Kussmaul's sign is a paradoxical rise in
jugular venous pressure
The jugular venous pressure (JVP, sometimes referred to as ''jugular venous pulse'') is the indirectly observed pressure over the venous system via visualization of the internal jugular vein. It can be useful in the differentiation of different f ...
(JVP) on inspiration, or a failure in the appropriate fall of the JVP with inspiration. It can be seen in some forms of
heart disease
Cardiovascular disease (CVD) is any disease involving the heart or blood vessels. CVDs constitute a class of diseases that includes: coronary artery diseases (e.g. angina pectoris, angina, myocardial infarction, heart attack), heart failure, ...
and is usually indicative of limited right ventricular filling due to right heart dysfunction.
Pathophysiology
Ordinarily the JVP falls with inspiration due to reduced pressure in the expanding
thoracic cavity
The thoracic cavity (or chest cavity) is the chamber of the body of vertebrates that is protected by the thoracic wall (rib cage and associated skin, muscle, and fascia). The central compartment of the thoracic cavity is the mediastinum. Ther ...
and the increased volume afforded to right ventricular expansion during diastole. Kussmaul sign suggests impaired filling of the
right ventricle
A ventricle is one of two large chambers located toward the bottom of the heart that collect and expel blood towards the peripheral beds within the body and lungs. The blood pumped by a ventricle is supplied by an atrium (heart), atrium, an adjace ...
due to a poorly compliant myocardium or pericardium. This impaired filling causes the increased blood flow to back up into the venous system, causing the jugular vein distention (JVD) and is seen clinically in the internal jugular veins becoming more readily visible.
Causes
The differential diagnoses of Kussmaul's sign includes
constrictive pericarditis
Constrictive pericarditis is a condition characterized by a thickened, fibrotic pericardium, limiting the heart's ability to function normally. In many cases, the condition continues to be difficult to diagnose and therefore benefits from a good ...
,
restrictive cardiomyopathy
Restrictive cardiomyopathy (RCM) is a form of cardiomyopathy in which the walls of the heart are rigid (but not thickened). Thus the heart is restricted from stretching and filling with blood properly. It is the least common of the three original s ...
,
pericardial effusion
A pericardial effusion is an abnormal accumulation of fluid in the pericardial cavity. The pericardium is a two-part membrane surrounding the heart: the outer fibrous Connective tissue, connective membrane and an inner two-layered serous membrane ...
, and severe
right-sided heart failure.
With
cardiac tamponade
Cardiac tamponade, also known as pericardial tamponade (), is a compression of the heart due to pericardial effusion (the build-up of pericardial fluid in the pericardium, sac around the heart). Onset may be rapid or gradual. Symptoms typically i ...
, jugular veins are distended and typically show a prominent x descent and an absent y descent as opposed to patients with constrictive pericarditis (prominent x and y descent); see
Beck's triad.
Other possible causes of Kussmaul's sign include:
* Right
ventricular infarction
Infarction is tissue death (necrosis) due to Ischemia, inadequate blood supply to the affected area. It may be caused by Thrombosis, artery blockages, rupture, mechanical compression, or vasoconstriction. The resulting lesion is referred to as a ...
- low ventricular compliance
*
Cardiac tumours
*
Tricuspid stenosis
Tricuspid valve stenosis is a valvular heart disease that narrows the opening of the heart's tricuspid valve. It is a relatively rare condition that causes stenosis (increased restriction of blood flow through the valve). Cause
Causes of tricuspi ...
*
Pulmonary embolism
Pulmonary embolism (PE) is a blockage of an pulmonary artery, artery in the lungs by a substance that has moved from elsewhere in the body through the bloodstream (embolism). Symptoms of a PE may include dyspnea, shortness of breath, chest pain ...
History
Kussmaul's sign is named after the
German
German(s) may refer to:
* Germany, the country of the Germans and German things
**Germania (Roman era)
* Germans, citizens of Germany, people of German ancestry, or native speakers of the German language
** For citizenship in Germany, see also Ge ...
doctor who first described it,
Adolph Kussmaul (1822–1902). He is also credited with describing
Kussmaul breathing
Kussmaul breathing is a deep and labored breathing pattern often associated with severe metabolic acidosis, particularly diabetic ketoacidosis (DKA) but also kidney failure. It is a form of hyperventilation, which is any breathing pattern that ...
.
See also
*
Pulsus paradoxus
References
{{Cardiovascular system symptoms and signs
Symptoms and signs: Vascular