Diseases of Pericardium

Seoul National University Hospital

Department of Thoracic & Cardiovascular Surgery

PericardiumAnatomy• The pericardial sac is composed of the fibrous and the se

rous pericardium• Fibrous pericardium is the tough external fibroelastic ou

termost layer of the pericardium & the inner layer, serous pericardium, is the smooth mesothelial layer

• The inner layer pericardium consists of outer parietal and inner visceral layer

• Outer parietal layer sits on the inner aspects of fibrous pericardium and the inner visceral layer of serous pericardium is the mesothelial component of epicardium

• Epicardium consists of an inner fibroelastic connective tissue layer & outer superficial mesothelial membrane

Pericardium

Developmental anatomy• During embryologic development, heart invaginates in

to the serous pericardium and is coated by the inner visceral layer of serous pericardium

• At the roots of great vessels, the investing inner visceral layer of serous pericardium is continuous with the outer parietal layer of serous pericardium

• The potential space between the outer parietal layer of serous pericardium and the inner visceral layer of serous pericardium is pericardial space and normally contains only a small amount of serous fluid and the pressure is subatmospheric under normal condition

Pericardium

Anatomy

PericardiumPhysical protection of adhesion1. Loss of mesothelial integrity 1) Mechanical damage surgical swabs, instruments desiccation, cold stretching, blood spilling 2) CPB2. Pathogenesis of adhesion 1) Deposition of fibrin in damaged area 2) Loss of fibrinolytic activity3. Methods of preventing adhesion 1) pericardial meshing, padding 2) Dextran pericardial washing 3) pericardial substitute 4) Pharmacologic agents (methyl PD)

5) primary closure

Diseases of PericardiumClassification• Effusive pericarditis• Constrictive pericarditis• Cardiac tamponade• Postoperative pericardial effusion• Postoperative cardiac tamponade• Postpericardiotomy syndrome• Congenital defect• Neoplastic process• Benign mass • Pericardial cyst• Pneumopericardium• Chylopericardium

Effusive Pericarditis

Definition Inflammatory stimulation of the pericardium which results

in the accumulation of appreciable amounts of pericardial fluid

Etiology• Idiopathic ; common • Viral ; often• Uremic• Tuberculosis• Purulent• Neoplastic• Traumatic• Traumatic with hemopericardium• Drug-induced

Effusive Pericarditis

Symptoms and signs• Vary depending on the etiology and the speed with whi

ch the pericardial fluid has accumulated• The quality of pericardial fluid also play a role in symp

tomatology Diagnosis• History, physical examination, chest radiography, echo

cardiography Theray• Medical and surgical management

Pericardium

• Computed tomography scan of chest – pericardial fluid is seen

around the heart

Constrictive Pericarditis Definition Chronic inflammatory process of fibrous and serous laye

rs of the pericardium that leads to pericardial thickening and compression of the cardiac chambers, ultimately with an associated significant reduction in cardiac function

Etiology• Idiopathic• Viral• Tuberculosis• Effusive• Postcardiotomy• Radiation induced

Constrictive Pericarditis

History• Galen ; Cicatrical thickening in animal in A.D 160

• Lower ; Acute & chronic pericarditis in 1669

• Lancisi ;Autopsy description in 1728

• Pick ; A clinical report in 1896

• Rehn & Sauerbruck ; Successful partial pericardiectomy in 1913 in German

• Church, Beck ; Successful series in 1929, 1931 in States

Constrictive Pericarditis

Pathophysiology• Clinical features derives from basic abnormalities of di

astolic function• Ventricular filling is impaired and ventricular stroke v

olume reduced as a result of reduced compliance of fused cardiac and pericardial mass.

• For a brief period in early diastole, ventricular filling is rapid. However, the limit of ventricular distensibility is reached rapidly, and right ventricular pressure pulse displays an early diastolic dip and then a high diastolic plateau ( square root sign )

Constrictive Pericarditis Symptoms and signs• Rare in childhood• Easy fatigability, DOE and even syncope on exertion• Jugular venous distention, distant heart sound, hepato

megaly, ascites in advanced cases Diagnosis• Lack of cardiac enlargement and calcification on chest

radiography , pericardial thickening but not always on echocardiography and CT

• Equalization of mean RA, PA wedge, RVED and LVED pressure help differentiate constrictive pericarditis to LV failure on cardiac catheterization

Therapy• Medical and surgical early pericardiectomy

Constrictive Pericarditis

Natural history• Knowledge of surgically untreated patients is incomplete• The interval between the etiologic event and onset of clin

ical evidence of constriction varies between the few months and many years, and the factors that determine rate of progression are unknown

• Atrial fibrillation commonly occurs at some stage and results in sudden deterioration in circulatory status

• Once signs and symptoms develop, only a semi-invalid life can be led over an interval of 5 to 15 more years

• When the clinical syndrome includes ascites, progression is more rapid, particularly in children

Constrictive Pericarditis

• The plateaued end-diastolic pressure of the right ventricle

& equalization of diastolic pressures in all cardiac chamber

Pressure tracing

Constrictive PericarditisClinical spectrum• The advent of antitubercular chemotherapy brought down t

he mortality for tuberculous pericarditis from 90% to about 40%

• In tuberculous pericarditis, institute the antitubercular therapy for a minimum period of 12 months

• The role of corticosteroids in the management of tuberculous pericarditis is controversial

• The diagnosis of constrictive pericarditis remains a challenge and is achieved by echocardiography, computed tomography, magnetic resonance imaging, and cardiac catheterization

Constrictive Pericarditis

Distinction to restrictive cardiomyopathy • Differentiation of constrictive pericarditis to from restr

ictive cardiomyopathy with diastolic ventricular dysfunction can be quite challenging

• Clinical assessment, MRI, cardiac catheterization, echocardiography, radionuclide ventriculography & endomyocardial biopsy neeeded

• For patients with constrictive pericarditis, early complete surgical pericardiectomy remains the only definitive treatment

Pericardiectomy

Definitions• Total pericardiectomy was defined as wide excision of the

pericardium with the phrenic nerves defining the posterior extent, the great vessels including the intrapericardial portion of and superior vena cava–right atrium junction defining the superior extent, and the diaphragmatic surface, including the inferior vena cava–right atrium junction defining the inferior extent of the pericardial resection.

• Constricting layers of the epicardium were removed whenever possible

• Any excision less than total was considered partial.

PericardiectomySurgical techniques• The objective is to remove all pericardium from the car

diac structures including the right & left ventricle, right & left atrium, aorta, pulmonary artery, SVC, IVC, pulmonary veins

• In epicardial peel is adherent, a cross-hatching waffle procedure, or multiple incision of peel ( turtle cage operation ) allow myocardial expansion.

• On the ventricular surface, dissection may proceed outside the epicardium in the area of coronary arteries, but the dissection over the great arteries and atria should remove the overlying epicardium

• Management is curative surgical pericardiectomy and should be instituted rapidly once diagnosis is made

PericardiectomySurgical approach• Left anterolateral thoracotomy was the preferred option in

the setting of purulent pericarditis to avoid sternal infection• Median sternotomy approach was preferred in the followin

g cases: (1) annular constrictive pericarditis, (2) calcific pericardial patch compressing the right atrium & right ventricular outflow tract, (3) extracardiac intrapericardial mass, (4) presence of a gradient between the superior & inferior venae cavae & right atrium 2 mm Hg or greater, (5) constriction after previous open heart surgery, and (6) recurrent constrictive pericarditis after partial pericardiectomy

Pericardiectomy

Surgical results• Regardless of the operative approach or the extent of peri

cardial resection, a subset of patients with constrictive pericarditis will develop low-output syndrome after pericardiectomy

• Poor results, with persistent elevation of ventricular filling pressures, have been variously attributed to inadequate decortication, fibrous invasion of the myocardium, atrophy of myocardial fibers, "remodeling" of the ventricles, worsening tricuspid regurgitation, and postoperative mitral regurgitation secondary to papillary muscle elongation

Constrictive Pericarditis

Results of surgical treatment• Survival Early death Time-related survival• Modes of death• Incremental risk factors Preoperative functional class High RV end-diastolic pressure Previous radiation therapy over chest• Hemodynamic results• Functional status• Reoperation

Cardiac Tamponade

Characteristics• Accumulation of fluid within the pericardial space can c

ause a rise in pericardial pressure that leads to impairment of ventricular diastolic filling as defined cardiac compression

• All intracardiac pressures will equalize during diastole• The end result of this physiologic state is a lack of left he

art filling, leading to a lack of cardiac output and shock• Initial compensatory mechanisms such as tachycardia w

ill precede & herald the eventual state of circulatory collapse

Cardiac Tamponade

Diagnosis• Beck’s triad

Elevated CVP

Decreased arterial pressure

Muffled heart tones

• Pulsus paradoxicus

• Kussmaul’s sign

Postpericardiotomy Syndrome

Definition• Pericardial inflammation secondary to either cardiac m

uscle damage or surgical cardiotomy associated with pain, friction rub, and occasionally ECG changes suggestive of ischemia.

• Unclear etiology & thought to be an autoimmune disorder

• Symptoms typically appear 3 – 6 weeks after pericardiotomy including fever, arthralgia, progressive pericardial effusion, and sometimes pleural effusion

• Usually lymphocytosis, elevated ESR; eosinophilia and elevated C reactive protein may be present

Postpericardiotomy Syndrome

Etiology• Unclear and is thought to be an autoimmune disorder;

concurrent fresh or reactivated viral illness is also felt to play a role in triggering the immunologic response & seasonal variation with a higher incidence in winter

Therapy• Initial bed rest & nonsteroidal antiinflammatory agents

Indomethacin or salicylates ( 60-80mmg/Kg/day )

• Prednisolone for refractory cases or recurred case

• Surgical intervention for difficult case

Pericardial DefectCongenital defects• A rare group of lesions which range from isolated defects

to complete absence of pericardium• Three types are complete absence, left-sided defect (most

common), and right-sided defect.• Patients may be completely asymptomatic or have exerti

onal or nonexertional chest pain, cardiac arrhythmias, syncope, sudden death, or incarceration of myocardium

• Surgery is indicated for arrhythmia, cardiac torsion, cardiac herniation, or debilitating chest pain

• Surgical repair involves pericardial reconstruction ( pericardioplasty) using xenograft or Gore-tex

ChylopericardiumDefinition• A chylous effusive process of the pericardium a

nd cause either acute or chronic cardiac tamponade

Etiology• Idiopathic• Posttraumatic• After thoracic surgery• Postpericardiotomy• Neoplastic in origin

ChylopericardiumAfter cardiac surgery• Incidence 0.2%, frequent after Fontan operation • Etiology Thoracic duct injury ; most common Damage of lymphatics of pericardium Elevation of systemic venous pressure Congenital lymphatic dysplasia (Down syndrome)• Treatment Diuresis & dietary modification ( low-fat medium- chain trigliceride ), parenteral nutrition, repeated centesis. Exploration to ligate after 2 weeks medical failure with pleuropericardial window or pericardiostomy

Pericardial Reentry

Surgical adhesion• Lead to more difficult sternal reentry and cardiac disse

ction, to blunted visibility of distinct cardiac structures, to potential injury of cardiac structures (including the atria, ventricles, and coronary arteries), as well as to an increased risk of surgical bleeding, all associated with an increase in morbidity and mortality.

• Reoperations due to adhesions are more time-consuming, and because of the increased difficulty they are associated with a greater level of stress for the individual surgeon.