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

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.

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Journal
JACC Advances
Published
2026-07-02
DOI
https://doi.org/10.1016/j.jacadv.2026.102974
Primary Topic
Pulmonary Hypertension Research and Treatments
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article
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article

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

WILLIAM M. WILSON, Eric V. Krieger, Pastora Gallego, Craig S. Broberg et al.
JACC Advances
Pulmonary Hypertension Research and Treatments
article

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

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

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.

JACC AdvancesVol. 5(8)
Children's Hospital of Wisconsin (US), Brigham and Women's Hospital (US), Cincinnati Children's Hospital Medical Center (US), Boston Children's Hospital (US), Seattle Children's Hospital (US), The University of Sydney (AU), The Royal Melbourne Hospital (AU), Royal Children's Hospital (AU), Mayo Clinic (US), Children's National (US), Johns Hopkins University (US), University of Alberta (CA), Oregon Health & Science University (US), Palo Alto University (US), University of Michigan (US), Royal Prince Alfred Hospital (AU), Ochsner Medical Center (US), Montreal Heart Institute (CA), Emory University Hospital (US), UC San Diego Health System (US), UCLA Medical Center (US), University of Washington Medical Center (US), Erasmus MC (NL), Jackson Memorial Hospital (US), University of Mississippi Medical Center (US), University Hospital in Motol (CZ), Regina General Hospital (CA), Stanford Medicine (US), Saint Luke's Hospital (US), Ospedale Monaldi (IT), St. Paul's Hospital (CA), Children's Hospital & Medical Center (US), Amsterdam University Medical Centers (NL), Hospital Universitario Virgen del Rocío (ES), University of Gothenburg (SE), University of Colorado Denver (US), Stanford University (US)
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Openalex Percentile: Top 8%
Pulmonary Hypertension Research and Treatments
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