Ventricularization of right atrial wave form in amyloid restrictive cardiomyopathy

Abstract

Two patients with biopsy-proven amyloid restrictive cardiomyopathy were presented. Both cases showed ventricularization of an elevated right atrial pressure wave form in absence of tricuspid regurgitation. Possible explanations for this finding as well as its clinical implications are discussed. This observation indicates that ventricularization of right atrial pressure wave form could be a useful hemodynamic sign in amyloid restrictive cardiomyopathy in absence of tricuspid regurgitation. Furthermore, such a finding does not seem to be specific for tricuspid regurgitation.

Persistent Left Superior Vena Cava Suggested by an Unusual Central Venous Pressure Waveform

“…Other causes of the ventricular pattern of the right atrial waveform include severe tricuspid regurgitation, restrictive cardiomyopathy, and the administration of retrograde cardioplegia through a coronary sinus catheter. 5,6 While generally asymptomatic and of no clinical consequences, rare clinical implications related to PLSVC include an abnormal course or malposition of a PA catheter as noted in our patient with the potential for coronary sinus perforation due to inadvertent coronary sinus cannulation. PLSVC has also been associated with an increased risk of cardiac arrhythmias especially atrial fibrillation, paradoxical embolization if there is an associated ASD or drainage into the left atrium, and dilatation of the coronary sinus leading to obstruction of the mitral valve with a predisposition to thrombus formation.…”

“…As was noted in our patient, the CVP waveform may be abnormal with a pattern described as a "ventricle pattern or ventricularization" if the opening of the CVP port is in the coronary sinus instead of the right atrium. 5,6 The abnormal pattern or ventricularization results from transmission of pressure from the left ventricle to the coronary sinus. Other causes of the ventricular pattern of the right atrial waveform include severe tricuspid regurgitation, restrictive cardiomyopathy, and the administration of retrograde cardioplegia through a coronary sinus catheter.…”

“…In a small percentage of patients, it may drain into the left atrium resulting in a right-to-left shunt with the potential for paradoxical emboli. 5,6 We present an 82-year-old man who presented for coronary artery bypass surgery and repair of an ASD. The presence of a PLSVC was suggested by an abnormal waveform on the central venous pressure (CVP) tracing and confirmed intraoperatively.…”