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