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Review
. 2019 Sep;11(Suppl 14):S1789-S1799.
doi: 10.21037/jtd.2019.08.54.

Evaluation and classification of pulmonary arterial hypertension

Affiliations
Review

Evaluation and classification of pulmonary arterial hypertension

Sandeep Sahay. J Thorac Dis. 2019 Sep.

Abstract

In early 2019, the 6th World Symposium on Pulmonary Hypertension (WSPH) released an updated document highlighting the advances in the last five years. During the quinquennial event many experts worked together to suggest new changes in the disease diagnosis and management. Since inception of the WSPH in 1973, this is the first time when the hemodynamic definition of pulmonary hypertension (PH) has been updated. These proceedings have re-defined the different hemodynamic types of PH that occur with the left heart disease along with introduction to the genetic testing as part of pulmonary arterial hypertension (PAH) evaluation. Objective of this review is to highlight the evaluation and diagnosis of PAH based on the proceedings of the 6th WSPH. Accurate early diagnosis and subsequent management of PH is necessary, as despite of treatment advances, survival remains suboptimal.

Keywords: 6th World Symposium on Pulmonary Hypertension (6th WSPH); Pulmonary hypertension (PH); chronic thromboembolic pulmonary hypertension (CTEPH); pulmonary arterial hypertension (PAH); pulmonary veno-occlusive disease (PVOD).

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Conflict of interest statement

Conflicts of Interest: The author serves on the speaker panel for Actelion pharmaceuticals, Bayer and United therapeutics; has received honoraria from these entities for consultancy and speaking engagements. Member of a data safety and monitoring board for an industry sponsored FDA approved study. The author is PI or Sub I on multiple clinical trials sponsored by Actelion and United therapeutics.

Figures

Figure 1
Figure 1
Algorithm highlighting PH evaluation at a community setting (Tables 4,5). PH, pulmonary hypertension.
Figure 2
Figure 2
Pulmonary angiogram of a patient with CTEPH showing classical lesions seen in CTEPH. CTEPH, chronic thromboembolic pulmonary hypertension.

References

    1. Galiè N, McLaughlin VV, Rubin LJ, et al. An overview of the 6th World Symposium on Pulmonary Hypertension. Eur Respir J 2019;53:1802148. 10.1183/13993003.02148-2018 - DOI - PMC - PubMed
    1. Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J 2019;53:1801913. 10.1183/13993003.01913-2018 - DOI - PMC - PubMed
    1. Benza RL, Miller DP, Barst RJ, et al. An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL registry. Chest 2012;142:448-56. 10.1378/chest.11-1460 - DOI - PubMed
    1. Zamanian RT, Hedlin H, Greuenwald P, et al. Features and outcomes of methamphetamine-associated pulmonary arterial hypertension. Am J Respir Crit Care Med 2018;197:788-800. 10.1164/rccm.201705-0943OC - DOI - PMC - PubMed
    1. Montani D, Bergot E, Günther S, et al. Pulmonary arterial hypertension in patients treated by dasatinib. Circulation 2012;125:2128-37. 10.1161/CIRCULATIONAHA.111.079921 - DOI - PubMed