Severe atrial enlargement is a classic, albeit nonspecific feature. 14. J Mol Cell Cardiol 2008; 44: 891. : "Early detection of Fabry cardiomyopathy by tissue Doppler imaging". (B) Patient with hemochromatosis. 70. 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. Saraiva M.J.: "Sporadic cases of hereditary systemic amyloidosis". Diuretic therapy is necessary for volume control; however, there is an increased likelihood of hypotension and renal insufficiency in these patients. Restrictive cardiomyopathy … Secondary restrictive cardiomyopathies are subclassified as infiltrative, noninfiltrative, and storage disorders. AU - Mookadam, Farouk. CMR provides additional diagnostic information by detecting subendocardial fibrosis. : "Endomyocardial biopsy in 30 patients with primary amyloidosis and suspected cardiac involvement". Koeppen A.H., Ramirez R.L., Becker A.B.et al. Cecchetti G., Binda A., Piperno A.et al. J Am Coll Cardiol 1996; 27: 108. 103. Br Heart J 1984; 52: 465. : "Outcome of idiopathic restrictive cardiomyopathy in children". Constrictive pericarditis versus restrictive cardiomyopathy… 48. The use of this drug is restricted by its limited availability and elevated cost. Impaired LV relaxation may be detected by Doppler echocardiography before the onset of symptoms. 13. Both the myocardium and liver (L) show decrease signal intensity (dark) compared with the trapezius (T) skeletal muscle (grey). Iglesias Cubero G., Rodriguez Reguero J.J. and Rojo Ortega J.M. Circulation 1996; 93: 2007. Troponin may also be increased, and elevated serum levels of troponin and B-type natriuretic peptide are associated with a worse prognosis (41). In constrictive pericarditis, the signal intensity of the thickened pericardium was similar or decreased compared with that of the myocardium. 45. : "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". Murtagh B., Hammill S.C., Gertz M.A.et al. 20. : "Worsening of congestive heart failure in amyloid heart disease treated by calcium channel-blocking agents". Cardiac biopsy shows abnormal deposits of granular, yellow-gray material within the sarcoplasm of the myocytes under light microscopy and Prussian blue stains positive for iron, which is diagnostic of iron overload (87). Sagristà-Sauleda J., Angel J., Sánchez A. Rajagopalan N., Garcia M.J., Rodriguez L. "Comparison of new Doppler echocardiographic methods to differentiate constrictive pericardial heart disease and restrictive cardiomyopathy", "Differentiation between restrictive cardiomyopathy and constrictive pericarditis by early diastolic Doppler myocardial velocity gradient at the posterior wall", "Differentiation of constrictive pericarditis from restrictive cardiomyopathy: assessment of left ventricular diastolic velocities in longitudinal axis by Doppler tissue imaging". : "Mechanism underlying Kussmaul's sign in chronic constrictive pericarditis". Parasternal long-axis (A), short-axis (B), and 4-chamber (C) views. Circulation 2000; 101: 2490. and Bing O.H. : "Primary restrictive cardiomyopathy: clinical and pathologic characteristics". N Engl J Med 2002; 346: 1818. Uchida T., Bando K., Minatoya K.et al. Heart 1999; 81: 141. 11. 21. On echocardiography studies, tissue Doppler typically demonstrates reduced early diastolic myocardial velocity (e′). Internationaf Journal of Cardiology, 31 (1991) 319-328 319 0 1991 Elsevier Science Publishers B.V. 0167-5273/91/%03.50 ADONIS 016752739100143W CARD10 01261 Constrictive pericarditis versus restrictive cardiomyopathy: the role of Doppler echocardiography in differential … : "Variant-sequence transthyretin (isoleucine 122) in late-onset cardiac amyloidosis in black Americans". : "Left ventricular structure and function in transthyretin-related versus light-chain cardiac amyloidosis". However, significant respiratory variation of mitral, tricuspid, pulmonary, and hepatic flows occurs only with constriction. : "Hydroxychloroquine-induced restrictive cardiomyopathy". Am J Med 1983; 75: 618. Schneider U., Jenni R., Turina J.et al. 27. Caines A.E., Kpodonu J., Massad M.G.et al. Ann Thorac Surg 2001; 72: 924. 38. Patients who received mediastinal radiation for malignancy develop a number of radiation-related complications including constrictive pericarditis and myocardial fibrosis, which may lead to a restrictive-type cardiomyopathy. Early in the course of the disease, ECG and echocardiographic findings resemble those of hypertrophic cardiomyopathy, including symmetric LV hypertrophy, abnormal myocardial relaxation, and LV outflow obstruction (90). N Engl J Med 2005; 352: 362. and Cohen G.I. Cardiac involvement may result in supraventricular arrhythmias, such as atrial fibrillation (83). Dr. Garcia has reported that he has no relationships relevant to the contents of this paper to disclose. † Highly specific findings with low sensitivity. : "Endomyocardial biopsy-proven light chain amyloidosis (AL) without echocardiographic features of infiltrative cardiomyopathy". A Doppler echocardiography study". Evidence for a disease specific abnormality of the myocardial interstitium". This is the case of a patient who presented with severe right-sided heart failure due to diastolic dysfunction that caused a dilemma of differential diagnosis between restrictive cardiomyopathy and constrictive pericarditis. : "Cyclophosphamide-bortezomib-dexamethasone (CyBorD) produces rapid and complete hematologic response in patients with AL amyloidosis". : "99mTc-pyrophosphate scintigraphy for differentiating light-chain cardiac amyloidosis from the transthyretin-related familial and senile cardiac amyloidoses". 102. Failure to improve or recurrence of symptoms is often due to incomplete pericardiectomy, thus warranting referral to experienced cardiac surgeons. Friedreich's ataxia is an autosomal recessive neurodegenerative disorder caused by a mutation of the frataxin gene that manifests in the second to third decade of life with diabetes mellitus, ataxia, and HF (89). Familial and sporadic cases are both described, and in familial cases, a skeletal myopathy may also be present. 100. Amaki M, Savino J, Ain DL, et al. Davis J., Wen H., Edwards T.et al. Quarta C.C., Solomon S.D., Uraizee I.et al. 66. Am J Cardiol 2002; 90: 501. Hypereosinophia syndromes, associated with parasitic infection or hematological malignancy result in a similar presentation with obliterative apical fibrosis and thrombus. Table 1. In contrast to CT, even significant foci of calcification can be missed on CMR. The magnitude of their variability will depend, however, on the severity of constriction, the volume status of the patient, and the inspiratory effort during the study acquisition. Ghersin E., Lessick J., Litmanovich D.et al. : "Fabry disease, an under-recognized multisystemic disorder: expert recommendations for diagnosis, management, and enzyme replacement therapy". Arad M., Benson D.W., Perez-Atayde A.R.et al. : "Cardiac manifestations of Anderson-Fabry disease: results from the international Fabry outcome survey". : "Clinical and molecular studies of a large family with desmin-associated restrictive cardiomyopathy". CMR may show a mid-myocardial pattern of late enhancement of the basal inferolateral wall, or a more diffuse pattern in patients with severe LVH (71). 5 Restrictive cardiomyopathy versus constrictive pericarditis: role of endomyocardial biopsy in avoiding unnecessary thoracotomy. Heart sounds may be reduced. Instead, they present with symptoms of heart failure and so this can often be a challenge for even very skilled sonographers to identify the subtle differences between Restrictive Cardiomyopathy and Constrictive Pericarditis. Worldwide, endomyocardial fibrosis is probably the most common cause of restrictive cardiomyopathy, but is rare in the United States. Chest x-ray in patients with constrictive pericarditis may show pleural effusions without significant alveolar edema and biatrial enlargement. Nat Clin Pract Cardiovasc Med 2008; 5: 176. : "Left ventricular systolic and diastolic function after pericardiectomy in patients with constrictive pericarditis: Doppler echocardiographic findings and correlation with clinical status". There is no specific treatment for this condition other than standard HF drugs. 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". : "Serum cardiac troponins and N-terminal pro-brain natriuretic peptide: a staging system for primary systemic amyloidosis". 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. Jacobson D.R., Pastore R.D., Yaghoubian R.et al. Chemotherapy and stem cell transplantation for AL amyloid may prolong survival and increase quality of life if started early. About one-half of the patients with congestive heart failure have preserved left ventricular ejection fraction (HFpEF). All rights reserved. Cardiol Clin 2007; 25: 111. vi. Despite clinical, noninvasive, and hemodynamic assessment, the differentiation of restrictive cardiomyopathy from constrictive pericarditis remains difficult in a small subset of patients that present with mixed constrictive/restrictive physiology. : "Senile systemic amyloidosis, cerebral amyloid angiopathy, and dementia in a very old Finnish population". 87. : "CT and MRI findings in a child with constrictive pericarditis". DeValeria P.A., Baumgartner W.A., Casale A.S.et al. 42. McCarthy G.M., Crowe J., McCarthy C.J.et al. 12. J Heart Lung Transplant 2005; 24: 486. Constrictive pericarditis … Patients with Constrictive Pericarditis do not present for typical pericarditis symptoms. : "Clinical profile and outcome of idiopathic restrictive cardiomyopathy". Katritsis D., Wilmshurst P.T., Wendon J.A.et al. 122. 30. Cardiac amyloid deposition also occurs in isolated atrial and dialysis-related (β2 microglobulin) amyloidosis. Diagnosis of systemic amyloidosis may be attempted with rectal submucosal or abdominal fat pad biopsy. N Engl J Med 1997; 336: 466. J Am Coll Cardiol 1999; 33: 1182. Case records of the Massachusetts General Hospital: "Weekly clinicopathological exercises. Weller R.J., Weintraub R., Addonizio L.J.et al. Five major clinical types of cardiac amyloidosis are recognized, each associated with a different precursor protein. : "Senile systemic amyloidosis presenting with heart failure: a comparison with light chain-associated amyloidosis". : "Diagnostic role of Doppler echocardiography in constrictive pericarditis". PERICARDITIS: Pericarditis is inflammation with fluid accumulation and reduced cardiac output. : "Electrocardiography and Doppler echocardiography in secondary (AA) amyloidosis". CMR has high accuracy in the diagnosis of myocardial iron overload. : "Magnetic resonance imaging of pericardial disease and cardiac masses". 56. Immunohistochemical staining using specific antibodies can discriminate between the different types of amyloidosis (52). 43. Atrial Fibrillation/Supraventricular Arrhythmias, Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Pulmonary Hypertension and Venous Thromboembolism. EMF is probably the most common cause of restrictive cardiomyopathy, affecting an estimated 12 million people worldwide (22). Owan T.E., Hodge D.O., Herges R.M.et al. Recent data support the benefit of implantable cardioverter-defibrillators for primary prevention of sudden cardiac death (SCD) (60). Case 31-1994. Cotroneo J., Sleik K.M., Rene Rodriguez E.et al. Kebbel A. and Röcken C.: "Immunohistochemical classification of amyloid in surgical pathology revisited". Echocardiography may show endomyocardial thickening, ventricular apical obliteration, and involvement of the posterior mitral leaflet (28). EMF is endemic in tropical and subtropical Africa, Asia, and South America, but is also occasionally encountered outside the tropics (23–25). Endomyocardial biopsy may be useful to avoid unnecessary thoracotomy in patients with significant myocardial involvement who may not respond to pericardiectomy (122). : "The usefulness of three-dimensional multidetector computed tomography to delineate pericardial calcification in constrictive pericarditis". : "Constrictive pericarditis in 26 patients with histologically normal pericardial thickness". Cardiac amyloidosis is an infiltrative form of restrictive cardiomyopathy and is often associated with a monoclonal gammopathy or multiple myeloma. Mitral 'annulus reversus ' to diagnose and therefore benefits from a good understanding of the myocardial interstitium.! D.Et al a median survival of 6 years ( 35 ) water and sodium, renal function, hemodynamics and! 40 ), Zamorano J.L.et al Pfeffer M.A., Benson D.W., Perez-Atayde A.R.et al ( 22.. Microscopy ( 73 ) common in patients with cardiac amyloidosis, cerebral angiopathy! 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