Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Restrictive Cardiomyopathy Workup

  • Author: Alan Vainrib, MD; Chief Editor: Henry H Ooi, MD, MRCPI  more...
 
Updated: Dec 18, 2014
 

Approach Considerations

Laboratory studies are performed to establish the diagnosis of restrictive cardiomyopathy (RCM), to quantitate the severity of the disease, and to monitor the patient.

Other investigative modalities are also employed in the workup, in particular to facilitate differentiation between RCM and constrictive cardiomyopathy (see Table 2 below).

Table 2. Investigation of Constrictive Cardiomyopathy and Restrictive Cardiomyopathy (Open Table in a new window)

Investigation Constrictive Cardiomyopathy Restrictive Cardiomyopathy
Chest radiograph Pericardial calcification Atrial dilatation causing increased cardiothoracic ratio, normal ventricular size
CT scan/MRI Pericardial thickening No pericardial thickening
Echocardiography Normal-sized ventricles and atria; pericardial thickening, pericardial effusion may be observed Nondilated, normally contracting, nonhypertrophied ventricles and marked dilatation of both atria; speckled texture of myocardium in cases of amyloid infiltration of the heart
Doppler flow velocities on echocardiography Respiratory changes (ie, decreased peak transmitral diastolic flow) during inspiration Equalization of the right- and left-sided filling pressures No respiratory changes Greater elevation in the left-sided filling pressures
Catheterization hemodynamics:



1) RVSP



2) RVEDP:RVSP ratio



3) RVEDP/LVEDP equalization



 



1) = 50 mm Hg



2) = 0.33



3) = 5 mm Hg difference



 



1) = 50 mm Hg



2) = 0.33



3) = 5 mm Hg difference



Cardiac biopsy Normal myocardium Often diagnostic, showing abnormal myocardium
CT = computed tomography; LVEDP = left ventricular end-diastolic pressure; MRI = magnetic resonance imaging; RVEDP = right ventricular end-diastolic pressure; RVSP = right ventricular systolic pressure.
Next

Laboratory Studies

A complete blood count (CBC) with peripheral smear helps establish eosinophilia. Blood gas analysis is performed to monitor hypoxia. Serum electrolyte, blood urea nitrogen (BUN) and creatinine levels should be obtained, as well as a liver function profile.

Serum iron concentrations, percent saturation of total iron-binding capacity, and serum ferritin levels are all increased in hemochromatosis.

Serum brain natriuretic peptide (BNP) levels should be assessed. Data suggest that serum BNP levels are nearly normal in patients with constrictive physiology of heart failure and grossly elevated in patients with restrictive physiology, despite nearly identical clinical and hemodynamic presentation.[13]

Previous
Next

Radiography and Angiography

Chest radiograph typically shows cardiomegaly with bilateral pleural effusions, absence of cardiomegaly, normal cardiac silhouette, no pericardial calcification (seen in constrictive pericarditis), and manifestations of pulmonary venous hypertension and pulmonary congestion

Angiography may show a small, thick-walled cavity in eosinophilic endomyocardial disease, which may be distorted significantly by a mural thrombus.

Previous
Next

Echocardiography

Two-dimensional imaging

Two-dimensional echocardiography shows a nondilated, normally contracting, nonhypertrophied left ventricle and marked dilatation of both atria. However, amyloidosis and glycogen storage diseases typically show diffuse increased left ventricular thickening.

The ventricular cavity size may be normal or reduced. The wall thickness may be increased in patients with infiltrative diseases. Mural thrombus and cavity obliteration are features of obliterative cardiomyopathy. In contrast, dilated cardiomyopathy shows dilatation of all the chambers of the heart, and increased wall thickness, especially of the ventricular septum, is observed in hypertrophic cardiomyopathy.

Abnormal myocardial textures can also be appreciated using echocardiography. For example, granular speckling of the ventricular walls suggests the presence of infiltrative disease, such as amyloidosis.

Pericardial thickening is not reliably observed on echocardiography; magnetic resonance imaging (MRI) is suggested for exclusion of a thick pericardium.

Doppler imaging

Doppler echocardiography shows features of restriction to diastolic filling. Accentuated early diastolic filling of the ventricles (E), shortened deceleration time, and diminished atrial filling (A), which results in a high E-to-A ratio on the mitral inflow velocities, are present. Variations of this diastolic (transmitral) blood flow with respiration help differentiate between constrictive pericarditis and RCM.

Because both of the ventricles are encased in a common constricting pericardial sac, an inspiratory increase in inflow to the right ventricle causes a reciprocal reduction in the transmitral inflow to the left ventricle. Thus, a pattern of respiratory variation, with a diminished peak transmitral diastolic flow during inspiration, is characteristic of pericardial constriction but not of RCM. In contrast, in RCM, the left-sided filling pressures are elevated further in inspiration.

Pulsed-wave tissue Doppler imaging

The use of pulsed-wave Doppler imaging is used in some centers as a noninvasive approach to distinguishing RCM from constrictive pericarditis. In addition to the information obtained by standard Doppler imaging, pulsed-wave Doppler imaging can define myocardial contraction and relaxation. This results in a measure referred to as the myocardial velocity gradient. Small studies have suggested that the myocardial velocity gradient is a specific measure that distinguishes these 2 entities well.

Previous
Next

Cardiac Catheterization

Ventricular pressure tracings of increased right heart pressures, typical venous wave pattern, and the dip-and-plateau or square-root contour of the ventricular diastolic pressures (deep and rapid early decline in ventricular pressure at the onset of diastole, with a rapid rise to a plateau in early diastole) obtained by cardiac catheterization are the same in pericardial constriction and in RCM. This dip-and-plateau or square-root sign of ventricular pressure is manifested in the atrial pressure tracing as a prominent descent followed by a rapid rise to a plateau.

A few criteria favor the pericardial disorder, as follows:

  • Equalization of left and right ventricular filling pressures, with a difference of no more than 5 mm Hg between the 2 sides
  • Right ventricular end-diastolic pressure (RVEDP) equal to or exceeding one third of the level of the right ventricular systolic pressure (RVSP)
  • RVSP lower than 50 mm Hg
  • Persistence of diastolic equalization of pressures under stress or exercise or fluid challenge

In RCM, the variance between right and left ventricular diastolic pressures is more likely to be greater than 5 mm Hg, RVEDP is more likely to be less than one third the RVSP, and RVSP is more likely to be higher than 50 mm Hg.

Previous
Next

Electrocardiography

The findings on electrocardiography (ECG) depend on the stage of the disease and the specific diagnosis. The ECG may be normal or just show some nonspecific ST-T wave changes, but rhythm disorders (notably atrial fibrillation) are common.

Conduction abnormalities are uncommon in amyloidosis. Low QRS voltage is common in amyloidosis, out of proportion to the thick left ventricle on echocardiography. A pseudoinfarct pattern is possible, secondary to myocardial infiltration and/or small vessel–induced ischemia or infarction.

Previous
Next

Other Studies

Radionuclide imaging

Radionuclide imaging shows increased diffuse uptake of technetium-99m (99m Tc) pyrophosphate and indium-111 (111 In) antimyosin in cardiac amyloidosis.

Cardiovascular magnetic resonance (CMR)

Cardiovascular magnetic resonance (CMR) has been used to assess abnormal myocardial interstitium. Preliminary reports suggest a characteristic pattern of global subendocardial late gadolinium enhancement coupled with abnormal myocardial and blood-pool gadolinium kinetics in RCM.

Previous
Next

Biopsy

Ventricular biopsy obtained from either the right or the left ventricle has proved useful in certain cases in establishing whether endocardial or myocardial disease is present. Growing experience in this technique indicates a high diagnostic yield in diseases that may present with restriction hemodynamics, when noninvasive studies have failed to establish a clear-cut diagnosis.

Amyloidosis demonstrates apple-green birefringence, stained with Congo red, viewed under a polarizing microscope. Fine-needle aspiration of abdominal fat is easier and safer than myocardial biopsy for determination of amyloidosis. Confirmation of the diagnosis of AL amyloidosis demands a search for a plasma cell dyscrasia.

Liver biopsy is performed for diagnosis of hemochromatosis.

Previous
 
 
Contributor Information and Disclosures
Author

Alan Vainrib, MD Fellow, Department of Cardiology, Stony Brook University Medical Center

Disclosure: Nothing to disclose.

Coauthor(s)

Asa William (Peter) Viccellio, MD Professor, Vice-Chair, Department of Emergency Medicine, State University of New York at Stony Brook

Asa William (Peter) Viccellio, MD is a member of the following medical societies: Medical Society of the State of New York, National Association of EMS Physicians, New York Academy of Medicine, New York Academy of Sciences, New York County Medical Society, American Association for the Advancement of Science, American College of Emergency Physicians, American Medical Association

Disclosure: Nothing to disclose.

Vivek J Goswami, MD Director of Nuclear Cardiology, Austin Heart; Clinical Assistant Professor, Texas A&M Health Science Center College of Medicine

Vivek J Goswami, MD is a member of the following medical societies: American College of Cardiology, American College of Physicians-American Society of Internal Medicine, American Heart Association, American Medical Association, Illinois State Medical Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

A Antoine Kazzi, MD Deputy Chief of Staff, American University of Beirut Medical Center; Associate Professor, Department of Emergency Medicine, American University of Beirut, Lebanon

A Antoine Kazzi, MD is a member of the following medical societies: American Academy of Emergency Medicine

Disclosure: Nothing to disclose.

Chief Editor

Henry H Ooi, MD, MRCPI Director, Advanced Heart Failure and Cardiac Transplant Program, Nashville Veterans Affairs Medical Center; Assistant Professor of Medicine, Vanderbilt University School of Medicine

Disclosure: Nothing to disclose.

Additional Contributors

Gary Edward Sander, MD, PhD, FACC, FAHA, FACP, FASH Professor of Medicine, Director of CME Programs, Team Leader, Root Cause Analysis, Tulane University Heart and Vascular Institute; Director of In-Patient Cardiology, Tulane Service, University Hospital; Visiting Physician, Medical Center of Louisiana at New Orleans; Faculty, Pennington Biomedical Research Institute, Louisiana State University; Professor, Tulane University School of Medicine

Gary Edward Sander, MD, PhD, FACC, FAHA, FACP, FASH is a member of the following medical societies: Alpha Omega Alpha, American Chemical Society, American College of Cardiology, American College of Chest Physicians, American College of Physicians, American Federation for Clinical Research, American Federation for Medical Research, American Heart Association, American Society for Pharmacology and Experimental Therapeutics, American Society of Hypertension, American Thoracic Society, Heart Failure Society of America, National Lipid Association, Southern Society for Clinical Investigation

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors Sarath Reddy, MD, Alan Forker, MD, Gunateet Goswami, MD, Nafisa Kuwajerwala, MD, Paul J Kaloudis, MD, and Andrew Wackett, MD, to the development and writing of the source articles.

References
  1. Kushwaha SS, Fallon JT, Fuster V. Restrictive cardiomyopathy. N Engl J Med. 1997 Jan 23. 336(4):267-76. [Medline].

  2. Goldstein JA. Differentiation of constrictive pericarditis and restrictive cardiomyopathy. ACC Ed Highlights. 1998 Fall. 14-22.

  3. Amaki M, Savino J, Ain DL, Sanz J, Pedrizzetti G, Kulkarni H, et al. Diagnostic concordance of echocardiography and cardiac magnetic resonance-based tissue tracking for differentiating constrictive pericarditis from restrictive cardiomyopathy. Circ Cardiovasc Imaging. 2014 Sep. 7(5):819-27. [Medline].

  4. Schlant RC, Alexander RW, eds. The Heart. McGraw-Hill; 1994. 1637-45.

  5. Higano ST, Azrak E, Tahirkheli NK, Kern MJ. Hemodynamic rounds series II: hemodynamics of constrictive physiology: influence of respiratory dynamics on ventricular pressures. Catheter Cardiovasc Interv. 1999 Apr. 46(4):473-86. [Medline].

  6. Towbin JA. Inherited Cardiomyopathies. Circ J. 2014 Sep 2. [Medline].

  7. Peled Y, Gramlich M, Yoskovitz G, Feinberg MS, Afek A, Polak-Charcon S, et al. Titin mutation in familial restrictive cardiomyopathy. Int J Cardiol. 2014 Jan 15. 171(1):24-30. [Medline].

  8. [Guideline] Lindenfeld J, Albert NM, Boehmer JP, et al, for the Heart Failure Society of America. HFSA 2010 Comprehensive Heart Failure Practice Guideline. J Card Fail. 2010 Jun. 16(6):e1-194. [Medline].

  9. Davies MJ, Mann JM. Systemic pathology. The Cardiovascular System. 1995. Vol 10: 1409-16.

  10. Wald DS, Gray HH. Restrictive cardiomyopathy in systemic amyloidosis. QJM. 2003 May. 96(5):380-2. [Medline].

  11. Sasaki N, Garcia M, Ko HH, Sharma S, Parness IA, Srivastava S. Applicability of published guidelines for assessment of left ventricular diastolic function in adults to children with restrictive cardiomyopathy: an observational study. Pediatr Cardiol. 2014 Sep 6. [Medline].

  12. Braunwald E, Abelmann WH. Atlas of Heart Diseases. 1994. Vol 2.: 53-61.

  13. Leya FS, Arab D, Joyal D, Shioura KM, Lewis BE, Steen LH, et al. The efficacy of brain natriuretic peptide levels in differentiating constrictive pericarditis from restrictive cardiomyopathy. J Am Coll Cardiol. 2005 Jun 7. 45(11):1900-2. [Medline].

  14. Tintinalli JE, Kelen GD, Stapczynski JS, eds. Emergency Medicine: A Comprehensive Study Guide. McGraw-Hill; 2004. 381.

  15. Topilsky Y, Pereira NL, Shah DK, et al. Left ventricular assist device therapy in patients with restrictive and hypertrophic cardiomyopathy. Circ Heart Fail. 2011 May. 4(3):266-75. [Medline].

  16. Uriel N, Vainrib A, Jorde UP, Cotarlan V, Farr M, Cheema FH. Mediastinal radiation and adverse outcomes after heart transplantation. J Heart Lung Transplant. 2010 Mar. 29(3):378-81. [Medline].

  17. [Guideline] Hershberger RE, Lindenfeld J, Mestroni L, Seidman CE, Taylor MR, Towbin JA. Genetic evaluation of cardiomyopathy--a Heart Failure Society of America practice guideline. J Card Fail. 2009 Mar. 15(2):83-97. [Medline].

 
Previous
Next
 
Table 1. Clinical Features of Constrictive Pericarditis and Restrictive Cardiomyopathy
Clinical Features Constrictive Pericarditis Restrictive Cardiomyopathy
History Prior history of pericarditis or condition that causes pericardial disease History of systemic disease (eg, amyloidosis, hemochromatosis)
General examination Peripheral stigmata of systemic disease
Systemic examination - Heart sounds Pericardial knock, high-frequency sound Presence of loud diastolic filling sound S3, Low-frequency sound
Murmurs No murmurs Murmurs of mitral and tricuspid insufficiency
Prior chest radiograph Pericardial calcification Normal results of prior chest radiograph
Table 2. Investigation of Constrictive Cardiomyopathy and Restrictive Cardiomyopathy
Investigation Constrictive Cardiomyopathy Restrictive Cardiomyopathy
Chest radiograph Pericardial calcification Atrial dilatation causing increased cardiothoracic ratio, normal ventricular size
CT scan/MRI Pericardial thickening No pericardial thickening
Echocardiography Normal-sized ventricles and atria; pericardial thickening, pericardial effusion may be observed Nondilated, normally contracting, nonhypertrophied ventricles and marked dilatation of both atria; speckled texture of myocardium in cases of amyloid infiltration of the heart
Doppler flow velocities on echocardiography Respiratory changes (ie, decreased peak transmitral diastolic flow) during inspiration Equalization of the right- and left-sided filling pressures No respiratory changes Greater elevation in the left-sided filling pressures
Catheterization hemodynamics:



1) RVSP



2) RVEDP:RVSP ratio



3) RVEDP/LVEDP equalization



 



1) = 50 mm Hg



2) = 0.33



3) = 5 mm Hg difference



 



1) = 50 mm Hg



2) = 0.33



3) = 5 mm Hg difference



Cardiac biopsy Normal myocardium Often diagnostic, showing abnormal myocardium
CT = computed tomography; LVEDP = left ventricular end-diastolic pressure; MRI = magnetic resonance imaging; RVEDP = right ventricular end-diastolic pressure; RVSP = right ventricular systolic pressure.
Previous
Next
 
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.