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

Publication Title

JACC Adv

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, Providence Spokane, Spokane, Washington, USA.Follow
• Craig S Broberg
• Michael Cheung

Document Type

Article

Publication Date

8-1-2026

Keywords

congenitally corrected transposition of the great arteries; pulmonary hypertension; transposition of the great arteries.; washington; spokane

Abstract

BACKGROUND: Little is known regarding pulmonary hypertension (PH) in adults with a systemic right ventricle.

OBJECTIVES: 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).

METHODS: 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.

RESULTS: 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).

CONCLUSIONS: 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.

Area of Special Interest

Cardiovascular (Heart)

Specialty/Research Institute

Cardiology

DOI

10.1016/j.jacadv.2026.102974