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Pediatric Dilated Cardiomyopathy Medication

  • Author: Poothirikovil Venugopalan, MBBS, MD, FRCPCH; Chief Editor: P Syamasundar Rao, MD  more...
 
Updated: Apr 25, 2014
 

Medication Summary

Medical therapy in dilated cardiomyopathy (DCM) includes diuretics, angiotensin-converting enzyme (ACE) inhibitors, and beta-blockers. Antibiotics for endocarditis prophylaxis are administered to patients with certain cardiac conditions, such as DCM, before performing procedures that may cause bacteremia. For more information, see Antibiotic Prophylactic Regimens for Endocarditis.

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Diuretics

Class Summary

These agents are used to eliminate retained fluid and preload reduction.

Furosemide (Lasix)

 

Furosemide is the drug of choice for diuresis in acute heart failure and in exacerbations of chronic heart failure. It is also used for long-term management of chronic heart failure.

Furosemide inhibits reabsorption of fluid from the ascending loop of Henle in the renal tubule. When administered intravenously, it produces venodilation and lowers preload even before diuresis sets in.

Spironolactone (Aldactone)

 

Spironolactone is a potassium-sparing diuretic that acts on the distal convoluted tubule of the kidney as an aldosterone antagonist. It exhibits synergistic action with furosemide.

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Angiotensin-Converting Enzyme Inhibitors

Class Summary

These drugs reduce afterload and decrease myocardial remodeling that worsens chronic heart failure.

Captopril

 

Captopril is accepted as an essential part of any therapy against heart failure, providing symptomatic improvement and prolonged survival. Captopril prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in lower aldosterone secretion.

Enalapril (Vasotec)

 

This agent is an ACE inhibitor with prolonged duration of action with oral administration. A competitive inhibitor of ACE, it reduces angiotensin II levels, decreasing aldosterone secretion.

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Cardiac Glycosides

Class Summary

These drugs provide improvement of symptoms with chronic administration. The role of cardiac glycosides is less clear than in the past.

Digoxin (Lanoxin)

 

Digoxin improves myocardial contractility, reduces heart rate, and lowers sympathetic stimulation in chronic heart failure. It inhibits the Na+-K+ ATPase pump. Sodium preferentially exchanges with calcium, increasing the intracellular calcium and resulting in an increase in contractility.

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Anticoagulants

Class Summary

These agents are administered to prevent recurrence of thromboembolic episodes of cardiac origin.

Warfarin (Coumadin, Jantoven)

 

Warfarin interferes with hepatic synthesis of vitamin K–dependent coagulation factors. It prevents thrombus formation within cardiac chambers and the venous circulation. Tailor the dose to maintain an International Normalized Ratio (INR) of 2-3.

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Beta-Adrenergic Blocking Agents

Class Summary

These agents block the beta-adrenergic receptor and are modulators of the autonomic system.

Propranolol (Inderal LA, Inderal XL)

 

Propranolol is a nonselective beta-adrenergic antagonist (ie, it inhibits both beta1- and beta2-adrenergic receptors).

Carvedilol (Coreg)

 

Carvedilol is a nonselective beta-blocker with additional direct vasodilator action.

Metoprolol (Lopressor, Toprol XL)

 

Metoprolol is a selective beta-1 adrenergic receptor blocker that decreases automaticity of contractions.

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Adrenergic Agonist Agents

Class Summary

These agents are used in resistant cases as intravenous infusions and stimulate beta1-adrenergic receptors in the myocardium. They are also useful for periodic home inotropic therapy in end-stage disease, in which cardiac transplant is not feasible, to improve the quality of life. However, studies have shown increased mortality related to arrhythmogenic potential.

Dobutamine

 

Dobutamine is a synthetic catecholamine with potent cardiac-stimulating properties; in addition, it has direct vasodilating action on peripheral blood vessels. Infusion with or without additional dopamine in renal dose would be appropriate therapy for cardiogenic shock secondary to dilated cardiomyopathy.

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Phosphodiesterase Enzyme Inhibitors

Class Summary

These agents elicit positive inotropic and vasodilatory effects.

Milrinone

 

Milrinone is a bipyridine with positive inotrope and vasodilator activity. Little chronotropic activity is observed. This agent differs in its mode of action from both digitalis glycosides and catecholamines. It selectively inhibits phosphodiesterase type III (PDE III) in cardiac and smooth vascular muscle, resulting in reduced afterload, reduced preload, and increased inotropy.

Milrinone is not approved by the US Food and Drug Administration (FDA) for use in pediatric patients. Nevertheless, it is often considered the drug of choice in pediatric patients in the intensive care unit setting.

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Contributor Information and Disclosures
Author

Poothirikovil Venugopalan, MBBS, MD, FRCPCH Consultant Pediatrician with Cardiology Expertise, Department of Child Health, Brighton and Sussex University Hospitals, NHS Trust; Honorary Senior Clinical Lecturer, Brighton and Sussex Medical School, UK

Poothirikovil Venugopalan, MBBS, MD, FRCPCH is a member of the following medical societies: Royal College of Paediatrics and Child Health, Paediatrician with Cardiology Expertise Special Interest Group, British Congenital Cardiac Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Ameeta Martin, MD Clinical Associate Professor, Department of Pediatric Cardiology, University of Nebraska College of Medicine

Ameeta Martin, MD is a member of the following medical societies: American College of Cardiology

Disclosure: Nothing to disclose.

Chief Editor

P Syamasundar Rao, MD Professor of Pediatrics and Medicine, Division of Cardiology, Emeritus Chief of Pediatric Cardiology, University of Texas Medical School at Houston and Children's Memorial Hermann Hospital

P Syamasundar Rao, MD is a member of the following medical societies: American Academy of Pediatrics, American Pediatric Society, American College of Cardiology, American Heart Association, Society for Cardiovascular Angiography and Interventions, Society for Pediatric Research

Disclosure: Nothing to disclose.

References
  1. Akagi T, Benson LN, Lightfoot NE, et al. Natural history of dilated cardiomyopathy in children. Am Heart J. 1991 May. 121(5):1502-6. [Medline].

  2. Andrews RE, Fenton MJ, Ridout DA, Burch M. New-onset heart failure due to heart muscle disease in childhood: a prospective study in the United kingdom and Ireland. Circulation. 2008 Jan 1. 117(1):79-84. [Medline].

  3. Arola A, Tuominen J, Ruuskanen O, Jokinen E. Idiopathic dilated cardiomyopathy in children: prognostic indicators and outcome. Pediatrics. 1998 Mar. 101(3 Pt 1):369-76. [Medline].

  4. Blauwet LA, Cooper LT. Myocarditis. Prog Cardiovasc Dis. 2010 Jan-Feb. 52(4):274-88. [Medline].

  5. Bowles NE, Ni J, Kearney DL, et al. Detection of viruses in myocardial tissues by polymerase chain reaction. evidence of adenovirus as a common cause of myocarditis in children and adults. J Am Coll Cardiol. 2003 Aug 6. 42(3):466-72. [Medline].

  6. Breinholt JP, Fraser CD, Dreyer WJ, et al. The efficacy of mitral valve surgery in children with dilated cardiomyopathy and severe mitral regurgitation. Pediatr Cardiol. 2008 Jan. 29(1):13-8. [Medline].

  7. Cheitlin MD, Armstrong WF, Aurigemma GP, Beller GA, Bierman FZ, Davis JL. ACC/AHA/ASE 2003 guideline update for the clinical application of echocardiography: summary article: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (ACC/AHA/ASE Committee to Update the 1997 Guidelines for the Clinical Application of Echocardiography). Circulation. 2003 Sep 2. 108(9):1146-62. [Medline].

  8. Chen C, Heusch A, Donner B, Janssen G, Göbel U, Schmidt KG. Present risk of anthracycline or radiation-induced cardiac sequelae following therapy of malignancies in children and adolescents. Klin Padiatr. 2009 May-Jun. 221(3):162-6. [Medline].

  9. Chen SC, Nouri S, Balfour I, Jureidini S, Appleton RS. Clinical profile of congestive cardiomyopathy in children. J Am Coll Cardiol. 1990 Jan. 15(1):189-93. [Medline].

  10. Connuck DM, Sleeper LA, Colan SD, et al. Characteristics and outcomes of cardiomyopathy in children with Duchenne or Becker muscular dystrophy: a comparative study from the Pediatric Cardiomyopathy Registry. Am Heart J. 2008 Jun. 155(6):998-1005. [Medline].

  11. Dimas VV, Denfield SW, Friedman RA, Cannon BC, Kim JJ, Smith EO, et al. Frequency of cardiac death in children with idiopathic dilated cardiomyopathy. Am J Cardiol. 2009 Dec 1. 104(11):1574-7. [Medline].

  12. Friedberg MK, Roche SL, Balasingam M, et al. Evaluation of mechanical dyssynchrony in children with idiopathic dilated cardiomyopathy and associated clinical outcomes. Am J Cardiol. 2008 Apr 15. 101(8):1191-5. [Medline].

  13. Fujioka S, Kitaura Y, Ukimura A, et al. Evaluation of viral infection in the myocardium of patients with idiopathic dilated cardiomyopathy. J Am Coll Cardiol. 2000 Nov 15. 36(6):1920-6. [Medline].

  14. Griffin ML, Hernandez A, Martin TC, et al. Dilated cardiomyopathy in infants and children. J Am Coll Cardiol. 1988 Jan. 11(1):139-44. [Medline].

  15. 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].

  16. Janousek J, Gebauer RA. Cardiac resynchronization therapy in pediatric and congenital heart disease. Pacing Clin Electrophysiol. 2008 Feb. 31 Suppl 1:S21-3. [Medline].

  17. John JB, Cron SG, Kung GC, Mott AR. Intracardiac thrombi in pediatric patients: presentation profiles and clinical outcomes. Pediatr Cardiol. 2007 May-Jun. 28(3):213-20. [Medline].

  18. Pahl E, Sleeper LA, Canter CE, et al. Incidence of and risk factors for sudden cardiac death in children with dilated cardiomyopathy a report from the pediatric cardiomyopathy registry. J Am Coll Cardiol. 2012 Feb 7. 59(6):607-15. [Medline].

  19. Kaushal S, Jacobs JP, Gossett JG, Steele A, Steele P, Davis CR, et al. Innovation in basic science: stem cells and their role in the treatment of paediatric cardiac failure--opportunities and challenges. Cardiol Young. 2009 Nov. 19 Suppl 2:74-84. [Medline].

  20. Kimura A. Molecular etiology and pathogenesis of hereditary cardiomyopathy. Circ J. 2008. 72 Suppl A:A38-48. [Medline].

  21. Kovacevic-Preradovic T, Jenni R, Oechslin EN, Noll G, Seifert B, Attenhofer Jost CH. Isolated left ventricular noncompaction as a cause for heart failure and heart transplantation: a single center experience. Cardiology. 2009. 112(2):158-64. [Medline].

  22. Labombarda F, Blanc J, Pellissier A, Stos B, Gaillard C, Bajolle F, et al. Health-e-Child project: mechanical dyssynchrony in children with dilated cardiomyopathy. J Am Soc Echocardiogr. 2009 Nov. 22(11):1289-95. [Medline].

  23. Liu W, Li WM, Sun NL. Relationship between HLA-DQA1 polymorphism and genetic susceptibility to idiopathic dilated cardiomyopathy. Chin Med J (Engl). 2004 Oct. 117(10):1449-52. [Medline].

  24. Malaisrie SC, Pelletier MP, Yun JJ, et al. Pneumatic paracorporeal ventricular assist device in infants and children: initial Stanford experience. J Heart Lung Transplant. 2008 Feb. 27(2):173-7. [Medline].

  25. McMahon CJ, Pignatelli RH, Nagueh SF, et al. Left ventricular non-compaction cardiomyopathy in children: characterisation of clinical status using tissue Doppler-derived indices of left ventricular diastolic relaxation. Heart. 2007 Jun. 93(6):676-81. [Medline].

  26. Michels VV, Driscoll DJ, Miller FA, et al. Progression of familial and non-familial dilated cardiomyopathy: long term follow up. Heart. 2003 Jul. 89(7):757-61. [Medline].

  27. Michels VV, Olson TM, Miller FA, et al. Frequency of development of idiopathic dilated cardiomyopathy among relatives of patients with idiopathic dilated cardiomyopathy. Am J Cardiol. 2003 Jun 1. 91(11):1389-92. [Medline].

  28. Morales DL, Dreyer WJ, Denfield SW, et al. Over two decades of pediatric heart transplantation: how has survival changed?. J Thorac Cardiovasc Surg. 2007 Mar. 133(3):632-9. [Medline].

  29. Norkiene I, Ringaitiene D, Rucinskas K, et al. Intra-aortic balloon counterpulsation in decompensated cardiomyopathy patients: bridge to transplantation or assist device. Interact Cardiovasc Thorac Surg. 2007 Feb. 6(1):66-70. [Medline].

  30. Rhee EK, Canter CE, Basile S, Webber SA, Naftel DC. Sudden death prior to pediatric heart transplantation: would implantable defibrillators improve outcome?. J Heart Lung Transplant. 2007 May. 26(5):447-52. [Medline].

  31. Senes M, Erbay AR, Yilmaz FM, et al. Coenzyme Q10 and high-sensitivity C-reactive protein in ischemic and idiopathic dilated cardiomyopathy. Clin Chem Lab Med. 2008. 46(3):382-6. [Medline].

  32. Shaddy RE, Boucek MM, Hsu DT, et al. Carvedilol for children and adolescents with heart failure: a randomized controlled trial. JAMA. 2007 Sep 12. 298(10):1171-9. [Medline].

  33. Singh TP, Sleeper LA, Lipshultz S, Cinar A, Canter C, Webber SA, et al. Association of left ventricular dilation at listing for heart transplant with postlisting and early posttransplant mortality in children with dilated cardiomyopathy. Circ Heart Fail. 2009 Nov. 2(6):591-8. [Medline].

  34. Sonne C, Sugeng L, Takeuchi M, Weinert L, Childers R, Watanabe N, et al. Real-time 3-dimensional echocardiographic assessment of left ventricular dyssynchrony: pitfalls in patients with dilated cardiomyopathy. JACC Cardiovasc Imaging. 2009 Jul. 2(7):802-12. [Medline].

  35. Stanton C, Bruce C, Connolly H, Brady P, Syed I, Hodge D, et al. Isolated left ventricular noncompaction syndrome. Am J Cardiol. 2009 Oct 15. 104(8):1135-8. [Medline].

  36. Sugiyama H, Hoshiai M, Naitoh A, Kadono T, Suzuki S, Sugita K. Outcome of non-transplant surgical strategy for end-stage dilated cardiomyopathy in young children. Circ J. 2009 Jun. 73(6):1045-8. [Medline].

  37. Tjang YS, Stenlund H, Tenderich G, et al. Risk factor analysis in pediatric heart transplantation. J Heart Lung Transplant. 2008 Apr. 27(4):408-15. [Medline].

  38. Towbin JA, Lowe AM, Colan SD, et al. Incidence, causes, and outcomes of dilated cardiomyopathy in children. JAMA. 2006 Oct 18. 296(15):1867-76. [Medline].

  39. Vatta M, Sinagra G, Brunelli L, Faulkner G. Remodeling of dystrophin and sarcomeric Z-band occurs in pediatric cardiomyopathies: a unifying mechanism for force transmission defect. J Cardiovasc Med (Hagerstown). 2009 Feb. 10(2):149-56. [Medline].

  40. Venugopalan P, Agarwal AK, Akinbami FO. Improved prognosis of heart failure due to idiopathic dilated cardiomyopathy in children. Int J Cardiol. 1998 Jul 1. 65(2):125-8. [Medline].

  41. Venugopalan P, Houston AB, Agarwal AK. The outcome of idiopathic dilated cardiomyopathy and myocarditis in children from the west of Scotland. Int J Cardiol. 2001 Apr. 78(2):135-41. [Medline].

  42. Pillekamp F, Khalil M, Emmel M, Brockmeier K, Hescheler J. Stem cells in pediatric heart failure. Minerva Cardioangiol. 2008 Jun. 56(3):335-48. [Medline].

  43. Alexander PM, Daubeney PE, Nugent AW, Lee KJ, Turner C, Colan SD, et al. Long-Term Outcomes of Dilated Cardiomyopathy Diagnosed During Childhood: Results from a National Population-Based Study of Childhood Cardiomyopathy. Circulation. 2013 Sep 13. [Medline].

  44. Boggs W. Good Long-Term Outcomes for Childhood Dilated Cardiomyopathy. Available at http://www.medscape.com/viewarticle/812122. Accessed: October 12, 2013.

 
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Chest radiograph of a child with idiopathic dilated cardiomyopathy.
Echocardiogram obtained from apical 4-chamber view revealing a dilated left atrium and left ventricle in a child with idiopathic dilated cardiomyopathy.
This is a color Doppler echocardiogram obtained from apical 4-chamber view revealing a dilated left atrium and left ventricle with the blue jet of mitral regurgitation in a child with idiopathic dilated cardiomyopathy. Mild tricuspid regurgitation is also shown.
This is an echocardiogram obtained from parasternal long axis view revealing a dilated left atrium and left ventricle in a child with idiopathic dilated cardiomyopathy.
Table 1. Factors Identified as Causes of Myocardial Damage
Category Of Factors Specific Factors
Viral infections (myocarditis) Coxsackievirus B, human immunodeficiency virus, echovirus, rubella, varicella, mumps, Epstein-Barr virus, cytomegalovirus, measles, polio
Bacterial infections Diphtheria, Mycoplasma, tuberculosis, Lyme disease, septicemia
Rickettsia Psittacosis, Rocky Mountain spotted fever
Parasites Toxoplasma, Toxocara, Cysticercus
Fungi Histoplasma, coccidioidomycoses, Actinomyces
Neuromuscular disorders Duchenne or Becker muscular dystrophies, Friedreich ataxia, Kearns-Sayre syndrome, other muscular dystrophies
Nutritional factors Kwashiorkor, pellagra, thiamine deficiency, selenium deficiency
Collagen vascular diseases Rheumatic fever, rheumatoid arthritis, systemic lupus erythematosus, dermatomyositis, Kawasaki disease
Hematological diseases Thalassemia, sickle cell disease, iron deficiency anemia
Coronary artery diseases Anomalous left coronary artery from pulmonary artery, infarction
Drugs Anthracycline, cyclophosphamide, chloroquine, iron overload
Endocrine diseases Hypothyroidism, hyperthyroidism, hypoparathyroidism, pheochromocytoma, hypoglycemia
Metabolic disorders Glycogen-storage diseases, carnitine deficiency, fatty acid oxidation defects, mucopolysaccharidoses
Malformation syndromes Cri-du-chat (cat-cry) syndrome
Table 2. Summary of Genetic Loci and Disease Genes for Familial Dilated Cardiomyopathy
Clinical Pattern Identified Genetic Loci Identified Disease Genes
Autosomal dominant (AD) 10q21-10q23, 9q13-q22, 1q32, 15q14, 2q31, 1q11-21 Actin, desmin, lamin A/C
AD with conduction defect 1p1-1q1, 3p22-3p25 ...
X-linked (XL) Xp21 Dystrophin
XL cardio-skeletal (Barth syndrome) Xq28 (gene G4.5) Tafazzin
Table 3. Diagnosis of Dilated Cardiomyopathy in Children - Step I: Diagnosis
Approach Findings Conclusion
Clinical suspicion Infants and young children: Shortness of breath, feeding difficulties, wheezing, failure to thrive, recurrent chest infections, hepatomegaly, cardiomegaly



Older children: Dyspnea, dependent edema, elevated jugular venous pressure, cardiomegaly



Probable heart disease with heart failure
Chest radiography Cardiomegaly, pulmonary plethora, prominent upper lobe veins, pulmonary edema, pleural effusion, collapsed left lower lobe High probability of heart failure with or without chest infection
Electrocardiography Low-voltage complexes Pericardial effusion
Presence of Q waves and inversion of T waves in leads I, II, aVL, and V4 through V6 (anterolateral infarction pattern) Anomalous left coronary artery from pulmonary artery
Significant arrhythmia Dilated cardiomyopathy secondary to arrhythmia
Left ventricular or biventricular hypertrophy with or without left ventricular strain pattern Often unhelpful
Doppler echocardiographic studies Significant congenital heart disease Diagnose primary disease
Significant pericardial effusion with satisfactory left ventricular ejection fraction Diagnose pericardial effusion
Left ventricular posterior wall hypokinesia with hyperechoic papillary muscles, retrograde continuous flow into proximal pulmonary artery Diagnose anomalous left coronary artery from pulmonary artery
Dilated left ventricle (>95th percentile) with global hypokinesia (fractional shortening < 25%, ejection fraction < 50%), and no demonstrable structural heart disease Diagnose dilated cardiomyopathy
Table 4. Diagnosis of Dilated Cardiomyopathy in Children - Step II: Identification of Any Underlying Etiology
Approach Findings Conclusion
Clinical features Positive family history Genetic cause for dilated cardiomyopathy
Acute or chronic encephalopathy, muscle weakness, hypotonia, growth retardation, recurrent vomiting, lethargy Inborn error of metabolism involving energy production
Coarse or dysmorphic features, organomegaly, skeletal abnormalities, short stature, chronic encephalopathy, cherry-red spot in eyes Storage diseases
Skeletal muscle weakness without encephalopathy Neuromuscular disorders
Blood investigations High blood urea nitrogen and creatinine levels, low calcium and magnesium levels, electrolyte disturbances Help in the initial management; occasionally point to a cause of dilated cardiomyopathy, especially in neonates
Elevated acute-phase reactants and cardiac enzyme levels Myocarditis
Positive viral titers Viral myocarditis
Low serum carnitine levels Systemic carnitine deficiency
Hypoglycemia with low or no acidosis (ketosis)



1. High insulin levels, low free fatty acid



2. Low insulin levels, high free fatty acid



1. Infant of diabetic mother, nesidioblastosis



2. Defect in fatty acid oxidation or carnitine metabolism



Hypoglycemia with moderate or high acidosis (ketosis)



1. Low or normal lactate and abnormal urine and serum organic acid levels



1. High lactate



1. Organic (propionic, methylmalonic) acidemias, or ß-ketothiolase deficiency



2. Glycogen storage disease, Bath and Sengers syndromes, pyruvate dehydrogenase deficiency, mitochondrial enzyme deficiency



Hyperammonemia with acidosis Organic acidemias (as above)
Specific enzyme assay Confirms enzymatic defect
Absence of above physical and biochemical abnormalities Post myocarditis or idiopathic dilated cardiomyopathy
Cardiac catheterization Evaluate hemodynamics Useful to predict prognosis and evaluate for transplant
Coronary angiography Abnormal origin of left coronary artery from pulmonary artery Anomalous left coronary artery from pulmonary artery
Myocardial biopsy Myocyte hypertrophy and fibrosis without lymphocytic infiltrate Dilated cardiomyopathy
Inflammatory cell infiltration, cell necrosis Myocarditis
Special stains Mitochondrial or infiltrative diseases
Molecular studies (on blood, fibroblasts, or myocardial cells) Nucleic acid hybridization studies



Polymerase chain reaction studies



Myocarditis
DNA mutation analysis Identifies specific genetic defect
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