History

Fact Explanation
Generalized body swelling In constrictive pericarditis, the heart is covered by a rigid, non flexible pericardium due to fibrosis and adhesion formation leading to impaired diastolic cardiac function. This will in turn leads to low cardiac output and systemic venous congestion causing fluid overload manifesting as body swelling. Degree of swelling vary from peripheral edema to anasarca. [1,2]
Abdominal distension Low cardiac output and systemic venous congestion causing fluid overload leading to accumulation of fluid in the peritoneal cavity causing abdominal distension.[1,2]
Ankle swelling Low cardiac output and systemic venous congestion causing fluid overload leading to accumulation of fluid around the ankle joint due to the effect of gravity and also as there are lax tissue around the ankle.[1,2]
Fatigability, weakness Decreased cardiac output and associated pleural effusions, ascites causing lung compromise in constrictive pericarditis will give rise to these symptoms.[1,2,3]
Dyspnea on exertion Decreased cardiac output in response to body exertion and associated pleural effusions, ascites causing lung compromise in constrictive pericarditis will cause shortness of breath on exertion.[1,2,3]
Chest pain Characteristic chest pain of acute pericarditis is not common in chronic constrictive pericarditis, but some patients may develop pleuritic type chest pain.[1,2,3]
History of a predisposing disease condition There are some disease conditions which predispose to the development of chronic constrictive pericarditis. They can be categorized as 1) infective causes such as viral pericarditis, tuberculosis, septic infections, histoplasmosis 2) trauma 3) history of cardiac surgery or interventions 4) systemic diseases such as systemic lupus erythematosus, rheumatoid arthritis, chronic kidney disease, malignancies 5) history of mediastinal irradiation.[1,2,3]
References
  1. Guidelines on the Diagnosis and Management of Pericardial Diseases Executive SummaryThe Task Force on the Diagnosis and Management of Pericardial Diseases of the European Society of Cardiology. European Heart Journal [online] 2004 April, 25(7):587-610 [viewed 19 June 2014] Available from: doi:10.1016/j.ehj.2004.02.002
  2. DOUSTKAMI HOSSEIN, HOOSHYAR AFSHIN, MALEKI NASROLLAH, TAVOSI ZAHRA, FEIZI IRAJ. Chronic Constrictive Pericarditis. Case Reports in Cardiology [online] 2013 December, 2013:1-4 [viewed 19 June 2014] Available from: doi:10.1155/2013/957497
  3. KHANDAKER MASUD H., ESPINOSA RAUL E., NISHIMURA RICK A., SINAK LAWRENCE J., HAYES SHARONNE N., MELDUNI ROWLENS M., OH JAE K.. Pericardial Disease: Diagnosis and Management. Mayo Clinic Proceedings [online] 2010 June, 85(6):572-593 [viewed 19 June 2014] Available from: doi:10.4065/mcp.2010.0046

Examination

Fact Explanation
Elevated jugular venous pressure (JVP) In constrictive pericarditis, JVP is always elevated and help to differentiate cardiac causes for ascites from noncardiac causes. In constrictive pericarditis there is deep, steep y descent. And also the Kussmaul sign (paradoxical rise in JVP on inspiration) is positive.[1,2,3]
Pericardial knock Characteristic finding in constrictive pericarditis which aid the diagnosis. The early diastolic sound of pericardial constriction occurs slightly earlier than the average third heart sound. Its frequency is somewhat higher, allowing you to hear it throughout the precordium using the diaphragm and the bell. However, it may be difficult to distinguish from a third heart sound, because the origins of both are similar in that the ventricular wall vibrates with rapid early diastolic filling.[1,2,3]
Muffled heart sounds Pericardial thickening will disrupt the sound transmission giving rise to muffled heart sounds.[1]
Ascites Due to fluid overload as a result of low cardiac out put.[1,3]
Pulsatile hepatomegaly Right heart failure due to systemic venous congestion with low cardiac output causes enlargement of the liver.[2,3]
Pitting ankle edema Due to fluid overload found in chronic constrictive pericarditis.[1,2,3]
Area of stony dullness in the thorax Most patients with constrictive pericarditis have associated pleural effusions either bilateral or unilateral due to fluid overload.[1,3]
Tachypnea Patients with chronic constrictive pericarditis have low cardiac output response to exertion and also associated pleural effusions and ascites cause reduction in the lung compliance making patients tachypneic.[1]
Muscle wasting Low cardiac output may give rise to cardiac cachexia with muscle wasting as these patients are frequently symptomatic for several years before the diagnosis is established.[3]
References
  1. DOUSTKAMI HOSSEIN, HOOSHYAR AFSHIN, MALEKI NASROLLAH, TAVOSI ZAHRA, FEIZI IRAJ. Chronic Constrictive Pericarditis. Case Reports in Cardiology [online] 2013 December, 2013:1-4 [viewed 19 June 2014] Available from: doi:10.1155/2013/957497
  2. KHANDAKER MASUD H., ESPINOSA RAUL E., NISHIMURA RICK A., SINAK LAWRENCE J., HAYES SHARONNE N., MELDUNI ROWLENS M., OH JAE K.. Pericardial Disease: Diagnosis and Management. Mayo Clinic Proceedings [online] 2010 June, 85(6):572-593 [viewed 19 June 2014] Available from: doi:10.4065/mcp.2010.0046
  3. LITTLE W. C.. Pericardial Disease. Circulation [online] 2006 March, 113(12):1622-1632 [viewed 19 June 2014] Available from: doi:10.1161/​CIRCULATIONAHA.105.561514

Differential Diagnoses

Fact Explanation
Right heart failure Symptoms and signs are similar in both constrictive pericarditis and right heart failure, therefore investigations are needed to differentiate each other. [1,2,3]
Restrictive cardiomyopathy Both constrictive pericarditis and restrictive cardiomyopathy limit diastolic filling and result in diastolic heart failure, with relatively preserved global systolic function. In constrictive pericarditis, diastolic filling is restricted by an rigid, non pliable pericardium after an initial expansion of the myocardium. The upper limit of ventricular volume is constrained by an inflamed, scarred, or calcified pericardium that is usually, but not always, thicker than normal. Restrictive cardiomyopathy is defined by a non dilated ventricle with a rigid myocardium that causes a major decrease in the effective operative compliance of the heart muscle itself. This decrease distinguishes it from constrictive pericarditis, in which no such decrease in myocardial compliance is usually seen.[1,2,3]
Cirrhosis Absence of increased jugular venous pressure favours cirrhosis and there will be more peripheral stigmata of chronic liver disease. Ascitic fluid analysis also will help in differentiating each other as in constrictive pericarditis the serum-ascites albumin gradient is high whereas in cirrhosis it is low.[1]
Cancer Ovarian malignancies, secondary deposits in the peritoneum and several other cancers can give rise to similar symptoms and signs. Imaging studies will help diagnosing the correct condition.[1]
Hematologic diseases - lymphomas Enlarged lymph nodes can cause systemic venous congestion and hepato-splenomegaly also found in lymphomas.[1]
Peritoneal tuberculosis Important to exclude as both constrictive pericarditis and peritoneal tuberculosis can co-exist.[1]
References
  1. DOUSTKAMI HOSSEIN, HOOSHYAR AFSHIN, MALEKI NASROLLAH, TAVOSI ZAHRA, FEIZI IRAJ. Chronic Constrictive Pericarditis. Case Reports in Cardiology [online] 2013 December, 2013:1-4 [viewed 19 June 2014] Available from: doi:10.1155/2013/957497
  2. KHANDAKER MASUD H., ESPINOSA RAUL E., NISHIMURA RICK A., SINAK LAWRENCE J., HAYES SHARONNE N., MELDUNI ROWLENS M., OH JAE K.. Pericardial Disease: Diagnosis and Management. Mayo Clinic Proceedings [online] 2010 June, 85(6):572-593 [viewed 19 June 2014] Available from: doi:10.4065/mcp.2010.0046
  3. Guidelines on the Diagnosis and Management of Pericardial Diseases Executive SummaryThe Task Force on the Diagnosis and Management of Pericardial Diseases of the European Society of Cardiology. European Heart Journal [online] 2004 April, 25(7):587-610 [viewed 19 June 2014] Available from: doi:10.1016/j.ehj.2004.02.002

Investigations - for Diagnosis

Fact Explanation
Cardiac catheterization The gold standard diagnostic investigation is cardiac catheterization with analysis of intracavitary pressure curves. Elevated and equalized diastolic pressures are the characteristic findings suggestive of constrictive pericarditis. Additionally “square root sign” or “dip and plateau sign” of the left ventricular waveforms also can be seen. Although these hemodynamic patterns can be observed in other causes of heart failure such as restrictive cardiomyopathy, discordance between changes in right and left ventricular systolic pressures during respiration, known as ventricular interdependence, reliably distinguishes constrictive pericarditis from other conditions. [1,2]
Echocardiography Generally the initial diagnostic imaging and hemodynamic study is Echocardiography, transesophageal echocardiogram (TEE) which is very useful for differentiate constrictive pericarditis and restrictive cardiomyopathy. Echocardiographic findings in constrictive pericarditis are:(1) pericardial thickness - increased (higher sensitivity by TEE and cardiac CT than transthoracic echocardiography). However, constrictive pericarditis can occur without increased pericardial thickness. (2) ventricular septal motion - abnormal; (3) inferior vena cava and hepatic veins - dilatation and absent/diminished collapse (4) mitral and tricuspid inflow velocities - restricted (5) preserved or increased medial mitral annulus early diastolic (e′) velocity, which is an important distinction from restrictive cardiomyopathy in which the e′ is diminished with a cutoff value of 7 cm/s95; and (6) increased hepatic vein flow reversal with expiration, reflecting the ventricular interaction and the dissociation of the intracardiac and intrathoracic pressures (7) Left ventricular systolic function/ejection fraction is usually normal but may be impaired in mixed constrictive-restrictive disease. [1,2]
Computed tomography (CT) and magnetic resonance imaging (MRI) CT is useful in diagnosing constrictive pericarditis and can detect increased pericardial thickness (>4 mm) and calcification with high accuracy. Inferior vena cava dilatation, deformed ventricular contours, and angulation of the ventricular septum also can be seen. Cardiac MRI has been shown to be useful in detecting increased pericardial thickness and dilatation of the IVC.[1,2]
Ascitic fluid analysis In chronic constrictive pericarditis the serum-ascites albumin gradient ≥1.1 g/dL and an ascites fluid total protein >2.5 g/dL. [1]
Abdominal sonography Congestive hepatomegaly, ascites and other possible causes such as malignancies can be seen.[1]
Magnetic Resonance Venography (MRV) To study abdominal vascular thrombosis, MRV is performed.[1]
Chest radiography As a baseline, simple, readily available investigation this should be done. Calcification of the pericardium strongly suggests constrictive pericarditis in patients with heart failure. It is best seen from a lateral view and located over the right ventricle and diaphragmatic surfaces of the heart. Pericardial calcification is associated with a longer duration of the constrictive process but not with a specific etiology. Therefore, absence of calcification does not exclude constrictive pericarditis. Additionally lung fields also can be observed.[2]
Pleural fluid analysis If the patient also has a pleural effusion, aspiration of that fluid can be done. The pleural fluid analysis includes adenosine deaminase, cytology, and mycobacteria.[3]
Special investigations - Antinuclear antibodies, Rheumatoid factor, Mycobacterial studies Should be done to find the cause if the history is suggestive of a underlying disease process. [3]
Electrocardiography (ECG) Commonly found abnormalities in constrictive pericarditis are nonspecific ST-segment and T-wave changes. Low voltage QRS complexes and isolated repolarization are more typical in constrictive pericarditis.[2]
References
  1. DOUSTKAMI HOSSEIN, HOOSHYAR AFSHIN, MALEKI NASROLLAH, TAVOSI ZAHRA, FEIZI IRAJ. Chronic Constrictive Pericarditis. Case Reports in Cardiology [online] 2013 December, 2013:1-4 [viewed 19 June 2014] Available from: doi:10.1155/2013/957497
  2. KHANDAKER MASUD H., ESPINOSA RAUL E., NISHIMURA RICK A., SINAK LAWRENCE J., HAYES SHARONNE N., MELDUNI ROWLENS M., OH JAE K.. Pericardial Disease: Diagnosis and Management. Mayo Clinic Proceedings [online] 2010 June, 85(6):572-593 [viewed 19 June 2014] Available from: doi:10.4065/mcp.2010.0046
  3. Guidelines on the Diagnosis and Management of Pericardial Diseases Executive SummaryThe Task Force on the Diagnosis and Management of Pericardial Diseases of the European Society of Cardiology. European Heart Journal [online] 2004 April, 25(7):587-610 [viewed 19 June 2014] Available from: doi:10.1016/j.ehj.2004.02.002

Management - Specific Treatments

Fact Explanation
Complete surgical pericardiectomy Complete surgical pericardiectomy is the only definitive treatment of chronic constrictive pericarditis. Removal of densely adherent fibrous pericardium is usually successful but it is associated with a significant operative mortality. Patients in whom the constriction has progressed to the point of abnormal ventricular function, severely reduced cardiac output, cachexia, or end-organ dysfunction derive the least benefit from the procedure.[1,2,3]
Laser shaving using an Excimer laser If severe calcified adhesions between peri and epicardium or a general affection of the epicardium are present surgery carries a high risk of either incomplete success or severe myocardial damage. In such cases this method can be used.[3]
Management of post operative complications Post operative low cardiac out put should be treated by fluid substitution, catecholamines, high doses of digitalis, and intra aortic ballon pump in most severe cases. [3]
Management of the underlying cause Specific treatment for underlying disease process, (if present) should be started.[4]
References
  1. DOUSTKAMI HOSSEIN, HOOSHYAR AFSHIN, MALEKI NASROLLAH, TAVOSI ZAHRA, FEIZI IRAJ. Chronic Constrictive Pericarditis. Case Reports in Cardiology [online] 2013 December, 2013:1-4 [viewed 19 June 2014] Available from: doi:10.1155/2013/957497
  2. KHANDAKER MASUD H., ESPINOSA RAUL E., NISHIMURA RICK A., SINAK LAWRENCE J., HAYES SHARONNE N., MELDUNI ROWLENS M., OH JAE K.. Pericardial Disease: Diagnosis and Management. Mayo Clinic Proceedings [online] 2010 June, 85(6):572-593 [viewed 19 June 2014] Available from: doi:10.4065/mcp.2010.0046
  3. Guidelines on the Diagnosis and Management of Pericardial Diseases Executive SummaryThe Task Force on the Diagnosis and Management of Pericardial Diseases of the European Society of Cardiology. European Heart Journal [online] 2004 April, 25(7):587-610 [viewed 19 June 2014] Available from: doi:10.1016/j.ehj.2004.02.002
  4. KLEYNBERG ROMAN L., KLEYNBERG VERA M., KLEYNBERG LEONID M., FARAHMANDIAN DANNY. Chronic Constrictive Pericarditis in Association with End-Stage Renal Disease. International Journal of Nephrology [online] 2011 December, 2011:1-4 [viewed 19 June 2014] Available from: doi:10.4061/2011/469602