Pulmonary Hypertension in Adults With a Systemic Right Ventricle (Biventricular Circulation).

Authors

William M Wilson, Nael Aldweib, David S Celermajer, Alexander R Opotowsky, Payam Dehghani, Jolien Roos-Hesselink, Mikael Dellborg, David Baker, Jamil Aboulhosn, Petra Antonova, Susan Fernandes, Salil Ginde, Frank Han, Clare O'Donnell, Carla P Rodriguez-Monserrate, Flavia Fusco, Anitha S John, Joshua Wong, Elizabeth Yeung, Berardo Sarubbi, Stephen Pylypchuk, Sangeeta Shah, Joseph Kay, Jonathan Cramer, Timothy Cotts, Tripti Gupta, Luke J Burchill, Paul Khairy, Isabelle Vonder Muhll, Alexandra van Dissel, Jasmine Grewal, Anthony Magalski, Pastora Gallego, Fred Rodriguez, Marissa Kuo, Robert M Kauling, Christopher DeZorzi, Eric V Krieger, Shelby Kutty, Jeremy Nicolarsen, Craig S Broberg, Michael Cheung

Published in

JACC. Advances. Volume 5. Issue 8. Pages 102974. Jul 02, 2026. Epub Jul 02, 2026.

Abstract

Little is known regarding pulmonary hypertension (PH) in adults with a systemic right ventricle.
This study evaluates the hemodynamic profile of PH in patients with transposition of great arteries palliated with an atrial switch repair (TGA-AS) and congenitally corrected TGA (CCTGA).
This was a retrospective cohort study of adults with TGA-AS or CCTGA who had undergone invasive hemodynamic assessment. Exclusion criteria were single ventricle anatomy, previous Rastelli, arterial switch, or double switch operation. PH was defined by a mean pulmonary artery pressure (mPAP) >20 mm Hg and subtypes according to 2022 European Society of Cardiology guidelines. The primary combined clinical endpoint was death, heart transplantation, or need for mechanical circulatory support.
A total of 261 patients were studied (TGA-AS, n = 161 and CCTGA, n = 100). PH prevalence was similar in both groups (65% vs 69%, P = 0.74). PH subtype was precapillary in 24%, isolated postcapillary (IpcPH) 25%, combined postcapillary and precapillary 51% (similar in both groups). The relationship between pulmonary capillary wedge pressure and mPAP was overall similar between 2 groups but there was greater variability in pulmonary capillary wedge pressure once mPAP >40 mm Hg in the TGA-AS group. The incidence of the primary endpoint was similar in both groups (24 v 29%; P = 0.39). Factors associated with the primary clinical endpoint were elevated PVR (in particular, in the TGA-AS subgroup) and lower aortic pulsatility index (both groups).
In this large study evaluating the hemodynamic phenotype in patients with a systemic RV referred for catheterization, PH was common and most commonly the combined postcapillary and precapillary subtype.

PMID:
42391650
Bibliographic data and abstract were imported from PubMed on 03 Jul 2026.

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