Contemporary Trends in the Diagnosis and Management of Pulmonary Arterial Hypertension

McLaughlin, Vallerie V.; Langer, Anatoly; Tan, Mary; Clements, Philip J.; Oudiz, Ronald J.; Tapson, Victor F.; Channick, Richard N.; Rubin, Lewis J.

doi:10.1378/chest.11-3060

Cited by 114 publications

(29 citation statements)

References 23 publications

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“…Subject attrition was likely a result of the limited observation period captured in our dataset, spanning a period of only three years; thus, patients who underwent RHC outside the study observation period would have been missed. Furthermore, published studies have consistently reported that many PH patients do not receive RHC as part of their diagnostic workup, despite being the diagnostic gold standard for PH [9, 29, 53]. …”

Section: Resultsmentioning

confidence: 99%

“…We have also conducted a secondary analysis of children who underwent RHC and were diagnosed with PH. However, given the limited study period, and the persistent underuse of recommended diagnostic tests in contemporary clinical practice [53–54], restricting the study population results in the exclusion of several important subtypes. Nonetheless, the ability to reproduce similar subtypes based on the RHC cohort further validates the approach.…”

Section: Discussionmentioning

confidence: 99%

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Learning a Comorbidity-Driven Taxonomy of Pediatric Pulmonary Hypertension

Ong

Mullen

Austin

et al. 2017

Circulation Research

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Rationale Pediatric pulmonary hypertension (PH) is a heterogeneous condition with varying natural history and therapeutic response. Precise classification of PH subtypes is therefore crucial for individualizing care. However, gaps remain in our understanding of the spectrum of PH in children. Objective We seek to study the manifestations of PH in children, and to assess the feasibility of applying a network-based approach to discern disease subtypes from comorbidity data recorded in longitudinal datasets. Methods and Results A retrospective cohort study comprising 6,943,263 children (<18 years of age) enrolled in a commercial health insurance plan in the US, between January 2010 and May 2013. A total of 1,583 (0.02%) children met the criteria for PH. We identified comorbidities significantly associated with PH compared with the general population of children without PH. A Bayesian comorbidity network was constructed to model the interdependencies of these comorbidities, and network clustering analysis was applied to derive disease subtypes comprising subgraphs of highly connected comorbid conditions. A total of 186 comorbidities were found to be significantly associated with PH. Network analysis of comorbidity patterns captured most of the major PH subtypes with known etiological basis defined by the World Health Organization and Panama classifications. The analysis further identified a number of subtypes documented in only a few case studies, including rare subtypes associated with several well-described genetic syndromes. Conclusions Application of network science to model comorbidity patterns recorded in longitudinal datasets can facilitate the discovery of disease subtypes. Our analysis relearned established subtypes, thus validating the approach, and identified rare subtypes that are difficult to discern through clinical observations, providing impetus for deeper investigation of the disease subtypes that will enrich current disease classifications.

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Section: Resultsmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Learning a Comorbidity-Driven Taxonomy of Pediatric Pulmonary Hypertension

Ong

Mullen

Austin

et al. 2017

Circulation Research

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“…[2] Analysis of a PH registry revealed that 43% of patients diagnosed with PAH never received a V/Q scan. [56]…”

Section: Determining Etiology Of Pulmonary Hypertensionmentioning

confidence: 99%

“…[535657] Other disadvantages include the high number of nondiagnostic or indeterminate scans and the necessity for further evaluation with cross-sectional imaging to confirm vascular and lung findings and assess the extent of anatomic disease. However, V/Q scans are advantageous in that they do not require iodine contrast and have less radiation exposure than CTPA; the estimated effective dose for thoracic imaging with V/Q is 0.6–3 mSV, whereas for 16-array or greater multi-detector CT, it is 8–20 msV.…”

Section: Determining Etiology Of Pulmonary Hypertensionmentioning

confidence: 99%

A review of imaging modalities in pulmonary hypertension

2017

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Pulmonary hypertension (PH) is defined as resting mean pulmonary artery pressure ≥25 mmHg measured by right heart catheterization. PH is a progressive, life-threatening disease with a variety of etiologies. Swift and accurate diagnosis of PH and appropriate classification in etiologic group will allow for earlier treatment and improved outcomes. A number of imaging tools are utilized in the evaluation of PH, such as chest X-ray, computed tomography (CT), ventilation/perfusion (V/Q) scan, and cardiac magnetic resonance imaging. Newer imaging tools such as dual-energy CT and single-photon emission computed tomography/computed tomography V/Q scanning have also emerged; however, their place in the diagnostic evaluation of PH remains to be determined. In general, each imaging technique provides incremental information, with varying degrees of sensitivity and specificity, which helps suspect the presence and identify the etiology of PH. The present study aims to provide a comprehensive review of the utility, advantages, and shortcomings of the imaging modalities that may be used to evaluate patients with PH.

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“…Increased systemic and pulmonary artery pressures indicated that old RBCs were more vasoactive than fresh RBCs (McLaughlin et al , 2013; Badesch et al , 2009). Pulmonary hypertension caused right ventricular dilatation and, by adversely affecting left ventricular filling, resulted in marked tachycardia to maintain cardiac output.…”

Section: Introductionmentioning

confidence: 99%

Does prolonged storage of red blood cells cause harm?

2014

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Summary Red blood cells (RBCs) degrade progressively during the weeks of refrigerated storage. No universally accepted definition of “fresh” or “old” RBCs exists. While practices vary from country to country, preservative solutions permitting shelf life as long as 7 weeks have been licensed. Transfusion of stored RBCs, particularly those at the end of the approved shelf life, has been implicated in adverse clinical outcomes. The results of observational analyses, animal models and studies in volunteers have proved provocative, controversial and contradictory. A recently completed randomized controlled trial (RCT) in premature infants exemplifies the difficulties with moderately sized clinical studies. Several other RCTs are in progress. The effect of RBC storage may well vary according to the clinical setting. Resolution of the importance of the storage lesion may require large pragmatic clinical trials. In the meantime, institutions involved in blood collection and transfusion should explore strategies that assure blood availability, while limiting the use of the oldest RBCs currently approved by regulation.

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Contemporary Trends in the Diagnosis and Management of Pulmonary Arterial Hypertension

Cited by 114 publications

References 23 publications

Learning a Comorbidity-Driven Taxonomy of Pediatric Pulmonary Hypertension

Learning a Comorbidity-Driven Taxonomy of Pediatric Pulmonary Hypertension

A review of imaging modalities in pulmonary hypertension

Does prolonged storage of red blood cells cause harm?

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