Abnormalities in hyperpolarized (129)Xe magnetic resonance imaging and spectroscopy in two patients with pulmonary vascular disease.


: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 themselves. Hyperpolarized (HP) (129)Xe magnetic resonance imaging (MRI) is a novel technique for assessing abnormalities in ventilation and gas exchange in the lungs. We applied this technique to two patients for whom there was clinical suspicion of PVD. Two patients who had significant hypoxemia and dyspnea with no significant abnormalities on computed tomography imaging or ventilation-perfusion scan and only mild or borderline pulmonary arterial hypertension at catheterization were evaluated. They underwent HP (129)Xe imaging and subsequently had tissue diagnosis obtained from lung pathology. In both patients, HP (129)Xe imaging demonstrated normal ventilation but markedly decreased gas transfer to red blood cells with focal defects on imaging, a pattern distinct from those previously described for idiopathic pulmonary fibrosis or obstructive lung disease. Pathology on both patients later demonstrated severe PVD. These findings suggest that HP (129)Xe MRI may be useful in the diagnosis of PVD and monitoring response to therapy. Further studies are required to determine its sensitivity and specificity in these settings.


Pulm Circ


Pulmonary circulation


Dahhan T,Kaushik SS,He M,Mammarappallil JG,Tapson VF,McAdams HP,Sporn TA,Driehuys B,Rajagopal S




Has Abstract


2016-03-01 00:00:00














  • Comparative effectiveness of oral prostacyclin pathway drugs on hospitalization in patients with pulmonary hypertension in the United States: a retrospective database analysis.

    abstract::Two oral medications targeting the prostacyclin pathway are available to treat pulmonary arterial hypertension in the United States: oral treprostinil and selexipag. We compared real-world hospitalization in patients receiving these medications. A retrospective administrative claims study was conducted using the Optum...

    journal_title:Pulmonary circulation

    pub_type: 杂志文章


    authors: McConnell JW,Tsang Y,Pruett J,Iii WD

    更新日期:2020-11-10 00:00:00

  • BMPR2 mutations and endothelial dysfunction in pulmonary arterial hypertension (2017 Grover Conference Series).

    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


    authors: Frump A,Prewitt A,de Caestecker MP

    更新日期:2018-04-01 00:00:00

  • Particulate matter disrupts human lung endothelial cell barrier integrity via Rho-dependent pathways.

    abstract::Increased exposure to ambient particulate matter (PM) is associated with elevated morbidity and mortality in patients with cardiopulmonary diseases and cancer. We and others have shown that PM induces lung microvascular barrier dysfunction which potentially enhances the systemic toxicity of PM. However, the mechanisms...

    journal_title:Pulmonary circulation

    pub_type: 杂志文章


    authors: Wang T,Shimizu Y,Wu X,Kelly GT,Xu X,Wang L,Qian Z,Chen Y,Garcia JGN

    更新日期:2017-07-01 00:00:00

  • Mechanical forces stimulate endothelial microparticle generation via caspase-dependent apoptosis-independent mechanism.

    abstract::Microparticle release by vascular endothelium has been implicated in various cardiovascular pathologies. Ventilator-induced lung injury (VILI) is a life-threatening complication of mechanical ventilation at high tidal volumes associated with excessive mechanical stretch of pulmonary vascular endothelial cells. However...

    journal_title:Pulmonary circulation

    pub_type: 杂志文章


    authors: Vion AC,Birukova AA,Boulanger CM,Birukov KG

    更新日期:2013-01-01 00:00:00

  • Impact of four times daily dosing of oral treprostinil on tolerability and daily dose achieved in pulmonary hypertension.

    abstract::Oral treprostinil (TRE) is a prostacylin that is approved for the treatment of patients with pulmonary arterial hypertension (PAH). Dosing is approved for two or three times daily (t.i.d.); however, adverse effects, including gastrointestinal-related symptoms, may limit the ability to reach optimal doses. We report ou...

    journal_title:Pulmonary circulation



    authors: Coons JC,Bunner C,Ishizawar DC,Risbano MG,Rivera-Lebron B,Mathier MA,Chan SY,Simon MA

    更新日期:2018-01-01 00:00:00

  • Molecular and functional significance of Ca(2+)-activated Cl(-) channels in pulmonary arterial smooth muscle.

    abstract::Increased peripheral resistance of small distal pulmonary arteries is a hallmark signature of pulmonary hypertension (PH) and is believed to be the consequence of enhanced vasoconstriction to agonists, thickening of the arterial wall due to remodeling, and increased thrombosis. The elevation in arterial tone in PH is ...

    journal_title:Pulmonary circulation

    pub_type: 杂志文章,评审


    authors: Leblanc N,Forrest AS,Ayon RJ,Wiwchar M,Angermann JE,Pritchard HA,Singer CA,Valencik ML,Britton F,Greenwood IA

    更新日期:2015-06-01 00:00:00

  • Furegrelate, a thromboxane synthase inhibitor, blunts the development of pulmonary arterial hypertension in neonatal piglets.

    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


    authors: Hirenallur-S DK,Detweiler ND,Haworth ST,Leming JT,Gordon JB,Rusch NJ

    更新日期:2012-04-01 00:00:00

  • Transforming growth factor-β inhibition and endothelin receptor blockade in rats with monocrotaline-induced pulmonary hypertension.

    abstract::Transforming growth factor-β (TGF-β) inhibition is an investigational therapy for pulmonary arterial hypertension with promising results in experimental studies. The present work compared this approach with endothelin-receptor blockade and evaluated the effects of combined administration. Pulmonary arterial hypertensi...

    journal_title:Pulmonary circulation

    pub_type: 杂志文章


    authors: Megalou AJ,Glava C,Vilaeti AD,Oikonomidis DL,Baltogiannis GG,Papalois A,Vlahos AP,Kolettis TM

    更新日期:2012-10-01 00:00:00

  • Dysregulation of ubiquitin-proteasome pathway and apolipoprotein A metabolism in sickle cell disease-related pulmonary arterial hypertension.

    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


    authors: Anjum F,Lazar J,Soh J,Albitar M,Gowda S,Hussain MM,Wadgaonkar R

    更新日期:2013-12-01 00:00:00

  • Medication adherence, hospitalization, and healthcare resource utilization and costs in patients with pulmonary arterial hypertension treated with endothelin receptor antagonists or phosphodiesterase type-5 inhibitors.

    abstract::Adherence to therapy for pulmonary arterial hypertension is essential to optimize patient outcomes, but data on real-world adherence to different pulmonary arterial hypertension drug classes are limited. This retrospective database analysis evaluated relationships between adherence, hospitalization, and healthcare cos...

    journal_title:Pulmonary circulation

    pub_type: 杂志文章


    authors: Frantz RP,Hill JW,Lickert CA,Wade RL,Cole MR,Tsang Y,Drake W 3rd

    更新日期:2020-03-18 00:00:00

  • The anticoagulant effects of warfarin and the bleeding risk associated with its use in patients with chronic thromboembolic pulmonary hypertension at a specialist center in Japan: a retrospective cohort study.

    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


    authors: Jujo-Sanada T,Tanabe N,Sakao S,Sugiura T,Sekine A,Nishimura R,Suda R,Naito A,Miwa H,Yamamoto K,Sasaki A,Matsumura A,Ema R,Kasai H,Kato F,Tatsumi K

    更新日期:2017-07-01 00:00:00

  • Left main bronchus compression due to main pulmonary artery dilatation in pulmonary hypertension: two case reports.

    abstract::Pulmonary arterial dilatation associated with pulmonary hypertension may result in significant compression of local structures. Left main coronary artery and left recurrent laryngeal nerve compression have been described. Tracheobronchial compression from pulmonary arterial dilatation is rare in adults, and there are ...

    journal_title:Pulmonary circulation



    authors: Jaijee SK,Ariff B,Howard L,O'Regan DP,Gin-Sing W,Davies R,Gibbs JS

    更新日期:2015-12-01 00:00:00

  • Pulmonary vasospasm in systemic sclerosis: noninvasive techniques for detection.

    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


    authors: Keir GJ,Nair A,Giannarou S,Yang GZ,Oldershaw P,Wort SJ,MacDonald P,Hansell DM,Wells AU

    更新日期:2015-09-01 00:00:00

  • Advanced risk stratification of intermediate risk group in pulmonary arterial hypertension.

    abstract::In 301 treatment-naïve patients with pulmonary arterial hypertension stratified by the European Society of Cardiology/European Respiratory Society risk score, further stratification of intermediate-risk patients based on six-minute walk distance and the tricuspid annular plane systolic excursion/systolic pulmonary art...

    journal_title:Pulmonary circulation

    pub_type: 杂志文章


    authors: Yogeswaran A,Richter MJ,Sommer N,Ghofrani HA,Seeger W,Tello K,Gall H

    更新日期:2020-10-07 00:00:00

  • Congenital heart disease and pulmonary arterial hypertension in South America (2013 Grover Conference series).

    abstract::South America is a territory of 17,819,100 km(2), where ∼388 million people live in 13 countries. In the region, access to medical assistance (e.g., for treatment of cardiovascular disorders) is relatively easy in metropolitan areas but difficult in remote places such as the Andes and the Amazon. Altitudes up to ∼6,70...

    journal_title:Pulmonary circulation

    pub_type: 杂志文章,评审


    authors: Lopes AA,Flores PC,Diaz GF,Mesquita SM

    更新日期:2014-09-01 00:00:00

  • Dynamic right ventricular-pulmonary arterial uncoupling during maximum incremental exercise in exercise pulmonary hypertension and pulmonary arterial hypertension.

    abstract::Despite recent advances, the prognosis of pulmonary hypertension (PH) remains poor. While the initial insult in PH implicates the pulmonary vasculature, the functional state, exercise capacity, and survival of such patients are closely linked to right ventricular (RV) function. In the current study, we sought to inves...

    journal_title:Pulmonary circulation

    pub_type: 杂志文章


    authors: Singh I,Rahaghi FN,Naeije R,Oliveira RKF,Vanderpool RR,Waxman AB,Systrom DM

    更新日期:2019-07-01 00:00:00

  • Endothelial nitric oxide synthase genotype is associated with pulmonary hypertension severity in left heart failure patients.

    abstract::The biological mechanisms behind the development of pulmonary hypertension in the setting of left heart failure (HF-PH), including combined pre- and post-capillary pulmonary hypertension (Cpc-PH), remains unclear. This study aimed to use candidate polymorphisms in nitric oxide synthase (NOS) genes to explore the role ...

    journal_title:Pulmonary circulation

    pub_type: 杂志文章


    authors: Duarte JD,Kansal M,Desai AA,Riden K,Arwood MJ,Yacob AA,Stamos TD,Cavallari LH,Zamanian RT,Shah SJ,Machado RF

    更新日期:2018-04-01 00:00:00

  • Nonmuscle myosin light chain kinase activity modulates radiation-induced lung injury.

    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


    authors: Wang T,Mathew B,Wu X,Shimizu Y,Rizzo AN,Dudek SM,Weichselbaum RR,Jacobson JR,Hecker L,Garcia JG

    更新日期:2016-06-01 00:00:00

  • BK channels in rat and human pulmonary smooth muscle cells are BKα-β1 functional complexes lacking the oxygen-sensitive stress axis regulated exon insert.

    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


    authors: Detweiler ND,Song L,McClenahan SJ,Versluis RJ,Kharade SV,Kurten RC,Rhee SW,Rusch NJ

    更新日期:2016-12-01 00:00:00

  • Relationship of left ventricular outflow tract velocity time integral to treatment strategy in submassive and massive pulmonary embolism.

    abstract::Pulmonary embolism is associated with high rates of mortality and morbidity. It is important to understand direct comparisons of current interventions to differentiate favorable outcomes and complications. The objective of this study was to compare ultrasound-accelerated thrombolysis versus systemic thrombolysis versu...

    journal_title:Pulmonary circulation

    pub_type: 杂志文章


    authors: Antoine D,Chuich T,Mylvaganam R,Malaisrie C,Freed B,Cuttica M,Schimmel D Jr

    更新日期:2020-09-28 00:00:00

  • Chemical and biological assessment of metal organic frameworks (MOFs) in pulmonary cells and in an acute in vivo model: relevance to pulmonary arterial hypertension therapy.

    abstract::Pulmonary arterial hypertension (PAH) is a progressive and debilitating condition. Despite promoting vasodilation, current drugs have a therapeutic window within which they are limited by systemic side effects. Nanomedicine uses nanoparticles to improve drug delivery and/or reduce side effects. We hypothesize that thi...

    journal_title:Pulmonary circulation

    pub_type: 杂志文章


    authors: Mohamed NA,Davies RP,Lickiss PD,Ahmetaj-Shala B,Reed DM,Gashaw HH,Saleem H,Freeman GR,George PM,Wort SJ,Morales-Cano D,Barreira B,Tetley TD,Chester AH,Yacoub MH,Kirkby NS,Moreno L,Mitchell JA

    更新日期:2017-07-01 00:00:00

  • Adverse physiologic effects of Western diet on right ventricular structure and function: role of lipid accumulation and metabolic therapy.

    abstract::Little is known about the impact of metabolic syndrome (MS) on right ventricular (RV) structure and function. We hypothesized that mice fed a Western diet (WD) would develop RV lipid accumulation and impaired RV function, which would be ameliorated with metformin. Male C57/Bl6 mice were fed a WD or standard rodent die...

    journal_title:Pulmonary circulation

    pub_type: 杂志文章


    authors: Brittain EL,Talati M,Fortune N,Agrawal V,Meoli DF,West J,Hemnes AR

    更新日期:2019-01-01 00:00:00

  • An observational study of inhaled-treprostinil respiratory-related safety in patients with pulmonary arterial hypertension.

    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


    authors: Zamanian RT,Levine DJ,Bourge RC,De Souza SA,Rosenzweig EB,Alnuaimat H,Burger C,Mathai SC,Leedom N,DeAngelis K,Lim A,De Marco T

    更新日期:2016-09-01 00:00:00

  • Echocardiographic parameters of right ventricular function predict mortality in acute respiratory distress syndrome: a pilot study.

    abstract::Right ventricular (RV) dysfunction in acute respiratory distress syndrome (ARDS) contributes to increased mortality. Our aim is to identify reproducible transthoracic echocardiography (TTE) parameters of RV dysfunction that can be used to predict outcomes in ARDS. We performed a retrospective single-center cohort pilo...

    journal_title:Pulmonary circulation

    pub_type: 杂志文章


    authors: Shah TG,Wadia SK,Kovach J,Fogg L,Tandon R

    更新日期:2016-06-01 00:00:00

  • Pulmonary capillary hemangiomatosis: the role of invasive cardiopulmonary exercise testing.

    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



    authors: DuBrock HM,Kradin RL,Rodriguez-Lopez JM,Channick RN

    更新日期:2015-09-01 00:00:00

  • Erratum.

    abstract::[This corrects the article DOI: 10.1086/688060.]. ...

    journal_title:Pulmonary circulation

    pub_type: 已发布勘误



    更新日期:2016-12-01 00:00:00

  • Impact of diabetes on survival and right ventricular compensation in pulmonary arterial hypertension.

    abstract::Insulin resistance is highly prevalent in pulmonary arterial hypertension (PAH) patients. However, the long-term impact of diabetes mellitus (DM) on survival in PAH is unclear. Insulin resistance and DM are associated with left ventricular steatosis and dysfunction, but whether the right ventricle (RV) might be affect...

    journal_title:Pulmonary circulation

    pub_type: 杂志文章


    authors: Benson L,Brittain EL,Pugh ME,Austin ED,Fox K,Wheeler L,Robbins IM,Hemnes AR

    更新日期:2014-06-01 00:00:00

  • Switching inhaled iloprost formulations in patients with pulmonary arterial hypertension: the VENTASWITCH Trial.

    abstract::Inhaled iloprost is an effective therapy for patients with pulmonary arterial hypertension (PAH); however, some patients experience extended inhalation times when using the V10 formulation (10.0 µg/mL) to deliver a 5 -µg dose (at mouthpiece) and are at risk of incomplete inhalations and reduced inhalation frequency. V...

    journal_title:Pulmonary circulation

    pub_type: 杂志文章


    authors: Richter MJ,Stollfuß B,Roitenberg A,Kleinjung F,Graeff V,Berghaus S,Müller C,Ghofrani HA

    更新日期:2018-10-01 00:00:00

  • Late-onset pulmonary arterial hypertension in repaired D-transposition of great arteries: an uncommon complication.

    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



    authors: Baskaran J,March KS,Thenappan T

    更新日期:2017-04-01 00:00:00

  • Atrial arrhythmias in chronic lung disease-associated pulmonary hypertension.

    abstract::Atrial arrhythmias are common during episodes of acute respiratory failure in patients with chronic lung disease-associated pulmonary hypertension. Expert opinion suggests that management of atrial arrhythmias in patients with pulmonary hypertension should aim to restore sinus rhythm. This is clinically challenging in...

    journal_title:Pulmonary circulation

    pub_type: 杂志文章,评审


    authors: Vahdatpour CA,Luebbert JJ,Palevsky HI

    更新日期:2020-03-09 00:00:00