Stiff right atrial syndrome? A complex clinical case report utilizing multimodality imaging and invasive hemodynamics.

ABSTRACT

Background: Stiff left atrial syndrome is a well-established cause of heart failure symptoms. A parallel entity involving the right atrium (RA) has not previously been described. We present a case of refractory right heart failure (RHF) 12 years following orthotopic heart transplantation. Case summary: Patient underwent annuloplasty ring placement for severe tricuspid regurgitation in 2018 and kidney transplantation in 2020. The use of multimodality imaging and a multidisciplinary approach suggested a stiff RA as a potential etiology to refractory symptoms. Redo-heart and kidney transplantation in March 2021 led to the resolution of symptoms without recurrence. Discussion: We propose stiff right atrial syndrome that may need to be considered in the setting of refractory RHF primarily suggested by significant right atrial enlargement and restrictive physiology.