Prognostic Implications of Right Atrial Dysfunction in Adults With Pulmonary Atresia and Intact Ventricular Septum

Pulmonary atresia with intact ventricular septum is associated with significant morbidity and mortality, but there are limited data to guide risk stratification in this population. The purpose of this study was to assess the role right atrial (RA) strain indices for prognostication in this population. This is a retrospective study of adults (aged ≥18 years) with pulmonary atresia with intact ventricular septum and biventricular repair who underwent echocardiogram (2003-2019). RA reservoir strain was used as the primary metric of RA function, and RA dysfunction was defined as RA reservoir strain 11); (3) incident atrial arrhythmias/heart failure hospitalization; (4) heart transplant/cardiovascular death. Of the 43 patients in the study, RA strain imaging was feasible in 95%, and RA dysfunction was present in 95%. Of the 43 patients, 67% and 49% had functional impairment and hepatorenal dysfunction, respectively; 44% developed incident atrial arrhythmia/heart failure hospitalization and 14% died during follow-up. RA reservoir strain was independently associated with all indices of clinical outcomes. Collectively, these data suggest that RA strain imaging was feasible in almost all patients and can be used for risk stratification in this population. There was a high prevalence of comorbidities including hepatorenal dysfunction. Further studies are needed to determine the prognostic implications of hepatorenal dysfunction (a previously unrecognized complication), and whether using RA function indices for clinical decision making will lead to improved outcomes in this population. L’atrésie pulmonaire à septum ventriculaire intact est associée à une morbidité et à une mortalité importantes, mais il existe peu de données pour guider la stratification des risques au sein de la population touchée. L’étude dont il est question dans notre article visait à évaluer le rôle des indices de déformation (strain) atriale droite (AD) dans la formulation d’un pronostic en présence de cette cardiopathie. Nous présentons ici une étude rétrospective portant sur un groupe d’adultes (âgés de 18 ans ou plus) atteints d’atrésie pulmonaire à septum ventriculaire intact qui avaient subi une réparation biventriculaire et une échocardiographie (2003-201