Kussmaul’s sign is characterized by paradoxical increase in right atrial pressure on inspiration due to decrease in RV compliance as in pericardial diseases like chronic constrictive pericarditis, cardiac tamponade, advanced heart failure and pulmonary hypertension. In this patient’s JVP, only prominent upstroke and downstroke is against the constrictive pericarditis which is the most common condition in which Kussmaul’s sign is seen. Constrictive Pericarditis has prominent X and Y descends in contrast to only prominent Y descend here. In this patient with a history of previous history of CTEPH, Kussmaul’s sign reflects advance disease with severe RV dysfunction. In patients with pulmonary hypertension, Kussmaul’s sign is thought to result due to decreased RV compliance, however in a study in patients with severe PAH, Kussmaul’s sign was shown to reflect severe pulmonary vascular physiology and correlated independently as a poor prognostic factor.1 In a meta-analysis in patients presenting with acute myocardial infarction, Kussmaul’s sign has been found to be very specific for RV involvement and portends an increased preload requirement with intravenous fluids.2 Correctly identifying these clinical signs in a patient presenting to ED, adds in the appropriate management of the patient. This would be most appropriate in patients presenting with inferior wall MI’s where Kussmaul’s sign identifies a subset of patients with RV involvement who have a much sinister prognosis. And in heart failure population, Kussmaul’s sign is common in patients referred for heart transplantation and is associated with adverse cardiopulmonary hemodynamics.3
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