Medication Summary
Medical therapy for supravalvar mitral ring consists of drugs to control pulmonary venous congestion and cardiac failure. The two main categories of drugs used are diuretics to promote excretion of excess water and positive inotropic drugs to improve myocardial function. Medical therapy helps to relieve symptoms of pulmonary edema and congestive heart failure (CHF) but does not correct the underlying anatomic problem of obstruction.
Continue treatment with diuretics and digoxin in patients with supravalvar mitral ring and CHF. Recommend use of a potassium supplement, especially in children receiving furosemide therapy.
Antibiotics are necessary for intercurrent bacterial infections and for prophylaxis of infective endocarditis during dental or surgical procedures. However, antibiotics for endocarditis prophylaxis are no longer recommended for most patients with congenital heart disease. Some significant exceptions are noted, including patients who have previously had endocarditis or patients within 6 months of their surgical repair. Subacute bacterial endocarditis (SBE) prophylaxis is also recommended for patients who have undergone mitral valve replacement. [16] For more information, see Antibiotic Prophylactic Regimens for Endocarditis.
See also the American Heart Association (AHA) and/or American College of Cardiology (ACC) guidelines on:
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Prevention of infective endocarditis in childhood (2015 update)
Diuretics
Class Summary
These agents are useful to remove excess water that accumulates in heart failure and to relieve symptoms associated with pulmonary edema and peripheral edema.
Furosemide (Lasix)
Drug of choice (DOC) for rapid relief of pulmonary congestion and edema caused by CHF. Useful for maintenance therapy for CHF in patients with supravalvar mitral ring. Promotes renal excretion of water by inhibiting electrolyte-transport system in ascending limb of loop of Henle. Can increase solute and water excretion, even with declining glomerular filtration rate.
Chlorothiazide (Diuril)
Thiazide that increases water excretion by inhibiting reabsorption of sodium chloride in distal renal tubule.
Less potent diuretic than furosemide.
Useful in maintenance therapy of CHF; in severe CHF or refractory edema, thiazides act synergistically with furosemide to promote diuresis.
Hydrochlorothiazide (Esidrix, HydroDIURIL)
Thiazide that increases water excretion by inhibiting reabsorption of sodium chloride in distal renal tubule.
Less potent diuretic than furosemide, useful in maintenance therapy of CHF; in severe CHF or refractory edema, thiazides act synergistically with furosemide to promote diuresis.
Spironolactone (Aldactone)
Counteracts secondary hyperaldosteronism that occurs in cardiac failure; inhibits sodium absorption in collecting duct and has a potassium-sparing diuretic effect. Used alone, produces relatively mild diuresis; however, it may be used with furosemide for synergistic action in severe CHF.
Inotropic agents
Class Summary
Positive inotropic agents increase the force of myocardial contraction and are used to treat acute and chronic CHF. Some may also provide vasodilatation, improve myocardial relaxation, or increase or decrease the heart rate (positive or negative chronotropic agents, respectively). These additional properties influence the choice of drug for specific circumstances.
Digoxin (Lanoxin)
DOC among inotropic agents. Improves CHF by positive effect on myocardial contraction. Also helps to control fast ventricular rate, especially in atrial arrhythmia. Preparations include elixir 0.05 mg/mL and tabs 0.125 or 0.25 mg.
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Supravalvular Ring Mitral Stenosis. Parasternal long-axis echocardiographic image showing a supra mitral variant of supravalvular mitral stenosis. A discrete membrane is visualized superior to but distinct from the mitral valve. The mitral valve appears otherwise normal.
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Supravalvular Ring Mitral Stenosis. Parasternal long-axis color flow image showing a supra mitral variant of supravalvular mitral stenosis. Turbulence is noted at the level of the supra mitral membrane. In this case, Doppler interrogation revealed only mild obstruction.
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Supravalvular Ring Mitral Stenosis. Apical four-chamber echocardiographic image showing an intramitral variant of supravalvular mitral stenosis. A membrane is visualized that is closely adherent to the mitral valve leaflets, restricting leaflet mobility.
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Supravalvular Ring Mitral Stenosis. Apical four-chamber color flow echocardiographic image showing an intramitral variant of supravalvular mitral stenosis. Color flow imaging demonstrates severe mitral valve stenosis.
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Supravalvular Ring Mitral Stenosis. Continuous wave Doppler interrogation of the mitral valve in a patient with supravalvular mitral stenosis demonstrates severe stenosis with a mean gradient of 25 mmHg.
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Supravalvular Ring Mitral Stenosis. Simultaneous recording of pressures in the pulmonary artery wedge (PAW) position and the left ventricle (LV) shows a large gradient in diastole across the mitral valve. PAW pressure is markedly elevated.
