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2014 ; 115
(1
): 131-147
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Current clinical management of pulmonary arterial hypertension
#MMPMID24951763
Zamanian RT
; Kudelko KT
; Sung YK
; Perez VJ
; Liu J
; Spiekerkoetter E
Circ Res
2014[Jun]; 115
(1
): 131-147
PMID24951763
show ga
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.