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.
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Lutembacher Syndrome. This is a two-dimensional transthoracic echocardiogram from a 74-year-old woman who presented with signs of right heart failure. Note the 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.
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Lutembacher Syndrome. This is a two-dimensional transesophageal echocardiogram during diastole in 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.
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Lutembacher Syndrome. This color-flow imaging of a 74-year-old woman who presented with signs of right-sided heart failure on transthoracic echocardiogram (TTE) demonstrates an anteriorly directed jet of moderate mitral regurgitation.
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Lutembacher Syndrome. This color-flow imaging (subcostal view) on transthoracic echocardiogram shows the left-to-right shunt across the atrial septum of a 74-year-old woman who presented with signs of right-sided heart failure.
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Lutembacher Syndrome. This 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 the anteriorly directed jet of moderate-to-severe mitral regurgitation during systole.
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Lutembacher Syndrome. This 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.
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Lutembacher Syndrome. These 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 mmHg.
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Lutembacher Syndrome. 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.