Related Experiment Video
Updated: Jul 4, 2026
10:03
Evaluation of Right Ventricular Function in Experimental Models of Pulmonary Arterial Hypertension
Published on: June 27, 2025
Pulmonary Hypertension in Adults With a Systemic Right Ventricle (Biventricular Circulation)
William M Wilson1, Nael Aldweib2, David S Celermajer3
1Department of Medicine, Royal Melbourne Hospital, Melbourne, Australia.
JACC. Advances
|July 2, 2026
Summary
Pulmonary hypertension (PH) is common in adults with a systemic right ventricle, often presenting as a combined postcapillary and precapillary subtype. This finding is crucial for understanding and managing PH in these complex cardiac conditions.
Area of Science:
- Cardiology
- Pulmonary Hypertension
- Congenital Heart Disease
Background:
- Pulmonary hypertension (PH) in adults with a systemic right ventricle is not well understood.
- Systemic right ventricle physiology presents unique challenges in diagnosing and managing PH.
Purpose of the Study:
- To evaluate the hemodynamic profile of PH in patients with transposition of great arteries palliated with atrial switch repair (TGA-AS) and congenitally corrected transposition of great arteries (CCTGA).
- To assess the prevalence and subtypes of PH in these specific adult congenital heart disease populations.
Main Methods:
- Retrospective cohort study of 261 adults with TGA-AS or CCTGA undergoing invasive hemodynamic assessment.
- PH defined as mean pulmonary artery pressure (mPAP) >20 mm Hg, with subtypes classified per 2022 ESC guidelines.
- Primary endpoint: death, heart transplantation, or need for mechanical circulatory support.
Main Results:
- PH prevalence was high and similar in both groups (65% TGA-AS vs. 69% CCTGA).
- The most common PH subtype was combined postcapillary and precapillary (51%), followed by isolated postcapillary (25%) and precapillary (24%).
- Elevated pulmonary vascular resistance (PVR) and lower aortic pulsatility index were associated with adverse clinical outcomes.
Conclusions:
- PH is a frequent finding in adults with a systemic right ventricle, regardless of TGA-AS or CCTGA.
- The combined postcapillary and precapillary PH subtype is most prevalent in this population.
- Hemodynamic assessment is critical for characterizing PH and identifying risk factors for adverse events.
Keywords:
congenitally corrected transposition of the great arteriespulmonary hypertensiontransposition of the great arteriesMore Related Videos
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