Pericardial constriction

Fundamentals Pericardial

cardiology

constriction

J. V. Hirschmann, Seattle,

of clinical

M.D.

Wash.

Definition

and terminology

Hereditary

When involved by inflammation, extensive fibrosis, calcification or neoplasm, the pericardium may become rigid and impede filling of the enclosed cardiac chambers. The usual term for this condition, chronic constrictivepericarditis, is somewhat inaccurate since inflammation may be absent.’ Pericardial constriction, however, is especially felicitous since it suggests both constriction of and constriction by the pericardium: its contraction and its compression of underlying structures. The two layers are frequently fused, but sometimes the constriction is mainly from the visceral pericardium (epicardium), often with a tense effusion, Usually labeled effusive-constrictiuepericarditis,pericardial constriction with effusion is appropriate for this condition, where cardiac compression by both pericardial fluid and a rigid pericardium can cause somewhat different clinical and hemodynamic findings.’ Etiology

(Table

I)

Most cases probably originate as pericardial inflammation with exudation of fluid into the pericardial sac. This is resorbed, and the visceral and parietal pericardial layers then fuse. Several disorders may cause the initial inflammation, but in most cases no underlying etiology is evident clinically or histologically.’ Instead, nonspecific fibrous tissue, sometimes accompanied by calcification, has replaced the original inflammatory process. From the Department of Medicine, of Medicine; and the Veterans Wash.

of Washington Hospital,

Received

for publication

Reprint Hospital,

requests: J. V. Hirschmann, M.D., Medicine 4435 Beacon Ave. South, Seattle, Wash. 98108.

110

July,

1978,

Apr.

University Administration

Vot.

School Seattle,

27, 1977.

96, No.

1

Dept.,

VA

“Mulibrey” nanism (dwarfism), found mostly in Finland, is an autosomal recessive disorder whose name is an acronym from muscle, liver, brain, and eye, the principal organs involved. Its diagnosis requires the presence of short stature plus at least two of the following four features: yellow dots in the ocular fundi, clinical evidence of pericardial constriction, fibrous dysplasia in the long bones, and a slightly increased basilar skull angle with a long, shallow, J-shaped sella turcica.3 Almost all patients have had clinical evidence of pericardial constriction; eight of 26 reported cases’ have had surgical confirmation, with resected tissue showing non-specific fibrosis. Infections Bacterial. A wide variety of bacteria-aerobic, anaerobic and higher bacteria like Actinomyces and Nocardia-can cause purulent pericarditis by several mechanisms: (1) contiguous spread from adjacent mediastinal, pulmonary, or pleural infections; (2) introduction of organisms by penetrating trauma, including surgery; (3) contiguous spread from an underlying myocardial abscess; (4) hematogenous involvement during bacteremia.5 Acute pericardial constriction can occur over several days to weeks in this setting, cardiac compression resulting from the combination of a thickened, adherent pericardium and purulent material in varying stages of organization6 Surgical drainage is usually necessary for survival, with pericardiectomy the optimal pr0cedure.j Rarely, chronic constriction may occur months to years after apparent recovery from acute purulent pericarditis,if other drainage procedures were used.’ Mycobacterial. Tuberculous pericarditis is now

0002~8703/78/0196-0110$01.20/0

Q 1978

The

C. V. Mosby

Co.

rape

in

deveioped countries. Untreated, this to have one of four outcomes: (1) rdiac compression from effusion and constriction, leading to death within several months; (2) development of fatal tuberculosis elsewhere; (3) apparent resolution with later occurrence of tuberculosis in other organs; (4) apparent resolution with possible late constriction.8 Several untreated patients with bacteriologically proved tuberculous pericarditis have had delayed constriction which on histologic and bacteriologic examination showed no evidence of its tuberculous etiology.’ This discovery emphasizes the frequent difficulty in delineating the etiology of pericardial constriction, but it is unlikely that tuberculosis is currently responsible for a substantial proport.ion of cases in the United States. The frequency of constriction, with or without &fusion, that occurs in treated tuberculous periearditis has ranged from 50 to 65 per cent.+l’ It may develop during therapy or years after apparent bacteriologic cure. In one retrospective study, four of 18 patients (22 per cent) receiving corticosteroid therapy in addition to antitubercu?ous agents uired pericardiectomy compared to five of patients (50 per cent) treated without corticosteroids.Y There are, however, no prospective, controlled studies that examine whether corticosteroid therapy reduces the incidence of subsequent constriction in this or any &her form of pericarditis. Funga!. Patients with disseminated coccidioidomycosis uncommonly have pericardial involvement, apparently by spread from an underlying myocardial abscess. T-his may rarely progress to constriction,” ne case has occurred a year following apparently uncomplicated acute coccidioidomycosis.“’ sma capsu2atum can cause an acute is characterized. by a prodrome of fever, malaise, rhinorrhea, and cough lasting several weeks followed by chest pain and frequently by pleural effusion.‘” The ilIness tends to last about ten weeks. Histoplasmosis has been demonstrated by serologic changes but has not been cultured. Pericardia! constriction has occurred three years following acute pericarditis in one case, in which organisms were demonstrated histologically but not by culture in the resected pericardium.‘” Because histoplasmosis is so common in the

__ Hereditary Mulibrey nanism Infections Bacterial Mycobacterial Fungal Virai Parasitic Connective tissue disorders Rheumatoid arthritis Systemic lupus erythematosur Polyarteritis nodosa IUetabolic Uremia Trauma Blunt or penetrating thoracic trauxx Surgery Radiation, therapy Neoplastic Benign or malignant pericsrdiai txnxors Metastatic malignancy --

--

this disease fndy ‘.se a more United &aces, frequent cause of pericardial const~ic;ion. than is currently recognized. In addiirion, ~~~d~~~t~~a~ fibrosis, an exuberant ~b~osi~g respoa-ase to histoplasma mediast~na~ node infection, may involve ium and cause constriction.‘” Virai. Several viruses, especially; Coxsackie B,‘i ears apparently cause acute rn~~~er~~~rd~t~s and are probably responsible for most Casey of “acute, benign, nonspecific pericarditis.“‘Y Only rare’ty, however; has the virus actually been gro-wn from the pesicardial fluid.‘!’ Instead, a viral etiology has been inferred from serologic changes or isolation of the organism from stool or pharynx at the time of the pericarditis. In some of these ciasco pericardial constriction has occurred several weeks to years following the acute event.‘” L1 An epidemic of Coxsackie pericarditis was apparently responsible for a sudden upsurge of pericardial con&cLion over a three-year period in Vancouverl British Columbia.“’ Infectious ~~o~o~:.u~leosis~ an rr virz3, has caused a ‘infection from Epstein ctiop with &?usion.z~~ case of pericardial cons ~a~as~t~~. Perforation of an amebic live] abscess into the pericardium causes a pericarditis that may rapidly ~onskt+t.“-~ culosis,“j rupture of an eehinococcsl cyst ink t,he pericardium,“’ and filariasis” have a’are1.y caused pericardial constrdction.

Hirschmann Table

II. Symptoms Symptom

Dyspnea Peripheral Abdominal Weakness, Orthopnea Paroxysmal

Table

Approximate

85% 70% 65% 30% 30% 10%

edema distension fatigue nocturnal

incidence”-“’

dyspnea

III. Signs

Sign Distended neck veins Hepatomegaly Ascites Peripheral edema Pulse pressure less mm Hg Third heart sound Pulsus paradoxus Cyanosis Splenomegaly

Connective

tissue

Approximate

than

35

incidence+“’ 95% 90% 70% 70% 50% 40% 35% 25% 10%

disorders

Clinical and necropsy studies indicate that pericarditis, usually asymptomatic, is common in rheumatoid arthritis.2s A few develop pericardial tamponade; more have pericardial constriction, with at least 46 casesreported.29 There is a male predominance, and most patients have had moderate or severe arthritis for several years, usually with subcutaneous nodules and a positive rheumatoid factor. The pericardial constriction has no distinctive features, although many have an accompanying pericardial effusion. Although pericarditis and effusions are frequent in systemic lupus erythematosus, pericardial constriction is rare. It has occurred, however, with both spontaneous30 and drug-induced”’ forms. It has been associated with polyarteritis nodosa,“2 but for unknown reasons probably does not occur from the pericarditis of acute rheumatic fever.33 Metabolic

Pericarditis occurs in the terminal stages of both acute and chronic renal failure.“’ Untreated patients usually die shortly after its appearance, but it typically responds well to dialysis. About 10 to 15 per cent of patients on maintenance chronic hemodialysis develop this disorder, some early in

112

the course, but many only after months of conventional dialysis. The prominent manifestations are pain and fever, the major complications, tamponade and constriction. The latter has occurred from six weeks to 11 months after the acute pericarditis, with an average of about six months. The pathogenesis of uremic pericarditis is unknown. Hemopericardium from the uremic coagulation abnormalities may be an important factor, in some cases perhaps exacerbated by systemic heparinization during hemodialysis. Trauma

The presence of blood in the pericardial sac seems to elicit inflammation which can sometimes evolve into fibrosis and pericardial constriction. Whole blood injected into the pericardial sac of dogs is rapidly absorbed, leaving minimal, if any, inflammation, pericardial thickening, and scarring. When superficial myocardial or epicardial injury is inflicted with a knife and bleeding occurs, however, pericardial constriction, may develop over many months.35 These results suggest that the formation of adhesions in the pericardial cavity requires damage to the mesothelial lining. This probably decreases both the mesothelial capacity to absorb blood and the normal mesothelial fibrinolytic activity. Since injection of the lipid fraction of blood into the pericardial sac can produce constriction.,“’ the lipid components of the blood may be the most important factor in the development of inflammation and eventual constriction. Alternatively, the traumatic pericardial injury may cause an immunologically-mediated inflammation analagous to the post-pericardiectomy syndrome.“’ One or both of these mechanisms may be involved in the constricting process sometimes observed months to years following penetrating or blunt thoracic trauma.“8 Unusual etiologies include foreign bodies (needles) in the myocardium3” and cardiac surgery, with constriction occurring several weeks postoperatively.“’ Radiation4’

Patients receiving therapeutic mediastinal radiation may develop pericardial constriction with or without pericardial effusion several months to years later. In some a preceding episode of symptomatic acute pericarditis has occurred. Most have received a minimum of 4,000 rads to the heart. Hodgkins’s diseaseor other lymphomas are

July, 1978, Vol. 96, No. 1

the com,monesc chnderlyrng malignancy, probably because they so often receive mediastinal irradiation and because tbeir frequent long-term survival allows the fibrosmg process to develop. 6x3

stk

Sometimes the neoplastic process rather than its treatment causes pericardial effusions and constriction. Primary pericardial tumors, both benign” and malignan,, + 43have led to constriction. Malignancy of other organs, most commonly breast or lung carcinoma, malignant melanoma, leukemia or lympboma, may cause cardiac ~ornpre~s~o~ by a large effusion or through neoplastic pericardial thickening. les II and

III)

~~o~~~~~io~ &nvolued. Pericardial constriction occurs in all decades of life, with most series reporting an average age in the thirties. Almost aU have an unexplained male predominance of about 3: 1. There seemsto be no racial predisposision, except in association with tuberculous perinarditis, which has a bigher incidence in blacks,55 ~~~~~~s~ The onset is typically insidious, symptoms frequently being present for months to years before the diagnosis is established. The most common and usually earliest complaint is exertional dyspnea, a consequence of several possible mechanisms: limited diaphragmatic excursion from ascites; restrictive lung disease from the pleural effusions; pulmonary venous hypertension. Bn addition, the arteriovenous oxygen difference mcreases abnormally on exercise because tachycardia cannot maintain an adequate cardiac output in the face of a stroke volume that is decreased and relatively fixed by the rigid pericardial shell. Increased ventilation, creating the sensation of dyspnea, is necessary to ensure full oxygenation of the abnormally desatmated blood arriving in the lungs. Orthopnea, present m a minority of patients, may be related to either the ascites or the pulmonary vascu.lar congestion. Paroxysmal nocturnal dyspnea is quite uncommon, perhaps because the rigid pericardium which inhibits diastolic filling prevents the increased venous return occurring with recumbency from reaching the pulmonary vasculature. Similarly, frank pulmonary edema is rare. Ascites, a prominent early finding, often

withers accompanying ~~~re~~ed from a ~Qrn~~atio~ o systemic venous pressure cau sion and impedes hepatic lym Lion, the decreased cardiac Q perC.rsion, resulting in increased sodium retention The varying contribution of determines whether the as&tic Bu protein (from protein-rich lym circumstances having been des Peripheral edema, alone or wa usuaily present as a rn~~~f~s~a~~~~ 0f the increased systemic venous pressure and sodium retention” Typically most prom ent in the lower extremities, it may o~~aa~o~~~~~occur in the face.57 ~o~stit~t~onal symptoms of reckless and fatigue are common, and so scribe e xhe weight ioss and anorexia. effects of hepatic congestjo d a cardiac output. Amenorrhea may occur in females. Unless the inflammatory pr0cess is s&ill active, chest discomfort is uncommon but abdominal pain in the right upper drank may occur from hepatic enlargement. toms include dizziness or sync which is probably due to left on the es0phagu.s.

constriction usually affects the pericardium diffusely and equally. ~~~as~~~~~~~, however, there may be a localized area of .constriction, most often in patients with ~~e~~~~~~~ ~~~~~q~ate ~eri~ardiecto~~es, but rarely without prior surgery.5s The clinica ~rese~~a~iQ~ has most commonly resembled infun ular ~U~mQ~ar~ stenosis, usually with ri.ght ntricular hypertrophy and failure and a cahcified ~~~st~ct~~~ band evident on chest r~e~t,~~~~~r~~~~ have resembled mitral ste~os~~-~~o~~a co mg band in the atri~ve~t~~~~~a.~ groove5’J or pulmonary veins- and aortic ste~~~~s--~r~rn NIBstriction at the aortic root.“” iTzi3stof the cases these unusual features have en associated with chnical evidence of typical ~~e~~ca~d~a~ c~nstrk-

vaced systemic venous lymphatic

ressure m

Hirschmann

Table

IV. Radiographic abnormalities

Abnormality Pleural thickening or effusion Pericardial calcification Enlarged cardiac silhouette

Approximate inCi(jenCei5“R.

Table 76-i!,. hi

55% 50% 40%

tein loss by bulk loss of lymph into the bowel lumen, often accompanied by diarrhea. The hypoproteinemia that results when gastrointestinal loss exceeds hepatic production reduces the intravascular osmotic pressure and thus further encourages the formation of peripheral edema. Small intestinal biopsy has demonstrated lymphangiectasia-dilated submucosal lymphatic vessels-and small bowel roentgenography may show the typical findings of that disorder: mild dilatation, diffusely thickened jejunal and ileal mucosal folds, and dilution, flocculation, and segmentation of the barium co1umn.63There may also be gastrointestinal loss of lymphocytes (mostly T cells) in the lymph that results in impaired cell-mediated immunity: delayed allograft rejection, cutaneous anergy, and .poor in vitro lymphocyte proliferative responses.@’The protein-losing enteropathy, the lymphangiectasia, and the lymphocytopenia with impaired cellular immunity disappear following pericardiectomy, although sometimes only after several months. Nephrdtic syndrome. The nephrotic syndrome has occurred with pericardial constriction and abated weeks to months following pericardiectoRenal biopsies have shown focal or diffuse my. 65-68 membranous glomerular thickening without hypercellularity.67s 68One case showed, in addition, diffuse tubular dilatation with focal interstitial scarring and round cell infiltration.6’ The pathogenesis of this syndrome is uncertain. The association of the nephrotic syndrome with renal vein thrombosis suggests that increased renal vein pressure may be responsible in both circumstances. In most cases,however, renal vein thrombosis is a complication, not a cause, of the nephrotic syndrome.69 Physical examination Many patients look chronically ill, the decreased muscle mass a sharp contrast to a protuberant abdomen and the prominent peripheral edema. This appearance

114

V. Electrocardiographic

abnormalities

Abnormality Flattened or inverted T waves Low QRS voltage Notched, prolonged P wave Persistent atria1 fibrillation

95% 65% 50% 25%

frequently suggests a serious systemic disease such as metastatic malignancy or a primary liver disorder. The patients are afebrile unless the underlying inflammatory process is still active. The rhythm is atria1 fibrillation in about 25 per cent, and atria1 flutter in about 5 per cent. These arrhythmias presumably arise from the stretch and distortion of the atria1 conduction system by an increased atria1 pressure, but may also be due to inflammatory or fibrotic involvement of the sinoatrial node. Hypertension is very uncommon, and the pulse pressue is often low (less than 35 mm. Hg), reflecting the decreased stroke volume. Pulsus paradoxus, an inspiratory decrease of greater than 10 mm. Hg in systolic pressure, is uncommon unless there is an associated pericardial effusion, presumably because the rigid pericardial shell is relatively unaffected by changes in intrathoracic pressure. Examination of the jugular venous pulse is the single most important physical observation. Unless the patient has undergone vigorous diuretic therapy, which can reduce the systemic venous pressure to normal, the jugular venous pressure is virtually always elevated. Frequently, the veins are distended to the mandibular angle in the upright position. Inspiratory distension of the cervical veins (Kussmaul’s sign), indicating an increased inspiratory venous return that the encased right ventricle is unable to accept, is present in a minority of patients. The high venous pressure may obscure this sign as well as the pulse waves.49 When visible, the jugular waves often show a rapid y descent occurring at the time of tricuspid valve opening. This results from the brisk, but brief, inflow of blood into the right ventricle from the high right atria1 pressure. The presence of a rapid y descent excludes the diagnosis of tricuspid stenosis,49which also has the clinical features of a high systemic venous pressure. The rapid y descent is followed by rapid

July,

1978,

Vol.

96, No.

1

Ta -

I. Semodynamic

differentiation

of pericardial

constriction

and cardiomyopathy _I

-_......-- .-._l-_x.--

~~rdia~y~~~~~~~ Left ventricular diastolic pressure-right ventricular diastolic pressure Right ventricular diastolic pressure Pulmonary artery systolic pressure ---

< 6 mm. 2 % of right

ventricular < 50 mm.

ascent in he jugular ulse as ventricular filling is abruptly terminated by the rigid pericardium. With normal sinus rhythm the x descent may also be prominent.70 The apical impulse is often inapparent because of a seduced stroke volume and the insulating and confining effect of the thickened pericardium. When the apical impulse is detectable, there may be systolic retraction. Coincident with rapid left ventricular fil&ng, an outward thrust may occur, causing an early diastolic apical impu1se.7’ The heart sounds are often distant, although clear, loud sounds do not exclude the diagnosis. A characteristic early diastolic sound, or “pericardial knock,” is present in many patients. Phonocardiographic’z and angiographic’3 studies indicate that it occurs from the abrupt halt in ventricular filling coincident with the steep upstroke qf the early diastolic dip, It is best heard at the center of the precordium, is snapping in quality (often louder than S, or S,) and can increase in intensity on inspiration. It occurs from 0.09 to 0.13 sec. following S,, the shorter the interval the more severe the constriction. It therefore occurs earlier than S, and later than the opening snap of mitral stenosis. A pericardial friction rub is very unusual. The chest examination may reveal dullness to percussion and decreased breath sounds from pleural thickening OF ef%usions,present in about 60 per cent. RBles from pulmonary vascular congestion are uncommon. ~epat~~ega~y occurs early from hepatic engorgement and is present in nearly all patients. ith prolonged disease, cardiac cirrhosis may velop and occasionally cause a decreased liver size. §p~e~omega~y may occur from portal hypertension.

outine laboratory data are usually unhelpful. The hematocrit may be decreased, perhaps from

Hg systolic Hg

> 6 mm. pressure

< ?4 of right

ventricular >50mm.Hg ___^--

Hg systolic

pressure

the effects of chronic illness or the 3x1 disease.The white count is ufiuakly mxmaB,4” as is the sedimentation rate.s’ ~~~~a~~s~smay proteinuria varying from trace to 4-r in with associated mir?emia may dysfunction, incre brotic syndrome, or from ~rot~~~~~~~~~ enteroathy. Bilirubin and liver f~~~t~o~ teses may be normal or show the changes ~~~~a~~er~s~~~of hepatic congestion: bilirubin below 5 mg./ 100 ml. and often predorn~~a~t~~ U~~Q~jug~~te~~ transaminase mildly elevated (40 to 8Q tl.) and rarely above 200; ~rothrombin time an phatase slightly increased,‘+ Liv shows evidence of hepatic conges times cardiac cirrhosis, but may be normaK5”

icardiai calcification, present in o3e-half 5f may be inapparent on ~os~e~oa~te~~o~ roentgenographs but detectable on la.teral or objiique projections. aisle ~~~~a~~~~ cal tion can occur without earssing my0 compression,‘” its resenee with the clinical features of pericar 1 constriction is virtual diagnostic. The most frequent sites of vzakifis: Lion are in the coronary su1cus between the left atrium and ventricle, along the left heart bar and on the sternal or ~~a~~~~g~~~~~ sarface the right ventricIe.‘6 The presence of ~~~~~~~~~~o~ the duration, rather than the cardial constriction. houette may be decrease demonstrate a d~rn~~~ti~~ in size oa serial films. An increased cardiac si~~5~~~~~~~~~ev~~, is common and may occur from the ~e~~~a~~~al t~~ke~ng itself o.r the presence of a, ~~~n~orn~ta~t pericardial effusion. The left ~~~~~rnmay appear enlarged. There is fre~nent~~ evidence of pulmonary vascular congestion and. often manifestations of ~~c~~ased systemic vem3us ~ress~r~~ a

Hirschmann

widened right upper mediastinum from an enlarged superior vena caval shadow and distended azygous vein.76-78 Pleural effusions, present in about 60 per cent, are usually bilateral; when unilateral, they are typically, though not uniformly, right-sided. Pleural thickening or scarring from the original inflammatory event may also be visible. On fluoroscopy the cardiac pulsations are usually, but not always, diminished or absent. Electrocardiogram79-82

(Table

V)

The electrocardiogram is rarely normal. Atria1 fibrillation is present in about 25 per cent, atria1 flutter in 5 per cent. Paroxysmal supraventricular tachycardia and both supraventricular and ventricular extrasystoles occasionally occur. The P- wave is often notched and prolonged (> 0.10 set), resembling “P mitrale,” and presumably occurring from left atria1 hypertension and enlargement. The P-R interval is usually normal. The QRS complex often shows decreased voltage, at times confined to the limb leads. Some patients may show abnormal Q waves characteristic of myocardial infarction. These patients have myocardial fibrosis in the involved area at autopsy.80 The QRS axis is usually normal but some show right axis deviation (> 100 degrees) with or without evidence of right ventricular hypertrophy (R/S > 1 in V,). These features are unexplained, since ventricular hypertrophy is not a pathologic feature of the disease. Left axis deviation and left ventricular hypertrophy are rare. A pattern of incomplete right bundle branch block occasionally occurs. The most common findings, seen in nearly all patients, are nonspecific T wave changes-low, flat, biphasic or inverted T waves in leads where they are normally upright. In a minority, there is accompanying ST segment depression. These changes may represent myocardial involvement from subepicardial penetration of the primary pericardial inflammatory process, myocardial atrophy and fibrosis, simultaneous involvement of the myocardium and pericardium by the same pathologic process, impairment of coronary flow or unrelated coronary artery disease.80 Echocardiography usually demonstrates an unexplained paradoxical motion of the interventricular septum similar to that seen in right ventricular tlow overload syndromes such as

116

atria1 septal defect. s3.*bEvaluation of pericardial thickness is usually difficult and unhelpful but the echocardiogram is sensitive in detecting any fluid in those with associated pericardial effusion. It may also be useful in assessingventricular size and function. Cardiac

catheterizations5

and angiographf”.

87

The most important diagnostic procedure is cardiac catheterization, best performed on both right and left sides of the heart and employing angiocardiography. Since all diastolic expansion is restricted by the rigid pericardium, all diastolic pressures are elevated and approximately equal, even following exercise. The difference between right and left diastolic pressures rarely exceeds 5 to 6 mm. Hg. Thus, the diastolic pressures in the left and right atria and ventricles and the pulmonary artery are nearly equal. The right atria1 pressure usually shows no respiratory variation, but an inspiratory increase (Kussmaul’s sign) may be present in some severe cases. In sinus rhythm the a and V waves are approximately equal and followed by rapid x and y descents because of the high filling pressure. This gives the typical M or W wave form. Concurrent with the right atria1 y descent is a deep diastolic dip in the right ventricular pressure as rapid filling begins. This pressure quickly rises to a plateau as the rigid pericardium impedes further filling. This “dip and plateau” pattern, resembling a square root sign, is also present in left ventricular diastolic tracings. Substantial pulmonary hypertension is rare, most pulmonary arterial systolic pressures being less than 45 mm. Hg. The pulmonary capillary wedge pressure tracing has a wave form similar to that of the right atrium. Unless vigorous diuresis has caused hypovolemia, the ventricular filling pressures are elevated, typically to levels between 12 and 25 mm. Hg, depending on the severity of constriction. Pulsus paradoxus occurs in a minority of patients, more frequently in those with an associated pericardial effusion. In most cases’* the cardiac index and stroke index are low-normal or decreased. The left ventricular end-diastolic volume is reduced in all but mild cases and reflects the severity of constriction. The ejection fraction is usually normal, but may sometimes be substantially diminished? Isovolumic and ejection indices of

July,

1978,

Vol.

96, No,

I

V,,,, and “eontractihty” have vazie : dp/dt, measurements of ~~~urnfer tial fiber shortening have been norma18”l 9o or decreased.89 The differences may, in part, reflect the degree of myocardial fibrosis and atrophy present. With exercisey’ the cardiac output is subnormal in relationship to the increased oxygen consumption. The AV 0, difference widens abnormally. The stroke volume fails to increase because of limitations to diastohc filling. There is a marked elevation in right ventricular diastolic pressure, but the difference between left and right diastolic pressures remains low and essentially unchanged.“” The a~~ogra~h~c findings may include: increased extra~~rn~~al soft tissue thickness along tbe lower right heart border, straightening and immobility of the right atria1 border, superior vena caval dilatation, and left atria1 enlargement. While coronary arteriography is not routinely indicated unless it is necessary to exclude the presence of ischemic heart disease, it does show extension of the heart shadow beyond the ventricular epicardium as delineated by the coronary arteries. This finding indicates pericardial thickening and/or fluid.9” ial constriction

with

Patients having pericardial constriction with effusion tend to be somewhat younger than those with pericardial constriction alone. The most frequent causes are idiopathic (many presumably viral), radiation therapy, uremia, neoplasm or rheumatoid arthritis. The duration of symptoms is @pica&y weeks to months, rather than years, and unhke perieardial constriction alone, there is frequently a precedin history of acute pericarditis, with fever, pleuri c chest pain, and pericardial friction rub. The symptoms are similar-dyspnea, abdominal distension, and ankle edema most prominently-but some physical features differ. Paradoxical pulse is more common, but Kussmaul’s sign and the diastolic sual. While there are T wave inversion and frequently low voltage, abnormal P waves and atria1 fibrillation are generally absent. The cardiac silhouette is usually enlarged on chest roentgenogram, and pericardial calcification is rare. The catheterization results show a generally higher right atria1

pressure thar; decreases to the usuai le%;elseen in pericardia”r ~o~~t~~tion following ~er~~~r~~o~~x~~ tesis. The x descent is p~edo~~~~~t or equ.al to e y descent; predominant y escent is not seen until perieardial fluid is rem ed. At o~er~t~o~ there is extensive tbicke~~~ cath visceral and parietal pericardial layers, the ~er~~~rd~a~ space generally costarring s 1 hundred c,c. of fluid, often bloody, always high in protein, and frequently tense. As with pe~cardial constriction alone, there may be underlying myocardial atrophy. Pericardial constriction with e&z&m can develop into pe~~ar~~al co~stri~~~~~ alsne over a period of weeks to nths and, in fact, may be a common stage in e evolntio~ of pericar constriction.

resk. Perhaps jn part from disuse, pericardial ~o~st~~tio~ can cau,semyocasdial fiber atrophy and fibrosis. In li patients dying with chronic pericardiai constriction, muscle fiber thickness was ~~~f~r~~~y reduced throughout both ventricles. The muscle ~tro~b~~ seemed related to neither the ~~~~at~~~of symptoms nor the thickness of the ~e~~~~r~~~rn.~~ patients may have re~~aeerne~t of muscle fibers by fibrous tissue, at times causnsg e~~~~o~~r~~ographic changes typical of a myoca.r&al infarction in the affected area. xo Myacardiai atrQ~h~1 Ok fibrosis may also be responsible for the refractor.y cardiac failure occasionally f~~~o~~~gpericardiectomy, when the rn~o~ard~u~ appears unable to handle the increased volume load.

k@eirr #&‘I. 95 The increased systemic venous pressure of pericardial constriction almost always cau hepatic congestkm which lead to cardiac cirrhosis. central atrophy with fibrous bridg?ng bet~~e~~ central vein areas. The hepatic congestion seenas to cause some ceIlu,lar destruction, promoting a reparative fibrogenesi n the damaged centrilrsbular areas. Clear hepatic congestion i biochemical features alone: cardiac c.3~rhosismay have no associated ab~orrn~l~t~~~ in liver ~~~~t~o~ tests and its presence does not seem to increase the incidence of portal ~ype~e~s~~~~~ ~~~~~Q~e~ galy, ascites, or e~opbagea~ varices, ~erb~~~ the

Hirschmann

only clue is a small liver, but definitive requires a liver biopsy. Differential

diagnosis

diagnosis

Unexplained exertional dyspnea, ascites, peripheral edema, or pleural effusions should suggest pericardial constriction. The diagnosis is missed with striking and disconcerting regularity. Hepatic cirrhosis, metastatic malignancy, and Meig’s syndrome in females are among the most frequent mistaken diagnoses. Careful examination of the jugular venous pulse, which is elevated in pericardial constriction but normal or low in these other disorders, should prevent such errors. Most difficult to distinguish from pericardial constriction are the cardiomyopathies, particularly the restrictive forms resulting from such processesas diffuse fibrosis following myocarditis, idiopathic hypertrophy, or infiltrative diseases like amyloidosis, hemochromatosis, or sarcoidosis. In the absence of pericardial calcification or some manifestation of the systemic disorder causing the cardiomyopathy, the sometimes strikingly similar clinical and hemodynamic findings may make clinical differentiation impossible without exploratory surgery. Both cardiomyopathies and pericardial constriction may have clinical features of increased systemic and pulmonary venous pressures, the S, of left ventricular failure may be mistaken for a “diastolic knock,” and the electrocardiographic findings may be identical. Cardiac catheterization in restrictive cardiomyopathy may also show increased systemic and pulmonary venous pressures displaying the M or W configuration and an early diastolic dip and plateau pattern of ventricular pressure.96 While certain findings on catheterization may help to distinguish these disorders, sometimes even this procedure is inconclusive, and surgical exploration becomes necessary. Findings favoring a cardiomyopathy are episodes of acute pulmonary edema, a prominent apical impulse displaced well to the left, an audible S,, and electrocardiographic evidence of left ventricular hypertrophy or bundle branch block.“” Of the hemodynamic findings (Table VI), the difference between right and left diastolic pressures is the most reliable point in the differential diagnosis, but even it may be misleading. yi. 98 In the cardiomyopathies the diastolic

118

pressures in both ventricles may be elevated but because the heart is not encased in a rigid shell, as in pericardial constriction, they are very unlikely to be equal. In restrictive cardiomyopathy the reduction in diastolic compliance is greater in left than right ventricle, giving a higher left ventricular diastolic pressure and a difference between the two of greater than 6 mm. Hg. Exercise enhances this difference. This value is almost always less than 6 in pericardial constriction, even with exercise, Other differential points include the pulmonary artery systolic pressure, which is usually less than 50 mm. Hg with pericardial constriction, but greater in the cardiomyopathies. In addition, left ventricular systolic function is usually normal in pericardial constriction, but abnormal in the cardiomyopathies. Several noninvasive techniques reflect these differences. Echocardiography or radionuclide angiography showing a small left ventricular cavity with good ventricular function in a patient with predominantly right-sided failure should suggest pericardial constriction and the need for cardiac catheterization. Systolic time intervals will usually show a short pre-ejection period (PEP), a long left ventricular ejection time (LVET), and a low PEP/LVET ratio (less than 0.5).9?Simultaneous jugular venous pulse tracing and phonocardiography demonstrate that the interval from the aortic component of the second sound to the peak of the jugular venous V wave is usually less than 0.03 sec. in pericardial constriction, greater in cardiomyopathies.loO These noninvasive tests are helpful but not completely reliable in differentiating the two disorders. In perplexing casescardiac catheterization or even exploratory surgery is necessary to exclude the eminently treatable entity of pericardial constriction. Noninvasive techniques may be more dangerous to the patient than invasive ones if they result in a missed or significantly delayed diagnosis. Treatment

Since no. reported large series of untreated patients exists, the natural history of pericardial constriction is uncertain. Young children seemed less capable of prolonged survival than adults, most dying within one to three years after the appearance of massive ascites. Adults could survive, albeit frequently with a valetudinarian

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existence, for 5 to 15 years with repeated paracentesis,‘“’ a procedure apparently unattended by any signidicant or deleterious hemodynamic consequences, despite the removal of large quantities of fluid.‘“’ An occasional patient has survived for several decades without surgery, one of Paul Dudley White’s cases having had symptoms for 44 years.‘4 Although some patients respond to vigorous diuretic therapy, the treatment of pericardial constriction is pericardiectomy, whose delay may risk the development of myocardial atrophy and fibrosis. The surgical mortality rate is small (5 to 10 per cenP ,,) and the outcome excellent, with about 80 to 90 per cent obtaining complete symptomatic relief. Few derive no benefit. The basic requirement for surgery is a thorough deeortication, with the removal of both visceral and parietal pericardial layers from the right and left ventricles. Resection over the left ventricle should proceed at least as far posterolaterally as the phrenic nerve.l’” The approach has varied, including median sternotomy, which most surgeons favor, and left thoractomy; the important point is obtaining excellent exposure. It is probably wise to free the left ventricle first to avoid pulmonary congestion when release of the right ventricle increases pulmonary blood flow and volume. Calcification may require bonecutting instruments and in places may so invade the myocardium that removal is impossible. The necessity of decorticating the atria and vena cava in the absence of obvious constricting bands is debated;““, lo5 this procedure increases the risk is probably unimportant in most patients. Memodynamic measurements immediately following perieardiectomy may show normal values in all chambers, normal left atria1 and ventricular values with improved but still abnormal. right atria1 and ventricular measurements, or no immediate changes.‘““, lo7 Over the next few days, however, central venous pressure begins to fall and ordinarily reaches normal levels within four weeks. The pulse pressure increases as stroke volume becomes normal. Cardiac catheterization studies two to five months following surgery demonstrate satisfactory hemodynamic indices in almost all patients. These findings suggest that in some patients there may be reversible myocardial impairmerpt requiring several days to abate following pericardiectomy.‘““. lo7

~le~tro~a~~~ogra~bi~ a~~~~~~~a~~~~~~ :Gten resolve ~0sto~erativeIy. The f~eq~e~tl~ ab~o~rn~l T wave usually reverts to normal, low voltage increases, most flat or inverted T we.ves become upright* and right axis eviation ~~s~~~~~~~” Those witb the pattern of i~~o~~~ete right bundle bran& block have no change, and atria1 fibrillation typically persistsb” A few patients have per~~a~e~~I~~ abnormal bemodynamic values following surgery. Probably the most common reason is ~~~orn~~e~e pericardiectomy.‘on Some patients, bowe~~er~ have substantial myocardial atrophy and fibrosis.“’ They may die shortly after surgery from ~~re~~~ti~~ cardiac failure; t&e peri~a~~i~~t~~~ effect of increasing ~~Irno~~~y blood the left ventricle cannot effectively accept. Others survive surgery but have ~~~~v~~ little or no benefit from it. Which patients will not respond to decortication is ~~~red~c~a~~le~significantly diminished left ventricular fun&on, which may persist postoperatively, does not preclude improvement in sympto or other hemodynamic measurements.8” v~t~ally all patients should receive su al therapy, with the recognition that 3m a very small number the results will be ~n~m~resaive or even ~atast~o~b~ iC.

1.

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