Kussmaul’s Sign in Right Ventricular Dysfunction
A 78-year-old woman had a mitral valve replacement for severe mitral stenosis. At the time, her tricuspid valve was also found to be severely regurgitant, but it appeared that the valve was structurally normal and that the problem was caused by right heart dysfunction secondary to her mitral valve disease. She also had a pacing implantable cardioverter-defibrillator for aborted sudden cardiac death on a background of slow atrial fibrillation.
Ten years later, the patient returned with symptoms and signs of right heart failure, which had gradually worsened over a period of several months. Echocardiography revealed normal pulmonary artery pressures and evidence of severe central tricuspid regurgitation (Figure 1); this was attributed to worsening right ventricular function.
The photographs (and the video from which they are taken) were obtained on admission to hospital. Cardiac catheterization was performed some days later, after aggressively induced diuresis.
The hemodynamic traces (Figure 2) depict tricuspid regurgitation seen at the level of the right atrium. This is mirrored by the cannon V wave seen in the internal jugular vein, as shown in Figure 3 and the Data Supplement Movie. There was mild pulmonary hypertension with a mean pulmonary artery pressure of 37 mm Hg and no significant rise with exertion.
The inspiratory increase in jugular venous pressure amplitude—Kussmaul’s sign—was first described in 18731 and is clearly evident in the Movie. Although commonly ascribed to pericardial constriction, it is also a marker of intrinsic right ventricular diastolic dysfunction. The fact that Kussmaul’s sign was not demonstrated at the time of cardiac catheterization is a salutary lesson that signs of hemodynamically significant cardiac pathology can be masked by relative hypovolemia.
The online-only Data Supplement, consisting of a movie, can be found at http://circ.ahajournals.org/cgi/content/full/115/4/e49 /DC1.