienced by both patients and carers. The study confirmed that a decline in a patient's World Health Organization functional class, which indicates an increase in clinical severity of the disease, is associated with greater limitations. Results from the survey demonstrate the need for multidisciplinary PAH management and a comprehensive standard of care to assess and improve all aspects of well-being for both patients and carers. In addition, they underline the need for updated PAH guidelines that address these needs. @ERSpublications PAH has a profound impact on numerous aspects of patients' and carers' lives http://ow.ly/pWWYf This article has supplementary material available from err.ersjournals.comFull survey results, along with a call to action, are available at www.phaeurope.org/projects-activities/pah-patient-andcarer-survey

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Section: Introductionmentioning

confidence: 99%

Understanding the impact of pulmonary arterial hypertension on patients' and carers' lives

Guillevin¹,

Armstrong²,

Aldrighetti³

et al. 2013

European Respiratory Review

129

215

View full text Add to dashboard Cite

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“…These progresses were accompanied by the development of drugs that target specific pathways in the pathophysiology of the disease [2]. Management in specialized centers and the use of pulmonary vasodilators lead to a significant impact on the survival and quality of life of PAH patients [3]. Unfortunately, survival rates are still unsatisfactory [4], signaling for the need of more effective treatments, which are under development [5].…”

Section: Introductionmentioning

confidence: 99%

Pulmonary Hypertension in Portugal: First Data from a Nationwide Registry

Baptista

Meireles

Agapito

et al. 2013

BioMed Research International

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Introduction. Pulmonary arterial hypertension (PAH) is a rare disease that must be managed in specialized centers; therefore, the availability of epidemiological national data is critical. Methods. We conducted a prospective, observational, and multicenter registry with a joint collaboration from five centers from Portugal and included adult incident patients with PAH or chronic thromboembolic pulmonary hypertension (CTEPH). Results. Of the 79 patients enrolled in this study, 46 (58.2%) were classified as PAH and 33 patients (41.8%) as CTEPH. PAH patients had a mean age of 43.4 ± 16.4 years. Idiopathic PAH was the most common etiology (37%). At presentation, PAH patients had elevated right atrial pressure (RAP) (7.7 ± 5.9 mmHg) and mean pulmonary vascular resistance (11.4 ± 6.5 Wood units), with a low cardiac index (2.7 ± 1.1 L·min−1 ·m−2); no patient was under selective pulmonary vasodilators; however, at follow-up, most patients were on single (50%), double (28%), or triple (9%) combination vasodilator therapy. One-year survival was 93.5%, similar to CTEPH patients (93.9%), that were older (60.0 ± 12.5 years) and had higher RAP (11.0 ± 5.2 mmHg, P = 0.015). Conclusions. We describe for the first time nationwide data on the diagnosis, management, and prognosis of PAH and CTEPH patients in Portugal. Clinical presentation and outcomes are comparable with those reported on other national registries.

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“…8‐10 With expanding research into the diagnosis and treatment of PH, it is important to provide updated surveillance on the impact of this disease on hospitalizations and mortality. The surveillance report by Hyduk et al 2 described trends in mortality and hospitalization rates associated with PH among adults aged 45 years and older from 1980 through 2002 by demographic characteristics.…”

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confidence: 99%

Pulmonary Hypertension Surveillance

George

Schieb

Ayala

et al. 2014

Chest

113

103

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Pulmonary hypertension (PH) is an uncommon but progressive condition, and much of what we know about it comes from specialized disease registries. With expanding research into the diagnosis and treatment of PH, it is important to provide updated surveillance on the impact of this disease on hospitalizations and mortality. This study, which builds on previous PH surveillance of mortality and hospitalization, analyzed mortality data from the National Vital Statistics System and data from the National Hospital Discharge Survey between 2001 and 2010. PH deaths were identified using International Classification of Diseases, Tenth Revision codes I27.0, I27.2, I27.8, or I27.9 as any contributing cause of death on the death certificate. Hospital discharges associated with PH were identified using International Classification of Diseases, Ninth Revision, Clinical Modification codes 416.0, 416.8, or 416.9 as one of up to seven listed medical diagnoses. The decline in death rates associated with PH among men from 1980 to 2005 has reversed and now shows a significant increasing trend. Similarly, the death rates for women with PH have continued to increase significantly during the past decade. PH-associated mortality rates for those aged 85 years and older have accelerated compared with rates for younger age groups. There have been significant declines in PH-associated mortality rates for those with pulmonary embolism and emphysema. Rates of hospitalization for PH have increased significantly for both men and women during the past decade; for those aged 85 years and older, hospitalization rates have nearly doubled. Continued surveillance helps us understand and address the evolving trends in hospitalization and mortality associated with PH and PH-associated conditions, especially regarding sex, age, and race/ethnicity disparities.

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A decade of achievement in pulmonary hypertension

Cited by 14 publications

References 30 publications

Understanding the impact of pulmonary arterial hypertension on patients' and carers' lives

Understanding the impact of pulmonary arterial hypertension on patients' and carers' lives

Pulmonary Hypertension in Portugal: First Data from a Nationwide Registry

Pulmonary Hypertension Surveillance

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