Why the study?
Little was known regarding pulmonary hypertension in adults with a systemic right ventricle, prompting evaluation of hemodynamic profiles in patients with TGA-AS and CCTGA.
Does TGA-AS compared to CCTGA affect the composite of death, heart transplantation, or need for mechanical circulatory support in adults with a systemic right ventricle?
Cohort (n=261)
Yes
Does TGA-AS compared to CCTGA affect the composite of death, heart transplantation, or need for mechanical circulatory support in adults with a systemic right ventricle?
Absolute Event Rate: 24% vs 29%
p-value: p=0.39
Pulmonary hypertension is common in adults with a systemic right ventricle and is associated with adverse clinical outcomes, with elevated PVR and lower aortic pulsatility index being key predictors.
Comparable outcomes in TGA-AS and CCTGA support unified risk assessment; leaves open whether pulmonary hypertension therapies alter prognosis in systemic right ventricle.
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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Wilson et al. (2026) conducted a cohort in Systemic right ventricle (TGA-AS and CCTGA) (n=261). Transposition of great arteries palliated with an atrial switch repair (TGA-AS) vs. Congenitally corrected TGA (CCTGA) was evaluated on Combined clinical endpoint of death, heart transplantation, or need for mechanical circulatory support (p=0.39).
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