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Review
. 2014 Jun 20;115(1):131-147.
doi: 10.1161/CIRCRESAHA.115.303827.

Current clinical management of pulmonary arterial hypertension

Roham T Zamanian #  1   2 , Kristina T Kudelko #  1   2 , Yon K Sung  1   2 , Vinicio de Jesus Perez  1   2 , Juliana Liu  1   2 , Edda Spiekerkoetter  1   2
Affiliations
Review

Current clinical management of pulmonary arterial hypertension

Roham T Zamanian et al. Circ Res. .

Abstract

During the past 2 decades, there has been a tremendous evolution in the evaluation and care of patients with pulmonary arterial hypertension (PAH). The introduction of targeted PAH therapy consisting of prostacyclin and its analogs, endothelin antagonists, phosphodiesterase-5 inhibitors, and now a soluble guanylate cyclase activator have increased therapeutic options and potentially reduced morbidity and mortality; yet, none of the current therapies have been curative. Current clinical management of PAH has become more complex given the focus on early diagnosis, an increased number of available therapeutics within each mechanistic class, and the emergence of clinically challenging scenarios such as perioperative care. Efforts to standardize the clinical care of patients with PAH have led to the formation of multidisciplinary PAH tertiary care programs that strive to offer medical care based on peer-reviewed evidence-based, and expert consensus guidelines. Furthermore, these tertiary PAH centers often support clinical and basic science research programs to gain novel insights into the pathogenesis of PAH with the goal to improve the clinical management of this devastating disease. In this article, we discuss the clinical approach and management of PAH from the perspective of a single US-based academic institution. We provide an overview of currently available clinical guidelines and offer some insight into how we approach current controversies in clinical management of certain patient subsets. We conclude with an overview of our program structure and a perspective on research and the role of a tertiary PAH center in contributing new knowledge to the field.

Keywords: pulmonary arterial hypertension.

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Figures

Figure 1
Figure 1
Updated Classification of Pulmonary Hypertension from the 5th World Symposium at Nice, France. Modifications are listed in bold. (Reproduced with permission, J Am Coll Cardiol 2013;62: D34-41).
Figure 2
Figure 2
Recommended Nice algorithm for diagnostic workup and initiation/continuation of therapies (Reproduced with permission, J Am Coll Cardiol 2013;62: D60-72).
Figure 3
Figure 3
Suggested evaluation and treatment algorithm for pulmonary hypertension in the intensive care unit (Modified with permission, Crit Care Med 2007;35:2037-50)
Figure 4
Figure 4
Schematic depicting a clinical and research collaborative model build around pulmonary hypertension academic programs.
See this image and copyright information in PMC

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