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Lindsey R. Baden, M.D., Editor

Kussmauls Sign

55-year-old woman with a remote history of Hodgkins lym- Andr M. Mansoor, M.D.
phoma treated with mantle-field radiation was admitted with subacute, pro- Sudhakar P. Karlapudi, M.B., B.S.
gressive dyspnea on exertion, which had developed over a period of 2 to
Oregon Health and Science University
3 weeks. On examination, the blood pressure was 97/59 mm Hg and the respiratory Portland, OR
rate 20 breaths per minute. Distention of the jugular veins and elevation of jugular mansooan@ohsu.edu
venous pressure during inspiration, known as Kussmauls sign, were observed (see
video). Heart sounds were normal. A pulsus paradoxus was not observed. The lungs
were clear on auscultation. A transthoracic echocardiogram showed a thickened
pericardium without pericardial effusion. Kussmauls sign is the paradoxical rise in
jugular venous pressure with inspiration. Decreased intrathoracic pressure during
inspiration normally leads to an increase in venous return to the right side of the
heart, with an associated decrease in jugular venous pressure. When there is impaired filling of the right ventricle, the jugular veins instead become engorged.
Causes of Kussmauls sign include right ventricular infarction, severe right ventricular failure, restrictive cardiomyopathy, constrictive pericarditis, and tricuspid
stenosis. It was postulated that this patient had constrictive pericarditis related to
mediastinal radiation therapy, leading to impaired filling of the heart and giving
rise to Kussmauls sign. She was treated with diuretics, which resulted in an improvement of symptoms and averted the need for more invasive therapy.
DOI: 10.1056/NEJMicm1310957
Copyright 2015 Massachusetts Medical Society.

n engl j med 372;2nejm.orgjanuary 8, 2015

The New England Journal of Medicine

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