Medication Summary
The goals of pharmacotherapy are to reduce morbidity and to prevent complications.
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.
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.
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.
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.
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].
Quash JA, Davia JE, de Castro CM, Bowen TE, Green DC. Echocardiography in Lutembacher's syndrome. Chest. Nov 1977;72(5):676-8. [Medline].
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].
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].
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].
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].
Joseph G, Abhaichand Rajpal K, Kumar KP. Definitive percutaneous treatment of Lutembacher's syndrome. Catheter Cardiovasc Interv. Oct 1999;48(2):199-204. [Medline].
Chau EM, Lee CH, Chow WH. Transcatheter treatment of a case of Lutembacher syndrome. Catheter Cardiovasc Interv. May 2000;50(1):68-70. [Medline].
Shabbir M, Ahmed W, Akhtar K. Transcatheter treatment of Lutembacher's syndrome. J Coll Physicians Surg Pak. Feb 2008;18(2):105-6. [Medline].
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].
Ansari A, Maron BJ. Lutembacher's syndrome. Tex Heart Inst J. 1997;24(3):230-1. [Medline].
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].
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].
Crawford MH. Iatrogenic Lutembacher's syndrome revisited. Circulation. Apr 1990;81(4):1422-4. [Medline].
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].
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].
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].
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].
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.
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].

