Circulation Journal
Online ISSN : 1347-4820
Print ISSN : 1346-9843
ISSN-L : 1346-9843
Reviews
Assessment of Operability of Patients With Pulmonary Arterial Hypertension Associated With Congenital Heart Disease
– Do We Have the Good Tools to Predict Success? –
Patrick O. MyersCécile TissotMaurice Beghetti
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2014 Volume 78 Issue 1 Pages 4-11

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Abstract

Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease, and is now predominantly among patients with uncorrected left-to-right shunts. A growing population is characterized by persistent or recurrent PAH after surgical or interventional correction of left-to-right shunts; the latter having a worse prognosis than other forms of PAH associated with congenital heart disease. New treatments for PAH have been shown to be effective in improving PAH exercise capacity and hemodynamics, raising the hope for making previously inoperable congenital heart defects operable and shifting the framework for the assessment of operability. This review focuses on current methods for assessing operability in PAH associated with congenital heart disease, and the possibility of “treat-and-repair” vs. “repair-and-treat” strategies for patients with inoperable or borderline PAH.  (Circ J 2014; 78: 4–11)

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© 2014 THE JAPANESE CIRCULATION SOCIETY
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