In some cases, restrictive cardiomyopathy may be confused with constrictive pericarditis, a condition in which the layers of the pericardium (the sac that surrounds the heart) become thickened, calcified and stiff. 229–30, 232–3 passim. 23. J Clin Invest 2002; 109: 357. Circulation 1989; 79: 357. However, significant respiratory variation of mitral, tricuspid, pulmonary, and hepatic flows occurs only with constriction. Am J Cardiol 1980; 45: 378. Patients with restrictive cardiomyopathy typically exhibit HF symptoms, such as dyspnea and fatigue. Palka P., Lange A., Donnelly J.E.et al. N Engl J Med 2005; 352: 362. 31. A T2* time <20 ms has been associated with reduced LV function (85). Richardson P., McKenna W., Bristow M.et al. Distinguishing a restrictive cardiomyopathy from constrictive pericarditis is often problematic. 112. In infiltrative disorders, abnormal deposits occur in the interstitial space, whereas in storage disorders, deposits occur within the cell. This paper reviews this latter group. Am J Cardiol 1989; 64: 1069. 17. J Mol Cell Cardiol 2008; 44: 891. Constrictive Pericarditis Versus Restrictive Cardiomyopathy? : "Constrictive pericarditis versus restrictive cardiomyopathy: a reappraisal and update of diagnostic criteria". : "Differentiation of constrictive pericarditis from restrictive cardiomyopathy: assessment of left ventricular diastolic velocities in longitudinal axis by Doppler tissue imaging". Weller R.J., Weintraub R., Addonizio L.J.et al. Echocardiography, CMR, and/or invasive catheterization can assess these pathophysiological changes (109,110). AL = amyloid light-chain; LA = left atrial; LV = left ventricular. 57. (A) Short axis and (B) 4-chamber views. Both familial and sporadic cases have been described (16,17). Chest 2005; 128: 3985. Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Pulmonary Hypertension and Venous Thromboembolism, CardioSource Plus for Institutions and Practices, Nuclear Cardiology and Cardiac CT Meeting on Demand, Annual Scientific Session and Related Events, ACC Quality Improvement for Institutions Program, National Cardiovascular Data Registry (NCDR). Utility of BNP in differentiating constrictive pericarditis from restrictive cardiomyopathy … Heart 2008; 94: 1478. A 25-year-old man with the recent onset of diabetes mellitus and congestive heart failure". 29. Klein A.L., Cohen G.I., Pietrolungo J.F.et al. Troponin may also be increased, and elevated serum levels of troponin and B-type natriuretic peptide are associated with a worse prognosis (41). 111. This condition is more frequently encountered in patients with radiation heart disease. Circulation 2003; 108: 1299. Buss S.J., Emami M., Mereles D.et al. 118. The definition of restrictive cardiomyopathies is on the basis of anatomic, histological, and physiological criteria, namely the presence of abnormal LV diastolic filling associated with intracellular or interstitial infiltration and/or fibrosis in the absence of LV dilation. Heart 1998; 79: 362. As many as 10-15% have either restrictive cardiomyopathy or constrictive pericarditis. : "Drastic Ca2+ sensitization of myofilament associated with a small structural change in troponin I in inherited restrictive cardiomyopathy". In both constrictive pericarditis and in advanced restrictive cardiomyopathy, the deceleration time of the LV early filling pulsed Doppler is short, consistent with a restrictive filling … (similar to restrictive cardiomyopathy or RV infarct) Elevated mean (CVP) pressure, prominent a and v waves, steep x and y … 77. About 90% of patients with primary amyloidosis have a monoclonal gammopathy. A Doppler echocardiography study". Echocardiographic Images Obtained From a Patient With Radiation Heart Disease Showing Calcification of the Aortic Valve and Mitral Valve Anterior Leaflet. The primary goal of treatment in cardiac amyloidosis remains relief of symptoms. A recent study reported that response to anti-inflammatory therapy is more likely to occur in patients with evidence of significant pericardial late enhancement and increased C-reactive protein and erythrosedimentation rate (104). Endomyocardial fibrosis: myocardial disease", "Clinical and echocardiographic features of hypereosinophilic syndromes", "Clinical, imaging, and pathological characteristics of left ventricular thrombus: a comparison of contrast-enhanced magnetic resonance imaging, transthoracic echocardiography, and transesophageal echocardiography with surgical or pathological validation", "Cardiac magnetic resonance imaging of eosinophilic endomyocardial disease", "Evaluation and management of the cardiac amyloidosis", "The clinical features of immunoglobulin light-chain (AL) amyloidosis with heart involvement". LV = left ventricular; RCM = restrictive cardiomyopathy. T1 - Constrictive pericarditis and restrictive cardiomyopathy. Although the etiology of HFpEF is most commonly related to long-standing hypertension and atherosclerosis, a significant number of suspected HFpEF patients have a restrictive cardiomyopathy … Chest x-ray in patients with constrictive pericarditis may show pleural effusions without significant alveolar edema and biatrial enlargement. 41. : "Differentiation between restrictive cardiomyopathy and constrictive pericarditis by early diastolic Doppler myocardial velocity gradient at the posterior wall". Multiple glycogen storage diseases including Fabry disease, Pompe’s disease, and lysosome-associated membrane protein disorders may result in cardiac involvement typically with ECG and echocardiographic features similar to that seen in hypertrophic cardiomyopathy. LV relaxation, seen as the rate of LV pressure decay in early diastole, is abnormal only in restriction. : "Hydroxychloroquine-induced restrictive cardiomyopathy". Eur Heart J 1991; 12: 224. Grizzard J.D. 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Familial and sporadic cases are both described, and in familial cases, a skeletal myopathy may also be present. Either CMR or computed tomography is essential for determining pericardial thickness, and computed tomography is highly sensitive for detecting pericardial calcification. Am J Transplant 2008; 8: 201. Familial cases are usually characterized by autosomal dominant inheritance with incomplete penetrance. Dutka D.P., Donnelly J.E., Nihoyannopoulos P.et al. : "Worsening of congestive heart failure in amyloid heart disease treated by calcium channel-blocking agents". PLoS One 2015; 10: e0116396. 50. The ECG and echocardiographic features are similar to those seen in hypertrophic cardiomyopathy (65,66). : "Differentiation of constrictive pericarditis and restrictive cardiomyopathy by Doppler echocardiography". The restrictive cardiomyopathies have been traditionally classified as primary or secondary to other diseases, such as storage or infiltrative disorders (3). Nat Clin Pract Cardiovasc Med 2008; 5: 176. On standard cine sequences (A), a large mass is seen in the basal posterolateral wall of the LV, involving the mitral valve apparatus. Siegel R.J., Shah P.K. : "Improvement of cardiac function during enzyme replacement therapy in patients with Fabry disease: a prospective strain rate imaging study". Heart 2008; 94: 384. Implantable cardioverter-defibrillators are used for prevention of SCD, but a survival benefit has not been demonstrated. "The abbreviated PR interval of Fabry's disease", "Early detection of Fabry cardiomyopathy by tissue Doppler imaging", "Gadolinium enhanced cardiovascular magnetic resonance in Anderson-Fabry disease. 95. The diminished venous return to the left heart during inspiration results in a septal shift to the left, with the opposite effect during expiration. (B) Patient with hemochromatosis. ∗ Highly sensitive findings with low specificity. Brain natriuretic peptide levels in constrictive pericarditis and restrictive cardiomyopathy. N Engl J Med 2004; 350: 469. Mavrogeni S.I., Markussis V., Kaklamanis L. "A comparison of magnetic resonance imaging and cardiac biopsy in the evaluation of heart iron overload in patients with β-thalassemia major", "Cardiovascular T2-star (T2*) magnetic resonance for the early diagnosis of myocardial iron overload", "A randomized, placebo-controlled, double-blind trial of the effect of combined therapy with deferoxamine and deferiprone on myocardial iron in thalassemia major using cardiovascular magnetic resonance", "Endomyocardial biopsy in hemochromatosis: clinicopathologic correlates in six cases", "Cardiac transplantation in patients with iron overload cardiomyopathy", "Clinical and genetic abnormalities in patients with Friedreich's ataxia". 13. © 2021 American College of Cardiology Foundation. The prognosis is better than with primary amyloidosis, with a median survival of 6 years (35). Talreja D.R., Edwards W.D., Danielson G.K. 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Circulation 1996; 93: 841. 37. 121. The reverse occurs during expiration. Case 31-1994. Approximately one-half of all patients with heart failure (HF) have preserved ejection fraction (HFpEF) (1). Eur Heart J 2001; 22: 2171. : "Clinical profile and outcome of idiopathic restrictive cardiomyopathy". Am J Cardiol 2005; 95: 535. Recent data support the benefit of implantable cardioverter-defibrillators for primary prevention of sudden cardiac death (SCD) (60). Chemotherapy and stem cell transplantation for AL amyloid may prolong survival and increase quality of life if started early. It typically presents as a systemic disorder, with infiltration also occurring in the liver, kidney, bowel, nerves, skin, and tongue. Images obtained during expiration (A and C) and during inspiration (B and D), demonstrating the changes in LV and RV volumes due to exaggerated ventricular interdependence. Severe atrial enlargement is a classic, albeit nonspecific feature. Sagristà-Sauleda J., Angel J., Sánchez A.et al. : "Implantable cardioverter-defibrillator placement in patients with cardiac amyloidosis". Transient constrictive pericarditis due to post-pericardiotomy syndrome, tuberculous, or viral pericarditis may respond to anti-inflammatory therapy (103). Biochem Biophys Res Commun 2005; 338: 1519. Patients with cardiac amyloidosis require a greater degree of scrutiny for complications of therapy than other patients. Clin Genet 2008; 73: 165. Enzyme replacement therapy with agalsidase beta has been reported to decrease LV wall thickness, decrease LV mass, and result in improved LV systolic and diastolic function (76–79). Bokhari S., Castaño A., Pozniakoff T.et al. 61. CMR = cardiac magnetic resonance; EMF = endomyocardial fibrosis; HFpEF = heart failure with preserved ejection fraction; LV = left ventricle. Anderson-Fabry disease is the most common glycogen storage disorder, affecting approximately 1 in 50,000 people. Endomyocardial biopsy shows disruption of normal muscle fiber architecture, with loss of z-lines and myosin filaments, and abundant curvilinear bodies, lysosomes, myeloid bodies, and glycogen granules located between myofibrils and perinuclear areas (61). A 25-year-old man with the recent onset of diabetes mellitus and congestive heart failure". 73. : "Morphologic spectrum of primary restrictive cardiomyopathy". Clin Genet 2004; 66: 158. : "Magnetic resonance imaging of pericardial disease and cardiac masses". : "Constrictive pericarditis in the modern era: evolving clinical spectrum and impact on outcome after pericardiectomy". Mutant TTR amyloidosis (m-TTR) is a systemic autosomal dominant disorder due to tissue deposition of various proteins, including TTR and apolipoproteins A-I and A-II (37), and is often associated with peripheral or autonomic neuropathy. Cardiac amyloidosis is an infiltrative form of restrictive cardiomyopathy and is often associated with a monoclonal gammopathy or multiple myeloma. Schoenfeld M.H., Supple E.W., Dec G.W.et al. Blood 1996; 88: 2801. HF is uncommon, although isolated atrial amyloidosis is associated with development of atrial fibrillation (40). 7. Circulation 2002; 106: 2091. Eur J Echocardiogr 2007; 8: 247. Echocardiography is crucial for staging the degree of diastolic abnormalities. : "Early detection of Fabry cardiomyopathy by tissue Doppler imaging". Nevertheless, none of these findings are sufficiently sensitive or specific. Circulation 1991; 83: 1880. Haley J.H., Tajik A.J., Danielson G.K.et al. Anand I.S., Ferrari R., Kalra G.S.et al. Pieroni M., Chimenti C., Ricci R.et al. Griffiths B.E., Hughes P., Dowdle R.et al. Pulmonary Hypertension and Venous Thromboembolism. Primary, or hereditary, hemochromatosis is a relatively common autosomal recessive disorder, affecting up to 0.8% of Caucasians, and results in increased intestinal absorption of iron (80). Another aspect of ventricular interdependence, characteristic of constrictive pericarditis in contrast to restrictive cardiomyopathy, is the discordant variation of right and left ventricular peak systolic pressure levels with respiration. 84. Constrictive pericarditis is long-term, or chronic, inflammation of the pericardium. Cardiac involvement is associated with a poor prognosis, with a median survival from diagnosis of 1 year (32). Anatomically, these patients are characterized by having normal or increased left ventricular wall thickness and normal or reduced left ventricular size with preserved left ventricular systolic function. : "Serum cardiac troponins and N-terminal pro-brain natriuretic peptide: a staging system for primary systemic amyloidosis". 4. LV inflow (A and B) and tissue Doppler myocardial velocities of the basal lateral wall (C and D) obtained from a patient with RCM (A and C) and a patient with constrictive pericarditis (B and D). Cueto-Garcia L., Reeder G.S., Kyle R.A.et al. Echocardiography may detect the presence of a thickened (>4 mm) pericardium, but is less useful than computed tomography (CT) and CMR to define the pericardial anatomy. Syed I.S., Martinez M.W., Feng D.L.et al. Pericardial calcifications are rare, occurring in 20% to 40% of constrictive cases and, more commonly, in tuberculous pericarditis (96,97). 26. : "Clinical and genetic abnormalities in patients with Friedreich's ataxia". Br J Haematol 1998; 101: 766. The presence of atrial dilation with normal ventricular chambers and a dilated inferior vena cava and hepatic veins, although nonspecific, support the diagnosis of constrictive pericarditis. : "Endomyocardial biopsy in hemochromatosis: clinicopathologic correlates in six cases". Am Heart J 1991; 122: 1431. Echocardiography to detect exaggerated respiratory interdependence and annular septal velocities may be diagnostic of pericardial constriction, but cannot determine pericardial thickness. 101. Anderson L.J., Holden S., Davis B.et al. Blood 2009; 114: 4957. Clinically, the patients present with a virtually identical constellation of signs and … 105. Dubrey S.W., Cha K., Simms R.W.et al. Advanced impairment of LV diastolic filling is invariably present. Hughes D.A., Elliott P.M., Shah J.et al. Studies of body water and sodium, renal function, hemodynamics, and plasma hormones before and after pericardiectomy". J Heart Lung Transplant 2005; 24: 486. J Am Coll Cardiol 1994; 23: 154. : "Respiratory ventricular area changes measured with real-time cardiac magnetic resonance: a new, accurate, and reproducible approach for the diagnosis of pericardial constriction". Clinically, the patients present with a virtually identical constellation of signs and symptoms. : "Glycogen storage diseases presenting as hypertrophic cardiomyopathy". 74. 53. : "Classification of amyloidosis by laser microdissection and mass spectrometry-based proteomic analysis in clinical biopsy specimens". Br Heart J 1977; 39: 399. Dubrey S.W., Cha K., Anderson J.et al. The echocardiographic features often mimic that of hypertrophic cardiomyopathy, and diastolic dysfunction is common. Int J Cardiol 1998; 64: 109. Normal (A), restrictive (B), and constrictive (C) hearts. Diagnosis and dynamic evaluation with multidetector CT". In addition, during respiration, changes in LV and RV systolic pressure are discordant. Am J Cardiol 2002; 90: 501. Circulation 2000; 102: 655. J Clin Oncol 2004; 22: 3751. At times, a calcified pericardium is seen with encasement of the heart impeding diastolic filling . Br Heart J 1984; 52: 465. Chew C.Y., Ziady G.M., Raphael M.J.et al. : "Endomyocardial biopsy in 30 patients with primary amyloidosis and suspected cardiac involvement". Bortezomib-based regimens have shown near complete remission of plasma cell dyscrasias and are now considered to be the preferred treatment option (58). and Fishbein M.C. : "Comparison of new Doppler echocardiographic methods to differentiate constrictive pericardial heart disease and restrictive cardiomyopathy". Treatment of the Restrictive Cardiomyopathies. 58. 104. Kaski J.P., Syrris P., Burch M.et al. Circulation 2011; 124: 1830. Ware S.M., Quinn M.E., Ballard E.T.et al. Home Circulation Vol. Failure to improve or recurrence of symptoms is often due to incomplete pericardiectomy, thus warranting referral to experienced cardiac surgeons. 32. Caines A.E., Kpodonu J., Massad M.G.et al. : "Pericardiectomy for constrictive pericarditis using the harmonic scalpel". : "Pitfalls in diagnosis and clinical, echocardiographic, and hemodynamic findings in endomyocardial fibrosis: a 25-year experience". As a result of pericardial encasement, patients with constrictive pericarditis exhibit exaggerated interventricular dependence and dissociation between intracardiac and intrathoracic pressures during respiration (Figure 6). 87. Sagristà-Sauleda J., Angel J., Sánchez A. Rajagopalan N., Garcia M.J., Rodriguez L. 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