Constrictive-like physiology in severe pulmonary hypertension: the effect of the Valsalva maneuver.

We describe the case of a 37-year-old female with severe pulmonary hypertension on intravenous Remodulin and Tracleer who experienced presyncope following a six-minute walk. A transthoracic echocardiogram, in addition to showing the usual findings of chronic pulmonary hypertension, also demonstrated a noticeable increase in both mitral annular systolic and early diastolic (E') velocities while performing the Valsalva maneuver. Most importantly, a significant increase in the propagation velocity to an almost perpendicular tilt was noted with Valsalva, resembling the propagation velocity profile that has only been previously described in cases of constrictive pericarditis. The clinical relevance of this finding and the pathophysiologic implications are reviewed and discussed.