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40. Leya FS, Arab D, Joyal D, Shioura KM, Lewis BE, Steen LH, Cho L.The efcacy of brain natriuretic peptide levels in differentiating constrictive pericarditis from restrictive cardiomy­opathy. J Am Coll Cardiol. 2005;45:1900–2.
41. Maisch B, Seferović PM, Ristić AD, Erbel R, Rienmüller R, Adler Y, Tomkowski WZ, Thiene G, Yacoub MH, Priori SG, Alonso Garcia MA.Guidelines on the diagnosis and management of pericardial diseases executive summary: the task force on the diagnosis and management of pericardial diseases of the European Society of Cardiology. Eur Heart J. 2004;25(7):587–610.
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43. Merlini G.AL amyloidosis: from molecular mechanisms to targeted therapies. Hematol Am Soc Hematol Educ Program. 2017;2017(1):1–12.
44. Mocumbi AO, Ferreira MB, Sidi D, etal. A population study of endomyocardial brosis in a rural area of Mozambique. N Engl J Med. 2008;359(1):43–9.
45. Muchtar E, Blauwet LA, Gertz MA.Restrictive cardiomyopathy: genetics, pathogenesis, clini­cal manifestations, diagnosis, and therapy. Circ Res. 2017;121(7):819–37.
46. Olsen CO, Tyson GS, Maier GW, Davis JW, Rankin JS.Diminished stroke volume during inspiration: a reverse thoracic pump. Circulation. 1985;72(3):668–79.
47. Olsen EGJ, CJF S. The pathogenesis of Loefer’s endomyocardial disease and its relation­ship to endomyocardial brosis. In: Yu P, Goodwin JF, editors. Progress in cardiology, vol. 8. Philadelphia: Lea & Febiger; 1979. p.281–303.
48. Olsen EGJ.Morphological overview and pathogenetic mechanism in endomyocardial brosis associated with eosinophilia. In: Olsen EGJ, Sekiguchi M, editors. Cardiomyopathy update 3. Tokyo: University of Tokyo Press; 1990. p.1–8.
49. Ruberg FL, Berk JL. Transthyretin (TTR) cardiac amyloidosis. Circulation. 2012;126(10):1286–300.
50. Schiavone WA.The changing aetiology of constrictive pericarditis in a large referral center. Am J Cardiol. 1986;58:373–5.
51. Schiavone WA, Calaore PA, Salcedo EE.Transesophageal Doppler echocardiographic dem­onstration of pulmonary venous ow velocity in restrictive cardiomyopathy and constrictive pericarditis. Am J Cardiol. 1989;63:1286–18.
52. Samuel I, Anklesaria X.Endomyocardial brosis in South India. Ind J Path Bact. 1960;3:157.
53. Smoedema JP, Katjitae I, Reuter H, Burgess L, Louw V, Pretorius M, etal. Twelve-lead elec­trocardiography in tuberculous pericarditis. Cardiovasc J South Afr. 2001;12:31–4.
54. Surawicz B, Lasseter KC.Electrocardiogram in pericarditis. Am J Cardiol. 1970;26:471–4.
55. Schoenfeld MH, Edwards WS, William GD Jr, etal. Restrictive cardiomyopathy versus con­strictive pericarditis-role of endomyocardial biopsy in avoiding unnecessary thoracotomy. Circulation. 1987;75:1012–7.
56. Schoenfeld MH.The differentiation of restrictive cardiomyopathy from constrictive pericardi­tis. Cardiol Clin. 1990;8:663–71.
57. Shaper AG. Cardiovascular disease in the tropics. II. Endomyocardial brosis. BMJ. 1972;3:743–6.
58. Shaper AG.The aaetiology of endomyocardial brosis. In: Valiathan MS.Somers K, Kartha CC (eds). Endomyocardial brosis. New Delhi: Oxford University Press, 1993: 111–120.
59. Sipe JD, Cohen AS.Review: history of the amyloid bril. J Struct Biol. 2000;130(2–3):88–98.
7 Clinical Presentation, Lab Investigations, andEndomyocardial Biopsy
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63. Spodick DH.Constrictive pericarditis. In: Spodick DH, editor. The pericardium: a comprehen­sive textbook. 1st ed. NewYork: Marcel Dekker, Inc; 1997. p.214–59.
64. Spodick DH.Diagnostic electrocardiographic sequences in acute pericarditis: signicance of PR segment and PR vector changes. Circulation. 1973;48:575–80.
65. Spodick DH. Infective pericarditis. Etiologic and clinical spectra. In: Reddy PS, Leon DF, Shaver JA, editors. Pericardial disease. NewYork: Raven Press; 1982. p.307–32.
66. Spodick DH.Pericardial rub: prospective, multiple observer investigation of pericardial fric­tion in 100 patients. Am J Cardiol. 1975;35:357–62.
67. Spodick DH.The normal and diseased pericardium: current concepts of pericardial physiol­ogy, diagnosis and treatment. J Am Coll Cardiol. 1983;1:240–51.
68. Spodick DH. The pericardium. A comprehensive textbook, vol. 233. New York, NY: M.Dekker; 1997. p.464.
69. Spodick DH. Pericardial diseases. In: Braunwald E, Zipes DP, Libby P, editors. Heart dis­ease: a textbook of cardiovascular medicine, vol. 6. Philadelphia, PA: WB Saunders Co; 2001. p.1823–70.
70. Spodick DH.Pericardial macro-and microanatomy: a synopsis. In: Spodick DH, editor. The pericardium: a comprehensive textbook. NewYork: Marcel Dekker; 1997. p.7–14.
71. Troughton RW, Asher CR, Klein AL.Pericarditis Lancet. 2004;363:717–27.
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73. Tharakan J, Bohora S. Current p erspe ctive on endomyocardial brosis. Curr Sci. 2009;97:405–10.
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75. Uemura H, Ho SY, Devine WA, Kilpatrick LL, Anderson RH.Atrial appendages and venoatrial connections in hearts with patients with visceral heterotaxy. Ann Thorac Surg. 1995;60:561–9.
76. Valiathan MS, Balakrishnan KG, Kartha CC.Endomyocardial brosis. In: Ahuja MMS, editor. Advances in clinical medicine. New Delhi: Churchill Livingstone; 1991. p.125–43.
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78. Valiathan MS, Shyamkrishnan KG. Surgical treatment of endomyocardial brosis: Kerala experience. In: Valiathan MS, Somers K, Kartha CC, editors. Endomyocardial brosis. New Delhi: Oxford University Press; 1993. p.220–7.
79. Valiathan MS, Kartha CC, Eapen JT, Dang HS, Sunta CM.A geochemical basis for endomyo­cardial brosis. Cardiovasc Res. 1989;23:647–8.
80. Vrana JA, Theis JD, Dasari S, etal. Clinical diagnosis and typing of systemic amyloido­sis in subcutaneous fat aspirates by mass spectrometry-based proteomics. Haematologica. 2014;99(7):1239–47.
81. Wood P.Chronic constrictive pericarditis. Am J Cardiol. 1961;7:48–61.
82. Wood JA.Tuberculous pericarditis; a study of fortyone cases with special reference to progno­sis. Am Heart J. 1951;42:737–45.
83. Wood DE, Crumbley AJ, Pereira NL. Reversible left ventricular dysfunction simulating a myocardial infarction after pericardiectomy. Heart. 2002;88:183–4.
84. Wise DE, Conti CR. Constrictive pericarditis. In Pericardial Diseases (Ed. Spodick DH). F.A.Davis, Philadelphia. Cardiovasc Clin. 1976;7(3):197–210.
85. White PD.Chronic constrictive pericarditis. Circulation. 1951;4(2):288–94.
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86. Wychulis AR, Connolly DC, McGoon DC. Surgical treatment of pericarditis. J Thorac Cardiovasc Surg. 1971;62:608–17.
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88. Westermark P, Benson MD, Buxbaum IN, etal. A primer of amyloid nomenclature. Amyloid Int J Exp Clin lnvestig. 2007;14(3):179–83.
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7 Clinical Presentation, Lab Investigations, andEndomyocardial Biopsy
Chapter 8
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Imaging Studies andHaemodynamics inChronic Constrictive Pericarditis
8.1 Chest Radiography
Chest radiography ndings are often suggestive of chronic constrictive pericarditis and sometimes help to differentiate it from restrictive cardiomyopathy [112, 113]. Typically the cardiothoracic ratio is normal and lung elds are clear in patients with chronic constrictive pericarditis. However, enlarged cardiac silhouette can be seen in effusive-constrictive pericarditis, co-existing pericardial effusion, or in the pres­ence of extracardiac masses [5762, 113]. Pleural effusions are common and can be an initial sign in 40% to 60% of cases [4143, 137, 159, 168184].
Findings suggestive of pulmonary tuberculosis have been reported in 30% to 70% of cases on chest roentgenogram. Patients with pulmonary venous congestion due to elevated left-sided lling pressures exhibit signs of in the form of cephaliza­tion or equalization. In the published literature, 47% of cardiac silhouette are nor­mal, 16% show mild, and 37% show moderate to massive enlargement (especially in cases of effusive-constrictive pericarditis) [5762, 113]. In 2001, Breen and asso­ciates reported evidence of occasional right-atrial and superior caval venous dilation [8]. On uoroscopic examination, the cardiac pulsation may be diminished or absent [104].
Presence of calcication over the right atrium, right ventricle, as well as atrio­ventricular grooves on lateral chest roentgenogram suggest tuberculosis [35]. Other features strongly suggestive of chronic constrictive pericarditis are presence of Egg shell calcication, Cocoon calcication, and amorphous calcication in the atrio­ventricular grooves (Figs.8.1, 8.2, 8.3, 8.4, and 8.5) [1825, 59, 63, 104, 105, 181,
185]. Although calcication along the cardiac silhouette suggest diagnosis of
chronic constrictive pericarditis, its presence is not always conrmative since calci­cation can also occur without cardiac compression [114]. As stated by Lorell, “A calcied pericardium is not necessarily a constricted one” [106, 186].
Ltd. 2023 U. K. Chowdhury, L. K. Sankhyan, Surgical Treatment of Chronic Constrictive Pericarditis, https://doi.org/10.1007/978-981-99-5808-5_8
89© The Author(s), under exclusive license to Springer Nature Singapore Pte
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8 Imaging Studies andHaemodynamics inChronic Constrictive Pericarditis
a
Fig. 8.1 Frontal (a) and left lateral (b) chest radiographs reveal thick, plaque- like calcications (arrowheads) over the diaphragmatic surface and free walls of both ventricles and along the atrio­ventricular groove
b
Fig. 8.2 Frontal (a) and left lateral (b) chest radiographs reveal plaque like calcications (arrow­heads) over the diaphragmatic surface, anterior surface of the right ventricle and along atrioven­tricular groove
According to study by Ling and associates, among 135 patients undergoing sur­gery for chronic constrictive pericarditis, 36 had radiological signs of pericardial calcication [104, 105]. In their study, 97% had calcication over the inferior/dia­phragmatic surface of the heart, 76% had calcication on the anterior surface of the heart over the right ventricle; and 62% had calcium deposits over the atrioventricu­lar groove [104, 105]. McCaughan and associates reported 40% incidence of calci­cation in their study group [112]. Bertog and associates found pericardial calcication in 55% of patients with idiopathic chronic constrictive pericarditis, in 10% of patients who were surgically treated, and in 6.7% with radiation-induced chronic constrictive pericarditis. Patchy thin areas of calcication is suggestive of adhesive pericarditis [9].
8.1 Chest Radiography
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Fig. 8.3 Frontal chest radiograph shows plaque like calcications (arrowheads) over the diaphragmatic surface of the heart
Fig. 8.4 Lateral chest roentgenogram reveals extensive circumferential pericardial calcication (indicated by white arrows)
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In our cumulative experience on 547 patients undergoing pericardiectomy for chronic constrictive pericarditis, chest roentgenograms revealed pericardial calci­cation in 37%, pleural effusion in 40%, and pulmonary inltrates in 16.6% of
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Fig. 8.5 Chest radiograph showing cardiac size within normal limits, pericardial calcication, biatrial enlargement and blunting of both CP angles
8 Imaging Studies andHaemodynamics inChronic Constrictive Pericarditis
patients [1825]. Among them, 22.2% had calcication distributed over the anterior and inferior surface of the heart, 10% had calcium around the atrioventricular groove and “calcium cocoon” in 14.8% of patients. However there were no cases of mitral annular calcication (Figs.8.1, 8.2, 8.3, 8.4, and 8.5) [1825].
8.1.1 Overview ofSpecic Imaging Modalities
Pericardial non-invasive multimodality imaging can be performed using echocar­diography, tissue Doppler imaging, computed tomography scan (CT), cardiac mag­netic resonance (CMR), radionuclide ventriculography, and/or Positron Emission Tomography. Primary investigation for diagnosis is echocardiography. Further investigations are done when echocardiography is non-diagnostic, or if additional information is required such as the degree of pericardial thickness, inammation, or calcication [26, 815, 1835, 37, 4056, 6578, 80103, 110, 111, 114167,
179, 180, 186, 187, 190194, 196205, 213218, 220222, 232237, 239, 240].
Although there exists no single echocardiographic nding to conrm the diagno­sis, a normal study denitely rules out the diagnosis of chronic constrictive pericar­ditis. Echocardiography remains the initial investigation of choice because of its wide spread availability, portability, low risk, and comparatively high resolution [40, 115]. Several echocardiographic parameters have been proposed to differenti­ate chronic constrictive pericarditis from restrictive cardiomyopathy and endomyo­cardial brosis. These parameters are measured on M-mode, 2D images, and Doppler imaging [817, 8592, 104111, 145147, 187, 188, 205218, 232239].
The ndings on Doppler imaging include diastolic ow reversal in the hepatic veins accentuated with expiration, rapid early (E) diastolic lling (restrictive type of
8.2 Echocardiography
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left ventricular lling pattern), increased tissue Doppler velocity of the medial mitral annulus and reciprocal respiratory variation of mitral and tricuspid inow Doppler velocities. About 15% of patients with chronic constrictive pericarditis have a negative, equivocal or technically conrmed echocardiogram [239].
Limitation of echocardiography are inability to perform a comprehensive study due to various patient factors and limited ability to assess pericardial thickness and pericardial calcication. Multimodality imaging help to conrm diagnosis in chal­lenging cases for echocardiography or suspected false negative cases on echocar­diogram and acquire more information on morphological features [127133].
Compared to cardiac magnetic resonance, computed tomography has higher spa­tial resolution, and is superior in visualizing pericardial thickness and calcication [80, 160, 189, 213]. Computed tomography is also useful for patients with magnetic resonance non-compatible implanted devices. Other advantages are ability to evalu­ate for pulmonary embolus and coronary artery disease.
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8.2 Echocardiography
(a) Evaluation of the pericardial morphology
1. Pericardial thickening and calcication
The characteristic feature of pericardial thickening on echocardiogram is parallel motion of the epicardium and parietal pericardium separated by approximately 1 mm wide relatively echo-free space [8591, 93, 94]. The best view to assess motion between the pericardial layers is the four-chamber subcostal view. Although assessment of pericardial thickness and calcication using transthoracic echocar­diography is inferior to computed tomography and cardiac magnetic resonance, assessment with transesophageal echocardiography (TEE) strongly correlates with cardiac tomography [26, 27, 79, 8591, 93, 94, 104, 105, 190].
Several investigators have demonstrated a pericardial thickness of more than 3mm obtained using transesophageal echocardiography having 95% sensitivity and 86% specicity for detection of the thickened pericardium [79, 104, 105]. The 2003 ACC/AHA/ASE Task Force recommends transesophageal echocardiography for assessment of pericardial thickness to support the diagnosis of chronic constrictive pericarditis (class IIB) [26, 27]. Echocardiography evaluation may be inadequate in assessing pericardial thickness anterior to the right ventricle and near the right atrio­ventricular groove [26, 27, 39, 85].
2. Pericardial tethering
In the presence of pericardial adhesions, normal relative motion of the visceral pericardium covering the heart inside the parietal pericardium is lost. It is difcult to demonstrate this pericardial tethering by two-dimensional echocardiography. However, tissue Doppler imaging and myocardial strain imaging offer diagnostic information with a reported sensitivity and specicity of 88.8% and 94.8% respec­tively [6269, 107, 160, 191203, 221, 222].
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8 Imaging Studies andHaemodynamics inChronic Constrictive Pericarditis
Unlike echo Doppler methods and tissue Doppler imaging which rely mostly on longitudinal motion data, speckle tracking echocardiography can examine several components or planes (i.e. radial, longitudinal and circumferential) in a single data set. It can assess the torsional deformation of the myocardium in addition to strain and strain rate. Speckle tracking echocardiography can also quantify the rocking or swinging motion of the heart by measuring septal-to-lateral rotation displacement (SLRD) [4749, 70, 71, 8591, 93, 94, 134, 135, 138144, 148, 187200, 220].
“Strain reversus” is a feature characterised by diminished negative peak systolic strain in the free walls of left and right ventricles when compared with septal peak systolic strain. It is seen when diseases affecting myocardial strain values are absent. The sensitivity and specicity of left ventricular lateral wall strain to left ventricular septal wall strain ratio<0.96 are 89% and 96% respectively [7]. The left and right ventricular strain abnormalities are noted to cease following surgery [93]. A charac­teristic pattern observed following pericardiectomy is impaired early diastolic strain rate of left atrial superior and lateral walls compared with the septal wall [116].
(b) Haemodynamic evaluation
Usually patients with chronic constrictive pericarditis have normal ventricular dimensions with normal ejection fraction. However, deterioration occurs in constrictive- restrictive disease and long-standing cases of chronic constrictive peri­carditis with myocardial brosis/atrophy [161163].
Doppler echocardiography relies on the presence of distinct atrial and ventricular lling characteristics in relation to the respiratory cycle [10, 70, 71, 138144]. In about 50% of patients with chronic constrictive pericarditis, mitral inow velocity decreases as much as 25% and tricuspid inow velocity increases considerably with the rst heart beat after inspiration. In contrast, respiratory variation in patients with restrictive cardiomyopathy does not differ signicantly from normal.
Echocardiographic correlates of the haemodynamic abnormalities of chronic constrictive pericarditis include (i) dilation and blunted respiratory uctuation of the hepatic veins and inferior caval vein, (ii) left atrial enlargement, (iii) abrupt displace­ment of the interventricular septum during early diastole (septal bounce), (iv) atten­ing of the posterior wall of the left ventricle, and (v) increased hepatic ow reversal with expiration. These ndings reect the dissociation of the intracardiac and intra­thoracic pressures and interventricular interaction. However, these ndings of abnor­mal ventricular diastolic lling are not sensitive and lack specicity to be utilised for clinical evaluation [2, 9, 10, 26, 27, 39, 4749, 6470, 138144, 191203].
(c) Abnormalities of interventricular septal motion
Due to constrictive pericarditis, the intracardiac pressures are dissociated from changes in intrathoracic pressure during respiratory cycle. Respirophasic ventricu­lar septal shift (VSS) is a characteristic diagnostic feature of constrictive pericarditis on echocardiogram [2, 32, 46, 8590, 122126]. Ventricular interdependence can be seen as diastolic interventricular septal shift posteriorly into the left ventricle with inspiration, reecting left ventricular under lling, and anteriorly into the right
8.2 Echocardiography
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ventricle with expiration, reecting recovery of left ventricular lling in the para­sternal long axis view.
Dissociation of intracardiac and intrathoracic pressure in chronic constrictive pericarditis means that with inspiration, the intrathoracic pressure declination is not not fully transferred to the intracardiac chambers since they are isolated by a con­stricting pericardial cover. Consequently, left ventricular diastolic lling is reduced. In chronic constrictive pericarditis, a relatively xed cardiac volume, coupled with an increased right ventricular diastolic lling during inspiration, results in a left­ward shift of the interventricular septum. The opposite change occurs during expira­tion [140, 141]. Although non-specic, another common nding in chronic constrictive pericarditis is a septal shudder or bounce, which is an abrupt displace­ment of the interventricular septum in early diastole during each cardiac cycle [40]. Septal shudder corresponds to the dip and plateau sign observed on the right ven­tricular pressure tracing.
The early rapid lling phase causes abrupt increase in pressure in the right ven­tricle, initially pushing the interventricular septum towards the left ventricle. When the right ventricular volume reaches its limit set by the non-compliant constrictive pericardium, there is an abrupt halt in the rise of pressure and coincides with the rise of pressure in the left ventricle, resulting in an abrupt anterior septal motion. However a septal shudder should not be misinterpreted as ventricular septal shift. The latter is a more specic diagnostic nding. Other characteristic features of interventricular septal motion are early diastolic high-velocity on tissue Doppler M-mode and polyphasic septal uttering motion on short-axis pulsed-wave tissue Doppler imaging. (Figs.8.6 and 8.7) [199].
(d) Inferior caval vein size and hepatic vein ow pattern
Constrictive pericarditis is almost always associated with a dilated inferior caval vein [120126]. Diastolic ow reversal of hepatic venous ow during expiration can be appreciated on pulsed Doppler interrogation. Velocity of hepatic vein diastolic reverse ow/forward velocity gives the diastolic expiratory hepatic vein ow rever­sal ratio.(Figs. 8.8 and 8.9) [120126, 138144, 232].
A combination of ventricular septal shift with medial e′ ≥9 cm/s and diastolic hepatic vein expiratory ow reversal ratio of 79% has a sensitivity and specicity of 64% and 97% respectively, while a combined evaluation of ventricular septal shift and medial e′ ≥9 cm/sec has a sensitivity and specicity of 87% and 91%, respectively [232].
In chronic obstructive pulmonary disease, there may be wide uctuations in intrathoracic pressure with similar patterns of transmitral and transtricuspid Doppler inow patterns. However, characteristic ndings in obstructive pulmonary disease which help in differentiating from constrictive pericarditis are: lower E/A, pro­longed deceleration time, and signicant increase in inspiratory systolic ow in pulsed Doppler evaluation of superior caval vein while there is prominent diastolic ow in constrictive pericarditis [10, 161].