PERICARDIAL

Kruti Pandya MD LEARNING OBJECTIVES:

1. Understand the function and description of the . 2. Identify the diagnostic criteria and etiologies of acute , and constrictive pericarditis. 3. Understand the hemodynamic consequences of pericardial effusion and constrictive pericarditis. 4. Integrate the information obtained from a history, , , and laboratory studies to determine the diagnosis of the various pericardial syndromes. 5. Understand the treatment strategies to decrease morbidity and improve survival in patients with pericardial based on the basis of evidence and clinical experience PERICARDIUM: ANATOMY

• Pericardial Layers: • Visceral layer • Parietal layer • Fibrous pericardium • Between the two layers lies the pericardial space, which contains approximately 10-50ml of fluid, which is an ultrafiltrate of plasma. FUNCTION OF THE PERICARDIUM

• 1. Stabilization of the within the thoracic cavity by virtue of its ligamentous attachments -- limiting the heart’s motion. • 2. Protection of the heart from mechanical trauma and infection from adjoining structures. • 3. The pericardial fluid functions as a lubricant and decreases friction of cardiac surface during and . • 4. Prevention of excessive dilation of heart especially during sudden rise in intra-cardiac volume (e.g. acute aortic or mitral regurgitation). PERICARDIAL DISEASES

• • Pericardial effusion, . • Chronic constrictive pericarditis. ACUTE PERICARDITIS:

• Common • of layers of pericardium • 0.1% of hosp. pts ; 5% of ER pts with diagnosed with noncardiac chest . ETIOLOGY OF ACUTE PERICARDITIS

• INFECTIVE • NEOPLASM • Viral - Coxsackie A and B, Influenza, adenovirus, HIV, etc. • Primary: mesothelioma, angiosarcoma • Bacterial - Staphylococcus, pneumococcus, TB, etc. • Metastatic – lung, breast, bone, lymphoma, melanoma • Fungal - Candida • Direct extension from adjoining tumor • Parasitic - Amoeba, candida, etc. • RADIATION PERICARDITIS • AUTOIMMUNE DISORDERS • RENAL FAILURE (uremia) • Systemic lupus erythematosus (SLE) • HYPOTHYROIDISM • Drug-Induced lupus (e.g. Hydralazine, Procainamide) • TRAUMATIC CARDIAC INJURY • Rheumatoid Arthritis • Penetrating - stab wound, bullet wound • Post Cardiac Injury Syndromes: • Blunt non-penetrating - automobile steering wheel accident • postmyocardial Infarction (Dressler's) Syndrome, postcardiotomy syndrome, etc. • IDIOPATHIC CLINICAL FEATURES: ACUTE PERICARDITIS

• : classic chest pain – sharp, pleuritic, is worse with supine position and improved on sitting upright, leaning forward. ( >85-90% of cases) • ( <33% of cases) • EKG: Sinus tachycardia, diffuse ST elevation; PR segment depression; PR seg elevation in lead aVR. ( upto 60% of cases) • Additional symptoms: , leukocytosis, SIRS, cancer); elevated CRP, ESR; • Rarely elevation of troponins ( sign of ) EKG FINDINGS IN ACUTE PERICARDITIS

4 Stages of ECG changes • Stage I • Diffuse ST elevation+PR depression(hrs to days) • Stage II • Normalization of ST/PR segments(days to weeks) • Stage III • Widespread inversion(several weeks) • Stage IV • Gradual resolution of T wave inversions(months) DIAGNOSTIC TESTS

• ECG is recommended in all patients. • Echocardiogram: Pericardial effusion • CXR is recommended in all patients • Blood tests : markers of inflammation- crp. And markers of myocardial injury ( troponin, CK) • Additional tests: if concern for other infections like TB. Serologic tests ( ANA, RF) to screen for connective tissue disease, screen for malignancy – lung, breast DIAGNOSIS/ TREATMENT: TREATMENT

• Aspirin or NSAIDS are first line treatment of acute pericarditis. ( class I A) • Colchicine is recommended as first line adjunct treatment of acute pericarditis ( class I A) • Could check Sr CRP to assess therapy/guide treatment duration. ( class II ) • Exercise restriction – until pain resolves, CRP, ECG, Echo normalizes.; for athletes at least 3 mo. ( class II ) ; 6mo for myopericarditis (class I) • Corticosteroids are not recommended as first line therapy for acute pericarditis ( class III C) TREATMENT: PROGNOSIS:

• Prognosis is generally good. • Bacterial/ TB pericarditis à high chances of developing constrictive pericarditis. • Patient who aren’t treated with colchicine à higher chances of developing recurrent pericarditis. • Recurrent pericarditis à may need addition of low dose steroids ( after excluding infection) ; for steroid dependent cases – consultation with rheum/ IVIG, anakinra, azathioprine may be considered. PERICARDIAL EFFUSION

• Normal 15-50 ml of fluid • Common etiologies include: • Acute pericarditis • Autoimmune disease • Chest trauma • Malignancy-most often due to noncardiac primary tumors. • Mediastinal radiation • Renal failure with uremia • Aortic dissection extending into the pericardium • Certain drugs: Hydralazine, Minoxidil, Procainamide,INH • CHF, Cirrhosis, Hypothyroidism • Fluid composition: Serous, Fibrinous, Hemorrhagic, Purulent • Imaging: Echo, CT, MRI, +/-CXR PATHOPHYSIOLOGY

• Symptoms of cardiac compression dependent on: • Volume of fluid • Rate of fluid accumulation • Compliance characteristics of the pericardium

A. Sudden increase of small amount of fluid (e.g. trauma) B. Slow accumulation of large amount of fluid (e.g. CHF) CLINICAL FEATURES:

• Small effusions do not produce hemodynamic Physical Findings: abnormalities. May be normal in patients without hemodynamic • Large effusions, in addition to causing compromise. hemodynamic compromise, may lead to When tamponade: compression of adjoining structures and produce symptoms of: • Muffled • dysphagia (compression of esophagus) • Elevated JVP • • hoarseness (recurrent laryngeal nerve Rarely pericardial friction rub compression) • ( if tamponade) • hiccups (diaphragmatic stimulation) • • dyspnea (pleural inflammation/effusion) • Fatigue, anorexia, cough, weakness, MECHANISM AND MEASUREMENT OF PULSUS PARADOXUS PERICARDIAL EFFUSION DIAGNOSTIC STUDIES

“Water Bottle” Sign

Electrical alternans CARDIAC TAMPONADE: ECHO

• Chamber collapse • Dilated IVC • Respiratory flow variation of mitral and tricuspid velocities CARDIAC TAMPONADE ECHO FINDINGS

IVC in Cardiac Tamponade CARDIAC TAMPONADE-ECHO FINDINGS CARDIAC TAMPONADE-ECHO FINDINGS CARDIAC TAMPONADE-ECHO FINDINGS

Mitral Inflow in Cardiac Tamponade PERICARDIAL EFFUSION: LABS

• Routine Labs: CMP, CBC, TFTs,ANA, screening for malignancy (age appropriate) • Echo: mainstay. Consider CT/ MRI if loculated, pericardial thickening, masses, chest abnormalities. • Diagnostic Pericardiocentesis yields diagnosis <40% of cases! • Fluid analysis : Cell count, protein, LDH, glucose, gram stain/CX, Cytology, AFB stain/Cx, PCR • PERICARDIAL EFFUSION: TREATMENT

• Treatment: • With hemodynamic compromise: • Urgent pericardiocentesis – percutaneous or surgical • Treat underlying cause ( class I c) • Volume expansion – temporary • If associated with inflammation – consider ASA, NSAIDS, colchicine ( class I c) • Avoid diuresis. • Pericardiocentesis – to determine etiology • If hemodynamically stable: or for tamponade or if suspicion for • Monitor unknown bacterial or neoplastic etiology ( • Serial echo class Ic) • Treat underlying c • if reaccumulates – pericardial window/ if biopsy needed. PERICARDIOCENTESIS CONSTRICTIVE PERICARDITIS

• Late complication of pericardial disease • Fibrous scar formation • Fusion of pericardial layers • Calcification further stiffens pericardium • A potentially curable entity in patients with with preserved ejection fraction but it is often underdiagnosed and missed. PATHOPHYSIOLOGY

Scarred Pericardium Inhibits diastolic filling of both ventricles

Venous Pressure

Pulmonary JVP Congestion

Hepatomegaly Ascites Peripheral Edema ETIOLOGY OF CONSTRICTIVE PERICARDITIS

Developed Nations: Presenting Symptoms: Post pericarditis Volume Overload: Mediastinal Radiation - peripheral edema, Underdeveloped Nations & Immunocompromised: - anascara, • Tuberculosis - cardiac cirrhosis • Connective tissue disorder • Malignancy Diminshed Cardiac Output: • Trauma - Fatigue, • Uremic pericarditis. - Dypnea on exertion VENTRICULAR INTERDEPENDENCE CONSTRICTIVE PERICARDITIS- PHYSICAL FINDINGS

• Elevated JVP • • Kussmaul’s Sign • Pericardial Knock • Pulsatile hepatomegaly • Pleural Effusion • Ascites • Peripheral Edema • Anascara DIAGNOSIS

CXR: calcified cardiac silhouette CT or MRI: pericardial thickening CONSTRICTIVE PERICARDITIS

. RIGHT HEART CATHETERIZATION

Equalization of pressures- RV – LV discordance -Less than 5 mmHg, difference between mean RA, RV diastolic, PA diastolic, LV diastolic and pericardial pressures CONSTRICTION VS. RESTRICTION: TREATMENT

• For chronic constrictive pericarditis- surgery is the treatment of choice. • Significant surgical mortality even in experienced centers(>6%) • Independent predictors of ↑mortality: • Older age • Higher NYHA class, • LVEF • PCWP • Worse renal failure • Lower serum Na • Certain etiologies

From: Bertog, C. JACC 2004 SUMMARY

• Acute pericarditis is most often of idiopathic or viral cause and is usually a self-limited illness. • Common clinical findings in acute pericarditis include pleuritic chest pain, fever, pericardial friction rub, and diffuse ST-segment elevation on the ECG, often accompanied by PR-segment depression. • Treatment of common acute pericarditis (i.e., viral or idiopathic pericarditis) consists of a nonsteroidal anti-inflammatory drug; the addition of colchicine may reduce the frequency of recurrences and shorten the duration of the acute illness. • Glucocorticoid drugs should not be used as initial therapy for acute pericarditis as they increase the likelihood of recurrences. • Complications of pericarditis include cardiac tamponade (accumulation of pericardial fluid under high pressure, which compresses the cardiac chambers) and constrictive pericarditis (restricted filling of the heart because of the surrounding rigid pericardium). • Distinguishing between constrictive pericarditis and restrictive is important because pericardial constriction is often correctable with surgical removal of the pericardium, whereas most cases of restrictive cardiomyopathy have very limited effective treatments. REFERENCES:

1. Kar, S.” Pericardial Cyst: A Review of Historical Perspective and Current Concept of Diagnosis and Management” Int Card J(2015).

2. Man, L.” Congenital Absence of the Pericardium” The Medical Forum. (2015) Retrieved from: http://jdc.jefferson.edu/cgi/viewcontent.cgi?article=1315&context=tmf

3. Kim,HJ “Congenital Absence of the Pericardium” J Cardiovasc Ultrasound. (2014 )ar; 22(1): 36–39.

4. Roy, C. “ Does this Parient with a Pericardial Effussion Have Cardiac Tamponade?” JAMA (2007);297(6):18110-18181.

5. Khandhaker, M”Pericardial Disease: Diagnosis and Management” Mayo Clinic Proceedings (2010) 85(6):572=593

6. Bertog SC, l” Constrictive pericarditis: etiology and cause-specific survival after pericardiectomy” J Am Coll Cardiol. 2004;43(8):1445-1452 Q1

A 55-year-old woman with a history of hypertension and End stage renal disease on dialysis comes to the emergency department complaining of dyspnea and chest pain. She has missed few dialysis sessions. She is noted to be hypotensive, tachycardic with elevated neck . A cuff is placed on her arm and the cuff is deflated slowly. Initially, the first Korotkoff sound is heard only during the expiratory phase. However, with further deflation, the are heard throughout both the expiratory and inspiratory cycles. What will be the ECG finding for this condition? a) Alternating QRS height with each beat b) Diffuse ST elevation c) Peaked T waves d) PR prolongation

40 Q2

• Same patient, What is the next best step in management of this patient? a) Cardiac catheterization b) Pericardiocentesis c) Nonsteroidal antiinflammatory drugs d) Thoracentesis

41 Q3.

A 21 year old male presents with complaints of sharp chest pain, worse on laying down, improved when sitting upright. Recent flu like illness +. What is the most likely physical exam finding in this patient? a) Pulsus paradoxus b) Pericardial friction rub c) Pericardial knock d) S3 gallop e) Kussmaul sign

42 Q4

This same patient, few years later presents with , dyspnea, Abdominal distension and LE edema. On exam, he has elevated JVP, clear lungs. On cardiac exam, you hear normal S1. S2 with a sharp early diastolic sound. On examination of his neck veins – you notice that JVP increases on inspiration. You perform echo which shows normal chamber size and function. • What is the most likely diagnosis? a) Cardiac tamponade b) Constrictive pericarditis c) Left heart failure. d) Mitral stenosis

43 Q5

A 24 year old male presents with sharp substernal chest pain that is worse when supine and improves with sitting forward. Cardiac is as below. An ECG, shown below, is obtained. What is the most likely diagnosis?

1. Acute 2. Acute pericarditis 3. Acute . 4. acute esophageal spasm. Q6

• A 65-year-old female with a history of metastatic breast cancer, receiving chemotherapy with doxorubicin, presents with complaints of shortness of breath. On examination, her blood pressure is 70/40 mmHg and HR is 120 bpm. Her heart sounds are muffled and distant and she has elevated . Lungs are clear to auscultation. A chest x-ray is obtained and results are shown below. What is the most likely diagnosis? a) Septic . b) Cardiac Tamponade c) Acute viral d) Doxorubicin induced cardiotoxicity