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

Eur Heart J Case Rep. 2024 Apr 29;8(4):ytae163. doi: 10.1093/ehjcr/ytae163. eCollection 2024 Apr.

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.

Keywords: Case report; Heart transplantation; Restrictive physiology; Right heart failure; Stiff right atrial syndrome.

Publication types

  • Case Reports