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 pathological diagnosis of PCH can be subtle and easily missed. Most reported cases of PCH have been associated with resting PAH. We report the cases of 3 patients who initially presented with exertional dyspnea with normal to mildly elevated resting pulmonary arterial pressures and marked intrapulmonary shunting. In all 3 patients, invasive cardiopulmonary exercise testing was suggestive of pulmonary vascular disease. Owing to abnormalities on invasive exercise testing, lung biopsies were performed; these were diagnostic of PCH, and the patients were referred for lung transplantation. We describe unique features of these 3 cases-including novel pathological findings and the presence of intrapulmonary shunting in all 3 patients-and we discuss the role of cardiopulmonary exercise testing in the evaluation of PCH.
journal_name
Pulm Circjournal_title
Pulmonary circulationauthors
DuBrock HM,Kradin RL,Rodriguez-Lopez JM,Channick RNdoi
10.1086/682227subject
Has Abstractpub_date
2015-09-01 00:00:00pages
580-6issue
3eissn
2045-8932issn
2045-8940pii
PC2013221journal_volume
5pub_type
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
doi:10.1086/685549
更新日期:2016-06-01 00:00:00
abstract::Limited data about the long-term prognosis and response to therapy in pulmonary arterial hypertension patients with World Health Organization functional class I/II symptoms are available. PubMed and Embase were searched for publications of observational registries and randomized, controlled trials in pulmonary arteria...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894020935291
更新日期:2020-11-25 00:00:00
abstract::Scleroderma-associated pulmonary arterial hypertension (SSc-PAH) is associated with worse outcome than idiopathic pulmonary arterial hypertension (IPAH), potentially due to worse right ventricular adaptation to load as suggested by pressure-volume loop analysis. The value of non-invasive load-adaptability metrics has ...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894018788268
更新日期:2018-07-01 00:00:00
abstract::There are limited data on the management of pulmonary arterial hypertension (PAH) in the elderly; therefore, this analysis compared the safety and efficacy of tadalafil between patients ≥65 and <65 years old. This was a post hoc analysis of the randomized, double-blind, placebo-controlled phase 3 Pulmonary Arterial Hy...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/674901
更新日期:2014-03-01 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::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::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
doi:10.4103/2045-8932.109921
更新日期:2013-01-01 00:00:00
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
doi:10.1086/675994
更新日期:2014-06-01 00:00:00
abstract::[This corrects the article DOI: 10.1086/688060.]. ...
journal_title:Pulmonary circulation
pub_type: 已发布勘误
doi:10.1086/689215
更新日期:2016-12-01 00:00:00
abstract::Patients with pulmonary hypertension are more sedentary than the general population, but attitudes and experiences that may influence their exercise behaviour remain poorly understood. This study identified patterns of behaviour, attitudes towards exercise, barriers and enablers of exercise for people living with pulm...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894020968023
更新日期:2020-11-16 00:00:00
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
doi:10.1177/2045894019880086
更新日期:2020-03-18 00:00:00
abstract::PH occurs alone or in association with many disorders. Many patients with transthoracic echocardiography (TTE) findings suggesting PH never receive additional evaluation. Patient characteristics and echocardiographic data associated with increased recognition of PH have not been fully evaluated. We evaluated TTE repor...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.4103/2045-8932.113184
更新日期:2013-04-01 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::Low-grade albuminuria, determined by the urinary albumin to creatinine ratio, has been linked to systemic vascular dysfunction and is associated with cardiovascular mortality. Pulmonary arterial hypertension is related to mutations in the bone morphogenetic protein receptor type 2, pulmonary vascular dysfunction and i...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894018824564
更新日期:2019-04-01 00:00:00
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
doi:10.1086/689906
更新日期:2017-07-01 00:00:00
abstract::Endothelial nitric oxide synthase (eNOS) plays a major role in maintaining pulmonary vascular homeostasis. Tetrahydrobiopterin (BH4), an essential cofactor that stabilizes the dimerization of eNOS and balances nitric oxide (NO) and superoxide production, may have therapeutic potential in pulmonary hypertension. In the...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/677361
更新日期:2014-09-01 00:00:00
abstract::Right ventricular (RV) function is a strong independent predictor of outcome in a number of distinct cardiopulmonary diseases. The RV has a remarkable ability to sustain damage and recover function which may be related to unique anatomic, physiologic, and genetic factors that differentiate it from the left ventricle. ...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.4103/2045-8932.101407
更新日期:2012-07-01 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::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::To shed light on the efficacy of oxygen therapy in pulmonary arterial hypertension (PAH) when hypoxemia is not present, we report seven years of observational data for a female patient recorded between February 2012 and February 2019 when she was aged 7.8-14.8 years. The patient was born with an atrial septal defect (...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894019845610
更新日期:2019-04-01 00:00:00
abstract::The aim of this study was to investigate whether a dual endothelin receptor antagonist bosentan modulates the kinetics of bone marrow-derived stem cells in inhibiting the development of pulmonary hypertension. Bone marrow chimeric mice, transplanted with enhanced green fluorescent protein (eGFP)-positive bone marrow m...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894020919355
更新日期:2020-05-14 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::Andeans and Tibetans have less altitude reduction in birth weight than do shorter-resident groups, but only Tibetans are protected from pulmonary hypertension and chronic mountain sickness (CMS). We hypothesized that differences in neonatal oxygenation were involved, with arterial O2 saturation (SaO2) being highest in...
journal_title:Pulmonary circulation
pub_type: 杂志文章,评审
doi:10.1086/679719
更新日期:2015-03-01 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::Current knowledge gaps pertaining to diagnosis and management of neonatal chronic pulmonary hypertension (cPH) may result in significant variability in clinical practice. The objective of the study is to understand cPH management practices in neonatal intensive care units affiliated with the Canadian Neonatal Network ...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894020937126
更新日期:2020-07-16 00:00:00
abstract::With the advancement of computed tomography pulmonary angiography, differentiating between acute and chronic thrombus in pulmonary embolism has become more feasible. However, whether pulmonary embolism with chronic thrombus contributes to a higher mortality than pulmonary embolism with acute thrombus remains undetermi...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894020905510
更新日期:2020-05-01 00:00:00
abstract::Pulmonary arterial hypertension (PAH) is a rapidly progressive and fatal disease for which there is an ever-expanding body of genetic and related pathophysiological information on disease pathogenesis. The most common single culprit gene known is BMPR2, and animal models of the disease in several forms exist. There is...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.4103/2045-8932.87293
更新日期:2011-07-01 00:00:00
abstract::Pulmonary hypertension (PH) comprises a group of pathophysiological syndromes characterized by elevated pulmonary artery pressure and pulmonary vascular resistance, which lead to right ventricular overload, and even right heart failure. PH has a poor prognosis and severely leads to a decline in quality of life. Histor...
journal_title:Pulmonary circulation
pub_type: 杂志文章,评审
doi:10.1177/2045894020937129
更新日期:2020-07-07 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::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