Lutembacher Syndrome Medication

  • Author: Kamran Riaz, MD; Chief Editor: Park W Willis IV, MD   more...
 
Updated: Apr 3, 2012
 

Medication Summary

The goals of pharmacotherapy are to reduce morbidity and to prevent complications.

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Diuretics

Class Summary

These agents inhibit sodium and chloride reabsorption. They are used to treat right-sided heart failure.

Furosemide (Lasix)

 

Increases excretion of water by interfering with chloride-binding cotransport system, which inhibits sodium and chloride reabsorption in ascending loop of Henle and distal renal tubule. Dose must be individualized to patient. Depending on response, administer at increments of 20-40 mg, no sooner than 6-8 h after previous dose, until desired diuresis occurs. When treating infants, titrate with 1-mg/kg/dose increments until satisfactory effect achieved.

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Cardiac glycosides

Class Summary

These agents are used to treat atrial arrhythmias. They have both direct and indirect effects.

Digoxin (Lanoxin)

 

Cardiac glycoside with direct inotropic effects in addition to indirect effects on cardiovascular system. Acts directly on cardiac muscle, increasing myocardial systolic contractions. Indirect actions result in increased carotid sinus nerve activity and enhanced sympathetic withdrawal for any given increase in mean arterial pressure.

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Antibiotics, prophylactic

Class Summary

These agents provide prophylaxis for subacute bacterial endocarditis (SBE). Patients with Lutembacher syndrome are at high risk for SBE owing to associated mitral stenosis.

Cephalexin (Keflex)

 

First-generation cephalosporin that inhibits bacterial replication by inhibiting bacterial cell wall synthesis. Bactericidal and effective against rapidly growing organisms forming cell walls.

Resistance occurs by alteration of penicillin-binding proteins. Effective for treatment of infections caused by streptococcal or staphylococcal infection, including penicillinase-producing staphylococci. May use to initiate therapy when streptococcal or staphylococcal infection is suspected.

Used orally when outpatient management is indicated. Primarily active against skin flora, including Staphylococcus aureus.

Amoxicillin (Amoxil, Trimox)

 

Interferes with synthesis of cell wall mucopeptides during active multiplication, resulting in bactericidal activity against susceptible bacteria. Used as prophylaxis in minor procedures.

Ampicillin (Marcillin, Omnipen)

 

For prophylaxis in patients undergoing dental, oral, or respiratory tract procedures. Coadministered with gentamicin for prophylaxis in GI or genitourinary procedures.

Clindamycin (Cleocin)

 

Used in penicillin-allergic patients undergoing dental, oral, or respiratory tract procedures. Useful for treatment against streptococcal and most staphylococcal infections.

Gentamicin (Garamycin)

 

Aminoglycoside antibiotic for gram-negative coverage. Used in combination with both an agent against gram-positive organisms and one that covers anaerobes. Used in conjunction with ampicillin or vancomycin for prophylaxis in GI or genitourinary procedures.

Vancomycin (Vancocin)

 

Potent antibiotic directed against gram-positive organisms and active against Enterococcus species. Useful in treatment of septicemia and skin structure infections. Indicated for patients who cannot receive, or have failed to respond to, penicillins and cephalosporins or have infections with resistant staphylococci. Use CrCl to adjust dose in patients diagnosed with renal impairment. Used in conjunction with gentamicin for prophylaxis in patients with penicillin allergy undergoing GI or genitourinary procedures.

Erythromycin (EES, E-Mycin, Eryc)

 

Used for prophylaxis in patients with penicillin allergy undergoing dental, oral, or respiratory tract procedures.

Cefazolin (Ancef)

 

First-generation semisynthetic cephalosporins that arrest bacterial cell wall synthesis, inhibiting bacterial growth. Primarily active against skin flora, including Staphylococcus aureus.

Azithromycin (Zithromax)

 

Macrolide antibiotics which inhibit bacterial growth, possibly by blocking dissociation of peptidyl tRNA from ribosomes, causing RNA-dependent protein synthesis to arrest.

Cefadroxil (Duricef)

 

First generation semisynthetic cephalosporin that arrests bacterial growth by inhibiting bacterial cell wall synthesis. Bactericidal activity against rapidly growing organisms. Primarily active against skin flora, including Staphylococcus aureus.

Clarithromycin (Biaxin)

 

Semisynthetic macrolide antibiotic that reversibly binds to P site of 50S ribosomal subunit of susceptible organisms and may inhibit RNA-dependent protein synthesis by stimulating dissociation of peptidyl t-RNA from ribosomes, causing bacterial growth inhibition.

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Antiarrhythmics

Class Summary

These agents alter the electrophysiologic mechanisms responsible for arrhythmia.

Amiodarone (Cordarone)

 

May inhibit AV conduction and sinus node function. Prolongs action potential and refractory period in myocardium and inhibits adrenergic stimulation. Prior to administration, control ventricular rate and CHF (if present) with digoxin or calcium channel blockers.

Diltiazem (Cardizem, Dilacor, Tiamate, Tiazac)

 

During depolarization, inhibits calcium ions from entering slow channels and voltage-sensitive areas of vascular smooth muscle and myocardium.

Verapamil (Calan, Covera, Verelan, Isoptin)

 

Can diminish PVCs associated with perfusion therapy and decrease risk of ventricular fibrillation and ventricular tachycardia. By interrupting reentry at AV node, can restore normal sinus rhythm in patients with paroxysmal supraventricular tachycardias (PSVT).

Sotalol (Betapace)

 

Class III anti-arrhythmic agent that blocks potassium channels, prolongs action potential duration (APD), and lengthens QT interval. Noncardiac selective beta-adrenergic blocker.

Esmolol (Brevibloc)

 

Excellent drug for patients at risk for complications from beta-blockade (particularly those with reactive airway disease, mild-to-moderate LV dysfunction, and/or peripheral vascular disease). Short half-life of 8 min allows for titration to desired effect and quick discontinuation if needed.

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Contributor Information and Disclosures
Author

Kamran Riaz, MD  Clinical Assistant Professor, Department of Internal Medicine, Section of Cardiology, Wright State University, Boonshoft School of Medicine

Kamran Riaz, MD is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Society of Echocardiography, Ohio State Medical Association, and Royal College of Physicians

Disclosure: Nothing to disclose.

Specialty Editor Board

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, Louisiana State Medical Society, National Lipid Association, and Southern Society for Clinical Investigation

Disclosure: Forest Labs Honoraria Speaking and teaching

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

Disclosure: Medscape Salary Employment

Marschall S Runge, MD, PhD  Charles and Anne Sanders Distinguished Professor of Medicine, Chairman, Department of Medicine, Vice Dean for Clinical Affairs, University of North Carolina at Chapel Hill School of Medicine

Marschall S Runge, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American College of Cardiology, American College of Physicians-American Society of Internal Medicine, American Federation for Clinical Research, American Federation for Medical Research, American Heart Association, American Physiological Society, American Society for Clinical Investigation, American Society for Investigative Pathology, Association of American Physicians, Association of Professors of Cardiology, Association of Professors of Medicine, Southern Society for Clinical Investigation, and Texas Medical Association

Disclosure: Pfizer Honoraria Speaking and teaching; Merck Honoraria Speaking and teaching; Orthoclinica Diagnostica Consulting fee Consulting

Amer Suleman, MD  Private Practice

Amer Suleman, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine, and Society of Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Chief Editor

Park W Willis IV, MD  Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North Carolina at Chapel Hill School of Medicine

Park W Willis IV, MD is a member of the following medical societies: American Society of Echocardiography

Disclosure: Nothing to disclose.

References
  1. Wiedemann HR. Earliest description by Johann Friedrich Meckel, Senior (1750) of what is known today as Lutembacher syndrome (1916). Am J Med Genet. Oct 15 1994;53(1):59-64. [Medline].

  2. Quash JA, Davia JE, de Castro CM, Bowen TE, Green DC. Echocardiography in Lutembacher's syndrome. Chest. Nov 1977;72(5):676-8. [Medline].

  3. Sadaniantz A, Luttmann C, Shulman RS, et al. Acquired Lutembacher syndrome or mitral stenosis and acquired atrial septal defect after transseptal mitral valvuloplasty. Cathet Cardiovasc Diagn. Sep 1990;21(1):7-9. [Medline].

  4. Vasan RS, Shrivastava S, Kumar MV. Value and limitations of Doppler echocardiographic determination of mitral valve area in Lutembacher syndrome. J Am Coll Cardiol. Nov 15 1992;20(6):1362-70. [Medline].

  5. Shigenobu M, Sano S. Surgical indications and treatment of mitral valve disease associated with secundum atrial septal defect with special reference to left ventricular geometry and function. J Cardiovasc Surg (Torino). Dec 1994;35(6):469-74. [Medline].

  6. Ruiz CE, Gamra H, Mahrer P, Allen JW, O'Laughlin MP, Lau FY. Percutaneous closure of a secundum atrial septal defect and double balloon valvotomies of a severe mitral and aortic valve stenosis in a patient with Lutembacher's syndrome and severe pulmonary hypertension. Cathet Cardiovasc Diagn. Apr 1992;25(4):309-12. [Medline].

  7. Joseph G, Abhaichand Rajpal K, Kumar KP. Definitive percutaneous treatment of Lutembacher's syndrome. Catheter Cardiovasc Interv. Oct 1999;48(2):199-204. [Medline].

  8. Chau EM, Lee CH, Chow WH. Transcatheter treatment of a case of Lutembacher syndrome. Catheter Cardiovasc Interv. May 2000;50(1):68-70. [Medline].

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  13. Cheng TO. Coexistent atrial septal defect and mitral stenosis (Lutembacher syndrome): An ideal combination for percutaneous treatment. Catheter Cardiovasc Interv. Oct 1999;48(2):205-6. [Medline].

  14. Crawford MH. Iatrogenic Lutembacher's syndrome revisited. Circulation. Apr 1990;81(4):1422-4. [Medline].

  15. Essop MR, Essop AR, Bedhesi S, Sareli PE. Cyanosis and clubbing in a patient with iatrogenic Lutembacher syndrome. Eur Heart J. Mar 1995;16(3):421-3. [Medline].

  16. Gopala Raju AR, Cherian G, Alurkar VM, Krishnaswami S, John S. Electrocardiographic features in Lutembacher's syndrome. Indian J Chest Dis Allied Sci. Jul-Sep 1979;21(3):125-9. [Medline].

  17. Horstkotte D, Niehues R, Strauer BE. Pathomorphological aspects, aetiology and natural history of acquired mitral valve stenosis. Eur Heart J. Jul 1991;12 Suppl B:55-60. [Medline].

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  19. Perloff JK. Atrial septal defect: Lutembacher syndrome. In: The Clinical Recognition of Congenital Heart Disease. 3rd ed. Philadelphia, Penn: WB Saunders Co; 1987:299-304.

  20. Turner SA, Paulus R, Massumi A, Duncan JM, Hernandez G, Hall RJ. Variant of Lutembacher's syndrome with intact atrial septum. Am Heart J. Jan 1994;127(1):224-7. [Medline].

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Shown is a 2-dimensional transthoracic echocardiogram of a 74-year-old woman who presented with signs of right heart failure. Note severely dilated left atrium, calcified and thickened mitral valve leaflets, doming of the anterior mitral valve leaflet, mitral annular calcification, and reduced opening of the mitral valve.
Shown is a 2-dimensional transesophageal echocardiogram during diastole of a 74-year-old woman who presented with signs of right-sided heart failure. Note the thickened, narrowed, and calcified mitral valve apparatus and doming of the anterior leaflet of the mitral valve.
Color-flow imaging of a 74-year-old woman who presented with signs of right-sided heart failure on transthoracic echocardiogram; this illustrates an anteriorly directed jet of moderate mitral regurgitation.
Color-flow imaging (subcostal view) on transthoracic echocardiogram showing the left-to-right shunt across the atrial septum of a 74-year-old woman who presented with signs of right-sided heart failure.
Shown is a color-flow image during transesophageal echocardiography at the mitral valve level of a 74-year-old woman who presented with signs of right-sided heart failure. Note anteriorly directed jet of moderate-to-severe mitral regurgitation during systole.
Color-flow imaging during transesophageal echocardiography shows blood flow across the atrial septum in a 74-year-old woman who presented with signs of right-sided heart failure.
Seen here are Doppler measurements at the mitral inflow level of a 74-year-old woman who presented with signs of right-sided heart failure. Note the reduced E-A slope and a peak transmitral velocity giving rise to a peak transmitral gradient of 21 mm Hg.
Doppler measurement across the atrial septum reveals a peak velocity of 4 m/s of a 74-year-old woman who presented with signs of right-sided heart failure.
 
 
 
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