scholarly journals Lupus-Associated Pulmonary Arterial Hypertension: Variable Course and Importance of Prompt Recognition

2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Kofi A. Mensah ◽  
Rajwardhan Yadav ◽  
Terence K. Trow ◽  
Cristina M. Brunet ◽  
Wassim H. Fares
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lupus Erythematosus ◽  
Treatment Approach ◽  
Long Term Outcomes ◽  
Care Level ◽  
Life Threatening ◽  
Pulmonary Arterial ◽  
Severe Pulmonary Arterial Hypertension

We describe a critically ill young woman with systemic lupus erythematosus (SLE) presenting with circulatory shock, multiorgan dysfunction, and elevated right-sided heart pressures. She was found to have recurrent acute severe pulmonary arterial hypertension (PAH) in the setting of an SLE flare. Our report highlights the variable course that SLE-associated PAH can take in the same patient and the implications of this for instituting the most effective treatment approach with each episode. This report also highlights the potential for SLE-associated PAH to present with life-threatening symptoms requiring critical care level interventions. We also describe evidence-based therapies, which can result in significant improvement in symptoms, function, and long-term outcomes.

Idiopathic Pulmonary Arterial Hypertension: Evolving Therapeutic Strategies

2017 ◽  
Vol 38 (05) ◽  
pp. 606-618 ◽  
Author(s):  
Manreet Kanwar ◽  
Amresh Raina ◽  
Michael Passineau ◽  
Raymond Benza
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Treatment Approach ◽  
Long Term Outcomes ◽  
Treatment Algorithms ◽  
The Past ◽  
Early Use ◽  
Short And Long Term ◽  
Pulmonary Arterial

AbstractThe field of pulmonary arterial hypertension (PAH) has seen substantial changes in the epidemiology, associated comorbidities, treatment algorithms, and prognosis in the past decade. Specifically for idiopathic PAH (IPAH), there has been a noted increase in the age of initial diagnosis. Increasing focus is being placed on appropriate categorization of patients with PAH, both in terms of diagnosis and individualized risk stratification. This is especially relevant given that patients being diagnosed with incident IPAH are now older and with multiple comorbidities, putting them at a higher risk of being mislabeled as PAH. The biggest impact has been seen in the number of pharmaceutical options available for these patients, resulting in improvement in both short- and long-term outcomes. In addition, there has been a shift in the treatment approach to the early use of combination therapies in PAH, since it has been demonstrated to improve clinical outcomes. Novel therapies are in the pipeline for patients with IPAH to impact both the morbidity and mortality of this deadly disease.

scholarly journals Occult catheter rupture causing episodic symptoms in a patient treated with epoprostenol

2017 ◽  
Vol 8 (1) ◽  
pp. 204589321774805 ◽  
Author(s):  
Barbara L. LeVarge ◽  
Anica C. Law ◽  
Blanche Murphy
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Adverse Events ◽  
Arterial Hypertension ◽  
Long Term Outcomes ◽  
Pulmonary Arterial

Infection, thrombosis, and catheter dislodgment are well-recognized potential complications of chronic intravenous prostanoid therapy for pulmonary arterial hypertension. As long-term outcomes of pulmonary hypertension patients improve, novel adverse events are likely to arise. We describe the sudden development of unexplained hypotension and lightheadedness in a patient receiving intravenous epoprostenol for several years, ultimately determined to be due to an unusual catheter complication, not previously described in this population.

Pulmonary arterial hypertension associated with connective tissue disease

ESC CardioMed ◽  
2018 ◽  
pp. 2531-2534
Author(s):  
Christopher P. Denton
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Connective Tissue Disease ◽  
Lupus Erythematosus ◽  
Heart Catheterization ◽  
Pulmonary Arterial ◽  
Detection And Diagnosis ◽  
Health Organization

Connective tissue disease-associated pulmonary arterial hypertension (PAH) falls within World Health Organization group 1. These patients are treated as others in this group, but there are important considerations regarding detection and diagnosis. Patients with connective tissue disease are at risk of PAH and should be screened with confirmation of diagnosis by right heart catheterization. Treatment follows the European Society of Cardiology guidelines for other forms of PAH. However, more information is available for systemic sclerosis PAH regarding screening, including the DETECT algorithm, and also in terms of long-term outcome of patients with borderline elevation of mean pulmonary arterial pressure. In cases of systemic lupus erythematosus or mixed connective tissue disease, immunosuppression should be given in conjunction with targeted PAH-specific therapy. Long-term outcomes for PAH in patients with connective tissue disease have improved since targeted specific therapies became available. Recent trials with morbidity–mortality endpoints and a high proportion of patients receiving combination treatment have shown comparable benefits for patients with connective tissue disease and PAH as for those with idiopathic PAH in contrast to the blunted response that was characteristic of earlier short-term studies assessing improvement in 6 min walk test distance.

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