Patients with systemic right ventricles (RV) are at risk for heart failure and sudden
cardiac death. Contributing factors to RV dysfunction include increased afterload
from the systemic circulation, coronary insufficiency, progressive tricuspid valve
regurgitation, the presence of residual lesions after palliation and arrhythmias.
While all patients with a systemic right ventricle (SRV) are vulnerable to heart failure,
there are distinct differences between patients with congenital dextro-transpostion
of the great arteries (d-TGA) repaired by atrial switch, unrepaired congenitally corrected
transposition of the great arteries (cc-TGA) and single systemic right ventricles
palliated with a Fontan operation. Herein, we explore both the similarities and differences
in progression of heart failure by phenotype as well as both the advancements and
limitations in treatment options by each type of SRV.
Keywords
Abbreviations:
RV (Right ventricle), LV (Left ventricle), SRV (Systemic right ventricle), ccTGA (Congenitally Corrected Transposition of the Great Arteries), dTGA (Dextrotransposition of the Great Arteries), NYHA (New York Heart Association)To read this article in full you will need to make a payment
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Article info
Footnotes
Disclosures: Consultant for W.L. Gore, Inc.
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