Pericardial Disease Dr. Mozhgan Parsaee Department of Echocardiography Shahid Rajaei Cardiovascular Center

Normal Function of Pericardium Fibrous sac surrounding heart-dense network of collagen fibres. Serous membrane – two continuous layers separated by a small amount of fluid lubricant , ultrafiltration of plasma (10-50 mL). Layers are called visceral and parietal Visceral is inner layer (epicardium) Parietal is continuous with diaphragm and outer walls of great arteries.

Normal Function of Pericardium Limit cardiac dilatation Limit cardiac displacement Reduce friction to cardiac movement Barrier to inflamation Not needed to sustain life

Acute Pericarditis

Acute Pericarditis Acute pericarditis, defined as symptoms or signs resulting from pericardial inflammation of no more than 1 to 2 weeks in duration. Serous Fibrinous Purulent Hemorrhagic

Etiology Infectious pericarditis Viral : coxackievirus A&B, echovirus, mumps,HIV, adenovirus, hepatitis Pyogenic: pneumococcus, streptococcus, staphylococcus Fungal: histoplasma, candida

Etiology Non infectious pericarditis acute MI Uremia Cancer (commonly lung or breast) Trauma Myxedema Postirradiation Aortic dissection Acute idiopathic Connective tissue disease

Etiology Pericarditis related to hypersensivity or autoimmunity Rheumatic fever Collagen vascular disease(SLE, RA) Drug induced (phenytoin, procainamide, hydralazine, isoniazide, minoxidil, anticoagulant) Post cardiac injury (posttraumatic, Dressler’s syn, post pericardiotomy)

Signs, Symptoms and Investigations How can we diagnose this? Clinical examination Auscultation Chest x-ray ECG Echo Catheter laboratory investigations

Acute Pericarditis Chest pain Pericardial friction rub ECG changes Pericardial effusion

Chest Pain Usually present in the acute infectious types and in many of the forms related to hypersensivity and autoimmunity Often absent pain in slowly developing TB, post irradiation, cancer, or uremic pericarditis

Chest Pain The pain of acute pericarditis is often severe, retrosternal and left precordial and reffered to the neck, arms or the left shoulder; the most characteristic radiation is to the trapezius ridge. Almost always the pain is pleuritric (sharp and aggravated by inspiration, coughing, and changes in body positions). Pain may be relieved by sitting up and leaning forward and is intensified by lying supine.

Pericardial Friction Rub Pericardial friction rub is audible in 85% of patients. Is high pitch and scratching. Has three components per cardiac cycle presystolic rub during atrial filling ventricular systolic rub (loudest) ventricular diastolic rub (after A2P2)

Pericardial Friction Rub Most frequently is audible at end-expiration with the patient upright and leaning forward. is audible when the diaphragm of the stethoscope is applied firm to the chest wall at the LLSB. The rub is often inconstant.

Clinical signs differential diagnosis - pleurisy Pleuritic pain has similar sharp quality but is usually more specific in location Pleural rub is heard over the area where the pain occurs A pericardial rub is heard throughout the respiratory cycle, while a pleural rub disappears when respiration is suspended.

ECG differential diagnosis - MI What leads is the ST elevation in? What shape is the elevation? Are there Q waves? Do the ST –T changes evolve with time? History of the patient Cardiac enzymes etc But REMEMBER that you can get more than one pathology at the same time!

ECG Features ECG display changes secondary to acute subepicardial inflammation and shows typically four stages. Stage I (first hours to days) Diffuse up sloping ST elevation with reciprocal ST depression(aVR,V1) PR depression in the inferolateral leads PR elevation in aVR

ECG Features Stage II (after several days) Stage III Stage IV Normalization of the ST and PR segments Stage III Diffuse T wave inversions, generally after the ST segment have become isoelectric, however, this phase is not seen in some patients Stage IV ECG may become normal or the T inversion may persist indefinitely

ECG in AMI ST elevation are convex Reciprocal depression is usually more prominent QRS changes occurs (development of Q wave and loss of R-wave amplitude) T wave inversion are seen within hours before the ST segment have become isoelectric.

ECG in Early Repolarization Early repolarization is a normal variant and may also associated with widespread ST-T segment elevation, most prominent in left precordial leads. T wave are usually tall. ST/T ratio is <0.25 (this ratio is higher in pericarditis)

ST/T ratio in Pericarditis

ECG in Early Repolarization

Diagnosis of pericarditis Patient will have 2 or more of the following; Characteristic chest pain Pericardial friction rub (auscultation) ECG showing characteristic ST elevation (caused by epicardial injury)

Treatment Search for the underlying disease Patients require bed rest NSAIDs: Iboprofen (600 to 800 mg orally three times daily) with discontinuation if pain is no longer present after 2 weeks Reliable patients with no more than small PE who respond well to NSAIDs need not to be admitted to the hospital.

Indication for Hospital Admission Patients who do not respond well initially to NSAIDs Patients have larger effusion Patients have a suspected cause other than idiopathic pericarditis Should be hospitalized for additional observation, diagnostic testing, and treatment as necessary

Prognosis Pericarditis is usually a benign and self-limited disease (usually over 1 to 3 weeks) without significant complication or recurrence in 70% to 90% of patients Diagnosis relates to underlying cause But any cause can lead to an effusion and tamponade which can lead to death Pericarditis can also progress to pericardial constriction and heart failure

Management In young women, it is not unreasonable to test for SLE. However, low ANA titers appear to be common in patients with recurrent idiopathic pericarditis who do not meet other criteria for SLE. Thus, the significance of low ANA titers in the setting of an initial presentation in somewhat uncertain.

Cardiac Enzymes Cardiac troponin I was detectable in 49% and above 1.5 ng/ml−1 in 22%. Elevated troponin values were felt to reflect superficial myocardial inflammation and were not an adverse prognostic marker after a mean follow‐up of 24 months.

Pericarditis of Renal Failure Occurs in to one-third of patients (uremic pericarditis) Occurs in patients undergoing chronic dialysis with normal level of BUN, CR (dialysis associated pericarditis) Pain is absent or mild. The ECG does not usually show the typical ST and T wave elevation. Treatment: NSAID, intensification of dialysis.

Early Post-MI Pericarditis It is associated with large, transmural MIs, but it is almost invariably a benign process that does not affect in-hospital mortality.Treatment is based on symptoms. Augmentation of usual post-MI aspirin doses (650 mg TID or QID for 2-5 days) or acetaminophen is usually effective. Because significant hemopericardium is rare with early post-MT pericarditis and there is no evidence that heparin or other antithrombotic drugs increase its risk, their administration need not be modified.

Pericardial Effusion

Pericardial Effusion It is important clinically when it develops within a relatively short time as it may lead to tamponade. Heart sounds may be fainter with PE. The friction rub may disappear. Apex impulse may vanish.

Pericardial Effusion: Symptoms Dull constant left chest ache Dyspnea (shortness of breath) Less common: Hiccups (phrenic nerve) Hoarseness (recurrent laryngeal nerve) Dysphagia (esophageal compression)

Pericardial Effusion Ewart’s sign: CXR: the base of the left lung may be compressed by PE, a patch of dullness and increased fremitus (egophony) beneath the angle of the left scapula. CXR: - Enlargemnt of cardiac silhouette (effusion>250 mL) - Water bottle configuration of the cardiac cilhouette.

Pericardial Effusion Echo: CT,MRI: Is the most effective imaging. A echo free space between the posterior pericardium and LV epicardium In the latter, the heart may swing freely within the pericardial space.(electrical alternans) CT,MRI: Loculated PE, pericardial thickening and mass

Myxedema 25-35% of patients with severe hypothyroidism develop PE. PE has a high concentration of protein-cholestrole and like other serous effusions ,its pathogenesis is not fully understood (accumulation of mucopolysaccharides or a combination of extravasation of albumin and decreased lymph flow( . The effusion gradually resolve with thyroid replacement. PE occasionally occur in subclinical hypothyrodism. Rarely, PE can occur in hyperthyrodism.

PE in hypothyroidy May cause of large chronic pericardial effusion (rarely is massive and lead to tamponade).

Management of Pericardial Effusion The management of small or moderate pericardial effusions, without tamponade, is usually conservative. The routine diagnostic pericardiocentesis in the absence of cardiac tamponade is not indicated. If the pericardial effusion is likely to be purulent then it should be drained. If the effusion is felt to be malignant, pericardiocentesis is recommended if confirmation would change management and can be performed safely.

Taomponade

Tamponade Tamponade is life-threatening, slow (2000 mL) or rapid (150-200 mL) compression of the heart due to the pericardial accumulation of the fluid. There are limitation of ventricular filling and reduction of cardiac output.

The causes of Raised Intra Pericardial Pressure Raised intrapericardial pressure can occur by 3 main mechanisms: Increased fluid within the intrapericardial space (PE) Increased volume of the cardiac chambers (PTE) Increased stiffness of the pericardium (CP)

Consequences of raised IPP Raised intrapericardial pressure has 3 potential adverse effect on the heart: A compressive effect with limits diastolic filling of the heart Increased diastolic filling pressure Reduced stroke volume and cardiac output

Etiology Causes of tamponade : Cancer Idiopathic pericarditis (receive anticoagulant) Renal failure Cardiac operation Trauma PCI Insertion PPM

Tamponade The clinical manifestation of tamponade may be resemble of heart failure (dyspnea, orthopnea, hepatic engorgement) When tamponade more slowly a high index of suspicious is necessary because sometimes no obvious cause for pericardial disease is apparent It should be considered in any patient with hypotention and increased JVP.

Suspicious to Tamponade Hypotention+increased JVP Unexplained enlargement of the cardiac silhouette Reduction in amplitude of the QRS complex Electrical alternance of the P,QRS,T

Beck’s triad Hypotension Soft or absent heart sounds Jugular venous distention (prominent x)

Ventricular interdependence Normal subjects – there is normally small augmentation of right-sided transvalvular flows accompanying inspiration. Explanation – combination of increased venous return due to decreases intrathoracic pressure fall in PVR

Enhanced Ventricular interdependence in Tamponade

Pulse paradox A more than 10 mmHg (normal) inspiratory decline in systolic arterial pressure When severe, it may be detected by palpating weakness or disappearance of the arterial pulse during inspiration. DD: RVMI, 1/3 patients with constrictive pericarditis, hypovolemic shock, PTE, acute and chronic obstructive lung disease

How to measure Pulsus Paradoxus When severe, it may be detected by palpating weakness or disappearance of the arterial pulse during inspiration. Ask the subject to breath normally Auscultate Korotkoff’s sounds as the BP cuff is slowly lowered. Time respiration simultaneously Mark when BP sounds are heard only in expiration Mark when BP sounds are heard both in expiration & inspiration. Korotkoff’s sounds seem to double at this point. The difference is the measured pulsus paradoxus

Echocardiography Both ventricles is a tight incompressible covery (pericardial sac) Inspiratory enlargement of the RV in tamponade Compress and reduce LV volume due to left ward bulging of the IVS Normal inspiratory augmentation of RV volume causes an exaggerated reciprocal reduction in LV volume Respiratory distress increases the fluctuation in transthoracic pressure

Ventricular interdependence in Tamponade

Echocardiography % respiratory variation= E exp- E ins E exp

Echocardiography An exaggerated respiratory variation in inflow and outflow velocities: MV:25% increase with expiration (normally 15%) TV :50% increase with inspiration (normally 25%) AV :14% increase with expiration (normally 4%) PV :16% increase with inspiration (normally 5%)

Echocardiography Doppler ultrasound shows the TV and PV flow velocities increase markedly in inspiration MV and AV and PVs flow velocities diminish RA and RV collapse in late diastole

Management In patient with clinical finding of tamponade if there is RV collapse , immediate therapy is needed In patient without clinical finding of tamponade if there is RV collapse it means intrapericardial pressure is greater than RV pressure and there is potential for acute hemodynamic deterioration, urgent therapy is needed

Management Medical emergency – intensive care environment needed. Oxygen Volume expansion Bed rest with leg elevation Inotropic drugs if necessary

Management Pericardiocentesis is the definitive therapy to remove the excessive fluid Commonly performed in the cath lab but may be done ‘blind’ in an intensive care environment

Constrictive Pericarditis

Chronic constrictive pericarditis Tuberculous constrictive pericarditis was common cause of constriction pre 1960 – decline in incidence. Post-radiotherapy constriction features prominently today along with post-surgical causes. Needs to be differentiated from restrictive cardiomyopathy when making diagnosis.

Chronic constrictive pericarditis Formation of granulation tissue after desorption of effusion or healing of a acute fibrinous pericarditis. The ventricles are enable to fill because of the limitations imposed by rigid, thickened pericardium Ventricular filling is only in early diastole and is reduced abruptly when the elastic limit of the pericardium is reached In tamponade ventricular filling is impeded throughout diastole

Investigations Clinical picture– prime symptoms weakness, fatigue, weight gain, edema, ascitis, exertional dyspnea, ortopnea (not severe) The cervical veins are distended and may remain so even after intensive diuretic treatment Auscultation may reveal a pericardial knock(0.09-0.12 after AV closure) , audible in apex, it occurs due to abrupt cessation of ventricular filling

Investigations Heart sounds may be distant Audible systolic murmur due to TR Congestive hepatomagaly that cause LFT impairment and jaundice Broadbent’s sign: reduced apical pulce and sometimes retract in systole

Kussmaul’s sign Normal subjects – inspiration causes a decrease in chest pressure. Increase in venous return – JVP falls Constrictive pericarditis – Increased venous return cannot be accommodated in RV because of high EDP So JVP rises on inspiration D.D: tricuspid stenosis, RVMI, RCM

Investigations ECG may not show characteristic ST elevation, display low voltage of the QRS complexes and diffuse flattening or inversion of the T waves, AF is present in one-third of patients CXR may see calcification(23-50%), calcification in CXR is more common in TB and there is normal or only mild enlarged heart that helps to rule out coexisting effusion Echo to identify hemodynamic effects on heart and coexisting effusion

Respiratory Variation in CP Ventricular interdependence is found in CP but not RCM

Respiratory Variation in CP

Echo Finding Normal subjects – increase in TV flow velocity on inspiration, and decrease in MV flow Due to increased vascular capacity of lungs venous return and RV output increases while return to LA is reduced This is exaggerated in tamponade/sig constriction – RV output can’t increase because of high EDP + pulmonary return is reduced further

Septal Bounce

TDI of the Mitral Annulus CP RCM

CT/MRI MRI/CT scan – data about the thickness of the pericardium and display calcification. Pericardial thickening and even calcification are not synonymous with CP since they may occur without seriously impairing ventricular filling.

Normal Right Atrial Pressure Tracing “a” wave Atrial systole “x” descent Relaxation of RA Downward pulling of tricuspid annulus by RV contraction “v” wave RV contraction “y” descent TV opening and RA emptying

CVP Tracing in Normal Subjects Bimodal with x > y

Constrictive Pericarditis

Constrictive Pericarditis vs. Tamponade Tamponade CP

Dip and Plateau in Diastolic Waveform

No respiratory variation in RCM

Treatment The only effective treatment for chronic constrictive pericarditis is complete surgical resection of the pericardium. Mortality for procedure ranges from 5-16% Symptomatic improvement in ~90% 5 year survival rate is 74-87% depending on co-morbidities pre-op

Treatment Radiation-induced disease is considered a relative contraindication for pericardiectomy. Healthy older patients with very mild constriction may also be managed nonsurgically (salt restriction and diuretics consumption).

Pericardial cyst Appear as rounded or lobulated deformities of the cardiac silhouette, most commonly at the right cardiophernic angle No symptom May be confused by tumor, ventricular aneurysm, or cardiomegaly Management is conservative

The End Result