Pulmonary arterial hypertension: right ventricular phenotyping to improve risk assessment at follow-up.
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Ghio Stefano, Badagliacca Roberto, D'Alto Michele, Acquaro Mauro, Ameri Pietro, Argiento Paola, Brunetti Natale Daniele, Casu Gavino, Cedrone Nadia, Confalonieri Marco, Corda Marco, Correale Michele, D'Agostino Carlo, De Tommasi Elisabetta, Filomena Domenico, Galgano Giuseppe, Greco Alessandra, Grimaldi Massimo, Lombardi Carlo, Madonna Rosalinda, Manzi Giovanna, Mercurio Valentina, Mulè Massimiliano, Paciocco Giuseppe, Papa Silvia, Recchioni Tommaso, Romaniello Antonella, Romeo Emanuele, Scelsi Laura, Stolfo Davide, Vatrano Marco, Vitulo Patrizio, Vizza Carmine Dario, Italian Pulmonary Hypertension NETwork (IPHNET)
Аннотация
METHODS: In 11 centres of the Italian Pulmonary Hypertension NETwork (IPHNET), data were prospectively collected from patients with PAH who underwent re-evaluation between 6 and 12 months after diagnosis. Echocardiographic variables were combined a priori to define four phenotypes representing different degrees of RV dilatation and right ventricular-pulmonary arterial (RV-PA) coupling: a mildly dilated right ventricle with preserved RV-PA coupling defined phenotype-1; a mildly dilated right ventricle with poor RV-PA coupling defined phenotype-2; a severely dilated right ventricle with preserved RV-PA coupling defined phenotype-3; a severely dilated right ventricle with poor RV-PA coupling, either with or without tricuspid regurgitation of moderate degree or more, defined phenotype-4. Patients were followed up for all-cause death for a median of 3.7 years.
RESULTS: These echocardiographic phenotypes were present in all European Society of Cardiology/European Respiratory Society or REVEAL 2.0 risk groups except for the high-risk groups, which included only phenotype-3 and phenotype-4. In each risk group, RV phenotype-4 identified patients with a poorer prognosis; RV phenotype-1 identified patients with better survival in intermediate risk groups.
CONCLUSIONS: Echocardiography-derived phenotypes describing different degrees of RV remodelling and dysfunction provide prognostic information which is independent of and additional to the clinically defined risk in PAH patients at first follow-up.