CHRONIC CONSTRICTIVE PERICARDITIS

CHRONIC CONSTRICTIVE PERICARDITISDr. NABAJYOTI HAZARIKACTVS DEPTT. (GMCH)

1

DEFINITION

Chronic constrictive pericarditis is a chronic inflammatory process that involves both fibrous and serous layers of the pericardium, and that leads to pericardial thickening and compression (constriction) of the ventricles. The resultant impairment in diastolic filling reduces cardiac function.

This disorder results when the healing of an acute fibrinous or serofibrinous pericarditis or the resorption of a chronic pericardial effusion is followed by obliteration of the pericardial cavity with the formation of granulation tissue, which latter gradually contracts and forms a firm scar, which may be calcified, encasing the heart and interfering with filling of the ventricles.Pericardial fluid is generally absent or of normal volume.Thickened wall of pericardial sac, may be 3-20mm thick, versus 1-2 mm thickness for normal pericardium.

CAUSES Idiopathic (most common in developed nations) Irradiation Postsurgical Infectious (most common in developing nations) Neoplastic Autoimmune (connective tissue) disorders Uremia Post-trauma Sarcoid Methysergide therapy Implantable defibrillator patches

The end result is dense fibrosis, often calcification, and adhesions of the parietal and visceral pericardium. Scarring is usually more or less symmetric and impedes filling of all heart chambers. In a subset of patients, the process develops relatively rapidly and is reversible. This variant is seen most commonly after cardiac surgery.

PATHOPHYSIOLOGYThe pathophysiologic consequence of pericardial scarring is markedly restricted filling of the heart.[This results in elevation and equilibration of filling pressures in all chambers and the systemic and pulmonary veins.Impairs cardiac filling only in late diastole.Early diastolic filling of RV occurs breifly in constrictive pericarditis untill the ventricle suddenly reaches the rigid constraint of the pericardium, producing the square root sign in the LV and RV diastolic filling pressure waveforms.CVP tracing has a prominent y descent that corresponds to the initial dip of the square root sign. This y descent results from exaggerated diastolic collapse of the normal venous pressure as rapid atrial filling occurs.

Systemic venous congestion results in hepatic congestion, peripheral edema, ascites and sometimes anasarca, and cardiac cirrhosis. Reduced cardiac output is a consequence of impaired ventricular filling and causes fatigue, muscle wasting, and weight loss. In absolute constriction, contractile function is preserved, although ejection fraction can be reduced because of reduced preload. The myocardium is occasionally involved in the chronic inflammation and fibrosis, leading to true contractile dysfunction that can at times be quite severe and predicts a poor response to pericardiectomy.

Representation of transvalvular and central venous flow velocities in constrictive pericarditis. During inspiration, the decrease in left ventricular filling results in a leftward septal shift, allowing augmented flow into the right ventricle. The opposite occurs during expiration

CLINICAL PRESENTATIONThe usual presentation consists of signs and symptoms of right-sided heart failure. At a relatively early stage, these include lower extremity edema, vague abdominal complaints, and passive hepatic congestion.Later on hepatic congestion worsens and can progress to ascites, anasarca, and jaundice due to cardiac cirrhosis.Elevated pulmonary venous pressures, leading to exertional dyspnea, cough, and orthopnea, may also appear with progressive disease.

Atrial fibrillation and tricuspid regurgitationIn end-stage constrictive pericarditis, the effects of a chronically low cardiac output are prominent, including severe fatigue, muscle wasting, and cachexia.Other findings include recurrent pleural effusions and syncope.Can be mistaken for any cause of right-sided heart failure as well as end-stage primary hepatic disease.

PHYSICAL EXAMINATIONMarkedly elevated jugular venous pressure with a prominent, rapidly collapsing y descent.Kussmaul sign.In cases with extensive calcification and adhesion of the heart to adjacent structures, the position of the cardiac point of maximal impulse may fail to change with changes in body position.Pericardial knock in around 50% cases.Widening of second heart sound splitting.Prominent S3 gallop may be present.Secondary tricuspid regurgitation with its characteristic murmur.Abdominal examination reveals hepatomegaly, often with palpable venous pulsations, with or without ascites. Other signs of hepatic congestion or cardiac cirrhosis include jaundice, spider angiomas, and palmar erythema.Lower extremity edema.

Inspiratory increase in right atrial pressure(Kussmauls sign)

"A" wave:atrial contraction (ABSENT in atrial fibrillation)

"C" wave: ventricular contraction (tricuspid bulges).

"X" descent:atrial relaxation

"V" wave:atrial venous filling (occurs at same of time of ventricular contraction)

"Y" descent:ventricular filling (tricuspid opens)

CHEST X-RAYShows. pericardial calcification

ECG with low voltage

ECHOCARDIOGRAPHYPericardial thickening,Abrupt displacement of the interventricular septum during early diastole (septal bounce).Premature pulmonic valve opening as a result of elevated right ventricular early diastolic pressure may also be observed. Exaggerated septal shifting during respiration is often present.Transesophageal echocardiography is superior to transthoracic echocardiography for measuring pericardial thickness and has an excellent correlation with CT.

CT SCANCT showing dialated SVC relative to AA and DA

CT with thickened pericardium

CARDIAC CATHETERIZATIONCVP greater than 14 mm hgNear equalization of CVP, PA diastolic pressure, and capillary wedge pressures.Decreased cardiac output.square root sign in RV and LV pressure tracingsProminent y descentAccentuated by 500ml volume change

Right sided pressure tracings showing near equalization of pressures

Simultaneous RV and LV tracings with early diastolic pressure dip (characteristic square root sign)

CONSTRICTIONRESTRICTIONProminent y descent in venous pressurePresentVariableParadoxical pulse1/3rd of casesAbsentPericardial knockPresentAbsentEqual right- and left-sided filling pressuresPresentLeft at least 3-5mmHg > rightFilling pressures >25mmHgRareCommonPulmonary artery systolic pressure >60mmHgNoCommonSquare root signPresentVariableRespiratory variation in left- and right-sided pressures or flowsExaggeratedNormalVentricular wall thicknessNormalUsually increasedAtrial sizePossible left atrial enlargementBiatrial enlargementSeptal bouncePresentAbsentTissue Doppler E velocityIncreasedReducedPericardial thicknessIncreasedNormal

MANAGEMENTWith the exception of patients with transient constriction, surgical pericardiectomy is the definitive treatment.Patients with major comorbidities or severe debilitation and radiation-induced disease are relatively contraindicated for pericardiectomy.Medical management with diuretics and salt restriction. Because sinus tachycardia is a compensatory mechanism, beta-adrenergic blockers and calcium antagonists that slow the heart rate should be avoided. In patients with atrial fibrillation and a rapid ventricular response, digoxin is recommended as initial treatment to slow the ventricular rate before resorting to beta blockers or calcium antagonists.

PERICARDIECTOMYUsually done through a median sternotomy.Possible through bilateral thoracotomies, and left anterolateral thoracotomy has been used to achieve effective pericardiectomy.For sternotomy a midline incision is made from just inferior to the suprasternal notch to the inferior extent of the xiphoid process.Sternum is divided with a sternal saw and opened with a sternal retractor.Relatively thin area of anterior pericardium is incised and a plane between the epicardium and the pericardium is established using sharp dissection.Pericardium is opened in the midline from the level of the diaphragm to the base of the great vessels.

LV should be freed first, to avoid RV distension.Ant. Pericardium is freed 1-2 cm anterior to both phrenic nerves.Dense epicardial scar can be removed with grid pattern of crossing lines 1-2 cm apart.Once the pericardium is freed from the epicardium, it is excised using low electrocautery to achieve hemostasis.Anterior pericardiectomy described above provides maximal hemodynamic benefit in relieving right ventricular constriction and usually can be performed without cardiopulmonary bypass. Resection of the diaphragmatic pericardium, atrial pericardium and posterior pericardiectomy remains unclear benefit.

Intraoperative findings of pericardial thickening.

OUTCOMEPericardiectomy provides excellent symptomatic improvement, with 85% of late survivors being NYHA class I and the remainder being class II.Operative mortality 14%, primarily as a result of low cardiac output in 70% of deaths.The operative mortality rate varies with NYHA class- 1% for class I-II 10% for class III 46% for class IV

TUBERCULOUS CONSTRICTIVE PERICARDITISPericardiectomy should be performed early and as radically as possible.A combination of chemotherapy and surgery yields gratifying results.Tuberculous pericarditis affects 1% to 2% of all patients with TBC by direct extension from the mediastinal lymph nodes and, occasionally, by hematogenous spread or by contiguous spread from the myocardium. Triple-drug antituberculous therapy is to be administered for a minimum of 9 months.In patients in whom pericardial effusion persists or recurs despite the use of anti-TBC drugs, 3 months of corticosteroid therapy may be a useful adjunct.

APPENDIX

Kussmaul's Sign

Kussmauls Sign In Effusive Constrictive Pericarditis

THANK YOU

107

Sara K. Johnson; Ramo K. Naidu; Ryan C. Ostopowicz; David R. Kumar;Satya Bhupathi, MD, MPH; Joseph J Mazza and Steven H. Yale, MD

Adolf Kussmaul: Distinguished Clinician and Medical Pioneer

History of Medicine

Clinical Medicine & ResearchVolume 7, Number 3: 107-1122009 Marshfield Clinic clinmedres.org

Increased jugular venous pressure with inspiration is commonly referred to as Kussmauls sign; and the disappearance of the radial pulse or a drop in systolic blood pressure of 10 mmHg or greater with inspiration is recognized as pulsus paradoxus. Both Kussmauls sign and pulsus paradoxus are commonly attributed to the discoveries of Dr. Adolf Kussmaul. Together these two clinical signs are important assessors of pericardial or mediastinal disease (table 1).

On February 22, 1822, Adolf Kussmaul (figure 1) was born in the farming community of Graben, Germany.1 Exposed to medicine at an early age, he accompanied his father, a physician, on house calls and autopsies. At the age of 18, Kussmaul began studying medicine at the University of Heidelberg where he received his doctorate of medicine.2 After graduation in 1845, the University of Heidelberg appointed Dr. Kussmaul to Professor of Medicine.3 His intellectual curiosity led him to study and write about many topics including aphasia, ophthalmologic anatomy, gastric lavage, obstetrics, and polyarteritis nodosa.2 Among his numerous contributions to the medical field, Kussmauls sign is considered one of his more significant discoveries and is still used today by astute clinicians, providing important information that frequently helps in directing further investigation.

Kussmauls SignIn his 1873 paper entitled Ueber Schwielge Mediastino-pericarditis und den Paradoxen Puls (Concerning Callous Mediastinopericarditis and the Paradoxical Pulse), Kussmaul described the phenomenon, originally discovered by Greisinger in 1854, in which the jugular veins became considerably swollen and by each inspiration, a slight increase of its contents could be noted.3 Defined by a visualized increase in jugular venous pressure or pulse with inspiration, this occurrence, contrary to the normal physiological event in which jugular venous pressure decreases with inspiration, can be validated with intravascular hemodynamic monitoring and quantitative measurements of pressures in the jugular venous system.

Clinicians use one of two options to determine the appearance of Kussmauls sign. The more invasive method measures central venous pressure. Central venous pressure monitoring approximates right arterial pressure, which rises during inspiration reflecting impaired venous return to the right heart. The more commonly preferred, non-invasive method to assess right ventricular filling pressure evaluates the jugular venous pressure with a simple flashlight and ruler. First, the clinician positions the patient at a 45-degree angle. Then shines light on the internal jugular vein at an oblique angle and then extends the flashlight

Keywords:Constrictive pericarditisJugular venous pressureKussmaul respirationComaKussmauls signPericardial effusionPericardial tamponadePulsus paradoxus

Reprint Requests:Steven H. Yale, MDDepartment of Internal MedicineMarshfield Clinic1000 North Oak AvenueMarshfield, WI 54449Tel: 715-387-9110Fax: 715-389-3808Email: yale.steven@mcrf.mfldclin.edu

Received: January 31, 2009Accepted: March 4, 2009

doi: 10.3121/cmr.2009.850

CM&R 2009 : 3 (September)108 Famous Names and Medical Eponyms

horizontally from the highest point of jugular venous pressure pulsations. The increased jugular venous pressure compared to pre-inspiration is consistent with the presence of Kussmauls sign.

Physiologically, in healthy individuals, pleural pressure approximates pericardial and mediastinal pressure. Inspiration creates negative intrapleural (intrathoracic) pressure, and enhances the pressure gradient and translocation of blood volume between the positive abdominal pressure and negative intrathoracic pressure within the thorax and superior vena cavae, increasing right ventricular pressure and volume, and decreasing right atrial pressure. Furthermore, the increase in negative intrathoracic pressure causes decreased left-atrial and left ventricular filling from the pulmonary venous system due to increased pulmonary pooling of blood volume which in turn causes a slight drop in systolic blood pressure.

The pathophysiological mechanisms responsible for Kussmauls sign can be explained by conditions which cause right ventricular dysfunction, impair right ventricular filling, and raise atrial pressure (figure 2).The inability for cardiac

chambers to expand due to (1) hypoelasticity or inelasticity of the myocardium caused by conditions such as infection and fibrosis (restrictive cardiomyopathy) (2) mechanical compartmentalization by constrictive pericardial diseases (constrictive pericarditis), or (3) impaired right ventricular function resulting from right-sided myocardial infarction, impede effective right ventricular filling and cause a paradoxical increase in jugular venous pressure during inspiration.4 Thus, Kussmauls sign is seen in conditions that restrict right ventricular filling such as constrictive pericarditis, right-sided heart failure, right-sided heart infarction, tricuspid stenosis and massive pulmonary embolism. Therefore, conditions that raise right atrial and venous pressure are a prerequisite to cause Kussmauls sign.

The presence of Kussmauls sign in patients with constrictive pericarditis and/or restrictive cardiomyopathy and not cardiac tamponade can be accounted for by the physiological differences in filling patterns and thus provides a physician with useful bedside information for diagnostic decision making. Kussmauls sign is not seen in patients with cardiac tamponade because even though the increase in pericardial pressure exerts an inward force compressing the entire heart during inspiration, the increase in negative intrathoracic pressure is still able to be transmitted to the right side of the heart and subsequent increase in blood flow to the right atrium ensues.5 Conversely, the restriction to diastolic filling of the right ventricle in constrictive pericarditis and restrictive cardiomyopathy by the fixed, less compliant constricting pericardium or myocardium respectively at higher chamber volumes, results in the paradoxical increase in jugular venous pressure referred to as Kussmauls sign.6

Effusive-constrictive pericarditis, a rare condition in which features of constriction by the visceral pericardium occurs concomitantly with pericardial effusion. Signs of constrictive pericarditis become unmasked after pericardiocentesis is performed to drain the exudative effusion. Another distinguishing feature of effusive-constrictive pericarditis is the presence of high right atrial pressure which persists after pericaridocentesis.7

Kussmaul Respiration and Coma In 1874, Dr. Kussmaul detailed physical examination findings he had observed in comatose diabetic patients:

A peculiar type of dyspnea...there is not the least suggestion...that the passage of air to or from the lung has to combat obstruction...everything is

Table 1. Signs commonly associated with Kussmaul.

Constrictive pericarditis Restrictive cardiomyopathy Pericardial tamponade

Kussmauls sign Yes Yes No

Pulsus paradoxus Yes Yes Yes

Figure 1. Adolf Kussmaul (1822-1902). Photo courtesy of the US National Library of Medicine.

CM&R 2009 : 3 (September) 109Johnson et al.

indicative of air hunger...even when the patients lies unconscious in deep coma (as translated in reference 2, pg 113 and 114).

The coma that may develop due to diabetic ketoacidosis is sometimes referred to as Kussmauls coma, and the abnormal respiratory pattern described is called Kussmaul breathing. The definition of Kussmaul breathing varies dependent on the source. Murray and Nadel8 define Kussmaul breathing as a pattern of hyperventilation with deep sighing breaths. Kussmaul breathing may be present in any form of metabolic acidosis. However, in less severe cases of metabolic acidosis, this pattern of breathing may not be detectable. The air hunger2 described by Kussmaul represents a compensatory increase in minute ventilation to expel carbon dioxide and increase the pH. This compensation occurs rapidly after the decrease in pH with the maximal response expected within 12 to 24 hours post-metabolic acidosis. On an interesting note, there is evidence that this respiratory compensation is not beneficial in chronic metabolic acidosis, as the change in pH towards normal results in less renal excretion of acid over time.9

Polyarteritis NodosaWorking alongside pathologist Rodolf Maier in 1866, Kussmauls description of what he termed periarteritis [polyarteritis] nodosa carried a greater significance than either of them could have envisioned.1 The lasting impact of this event on medicine is a result of a methodology employed by Kussmaul that was light-years ahead of its time, and would become a model for future physicians and researchers. Understanding and defining disease through laboratory testing coupled with complete clinical evaluation, including patient history and physical, became the standard for future clinicians.10 With the use of the microscope, Maier and Kussmaul were the first to analyze the histological characteristics of a disease and combine them with the clinical description to arrive at an appropriate diagnosis.10 While Viennese pathologist Carl von Rokitansky (1804-1878) published the first case of polyarteritis nodosa, his narrow focus on the histopathology

prevented him from fully understanding the true nature of the disease.10

Pulsus ParadoxusIn the same 1873 manuscript in which he describes Kussmauls sign, Kussmaul writes of another phenomenon seen in patients with constrictive pericarditis (table 2):

...this affliction results in the symptoms associated with chronic infection of the pericardium, as well as...a pronounced arterial pulse phenomenon...the pulsations of all arteries become smaller or disappear completely...and return with expiration...I propose to call this pulse action paradoxical (as translated in an editorial in reference 1).

Figure 2. Pathophysiology of Kussmauls Sign.

Table 2. Causes of pulsus paradoxus.

Cardiac causes

Cardiac tamponade

Pericardial effusion

Constrictive pericarditis

Pulmonary embolism

Cardiogenic shock

Restrictive cardiomyopathy

Acute myocardial infraction

Extracardiac pulmonary causes

Bronchial asthma

Tension pneumothorax

Extracardiac non-pulmonary causes

Extreme obesity

Superior vena caval obstruction

Volvolus of stomach

Diaphragmatic hernia

Anaphylactic shock

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Thus, the paradox Kussmaul describes is that the peripheral pulse disappears while the central heartbeat is still present. Formally defined as a decrease in systolic blood pressure of more than 10 mmHg during inspiration, pulsus paradoxus is an exaggeration of the cardiovascular physiological response to arterial pressure with normal inspiration. The paradox Kussmaul refers to is twofold: (1) a strongly beating heart with weak or absent peripheral pulses, and (2) pulses that seem irregular but actually follow a pattern.

This sign can be tested by placing a sphygmomanometer over the arm and deflating the cuff slowly. The systolic pressure at which the first Korotkoff sounds can be heard during expiration should be recorded. The cuff is then deflated (1 mm per heartbeat) while listening for uniformly loud Korotkoff sounds in both expiration and inspiration. The systolic blood pressure at which Korotkoff sounds can be heard during both phases of respiration should be recorded. The second recorded systolic pressure should be subtracted from the first, and if this value is >10 mmHg, pulsus paradoxus is considered to be present. Palpation of peripheral pulses, especially radial pulses, for decreased force during inspiration, as Kussmaul initially did, is a less formal evaluation for pulsus paradoxus.11 If a patient has an arterial catheter in place, pulsus paradoxus can be detected on the arterial waveform, and this method is the most sensitive for detecting pulsus paradoxus.11 Studying pulse-oximetry waveforms for a reduced plethysmographic peak with inspiration is another noninvasive method for evaluating for the presence of pulsus paradoxus.12,13

Alteration in the normal physiologic mechanisms that govern ventricular filling provides an explanation of the pathophysiology of pulsus paradoxus. During inspiration there is a decrease in intrathoracic pressure accompanied by an increase in the venous flow to the right ventricle. The increased right ventricular volume and pressure from increased venous return causes the interventricular septum to be displaced into the left ventricle reducing its size and volume thereby compromising ventricular filling and compliance.14 This phenomenon is referred to as ventricular interdependence.

Additionally, the increased negative intrathoracic pressure (1) enhances the pressure gradient (afterload) between the heart and the systemic arterial and venous system and (2) increases pulmonary blood flow, volume, and pooling of blood resulting in decreased left ventricular filling and stroke volume. The final consequence is a decrease in arterial pressure and pulse during inspiration.15,16

Pulsus paradoxus thus occurs as a result of conditions that exaggerate the basic physiological mechanism that occurs during inspiration (figure 3). The following pathologic events operate either alone or concurrently to cause pulsus paradoxus: (1) larger increases in venous blood flow to the right ventricle, (2) greater than normal pooling of blood in the pulmonary venous circulation, (3) decreased pressure gradient between the pulmonary veins and left atrium, and (4) wide deviations in the intrathoracic pressure during inspiration.6,14 Apart from being an indicator of pericardial tamponade, increasing pulsus paradoxus magnitude has also been shown to correlate with decreased stroke volume and cardiac output. Therefore, pulsus paradoxus may be used clinically to follow the status of patients with pericardial tamponade.17

Sensitivity and Specificity of Pulsus Paradoxus Pulsus paradoxus is often seen in patients with pericardial tamponade because ventricular interdependence is often exaggerated in such cases. Additionally, since there is no impedance towards venous flow to the right ventricle upon inspiration, adequate flow exists in order for the interventricular septum to be displaced.5 Pulsus paradoxus shows a sensitivity of 79% for predicting pericardial tamponade, but its specificity is quite low.17 However, in another study, 98% of patients with pericardial tamponade had pulsus paradoxus.18 This discrepancy may be explained by the fact that pulsus paradoxus is less likely to be seen in cardiac tamponade when certain comorbidities are present (eg, ventricular hypertrophy, heart failure, severe aortic regurgitation, large atrial septal defect, cardiac adhesions, severe hypotension, and acute left ventricular myocardial infarction).11 These certain conditions mask the presence of pulsus paradoxus because they counteract

Figure 3. Pathophysiology of Pulsus Paradoxus.

CM&R 2009 : 3 (September) 111Johnson et al.

the mechanisms responsible for pulsus paradoxus by either decreasing right-sided filling of the heart, increasing filling of the left atrium during diastole, or causing a more subtle shift of the interventricular septum.14 Echocardiography showing evidence of right ventricular collapse has been more sensitive (93%) and much more specific (100%) than pulsus paradoxus for diagnosing tamponade.17

Constrictive Pericarditis and Restrictive CardiomyopathyWhen it comes to differentiating between constrictive pericarditis and other cardiac restrictive disorders, such as restrictive cardiomyopathy, the challenge lies in the hands of the physician. A lack of available clinical resources or tools at the bedside to aid in making the diagnosis and differentiating between the two is greatly enhanced by diagnostic imaging studies, eg, magnetic resonance imaging (MRI) and computerized tomography (CT), and echocardiograms. A late S3 and in some cases S4 during diastole and a mitral or tricuspid regurgitation murmur, are subtle signs which may be present in restrictive cardiomyopathy. A pericardial knock or diastolic sound caused by rapid inflow of blood followed by an abrupt cessation of flow may be heard on auscultation in patients with constrictive pericarditis. Management and outcomes are critically dependent on defining the correct diagnosis, since patients with constrictive pericarditis can be cured by pericardectomy, resulting in significantly better outcomes than those seen in patients with restrictive cardiomyopathy.19

In one study by Ling et al , only 19% of patients with constrictive pericarditis were found to have pulsus paradoxus.20 However, in an older series of patients with constrictive pericarditis, 44% of patients had pulsus paradoxus by the modern definition.21 Patients with severe constrictive pericarditis are more likely to display a decrease in pulses with inspiration and have a positive Kussmaul sign.22 Pulsus paradoxus is more commonly seen in the syndrome of effusive-constrictive pericarditis, in which tamponade and pericardial constriction coexist and in one 66% of such patients displayed pulsus paradoxus.7 More importantly, pulsus paradoxus is usually absent in patients with more advanced restrictive cardiomyopathy as a result of a non-compliant septum that compromises ventricular interdependence.23 However, it may be seen in patients with constrictive pericarditis because of the interventricular septal displacement towards the left ventricle during diastole, thus impeding left ventricular filling.

Gastroenterology, Esophagogastroscopy, and the First OphthalmoscopeIn an article published in 1869, Kussmaul described the treatment of gastric outlet obstruction with associated dilation by means of a stomach pump.10 The successful use and treatment of a patient with gastric ectasia through such methods was described, with the patients symptoms subsided after only a few days. While the stomach pump was used by others at the time, Kussmaul played a key role in popularizing the device.1 In another example of Kussmauls sagacity; he

addressed the possibility of treating more complex cases of gastric outlet obstruction:

...whether perhaps bolder species of a distant future will attempt, in such cases through gastrotomy, creation of a gastric fistula and dilatation of the stricture with a knife or probe, to achieve radical successes...who dares today to decide this question? One must fear being softly or loudly ridiculed for just posing it (as translated in reference 10).

In 1881, Christian Billroth (1829-1894) acted upon this suggestion and attempted what was considered at that time an almost impossible feat; a full gastric resection in order to treat a gastric outlet obstruction.24 Also described within the article are the first references towards performing esophageal and gastric endoscopy using a rigid instrument.10

In a letter written in 1899 to one of his former assistants, Kussmaul described that he had incorporated the use of an endoscope into his clinical practice, visualizing a proximal esophageal carcinoma.10 After he introduced the idea and demonstrated the benefits of using a gastroscope, subsequent physicians such as Rudolf Schindler and Basil Hirschowitz expanded on the ideas set forth by Kussmaul and advanced the field of endoscopy to where it is today.10

Even though Kussmaul was unsuccessful in creating a successful final product, he is credited with being the first person to recognize the need for the ophthalmoscope. Kussmaul began his work on creating a tool that could view the interior of the eye at the age of 23, at which time he was a medical student in Heidelberg, Germany.25 In 1845, he wrote a dissertation entitled Die Farbenerscheinungen im Grunde des menschlichen Auges (An Important Description of Colour Phenomena in the Fundus Oculi), which contained the blueprint for construction of an ophthalmoscope.25 Despite a problem with the fundamental optics of the device, which would later be corrected by Hermann von Helmholtz, Kussmaul undoubtedbly played an integral role in the creation of the ophthalmoscope and provided physicians with a tool that enabled them to better understand and treat diseases associated with the interior of the eye.

In the early morning of May 28, 1902, Dr. Kussmaul succumbed to his bout with coronary sclerosis, leaving behind his many contributions to medical science.2 In his 80 years of life, Adolf Kussmaul made discoveries seemingly beyond the capabilities of one man. He wrote prolifically in diverse fields (for example, cardiology, rheumatology, endocrinology, gastroenterology, psychiatry, and neurology). Dr. Kussmaul serves as a model for medical professionals and students exemplifying that there are no limits to exploration. Adolf Kussmaul predicated his achievements on the foundations of science: observation, hypothesis, experimentation and analysis, as will future discoverers in the medical sciences.

CM&R 2009 : 3 (September)112 Famous Names and Medical Eponyms

References1. Adolf Kussmaul (1822-1902)--Country doctor to clinical

professor. JAMA 1964;189:58-59.2. Bast TH. The life and time of Adolf Kussmaul. New York, NY:

Paul B. Hoeber; 1926.3. Bilchick KC, Wise RA. Paradoxical physical findings described

by Kussmaul: pulsus paradoxus and Kussmauls sign. Lancet 2002;359:1940-1942.

4. Meyer TE, Sareli P, Marcus RH, Pocock W, Berk MR, McGregor M. Mechanism underlying Kussmauls sign in chronic constrictive pericarditis. Am J Cardiol 1989;64:1069-1072.

5. Roy, CL, Minor, MA, Brookhart, MA, Choudhry, NK. Does This Patient With a Pericardial Effusion Have Cardiac Tamponade? JAMA 2007;297:1810-1818.

6. Myers, RBH, Spodick, DH. Constrictive pericarditis: Clinical and pathophysiologic characteristics. Am Heart J 1999;138: 219-232.

7. Sagrist-Sauleda J, Angel J, Snchez A, Permanyer-Miralda G, Soler-Soler J. Effusive-constrictive pericarditis. N Engl J Med 2004;350:469-475.

8. Murray JF, Nadel JA, Mason RJ, Boushey HA Jr. Textbook of respiratory medicine. 3rd ed. Philadelphia, PA: W.B. Saunders; 2000.

9. Madias NE, Schwartz WB, Cohen JJ. The maladaptive renal response to secondary hypocapnia during chronic HCl acidosis in the dog. J Clin Invest 1977;60:1393-1401.

10. Matteson, EL, Kluge, FJ. Think clearly, be sincere, act calmly: Adolf Kussmaul (February 11, 1822-May 28, 1902) and his relevance to medicine in the 21st century. Curr Opin Rheumatol 2003;15:29-34.

11. Swami A, Spodick DH. Pulsus paradoxus in cardiac tamponade: a pathophysiologic continuum. Clin Cardiol 2003;26: 215-217.

12. Chadwick V, Pearce S, Taylor B, Galland BC. Continuous non-invasive assessment of pulsus paradoxus. Lancet 1992;339:495-496.

13. Tamburro RF, Ring JC, Womback K. Detection of pulsus paradoxus associated with large pericardial effusions in pediatric patients by analysis of the pulse-oximetry waveform. Pediatrics 2002;109:673-677.

14. Khasnis, A, Lokhandwala, Y. Clinical signs in medicine: pulsus paradoxus. J Postgrad Med 2002;48:46-49.

15. McGregor M. Current concepts: pulsus paradoxus. N Engl J Med 1979;301:480-482.

16. Spodick DH. The normal and diseased pericardium: current concepts of pericardial physiology, diagnosis and treatment. J Am Coll Cardiol 1983;1:240-251.

17. Singh S, Wann LS, Klopfenstein HS, Hartz A, Brooks HL. Usefulness of right ventricular diastolic collapse in diagnosing cardiac tamponade and comparison to pulsus paradoxus. Am J Cardiol 1986;57:652-656.

18. Guberman BA, Fowler NO, Engel PJ, Gueron M, Allen JM. Cardiac tamponade in medical patients. Circulation 1981;64:633-640.

19. Hancock, EW. Cardiomyopathy: Differential diagnosis of restrictive cardiomyopathy and constrictive pericarditis. Heart 2001;86:343-349.

20. Ling LH, Oh JK, Schaff HV, Danielson GK, Mahoney DW, Seward JB, Tajik AJ. Constrictive pericarditis in the modern era: evolving clinical spectrum and impact on outcome after pericardiectomy. Circulation 1999;100:1380-1386.

21. Lange RL, Botticelli JT, Tsagaris TJ, Walker JA, Gani M, Bustamante RA. Diagnostic signs in compressive cardiac disorders. Constrictive pericarditis, pericardial effusion, and tamponade. Circulation 1966;33:763-777.

22. Spodick DH. Chronic and constrictive pericarditis. New York, NY: Grune & Stratton; 1964.

23. Chinnaiyan, KM, Leff, CB, Marsalese, DL. Constrictive Pericarditis Versus Restrictive Cardiomyopathy: Challenges in Diagnosis and Management. Cardiology in Review 2004;12:314-320.

24. Kazi, RA, Peter, RE. Christian Albert Theodor Billroth: Master of surgery. J Postgrad Med 2004;50:82-83.

25. Mark, HH. The First Ophthalmoscope? Adolf Kussmaul 1845. Arch Ophthal 1970;84:520-521.

Author AffiliationsSara K. JohnsonUniversity of Wisconsin School of Medicine and Public HealthMadison, Wisconsin

Ramo K. NaiduUniversity of Wisconsin School of Medicine and Public HealthMadison, Wisconsin

Ryan C. OstopowiczUniversity of WisconsinStevens Point, Wisconsin andOffice of Scientific Writing and PublicationsMarshfield Clinic Research FoundationMarshfield, Wisconsin

David R. KumarResearch AssociateMarshfield Clinic Research FoundationMarshfield, Wisconsin

Satya Bhupathi, MD, MPHDepartment of Internal MedicineMarshfield ClinicMarshfield, Wisconsin

Joseph J. Mazza, MD, MACPEmeritus Physician, Department of Clinical ResearchMarshfield Clinic Research Foundation Marshfield, Wisconsin

Steven H. Yale, MDDepartment of Internal MedicineMarshfield Clinic andDepartment of Clinical ResearchMarshfield Clinic Research FoundationMarshfield, Wisconsin

IMAGES IN CARDIOVASCULAR MEDICINE 32

A paradoxical rise in the jugular venous pressure on inspiration

Kussmauls sign in effusive constrictive pericarditisMattia Cattaneo a, Stefano Muzzarellib, Francesco Faletrab, Alessandra Pia Porrettaa, Francesco Siclaric, Augusto Gallino a, d a Cardiovascular Medicine department, Ospedale Regionale di Bellinzona e Valli Ospedale San Giovanni, Bellinzona, Switzerland b Cardiology department, Fondazione Cardiocentro Ticino, Lugano, Switzerlandc Cardiac Surgery department, Fondazione Cardiocentro Ticino, Lugano, Switzerlandd University of Zurich, Switzerland

Case report

A 67-year old man with mitral valve prolapse and moderate regurgitation was admitted because of dyspnoea, bilateral ankle swelling and hypotension. Close inspection of the jugular veins identified Kuss-mauls sign, a typical increase in the central venous pressure during inspiration (fig. 1; arrows). He had no history or clinical evidence of infection, tumours, uraemia, trauma, surgery or radiation. Transthoracic echocardiography revealed moderate diffuse pericar-dial effusion (PE) (fig. 2, arrows) with paradoxical in-terventricular septum bounce (see video 1*). Persis-

* You can find the videos on http://www.cardiovasc-med.ch/for-readers/multimedia

Figure 1: Kussmauls sign (arrows) is a paradoxical rise in

the jugular venous pressure (JVP) (arrows) when the patient

breathes in, due to impaired venous flow toward the heart

associated with right ventricular constrictive diastolic

impairment.

Cl = clavicle; Sc = sternocleidomastoid muscle.

Figure 2: Transthoracic echocardiography; 4 chamber view:

it displays a moderate (2 cm) diffuse pericardial effusion (PE),

more pronounced on the left side due to partial adhesions.

Also ventricular septal bounce due to a paradoxical interven-

tricular septum shift prompted by respiration phases is dis-

played (see video 1*).

tence of Kussmauls sign and symptoms of acute right heart failure after pericardiocentesis (170 ml ex-udate, no infections and neoplastic cells) prompted the clinical suspicion of idiopathic effusive-constric-tive pericarditis. Diagnosis was supported by cardiac magnetic resonance (CMR), showing mild residual PE and diffuse thickening of the pericardium (fig. 3, ar-rows) with contrast enhancement at the pericardial edges (fig. 4, arrows) and septal bounce (see video 2*). Diagnosis of effusive-constrictive pericarditis was confirmed by typical elevated ventricular filling pressures at cardiac catheterisation (equilibration of ventricular diastolic pressures with dip-plateau waveform) and open surgery (pericardi ectomy) showing diffuse parietal (fig. 5AB) and visceral peri-cardial thickening (fig. 5CD). One year follow-up showed complete clinical relief with almost no resid-ual pericardial thickening at CMR.

Funding / potential competing interests: No financial support and no other potential conflict of interest relevant to this article were reported.

CARDIOVASCULAR MEDICINE KARDIOVASKULRE MEDIZIN MDECINE CARDIOVASCULAIRE 2015;18(1): 3233

IMAGES IN CARDIOVASCULAR MEDICINE 33

Correspondence: Mattia Cattaneo, MD Clinical and Research fellow Department of Cardiovascu-lar Medicine Ospedale Regionale di Bellinzona e Valli Ospedale San Giovanni (EOC) CH-6500 Bellinzona Switzerland mattia.cattaneo[at]eoc.ch

Figure 3: CMR in the 4 chamber orientation showing mild

residual pericardial effusion (moderate bright space between

pericardial layers), diffuse thickening of pericardial leaflets all

around the heart (arrows) and septal bounce (arrows) (see

video 2*).

Figure 4: Late-enhancement 4 chamber CMR showing

enhancement of the pericardial edges (arrows).

Figure 5: Surgical field and specimens of the thickened pericardium. On the left (A, B) thickened parietal pericardium (arrows)

is displayed, while on the right (C, D) visceral thickened pericardium is displayed.

CARDIOVASCULAR MEDICINE KARDIOVASKULRE MEDIZIN MDECINE CARDIOVASCULAIRE 2015;18(1): 3233