scholarly journals Health Disparities and Treatment Patterns in Portopulmonary Hypertension and Idiopathic Pulmonary Arterial Hypertension: An Analysis of the Pulmonary Hypertension Association Registry

2020 ◽  
Author(s):  
H.M. DuBrock ◽  
C.D. Burger ◽  
S. Bartolome ◽  
J.P. Feldman ◽  
E.B. Rosenzweig ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Health Disparities ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Treatment Patterns ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Portopulmonary Hypertension ◽  
Pulmonary Arterial

scholarly journals EXPRESS: Health disparities and treatment approaches in portopulmonary hypertension and idiopathic pulmonary arterial hypertension: An analysis of the Pulmonary Hypertension Association Registry

2021 ◽  
pp. 204589402110209
Author(s):  
Hilary Megan DuBrock ◽  
Charles D. Burger ◽  
Sonja Bartolome ◽  
Jeremy Feldman ◽  
David D Ivy ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Health Disparities ◽  
Pulmonary Arterial Hypertension ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Portopulmonary Hypertension ◽  
Similar Treatment ◽  
Pulmonary Arterial ◽  
Ed Visits ◽  
To Receive

Compared to idiopathic pulmonary arterial hypertension (IPAH), patients with portopulmonary hypertension (POPH) have worse survival. Health disparities may contribute to these differences but have not been studied. We sought to compare socioeconomic factors in patients with POPH and IPAH and to determine whether socioeconomic status (SES) and/or POPH diagnosis were associated with treatment and healthcare utilization. We performed a cross-sectional study of adults enrolled in the Pulmonary Hypertension Association Registry. Patients with IPAH (n=344) and POPH (n=57) were compared. Compared with IPAH, patients with POPH were less likely to be college graduates (19.6 versus 34.9%, p=0.02) and more likely to be unemployed (54.7 versus 30.5%, p<0.001) and have an annual household income below poverty level (45.7 versus 19.0%, p<0.001). Patients with POPH had similar functional class, quality of life, 6MWD and mean pulmonary arterial pressure with a higher cardiac index. Compared with IPAH, patients with POPH were less likely to receive combination therapy (46.4 versus 62.2%, p=0.03) and endothelin receptor antagonists (ERAs) (28.6 versus 55.1%, p<0.001) at enrollment with similar treatment at follow-up. Patients with POPH had more emergency department (ED) visits (1.7±2.1 versus 0.9±1.2, p=0.009) and hospitalizations in the 6 months preceding enrollment (1.5±2.1 versus 0.8±1.1, p=0.02). Both POPH diagnosis and lower education level were independently associated with a higher number of ED visits. Compared to IPAH, patients with POPH have lower SES, are less likely to receive initial combination therapy and ERAs but have similar treatment at follow-up and have increased healthcare utilization.

scholarly journals Association between splenectomy and portal hypertension in the development of pulmonary hypertension

2020 ◽  
Vol 10 (1) ◽  
pp. 204589401989542
Author(s):  
Li Huang ◽  
Wen Li ◽  
Tao Yang ◽  
Changming Xiong ◽  
Xinhai Ni ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Portal Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Previous History ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Rank Test ◽  
Portopulmonary Hypertension ◽  
Distribution Width ◽  
Pulmonary Arterial

Both portal hypertension and splenectomy are risk factors for pulmonary hypertension. However, the interactions between portal hypertension and splenectomy in the development of pulmonary hypertension remain unclear. Twelve newly diagnosed pulmonary hypertension patients with a previous history of splenectomy induced by portal hypertension were recruited between November 2008 and May 2017. We compared their clinical features, hemodynamics, and prognosis with idiopathic pulmonary arterial hypertension patients, who were matched by cardiac index, mean pulmonary arterial pressure, and pulmonary vascular resistance. We also compared the clinical characteristics of portal hypertension-post-splenectomy-pulmonary hypertension patients with eight portopulmonary hypertension patients. Compared with the matched idiopathic pulmonary arterial hypertension patients, the portal hypertension-post-splenectomy-pulmonary hypertension patients showed significantly wider red blood cell distribution width (16.7 ± 2.8% versus 13.3 ± 1.7%, p = 0.004), higher total bilirubin concentration (31.0 ± 13.8 µmol/l versus 18.9 ± 10.0 µmol/l, p = 0.010), and higher lactate dehydrogenase concentration (321.5 ± 41.2 IU/l versus 229.2 ± 69.4 IU/l, p = 0.001). Kaplan–Meier survival analyses showed that the portal hypertension-post-splenectomy-pulmonary hypertension patients tended to have poorer prognosis than the matched idiopathic pulmonary arterial hypertension patients (log-rank test: p = 0.010). Compared with the portal hypertension-post-splenectomy-pulmonary hypertension patients, the portopulmonary hypertension cohort appeared to exhibit poorer clinical conditions, including significantly lower mixed venous oxygen saturation (62.9 ± 8.0% versus 73.9 ± 6.5%, p = 0.004) and a significantly higher proportion of pericardial effusion (75.0% versus 8.3%, p = 0.004), even though the two cohorts showed similar hemodynamics. The mean intervals from diagnosis of portal hypertension to pulmonary hypertension in portopulmonary hypertension patients were significantly shorter than the intervals from splenectomy to diagnosis of pulmonary hypertension in portal hypertension-post-splenectomy-pulmonary hypertension patients (5.5 ± 5.2 years versus 13.1 ± 5.9 years, p = 0.008). Splenectomy might be involved in the initiation and development of pulmonary hypertension in patients with portal hypertension, although the precise mechanisms involved remain unknown. Portal hypertension-post-splenectomy-pulmonary hypertension patients might have poorer prognosis even with mild hemodynamics.

scholarly journals Idiopathic Pulmonary Arterial Hypertension Unmasked by Pregnancy

2020 ◽  
Vol 19 (4) ◽  
pp. 240-243
Author(s):  
Adam Maxwell ◽  
◽  
Thomas Holman ◽  
Timea Novak ◽  
◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Post Partum ◽  
Right Heart Failure ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Shortness Of Breath ◽  
Acute Medical Unit ◽  
Pulmonary Angiogram ◽  
Pulmonary Arterial

A 31-year old woman presented to the acute medical unit 9 days post-partum with shortness of breath and peripheral oedema. Initially suspected to have either a pulmonary embolism or post-partum cardiomyopathy, she proceeded to have imaging including a CT Pulmonary angiogram and echocardiogram, which were suggestive of pulmonary hypertension and severe right heart failure. Her history and other investigations did not reveal any obvious cause for this. She was transferred to a specialist centre where she was diagnosed with Idiopathic Pulmonary Arterial Hypertension (IPAH), previously known as primary pulmonary hypertension. Shortness of breath during pregnancy and in the postpartum period is a relatively common acute medical presentation. Whilst IPAH is a rare diagnosis, it carries a high mortality rate, particularly in pregnancy, and requires prompt specialist investigation, diagnosis and management.

scholarly journals Acute vasoreactivity testing in pediatric idiopathic pulmonary arterial hypertension: an international survey on current practice

2019 ◽  
Vol 9 (4) ◽  
pp. 204589401985753
Author(s):  
Lina Caicedo ◽  
Rachel Hopper ◽  
Humberto Garcia Aguilar ◽  
Dunbar Ivy ◽  
Dora Haag ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Functional Class ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Clinical Criteria ◽  
First Line Therapy ◽  
Specific Protocol ◽  
The World ◽  
Pulmonary Arterial

The aim of this study was to determine practice patterns and inter-institutional variability in how acute vasoreactivity testing (AVT) is performed and interpreted in pediatrics throughout the world. A survey was offered to physicians affiliated with the Pediatric & Congenital Heart Disease Taskforce of the Pulmonary Vascular Research Institute (PVRI), the Pediatric Pulmonary Hypertension Network (PPHNET), or the Spanish Registry for Pediatric Pulmonary Hypertension (REHIPED), from February to December 2016. The survey requested data about the site-specific protocol for AVT and subsequent management of pediatric patients with idiopathic pulmonary arterial hypertension (IPAH) or heritable PAH (HPAH). Twenty-eight centers from 13 countries answered the survey. AVT is performed in most centers using inhaled nitric oxide (iNO). Sitbon criteria was used in 39% of the centers, Barst criteria in 43%, and other criteria in 18%. First-line therapy for positive AVT responders in functional class (FC) I/II was calcium channel blocker (CCB) in 89%, but only in 68% as monotherapy. Most centers (71%) re-evaluated AVT-positive patients hemodynamics after 6–12 months; 29% of centers re-evaluated based only on clinical criteria. Most centers (64%) considered a good response as remaining in FC I or II, with near normalization of pulmonary arterial pressure and pulmonary vascular resistance, but a stable FC I/II alone was sufficient criteria in 25% of sites. Protocols and diagnostic criteria for AVT, and therapeutic approaches during follow-up, were highly variable across the world. Reported clinical practice is not fully congruent with current guidelines, suggesting the need for additional studies that better define the prognostic value of AVT for pediatric IPAH patients.

scholarly journals Genomewide RNA expression profiling in lung identifies distinct signatures in idiopathic pulmonary arterial hypertension and secondary pulmonary hypertension

2010 ◽  
Vol 298 (4) ◽  
pp. H1235-H1248 ◽  
Author(s):  
Revathi Rajkumar ◽  
Kazuhisa Konishi ◽  
Thomas J. Richards ◽  
David C. Ishizawar ◽  
Andrew C. Wiechert ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Estrogen Receptor ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Rna Expression ◽  
Data Set ◽  
Platelet Factor ◽  
Protein Ubiquitination ◽  
Pulmonary Arterial

Idiopathic pulmonary arterial hypertension (PAH) is a life-threatening condition characterized by pulmonary arteriolar remodeling. This investigation aimed to identify genes involved specifically in the pathogenesis of PAH and not other forms of pulmonary hypertension (PH). Using genomewide microarray analysis, we generated the largest data set to date of RNA expression profiles from lung tissue specimens from 1) 18 PAH subjects and 2) 8 subjects with PH secondary to idiopathic pulmonary fibrosis (IPF) and 3) 13 normal subjects. A molecular signature of 4,734 genes discriminated among these three cohorts. We identified significant novel biological changes that were likely to contribute to the pathogenesis of PAH, including regulation of actin-based motility, protein ubiquitination, and cAMP, transforming growth factor-β, MAPK, estrogen receptor, nitric oxide, and PDGF signaling. Bone morphogenic protein receptor type II expression was downregulated, even in subjects without a mutation in this gene. Women with PAH had higher expression levels of estrogen receptor 1 than normal women. Real-time quantitative PCR confirmed differential expression of the following genes in PAH relative to both normal controls and PH secondary to IPF: a disintegrin-like and metalloprotease with thrombospondin type 1 motif 9, cell adhesion molecule with homology to L1CAM, cytochrome b558and β-polypeptide, coagulation factor II receptor-like 3, A-myb myeloblastosis viral oncogene homolog 1, nuclear receptor coactivator 2, purinergic receptor P2Y, platelet factor 4, phospholamban, and tropomodulin 3. This study shows that PAH and PH secondary to IPF are characterized by distinct gene expression signatures, implying distinct pathophysiological mechanisms.

scholarly journals Circulating Plasma Metabolomic Profiles Differentiate Rodent Models of Pulmonary Hypertension and Idiopathic Pulmonary Arterial Hypertension Patients

2019 ◽  
Vol 32 (11) ◽  
pp. 1109-1117
Author(s):  
Jun-Han Zhao ◽  
Yang-Yang He ◽  
Shan-Shan Guo ◽  
Yi Yan ◽  
Zhe Wang ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Progressive Disease ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Rodent Models ◽  
Preclinical Models ◽  
Metabolic Dysregulation ◽  
Pulmonary Arterial ◽  
Significant Pathway

Abstract BACKGROUND Pulmonary arterial hypertension (PAH) is a severe progressive disease with systemic metabolic dysregulation. Monocrotaline (MCT)-induced and hypoxia-induced pulmonary hypertension (PH) rodent models are the most widely used preclinical models, however, whether or not these preclinical models recapitulate metabolomic profiles of PAH patients remain unclear. METHODS In this study, a targeted metabolomics panel of 126 small molecule metabolites was conducted. We applied it to the plasma of the 2 preclinical rodent models of PH and 30 idiopathic pulmonary arterial hypertension (IPAH) patients as well as 30 healthy controls to comparatively assess the metabolomic profiles of PAH patients and rodent models. RESULTS Significantly different metabolomics profiling and pathways were shown among the 2 classical rodent models and IPAH patients. Pathway analysis demonstrated that methionine metabolism and urea cycle metabolism were the most significant pathway involved in the pathogenesis of hypoxia-induced PH model and MCT-induced model, respectively, and both of them were also observed in the dysregulated pathways in IPAH patients. CONCLUSIONS These 2 models may develop PAH through different metabolomic pathways and each of the 2 classical PH model resembles IPAH patients in certain aspects.

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