Abstract:
:Pregnancy is not advised for patients with Pulmonary hypertension (PH) because of high risk of PH crisis. However, some patients have undiagnosed idiopathic pulmonary arterial hypertension (IPAH) before pregnancy. Upfront combination therapy has high efficacy for patients with IPAH. However, some patients are unable to stand until upfront combination therapy has worked sufficiently. The extracorporeal membrane oxygenation (ECMO) system has been proposed as a bridging therapy to recovery for patients with IPAH. Here, we report a case where a novel form of peripheral ECMO assist plus upfront combination therapy containing intravenous epoprostenol rescued a female patient diagnosed with IPAH just after childbirth. Following this treatment, the patient could successfully transition from intravenous epoprostenol to oral selexipag.
journal_name
Pulm Circjournal_title
Pulmonary circulationauthors
Adachi S,Shimokata S,Yoshida M,Imai R,Nakano Y,Okumura N,Suzuki S,Murohara T,Kondo Tdoi
10.1177/2045894020910140subject
Has Abstractpub_date
2020-02-28 00:00:00pages
2045894020910140issue
1eissn
2045-8932issn
2045-8940pii
10.1177_2045894020910140journal_volume
10pub_type
abstract::Patients with chronic thromboembolic pulmonary hypertension (CTEPH) require lifelong anticoagulation therapy. However, the bleeding risk and recurrence of venous thromboembolism (VTE) in CTEPH patients who are administered warfarin have not been adequately evaluated. The purpose of this study was to evaluate the risk ...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045893217717258
更新日期:2017-07-01 00:00:00
abstract::The maintenance of endothelial barrier integrity is absolutely essential to prevent the vascular leak associated with pneumonia, pulmonary edema resulting from inhalation of toxins, acute elevation to high altitude, traumatic and septic lung injury, acute lung injury (ALI), and its life-threatening complication, acute...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894018773044
更新日期:2018-04-01 00:00:00
abstract::Pulmonary arterial hypertension (PAH) is a major complication of sickle cell disease (SCD). Low levels of apolipoprotein A1 (Apo-A1) have been implicated in the development of PAH in SCD. We speculate that lower levels of Apo-A1 are related to dysregulation of the ubiquitin-proteasome pathway (UPP). Of 36 recruited pa...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/674763
更新日期:2013-12-01 00:00:00
abstract::In pulmonary hypertension, as in many other diseases, there is a need for a smarter approach to evaluating new treatments. The traditional randomized controlled trial has served medical science well, but constrains the development of treatments for rare diseases. A workshop was established to consider alternative clin...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.4103/2045-8932.109933
更新日期:2013-01-01 00:00:00
abstract::The development of pulmonary arterial hypertension (PAH) in pediatric patients has been linked to the production of the arachidonic acid metabolite, thromboxane A(2) (TxA(2)). The present study evaluated the therapeutic effect of furegrelate sodium, a thromboxane synthase inhibitor, on the development of PAH in a neon...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.4103/2045-8932.97605
更新日期:2012-04-01 00:00:00
abstract::Pulmonary arterial hypertension can be associated with exposure to certain drugs or toxins. However, only a few cases of drug-induced pulmonary arterial hypertension have been previously reported. Anagrelide is an oral imidazoquinazoline agent that is prescribed for reducing elevated platelet counts in patients with m...
journal_title:Pulmonary circulation
pub_type:
doi:10.1177/2045894019896682
更新日期:2019-12-27 00:00:00
abstract::Acute respiratory distress syndrome in patients with Coronavirus disease 19 is associated with an unusually high incidence of pulmonary embolism and microthrombotic disease, with evidence for reduced fibrinolysis. We describe seven patients requiring invasive ventilation for COVID-19-associated acute respiratory distr...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894020973906
更新日期:2020-12-15 00:00:00
abstract::Carboxyl terminus of Hsc70-interacting protein (CHIP) is a 35-kDa cytoplasmic protein expressed in human striated muscle, brain, aortic smooth muscle, endothelial cells, and other tissues. Studies have confirmed that CHIP regulates cell growth, apoptosis, cell phenotype, metabolism, neurodegeneration, etc. However, wh...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894019875343
更新日期:2019-09-09 00:00:00
abstract::Pulmonary capillary hemangiomatosis (PCH) is a rare form of pulmonary arterial hypertension (PAH) characterized by pulmonary capillary proliferation and pseudoinvasion of collagenous septal structures. PCH is often accompanied by veno-occlusive changes and pulmonary hypertensive arterial remodeling. The clinical and p...
journal_title:Pulmonary circulation
pub_type:
doi:10.1086/682227
更新日期:2015-09-01 00:00:00
abstract::Endothelial dysfunction plays an important role in the pathogenesis of pulmonary arterial hypertension (PAH) in sickle cell disease (SCD). A variety of evidence suggests that circulating endothelial progenitor cells (EPCs) play an integral role in vascular repair. We hypothesized that SCD patients with PAH are deficie...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.4103/2045-8932.94834
更新日期:2012-01-01 00:00:00
abstract::In a subgroup of patients with systemic sclerosis (SSc), vasospasm affecting the pulmonary circulation may contribute to worsening respiratory symptoms, including dyspnea. Noninvasive assessment of pulmonary blood flow (PBF), utilizing inert-gas rebreathing (IGR) and dual-energy computed-tomography pulmonary angiograp...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/682221
更新日期:2015-09-01 00:00:00
abstract::Riociguat is approved for the treatment of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Some patients have difficulty swallowing tablets; therefore, 2 randomized, nonblinded, crossover studies compared the relative bioavailability of riociguat oral suspensions and immediate-releas...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/685020
更新日期:2016-03-01 00:00:00
abstract::This manuscript on endpoints incorporates the broad experience of members of Pulmonary Vascular Research Institute's Innovative Drug Development Initiative as an open debate platform for academia, the pharmaceutical industry and regulatory experts surrounding the future design of clinical trials in pulmonary hypertens...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894020962960
更新日期:2020-11-18 00:00:00
abstract::Radiotherapy as a primary treatment for thoracic malignancies induces deleterious effects, such as acute or subacute radiation-induced lung injury (RILI). Although the molecular etiology of RILI is controversial and likely multifactorial, a potentially important cellular target is the lung endothelial cytoskeleton tha...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/686491
更新日期:2016-06-01 00:00:00
abstract::Hypereosinophilic syndrome is a myeloproliferative disorder characterized by persistent eosinophilia with involvement of multiple organs. The occurrence of severe pulmonary hypertension (PH) in the setting of hypereosinophilic syndrome is very uncommon. A 43-year-old man with documented idiopathic hypereosinophlic syn...
journal_title:Pulmonary circulation
pub_type:
doi:10.1177/2045894018793999
更新日期:2019-01-01 00:00:00
abstract::Pulmonary arterial hypertension (PAH) patients have distinct disease courses and responses to treatment, but current diagnostic and treatment schemes provide limited insight. We aimed to see if cluster analysis could distinguish clinical phenotypes in PAH. An unbiased cluster analysis was performed on 17 baseline clin...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045893217705891
更新日期:2017-04-01 00:00:00
abstract::Pulmonary endarterectomy (PEA) is the standard therapy for chronic thromboembolic pulmonary hypertension (CTEPH). Balloon pulmonary angioplasty (BPA) is an alternative therapy for such patients. Here we report the case of a 60-year-old woman who presented with severe CTEPH resulting in low cardiac output and liver fai...
journal_title:Pulmonary circulation
pub_type:
doi:10.1086/675643
更新日期:2014-03-01 00:00:00
abstract::Late-onset pulmonary arterial hypertension (PAH) is a rare but fatal complication in patients with childhood surgical repair of dextro-transposition of great arteries (D-TGA), especially with the Mustard and Senning procedures. The pathogenic mechanisms of PAH in patients with repaired D-TGA are not well understood an...
journal_title:Pulmonary circulation
pub_type:
doi:10.1177/2045893217699228
更新日期:2017-04-01 00:00:00
abstract::Our aim is to assess the safety and potential efficacy of a novel treatment paradigm in pulmonary arterial hypertension (PAH), immunomodulation by blocking interleukin-6 (IL6) signaling with the IL6 receptor antagonist, tocilizumab. Inflammation and autoimmunity are established as important in PAH pathophysiology. One...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045893217735820
更新日期:2018-01-01 00:00:00
abstract::Inhaled treprostinil (Tyvaso) has been shown to be a safe and effective addition to pulmonary arterial hypertension (PAH) oral therapies; however, the respiratory-related safety profile of inhaled treprostinil required further elucidation in the setting of routine clinical care. The objectives of this study were to ch...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/688059
更新日期:2016-09-01 00:00:00
abstract::A loss of K+ efflux in pulmonary arterial smooth muscle cells (PASMCs) contributes to abnormal vasoconstriction and PASMC proliferation during pulmonary hypertension (PH). Activation of high-conductance Ca2+-activated (BK) channels represents a therapeutic strategy to restore K+ efflux to the affected PASMCs. However,...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/688838
更新日期:2016-12-01 00:00:00
abstract::Despite the discovery more than 15 years ago that patients with hereditary pulmonary arterial hypertension (HPAH) inherit BMP type 2 receptor ( BMPR2) mutations, it is still unclear how these mutations cause disease. In part, this is attributable to the rarity of HPAH and difficulty obtaining tissue samples from patie...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894018765840
更新日期:2018-04-01 00:00:00
abstract::We hypothesized that vibrations created by the pulmonary circulation would create sound like the vocal cords during speech and that subjects with pulmonary artery hypertension (PAH) might have a unique sound signature. We recorded heart sounds at the cardiac apex and the second left intercostal space (2LICS), using a ...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/683694
更新日期:2015-12-01 00:00:00
abstract::Optimal pulmonary hypertension (PH) management relies on a timely, accurate diagnosis and follow-up in specialized clinics by multidisciplinary teams that have clearly defined responsibilities and protocols. Internationally agreed criteria for expert center staff are lacking, particularly with respect to nurses, who o...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894019855611
更新日期:2019-04-01 00:00:00
abstract::This study aimed to identify receptors mediating sphingosine-1-phosphate (S1P)-induced vasoconstriction in the normotensive and chronic hypoxia-induced hypertensive rat pulmonary circulation. In isolated perfused lungs from normoxic rats, infusion of S1P caused a sustained vasoconstriction, which was not reduced by co...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.4103/2045-8932.87309
更新日期:2011-07-01 00:00:00
abstract::Pulmonary hypertension currently has minimal guidelines for outpatient disease management. Congestive heart failure studies, however, have shown effectiveness of disease management plans in reducing all-cause mortality and all-cause and congestive heart failure-related hospital readmissions. Heart failure exacerbation...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894020968471
更新日期:2020-12-07 00:00:00
abstract::Obesity is causally linked to a number of comorbidities, including cardiovascular disease, diabetes, renal dysfunction, and cancer. Obesity has also been linked to pulmonary disorders, including pulmonary arterial hypertension (PAH). It was long believed that obesity-related PAH was the result of hypoventilation and h...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/678510
更新日期:2014-12-01 00:00:00
abstract::Right ventricular failure (RVF) is the most important prognostic factor for both morbidity and mortality in pulmonary arterial hypertension (PAH), but also occurs in numerous other common diseases and conditions, including left ventricle dysfunction. RVF remains understudied compared with left ventricular failure (LVF...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045893217714463
更新日期:2017-07-01 00:00:00
abstract::The diagnosis of pulmonary vascular disease (PVD) is usually based on hemodynamic and/or clinical criteria. Noninvasive imaging of the heart and proximal vasculature can also provide useful information. An alternate approach to such criteria in the diagnosis of PVD is to image the vascular abnormalities in the lungs t...
journal_title:Pulmonary circulation
pub_type:
doi:10.1086/685110
更新日期:2016-03-01 00:00:00
abstract::Echocardiography, a non-invasive and cost-effective method for monitoring cardiac function, is commonly used for evaluation and pre-clinical diagnostics of pulmonary hypertension (PH). Previous echocardiographic studies in experimental models of PH are fragmentary in terms of the evaluation of right ventricle (RV) fun...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894019841987
更新日期:2019-04-01 00:00:00