Lutembacher Syndrome Treatment & Management

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

Medical Care

  • Symptomatic relief
    • Right-sided heart failure - Diuretics
    • Atrial arrhythmias - Digoxin, beta-blockers, and calcium channel blockers used mainly for rate control, while amiodarone and sotalol used not only for rate control but also for conversion into and maintenance of normal sinus rhythm
  • Subacute bacterial endocarditis (SBE) prophylaxis: Patients with Lutembacher syndrome, unlike those with isolated ASD, are at high risk for SBE owing to associated mitral stenosis; thus, SBE prophylaxis is warranted.
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Surgical Care

Until recently, surgery was the only definite curative treatment available and involved closure of the ASD and mitral commissurotomy or mitral valve replacement.[5]

  • Percutaneous closure of ASD and mitral balloon valvuloplasty[6, 7, 8, 9, 10]
    • Percutaneous closure of the ASD with a clamshell device and mitral valvuloplasty provides a nonsurgical approach to correct these defects. Although mitral valvuloplasty has been performed for several decades, percutaneous closure of an ASD with a device represents a still-developing technology.
    • As already described, mitral valvuloplasty alone can be complicated by development of ASD secondary to transseptal puncture performed as a part of the procedure.
  • Indications for surgery or percutaneous intervention
    • ASD with a Qp/Qs ratio of more than 1.5
    • Moderate-to-severe mitral stenosis
    • Any degree of pulmonary hypertension, except individuals with irreversible pulmonary hypertension (Eisenmenger syndrome, see below)
  • Surgery is now performed early rather than late because the rates of heart failure and cardiac arrhythmia increase with age. Patients with pulmonary hypertension should demonstrate reversibility of pulmonary vascular resistance prior to surgical (or percutaneous) correction of ASD. Patients with pulmonary hypertension and irreversibly increased pulmonary vascular resistance (ie, Eisenmenger physiology) invariably develop progressive right-sided heart failure after ASD closure and die.
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Consultations

  • Cardiothoracic surgeon
  • Interventional cardiologist
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Diet

Patients should adhere to a low-sodium diet.

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Activity

Activity should be as tolerated by the patient.

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Proceed to Medication
 
 
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].

  9. Shabbir M, Ahmed W, Akhtar K. Transcatheter treatment of Lutembacher's syndrome. J Coll Physicians Surg Pak. Feb 2008;18(2):105-6. [Medline].

  10. Ho CL, Liang KW, Fu YC, Jan SL, Lin MC, Chi CS, et al. Transcatheter therapy of Lutembacher syndrome. J Chin Med Assoc. Jun 2007;70(6):253-6. [Medline].

  11. Ansari A, Maron BJ. Lutembacher's syndrome. Tex Heart Inst J. 1997;24(3):230-1. [Medline].

  12. Chen CH, Lin SL, Hsu TL, Chen CC, Wang SP, Chang MS. Iatrogenic Lutembacher's syndrome after percutaneous transluminal mitral valvotomy. Am Heart J. Jan 1990;119(1):209-11. [Medline].

  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].

  18. Kauffmann R, Bianchi C, Meruane J, Thumala A, Goich J. [Lutembacher's syndrome: clinical, echocardiographic and hemodynamic aspects in 6 cases]. Rev Med Chil. May 1987;115(5):433-9. [Medline].

  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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