Challenges in pulmonary hypertension: managing the unexpected

Olsson, Karen M.; Palazzini, Massimiliano

doi:10.1183/16000617.0060-2015

Cited by 11 publications

(4 citation statements)

References 39 publications

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“…In contrast to these previous reports, our case responded to treatment with glucocorticoid and IVCY despite WHO functional class IV PAH, a markedly decreased cardiac index, and refractoriness to combined vasodilators. Currently there are two previous case reports of SLE- or MCTD-associated WHO functional class IV PAH successfully treated with glucocorticoid and IVCY in conjunction with up-front triple combination therapy comprising intravenous prostacyclin analogue, endothelin receptor antagonist, and phosphodiesterase type 5 inhibitor ( 5 , 6 ). Our case, particularly with regard to the refractoriness to combined vasodilation, further supports the efficacy of immunosuppressive therapy for severe PAH associated with SLE or MCTD.…”

Section: Discussionmentioning

confidence: 99%

Treatment of Vasodilator-resistant Mixed Connective Tissue Disease-associated Pulmonary Arterial Hypertension with Glucocorticoid and Cyclophosphamide

et al. 2017

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Pulmonary arterial hypertension (PAH) associated with systemic lupus erythematosus (SLE) or mixed connective tissue disease (MTCD), in contrast to other types of PAH, may respond to immunosuppressive therapy. Most PAH cases with an immunosuppressant response were in the early stages of the disease (WHO functional class III or less). The present case was a 34-year-old woman with MCTD-associated PAH (WHO functional class IV) who was resistant to a combination of three vasodilators. Afterwards, she was treated with glucocorticoid and cyclophosphamide. This case suggested the potential benefit of immunosuppressants in patients with severe MCTD-associated PAH.

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Section: Discussionmentioning

confidence: 99%

Treatment of Vasodilator-resistant Mixed Connective Tissue Disease-associated Pulmonary Arterial Hypertension with Glucocorticoid and Cyclophosphamide

et al. 2017

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“…With associated forms of PAH and other forms of PH, control of the underlying disease is frequently a precondition for controlling RHF (e.g. immunosuppressive therapies in SLE) [23].…”

Section: Infectionsmentioning

confidence: 99%

Decompensated right heart failure, intensive care and perioperative management in patients with pulmonary hypertension: Updated recommendations from the Cologne Consensus Conference 2018

Olsson

Halank

Egenlauf

et al. 2018

International Journal of Cardiology

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In June 2016, members of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Pediatric Cardiology (DGPK) met for a Consensus Conference in Cologne, Germany. Aim of this Conference was to compile consensus based practice recommendations based on the 2015 European Pulmonary Hypertension guidelines, aiming at their practical implementation, considering country-specific issues, and including new evidence, where available. This article summarizes the results and updated recommendations 2018 of the working group on decompensated right heart failure (RHF), intensive care and perioperative management in patients with pulmonary hypertension. The RHF section comprises definition and pathophysiology, diagnosis and monitoring, identification of triggering factors and supportive therapy of RHF, volume management as well as PAH targeting therapy, therapy with inotropic, inodilator and vasopressor drugs, extracorporeal support and transplantation. The second part of this article summarizes preoperative management, perioperative monitoring and choice of anesthesia.

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“…Real-life case studies, to further illustrate evidence gaps for identifying and managing complex forms of PH, are presented in the case-based review by OLSSON and PALAZZINI [6]. The authors refer to diverse cases, each reminding us of the importance of a multidisciplinary approach to patient management and that all presentations of PH warrant careful consideration.…”

Section: @Erspublicationsmentioning

confidence: 99%

Improving patient outcomes in pulmonary hypertension

et al. 2015

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@ERSpublicationsThe updated management of PAH patients is leading to improvements in quality of life and outcome http://ow.ly/TAvMfIn recent years we have come a long way in our understanding of pulmonary hypertension (PH). As a result of ever-increasing experience in treating pulmonary arterial hypertension (PAH), and the availability of new treatment options, there have been continual improvements in patient outcomes. As patients are today living longer with this disease, there is now a focus on assessing the impact that PAH has on quality of life, and increased recognition that PAH management must aim to reduce this impact. The articles in this issue of European Respiratory Review discuss practical aspects of managing patients with PH, look at the disease from the patient perspective, and review some of the latest strategies for diagnosis and treatment of this group of conditions. The authors, each of them experts in the field of PH, delivered presentations at the 14th International Pulmonary Hypertension Forum held in March 2015 in Copenhagen, Denmark, upon which this series of reviews is based.Patients with PAH face numerous challenges that impact their day-to-day living as a result of the debilitating and progressive nature of the condition. While the ultimate goal of PAH treatment is to improve survival, it should also be to improve patients' quality of life. We should remember that many PAH patients often do not look sick to others and it is thus important to question how often the impact of PAH on health-related quality of life (HRQoL) is considered. DELCROIX and HOWARD [1] describe the burden of PAH on patients' physical, psychological and social well-being, and discuss the potential benefits of new tools in development for measuring the effect of PAH on HRQoL.One way to reduce the burden of PAH is to diagnose patients early. To help achieve this goal, noninvasive imaging techniques have been developed to assist with screening, risk stratification and monitoring treatment outcomes in patients with PH. The review by GRÜNIG and PEACOCK [2] focuses on how imaging techniques, including echocardiography and cardiac magnetic resonance, can provide complementary information about the volumetric and muscle mass limits of the heart. This review also highlights how improvements in imaging software and novel methodologies have the potential to influence how PH is diagnosed.Although the diagnostic work-up of suspected PH/PAH relies on noninvasive imaging techniques, confirmatory testing with right heart catheterisation (RHC) is essential [3]. RHC is an established and

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Challenges in pulmonary hypertension: managing the unexpected

Cited by 11 publications

References 39 publications

Treatment of Vasodilator-resistant Mixed Connective Tissue Disease-associated Pulmonary Arterial Hypertension with Glucocorticoid and Cyclophosphamide

Treatment of Vasodilator-resistant Mixed Connective Tissue Disease-associated Pulmonary Arterial Hypertension with Glucocorticoid and Cyclophosphamide

Decompensated right heart failure, intensive care and perioperative management in patients with pulmonary hypertension: Updated recommendations from the Cologne Consensus Conference 2018

Improving patient outcomes in pulmonary hypertension

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