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

BACKGROUND: 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. METHODS: 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 <31%. Clinical outcomes were assessed using 4 different indices: (1) functional impairment (New York Heart Association II-IV); (2) hepatorenal dysfunction (model for end-stage liver disease excluding international normalized ratio score >11); (3) incident atrial arrhythmias/heart failure hospitalization; (4) heart transplant/cardiovascular death. RESULTS: 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. CONCLUSION: 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.