eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology
Sinus of Valsalva Aneurysm: Treatment & Medication
Updated: Oct 10, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
Direct the medical care of the patient with a ruptured sinus of Valsalva aneurysm toward hemodynamic stabilization, prevention or treatment of endocarditis, and management of arrhythmias, cardiac ischemia, or both using the following indications and medications:3,9
- Heart failure: Administer diuretics, digitalis, and ACE inhibitors, and perform stabilization of cardiac rhythm (as indicated).
- Cardiac ischemia: Administer nitrates and beta-blockers.
- Endocarditis: Standard prophylaxis is no longer routinely recommended. Standard prophylaxis should be used after an episode of bacterial endocarditis has occurred to prevent reoccurrence.11 For more information, see Antibiotic Prophylactic Regimens for Endocarditis.
Surgical Care
Prompt surgical therapy is recommended when a ruptured sinus of Valsalva aneurysm is diagnosed. A combined approach from the affected chamber and from inside the aorta is most helpful to allow inspection of the aortic valve and to avoid injury to the coronary vessels. The procedure is described as follows:12,13
- The fistula tract from the ruptured aneurysm is closed, and an associated ventricular septal defect can be repaired.
- The aorta is reunited with the valve annulus either by direct anastomosis or by the interposition of a graft, if required.
- Competency of the aortic valve is tested using transesophageal Doppler ultrasound, and valve repair can be undertaken, if necessary.
- Preservation of the aortic valve, particularly in children, is of paramount importance; therefore, early surgical intervention may be warranted.
- No consensus as to when to perform surgery on a fortuitously discovered unruptured sinus of Valsalva aneurysm has been reached.
- Regular follow-up of these patients using echocardiography or MRI to document the size of the aneurysm is indicated.
- Undertake elective repair of a known sinus of Valsalva aneurysm at the same time as surgical repair of any other intracardiac shunt or defect.
- Percutaneous, transcatheter closure of a ruptured sinus of Valsalva aneurysm was first described in 1994.14 Numerous occluder devices have been used, especially the Amplatzer device.15,16,17,18 Guidance for transcatheter closure is provided by 2-dimensional or 3-dimensional transesophageal echocardiographic guidance.17
Activity
- Patients with a sinus of Valsalva aneurysm should avoid participation in contact sports or activities involving vigorous exertion or sustained heavy lifting. Chest trauma may precipitate rupture of a sinus aneurysm.
- Patients with ruptured aneurysms who are awaiting surgical repair can be allowed activity to tolerance levels. Activity may be limited because of symptoms of congestive heart failure.
Medication
No specific medical therapy is indicated for sinus of Valsalva aneurysm. Treatment of congestive heart failure may be required if rupture of the aneurysm occurs into the right heart chambers; standard therapy for heart failure is recommended,19,3 although surgery is the treatment of choice.
Angiotensin-converting enzyme (ACE) inhibitors
Chronic rupture of a sinus of Valsalva aneurysm may produce protracted symptoms and findings of congestive heart failure. ACE inhibitors have been shown to be effective in the treatment of long-standing aortic insufficiency. ACE inhibitors are beneficial in all stages of chronic heart failure. Pharmacologic effects provide both preload and afterload reduction and may have beneficial effects in the prevention of pathologic hypertrophy from volume overload.
Captopril (Capoten)
Prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in lower aldosterone secretion.
Note: May be placed into stabilized suspension with water and ascorbic acid.
Adult
12.5-25 mg PO bid/tid initially; may titrate upward; not to exceed 450 mg/d
Pediatric
Infants: 0.25 mg/kg/dose PO q6h initially; may titrate upward; not to exceed 1 mg/kg/dose PO q6h
Children: 0.4 mg/kg/dose PO bid/qid initially; titrate up to 6 mg/kg/d PO divided bid/qid
NSAIDs may reduce hypotensive effects of captopril; ACE inhibitors may increase digoxin, lithium, and allopurinol levels; rifampin decreases captopril levels; probenecid may increase captopril levels; hypotensive effects of ACE inhibitors may be enhanced when administered concurrently with diuretics
Documented hypersensitivity; renal impairment
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in renal impairment, valvular stenosis, severe congestive heart failure, connective tissue disorders, and bilateral renal artery stenosis
Lisinopril (Prinivil, Zestril)
Prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in lower aldosterone secretion.
Adult
2.5-5 mg/d PO initially; may titrate upward; not to exceed 40 mg/d PO divided bid
Pediatric
0.1 mg/d PO initially; may titrate upward; not to exceed 0.5 mg/d divided bid
May increase digoxin, lithium, and allopurinol levels; probenecid may increase levels; coadministration with diuretics increases hypotensive effects; hypotensive effects of lisinopril may be enhanced when administered concurrently with diuretics and NSAIDs
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in renal impairment, valvular stenosis, severe congestive heart failure, connective tissue disorders, and bilateral renal artery stenosis
Cardiac glycosides
Digitalis remains useful in the treatment of chronic heart failure. Cardiac glycosides are positive inotropic agents that increase the force of contraction of the myocardium and are used to treat acute and chronic congestive heart failure. Cardiac glycosides have been implicated in improving chemoreceptor function, thus potentially increasing exercise tolerance in patients with heart failure.
Digoxin (Lanoxin, Lanoxicaps)
Useful in slowing and stabilizing heart rate, particularly at the atrioventricular node. 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.
Adult
Total digitalizing dose (TDD): 0.75-1.5 mg PO
Divided TDD: Initially administer 50%, then remaining two 25% portions at 6- to 12-h intervals (ie, one-half, one-quarter, one-quarter)
Maintenance dose: 0.125-0.5 mg/d PO
Pediatric
TDD:
Preterm infants: 20-30 mcg/kg PO
Term infants: 25-35 mcg/kg PO
1 month to 2 years: 35-60 mcg/kg PO
2-5 years: 30-40 mcg/kg PO
5-10 years: 20-35 mcg/kg PO
>10 years: Administer as in adults
Divided TDD: Initially administer 50%, then administer remaining two 25% portions at 6- to 12-h intervals (ie, one-half, one-quarter, one-quarter)
Maintenance dose:
Preterm infants: 5-7.5 mcg/kg PO divided bid
Term infants: 6-10 mcg/kg PO divided bid
1 month to 2 years: 10-15 mcg/kg PO divided bid
2-5 years: 7.5-10 mcg/kg PO divided bid
5-10 years: 5-10 mcg/kg PO divided bid
>10 years: Administer as in adults
Digoxin levels may be increased by alprazolam, benzodiazepines, bepridil, captopril, cyclosporine, propafenone, propantheline, quinidine, diltiazem, aminoglycosides, PO amiodarone, anticholinergics, diphenoxylate, erythromycin, felodipine, flecainide, hydroxychloroquine, itraconazole, nifedipine, omeprazole, quinine, ibuprofen, indomethacin, esmolol, tetracycline, tolbutamide, and verapamil
Serum digoxin levels may be decreased by aminoglutethimide, antihistamines, cholestyramine, neomycin, penicillamine, aminoglycosides, PO colestipol, hydantoins, hypoglycemic agents, antineoplastic treatment combinations (including carmustine, bleomycin, methotrexate, cytarabine, doxorubicin, cyclophosphamide, vincristine, procarbazine), aluminum or magnesium antacids, rifampin, sucralfate, sulfasalazine, barbiturates, kaolin/pectin, and aminosalicylic acid
Documented hypersensitivity; beriberi heart disease, idiopathic hypertrophic subaortic stenosis, constrictive pericarditis, and carotid sinus syndrome
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Hypokalemia may reduce positive inotropic effects of digitalis; IV calcium may produce arrhythmias in patients taking digitalis; hypercalcemia predisposes patients to digitalis toxicity, and hypocalcemia can make digoxin ineffective until serum calcium levels are within reference range; magnesium replacement therapy must be instituted in patients with hypomagnesemia to prevent digitalis toxicity; patients diagnosed with incomplete AV block may progress to complete block when treated with digoxin; caution in hypothyroidism, hypoxia, and acute myocarditis; adjust dose in renal failure
Diuretic agents
These agents promote excretion of water and electrolytes by the kidneys and are used to treat heart failure or hepatic, renal, or pulmonary disease when sodium and water retention have resulted in edema or ascites. Both oral and parenteral diuretics may be helpful in the management of congestive heart failure.
Furosemide (Lasix)
Loop diuretic that increases excretion of water by interfering with chloride-binding cotransport system, which, in turn, inhibits sodium and chloride reabsorption in the ascending loop of Henle and distal renal tubules.
Used commonly for acute and long-term management of congestive heart failure.
Adult
20-80 mg/d PO/IV divided bid/tid
Pediatric
1 mg/kg PO/IV q8-12h; not to exceed 5-6 mg/kg/d PO or 2 mg/kg/dose IV
Metformin decreases furosemide concentrations; furosemide interferes with hypoglycemic effect of antidiabetic agents and antagonizes muscle-relaxing effect of tubocurarine; auditory toxicity appears to be increased with coadministration of aminoglycosides and furosemide; hearing loss of varying degrees may occur; anticoagulant activity of warfarin may be enhanced when administered concurrently; increased plasma lithium levels and toxicity are possible when administered concurrently
Documented hypersensitivity; hepatic coma; anuria; severe electrolyte depletion
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Perform frequent serum electrolyte, carbon dioxide, glucose, creatinine, uric acid, calcium, and BUN determinations during first few months of therapy and periodically thereafter
Hydrochlorothiazide (HydroDIURIL, Microzide)
Inhibits reabsorption of sodium in distal tubules, causing increased excretion of sodium and water, as well as potassium and hydrogen ions.
Adult
25-100 mg/d PO qd or divided bid
Pediatric
2-4 mg/kg/d PO divided bid
Thiazides may decrease effects of anticoagulants, antigout agents, and sulfonylureas; thiazides may increase toxicity of allopurinol, anesthetics, antineoplastics, calcium salts, loop diuretics, lithium, diazoxide, digitalis, amphotericin B, and nondepolarizing muscle relaxants
Documented hypersensitivity; anuria or renal decompensation
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in renal disease, hepatic disease, gout, diabetes mellitus, and erythematosus
Spironolactone (Aldactone)
Competes with aldosterone for receptor sites in distal renal tubules, increasing water excretion while retaining potassium and hydrogen ions. Has positive effect on neurohumoral mechanisms in congestive heart failure and may be helpful in remodeling in pathologic hypertrophy.
Adult
25-200 mg/d PO divided bid/qid
Pediatric
2-4 mg/kg/d PO divided bid/qid
May decrease effects of anticoagulants; potassium and potassium-sparing diuretics may increase toxicity of spironolactone
Documented hypersensitivity; anuria, renal failure, or hyperkalemia
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Caution in renal and hepatic impairment
Beta-adrenergic receptor blockers
These agents inhibit chronotropic, inotropic, and vasodilatory responses to beta-adrenergic stimulation. They are used for their effect on reducing myocardial oxygen consumption in congestive heart failure. Beta-blockers also counteract the sympathetic overdrive of congestive heart failure.
Metoprolol (Lopressor)
Selective beta1-adrenergic receptor blocker that decreases automaticity of contractions.
Adult
25 mg/d PO initially; may titrate slowly upward; not to exceed 200 mg/d
Pediatric
0.1-0.2 mg/kg/d PO divided bid initially; may titrate slowly upward; not to exceed 1 mg/kg/d divided bid
Aluminum salts, barbiturates, NSAIDs, penicillins, calcium salts, cholestyramine, and rifampin may decrease bioavailability and plasma levels of metoprolol, possibly resulting in decreased pharmacologic effects; toxicity of metoprolol may increase with coadministration of sparfloxacin, phenothiazines, astemizole, calcium channel blockers, quinidine, flecainide, and contraceptives; metoprolol may increase toxicity of digoxin, flecainide, clonidine, epinephrine, nifedipine, prazosin, verapamil, and lidocaine
Documented hypersensitivity; uncompensated congestive heart failure, bradycardia, asthma, cardiogenic shock, and AV conduction abnormalities
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Beta-adrenergic blockade may reduce signs and symptoms of acute hypoglycemia and may decrease clinical signs of hyperthyroidism; abrupt withdrawal may exacerbate symptoms of hyperthyroidism, including thyroid storm; monitor patient closely and withdraw metoprolol slowly; during IV administration, carefully monitor blood pressure, heart rate, and ECG
Carvedilol (Coreg)
Blocks beta1-adrenergic, alpha-adrenergic, and beta2-adrenergic receptor sites. Recently introduced to treat congestive heart failure. Therapeutic trials are currently underway in pediatric patients in the United States.
Adult
3.125 mg PO bid initially; may slowly titrate upward q2wk; not to exceed 25 mg bid
Pediatric
Not established
Concurrent use with cyclosporine may result in elevated cyclosporine concentrations (increasing risk of nephrotoxicity and neurotoxicity); coadministration with digoxin may increase digoxin concentrations, and synergistic bradycardia may occur
Documented hypersensitivity; cardiogenic shock, pulmonary edema, bradycardia, atrioventricular block, reactive airway disease, and severe bradycardia
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in impaired hepatic function; discontinue therapy if signs of liver dysfunction are present
Nitrates
Nitrates are peripheral and coronary vasodilators used in the management of angina pectoris, heart failure, and myocardial infarction. When given orally or sublingual, these agents reduce preload and improve myocardial oxygen supply and demand.
Nitroglycerin (Nitrostat)
Causes relaxation of vascular smooth muscle by stimulating intracellular cyclic guanosine monophosphate production. Administered acutely or in SR preparations for relief of myocardial ischemia and for reduction of preload and afterload. PO/SL forms rarely are administered in infants or children.
Adult
Acute dose: 0.2-0.6 mg SL q5min for up to 15 min
SR: 2.5-9 mg PO bid/tid
Pediatric
0.25-0.5 mcg/kg/min IV; may titrate upward to 1-5 mcg/kg/min
Aspirin may increase nitrate serum concentrations; marked symptomatic orthostatic hypotension may occur with coadministration of calcium channel blockers (dose adjustment of either agent may be necessary)
Documented hypersensitivity; severe anemia; shock; postural hypotension; head trauma; closed-angle glaucoma; cerebral hemorrhage
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in coronary artery disease and low systolic blood pressure
More on Sinus of Valsalva Aneurysm |
| Overview: Sinus of Valsalva Aneurysm |
| Differential Diagnoses & Workup: Sinus of Valsalva Aneurysm |
 Treatment & Medication: Sinus of Valsalva Aneurysm |
| Follow-up: Sinus of Valsalva Aneurysm |
| Multimedia: Sinus of Valsalva Aneurysm |
| References |
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References
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Further Reading
Keywords
sinus of Valsalva fistula, aortocameral fistula, Valsalva sinus rupture, congenital Valsalva sinus aneurysm, Valsalva sinus fistula, aortic sinus, ruptured Valsalva sinus aneurysm, unruptured Valsalva sinus aneurysm, heart murmur, diastolic murmur, heart failure, Marfan syndrome, syphilitic aortitis, ventricular septal defect, supracristal ventricular septal defect, aortic insufficiency, heart block, subpulmonic ventricular septal defect, cardiac tamponade, dysrhythmia, coronary ischemia, acute myocardial infarction, angina, syncope, Adams-Strokes syndrome, syphilis, Ehlers-Danlos syndrome, Turner syndrome, Williams syndrome, bicuspid aortic valve, osteogenesis imperfecta
