Constrictive pericarditis: Morphological, functional and haemodynamic evaluation

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Constrictive pericarditis: Morphological, functional and haemodynamic evaluation Poster No.: C-0743 Congress: ECR 2010 Type: Educational Exhibit Topic: Cardiac Authors: B. Graca, P. Donato, M. Ferreira, F. Caseiro-Alves; Coimbra/PT Keywords: constrictive, pericarditis, MRI DOI: 10.1594/ecr2010/C-0743 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. www.myesr.org Page 1 of 23

Learning objectives To demonstrate the spectrum of appearances of contrictive pericarditis on MRI, plain films and CT. To discuss the pathophysiology of the septal bounce, a common finding seen in contrictive pericarditis. Background Basics Inability of the ventricles to fill because of physical constraints imposed by a rigid, thickened, and sometimes calcified pericardium. Causes Tuberculosis: in less developed countries Malignant disease (lung cancer, breast cancer, lymphoma) Mediastinal irradiation Histoplasmosis Purulent or recurrent viral pericarditis Rheumatoid arthritis Uremia Chest trauma or hemopericardium Cardiac surgery Pathophysiology normal pericardium is 3 mm or less thick with chronic constriction, the pericardium may thicken to 6 mm or more with constriction, the impairment in ventricular filling is minimal in early diastole subsequently, diastolic pressure rises abruptly when cardiac volume reaches the anatomic limit set by the noncompliant pericardium stroke volume and cardiac output are reduced because of impaired filling, whereas intrinsic systolic function of the ventricles may be normal or only minimally impaired. Clinical Manifestations Page 2 of 23

dyspnea, weakness, anorexia, peripheral edema, hepatomegaly, splenomegaly, and ascites the pulse pressure is often narrowed pleural effusions are common the clinical picture may mimic hepatic cirrhosis, but with distended neck veins venous pressure often fails to fall with inspiration (Kussmaul's sign) heart sounds may be distant Imaging findings OR Procedure details Morphologic Abnormalities Pericardial thickening and calcification 1. 2. 3. 4. This thickening is usually most pronounced over the right heart side (right ventricle and anterior atrioventricular groove), and the pericardial delineation is often irregular Greater than 4mm is suggestive; greater than 6mm: high specificity though suggestive, the finding of pericardial calcification is not diagnostic of constriction about 1 in 5 patients with CP have normal pericardium, and the isolated finding of thickened pericardium does not imply constrictive physiology Chest x-ray may show pericardial calcification in tuberculous constriction Page 3 of 23

Echocardiography M-mode and 2-dimensional echocardiography show poor sensitivity to detect a pericardium more than 3 mm thick Transesophageal echocardiography, because of superior resolution, showed much better results CT the most appropriate technique to depict pericardial calcification Page 4 of 23

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MRI Abnormal pericardium with low signal on all sequence Occasionally in subacute forms, thickened pericardium may have moderate to high signal on T2wi and no enhancement after gadolinium delayed enhancement may be present, related to acute / subacute inflammation or fibrous thickening Page 8 of 23

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Flattened or tubular-shaped appearance of the ventricles Ventricles constricted by the abnormal pericardium CT may show flattening of right ventricle in static image Cine MR more easily depicts small LV + tubular appearing RV Page 11 of 23

: Four-chamber cine MR images. Focal thickening of pericardium compressing RV free wall and causing impaired RV diastolic filling Atrial enlargement; enlargement of superior and inferior vena cavae as a result of the increased cardiac filling pressures Chest x-ray: Radiographic signs of atrial enlargement not sensitive or specific; Widened superior mediastinum (enlargement of SVC) US can show dilation of hepatic veins and distention of the coronary sinus and inferior vena cava CT and RM the best imaging modalities to detect these alterations Page 12 of 23

Functional and Haemodynamic Abnormalities The septal bounce The rate of #lling is rapid in early diastole, and the rate of change in ventricular pressure at this time in the cycle is particularly rapid. Slight asymmetry of right and left ventricular #lling rate can result in rapid changes in the pressure differential between the two sides of the ventricular septum. The septum may therefore shift in position very abruptly, responding to such rapid changes in pressure. Normally the septum is convex to the right The septal bounce reflects the limitation of RV filling secondary to the rigid pericardium, with resultant shift of the septum to the left during early diastole Page 13 of 23

CMR with cine images can show early diastolic septal flattening or inversion in the majority of constrictive pericarditis patients : Four-chamber cine MR movie. Abnormal septal curvature with early diastolic septal convexity towards the LV. Pericardial thickening and right pleural fluid. Page 14 of 23

: Four-chamber cine MR movie. Septal bounce with early diastolic septal convexity towards the LV. Pericardial thickening. Ventricular septal shift with respiration Reciprocal changes in left and right ventricular volumes with respiration are one aspect of the increased degree of ventricular interdependence that is characteristic of constrictive pericarditis. Because the heart is enclosed within a relatively #xed volume, enlargement of one ventricle tends to be associated with a corresponding decrease in volume of the other ventricle. The septal inversion is indicative of the diminished compliance of the pericardium, such that any increase in RV filling (such as that caused by a deep inspiration) results in flattening or shift of the septum to the left Page 15 of 23

: Constrictive pericarditis. Doppler schema of respirophasic changes in mitral and tricuspid inflow. Reciprocal patterns of ventricular filling are assessed on pulsed Doppler examination of mitral (MV) and tricuspid (TV) inflow. References: Copyright The McGraw-Hill Companies. All rights reserved. Page 16 of 23

Exaggerated variation in the velocity of early diastolic #lling of the two ventricles with respiration The variation is reciprocal, the tricuspid velocity increasing in inspiration and the mitral velocity decreasing. The reciprocal ventricular variation re#ects ventricular interdependence Velocity-encoded CMR typically shows a restrictive filling pattern with an enhanced early filling, and decreased or absent late filling Real-time velocity CMR is a potential alternative to echo-doppler to assess the effects of respiration on cardiac filling, though ideally slice-tracking techniques are needed to compensate for through-plane motion Page 17 of 23

References: Constrictive pericarditis in the modern era: novel criteria for diagnosis in the cardiac catheterization laboratory. Talreja DR, Nishimura RA, Oh JK, Holmes DR. J Am Coll Cardiol. 2008 Jan 22;51(3):315-9. Equilibration of diastolic pressures Nearly equal levels of diastolic pressure in all chambers of the heart are a hallmark of constrictive pericarditis This re#ects the usually symmetrical pathological process around the entire heart Page 18 of 23

: RA - right atrium; RV - right ventricle; PA - pulmonary artery; PCWP - pulmonary capillary wedge pressure Diagnosis The first test should be plain posteroanterior (PA) and lateral chest radiography. If the results show characteristic pericardial calcification, the diagnosis of constrictive pericarditis is essentially established. If the plain radiographic findings are negative and clinical suspicion of constrictive pericarditis is present, echocardiography with an evaluation of Doppler inflow velocities of the mitral and tricuspid valves during inspiration and expiration should be performed. Ventricular interdependence is present in all patients with significant constrictive physiologies. Echocardiography is limited in its ability to detect pericardial thickening Both spin echo CMR and CT are superior to echocardiography in measuring pericardial thickness but CMR has the additional advantage of permitting assessment of haemodynamic impairment. CT is superior to MRI in detecting calcification But ultimately the diagnosis of constriction is one of altered physiology Therefore, cine images demonstrating altered hemodynamics are a more direct means of confirming the diagnosis MR imaging is the preferred technique for differentiation between constrictive pericarditis and restrictive cardiomyopathy Page 19 of 23

Differentiating Constrictive Pericarditis from Restrictive Cardiomyopathy Their clinical presentation and course overlap in many respects. Because treatment is radically different, distinguishing constrictive pericarditis from restrictive cardiomyopathy is extremely important. Hemodynamic and Imaging Features of Constrictive Pericarditis Compared with Restrictive Cardiomyopathy Constriction Restriction Equal right-left side filling Present pressures Left at least 3-5 mm Hg > right Respiratory variation in left- Exaggerated right pressures/flows Normal Ventricular wall thickness Normal Usually increased Atrial size Possible LA enlargement Biatrial enlargement Septal "bounce" Present Absent Pericardial thickness Increased Normal Page 20 of 23

: Patient who has systemic amyloidosis and cardiac involvement. Cine imaging shows thickened LV walls with preserved systolic function. Bilateral pleural fuid. Management Constrictive pericarditis is a progressive disease. With the exception of patients with transient constrictive pericarditis, surgical pericardiectomy is the only definitive treatment. Transient constriction should be suspected in patients presenting relatively earlier after cardiac surgery or with relatively rapid development of symptoms. Such patients can be monitored for several months to look for spontaneous improvement. They may respond to a course of corticosteroids, and there is little to lose by managing them in this way. Pericardiectomy involves radical excision of as much of the parietal pericardium as possible. Hemodynamic and symptomatic improvement is achieved in some patients immediately after operation. In others symptomatic improvement may be delayed for weeks to months. Page 21 of 23

From 70 to 80 percent of patients remain free from adverse cardiovascular outcomes at 5 years, and 40 to 50 percent at 10 years after pericardiectomy. Conclusion Despite a better understanding of the pathophysiologic basis of the imaging findings in constrictive pericarditis and the recent advent of MRI, which has dramatically improved the morphologic and dynamic evaluation of the pericardium, the diagnosis of constrictive pericarditis remains a challenge in many cases. Personal Information 1 Bruno M Graça, MD 1 Paulo J Donato, MD 2 Maria J Ferreira, MD PhD 1 Filipe Caseiro-Alves, MD PhD Faculdade de Medicina Da Universidade de Coimbra Hospitais da Universidade de Coimbra Coimbra, Portugal 1- Clínica Universitária de Radiologia 2- Clínica Universitária de Cardiologia References Cardiovascular magnetic resonance in pericardial diseases. Bogaert J, Francone M. J Cardiovasc Magn Reson. 2009 May 4;11(1):14. Page 22 of 23

Differential diagnosis of restrictive cardiomyopathy and constrictive pericarditis. Hancock EW. Heart. 2001 Sep;86(3):343-9. Role of echocardiography in the diagnosis of constrictive pericarditis. Dal-Bianco JP, Sengupta PP, Mookadam F, Chandrasekaran K, Tajik AJ, Khandheria BK. J Am Soc Echocardiogr. 2009 Jan;22(1):24-33; Imaging features of constrictive pericarditis: beyond pericardial thickening. Napolitano G, Pressacco J, Paquet E. Can Assoc Radiol J. 2009 Feb;60(1):40-6. Epub 2009 Apr 10. Page 23 of 23