eMedicine Specialties > Cardiology > Coronary Artery Disease
Isolated Coronary Artery Anomalies: Treatment & Medication
Updated: Mar 13, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
The goal of medical therapy is to improve and preserve the hemodynamic status through acting on myocardial contractility, reducing congestion, and decreasing myocardial energy expenditure.
- Cardiac glycosides are a mainstay of inotropic therapy in those patients with congestive heart failure. Digoxin improves cardiac function because of its positive inotropic effect and negative chronotropic effect, but it must be used with caution in patients with myocardial ischemia.
- Dobutamine (primarily a beta1-adrenergic agonist) is particularly useful for treatment of congestive heart failure in children because of its limited effect on heart rate and peripheral vasculature.
- Phosphodiesterase inhibitors (ie, inamrinone, milrinone) can be used as alternatives to dobutamine because of their inotropic effect on the heart and the peripheral vasodilation that reduces the afterload. However, they should be avoided in patients with anomalous origin of a coronary artery from the pulmonary trunk because of the unpredictable vasodilatory effect of these agents on the pulmonary arterial system.
- Loop diuretics (ie, furosemide) may be used in the presence of congestive heart failure.
- Antibiotic prophylaxis for endocarditis is recommended for coronary artery fistulas.
- Beta-adrenergic blocking agents have been used in isolated cases of symptomatic coronary artery anomaly to reduce myocardial oxygen demand and, thus, prevent ischemia.
Surgical Care
Surgery is the only definitive treatment for coronary artery anomalies.
- Coronary arteries originating from the pulmonary trunk are resected optimally from the pulmonary trunk and reimplanted into the ascending aorta. Alternative methods of revascularization include bypass grafts using the internal mammary artery or saphenous veins. In some patients with the origin of the coronary artery from the pulmonary trunk, an intrapulmonary tunnel may be produced to connect the ostium of the anomalous artery to the aorta.
- Coronary artery fistulas can be treated with percutaneous transcatheter occlusion using a detachable balloon, detachable coils, double-umbrella devices, and microparticles of polyvinyl alcohol foam, or they can be treated surgically with a simple ligation. When possible, ligation is performed preferably at the point of entry of the coronary artery to the cardiac chamber. When this is not possible, ligation is performed internally. In patients with multiple lateral communications between the coronary artery and the cardiac chambers, a tangential arteriorrhaphy can be performed. The great risk in coronary ligation is postsurgical myocardial ischemia or infarction.
- Coronary angioplasty with placement of stent is the treatment of choice for myocardial bridges if convincing evidence of myocardial ischemia exists. However, the vast majority of myocardial bridges do not appear to cause myocardial ischemia.
- Coronary artery bypass grafting, preferably using the internal mammary artery, is the surgical treatment of choice for coronary artery atresia.
Consultations
- Pediatric or adult cardiologist
- Cardiothoracic surgeon
Activity
Discourage heavy exercise and competitive sports in patients with significant coronary artery anomalies at least until surgical correction is performed.
Medication
The goals of pharmacotherapy are to reduce morbidity and to prevent complications.
Cardiovascular agents
Used to improve and preserve hemodynamic status by acting on myocardial contractility, reducing congestion, and decreasing myocardial energy expenditure.
Digoxin (Lanoxin)
Cardiac glycoside with direct inotropic effects in addition to indirect effects on the cardiovascular system. Acts directly on cardiac muscle, increasing myocardial contractility. Its indirect actions result in increased carotid sinus nerve activity and enhanced sympathetic withdrawal for any given increase in mean arterial pressure.
Adult
0.125-0.375 mg PO qd
Pediatric
<5 years: Not established
5-10 years: 20-35 mcg/kg PO
>10 years: 10-15 mcg/kg PO
Maintenance dose: 25-35% of PO loading dose
Medications that may increase digoxin levels include alprazolam, benzodiazepines, bepridil, captopril, cyclosporine, propafenone, propantheline, quinidine, diltiazem, aminoglycosides, oral amiodarone, anticholinergics, diphenoxylate, erythromycin, felodipine, flecainide, hydroxychloroquine, itraconazole, nifedipine, omeprazole, quinine, ibuprofen, indomethacin, esmolol, tetracycline, tolbutamide, and verapamil; medications that may decrease serum digoxin levels include aminoglutethimide, antihistamines, cholestyramine, neomycin, penicillamine, aminoglycosides, oral colestipol, hydantoins, hypoglycemic agents, antineoplastic treatment combinations (including carmustine, bleomycin, methotrexate, cytarabine, doxorubicin, cyclophosphamide, vincristine, and 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; carotid sinus hypersensitivity 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 effect of digitalis; IV calcium may produce arrhythmias in digitalized patients; hypercalcemia predisposes patient to digitalis toxicity, and hypocalcemia can make digoxin ineffective until serum calcium levels are within the 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; exercise caution in hypothyroidism, hypoxia, and acute myocarditis
Dobutamine (Dobutrex)
Produces vasodilation and increases inotropic state. At higher dosages may cause increased heart rate, exacerbating myocardial ischemia.
Adult
0.5 mcg/kg/min IV initially; titrate to desired therapeutic effect
Pediatric
Administer as in adults
Beta-adrenergic blockers antagonize effects of dobutamine; general anesthetics may increase toxicity
Documented hypersensitivity; idiopathic hypertrophic subaortic stenosis; atrial fibrillation or flutter
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Following MI, use with extreme caution; hypovolemic state should be corrected before using this drug
Inamrinone (Inocor)
Formerly amrinone. Bi-pyridine positive inotrope and vasodilator with little chronotropic activity. Different in mode of action from both digitalis glycosides and catecholamines. More likely to cause tachycardia than dobutamine. May exacerbate myocardial ischemia. Adjust dose according to patient response.
Adult
0.75 mg/kg IV bolus slowly over 2-3 min; maintenance infusion is 5-10 mcg/kg/min; not to exceed 10 mg/kg
Pediatric
Administer as in adults
Coadministration with diuretics may result in hypovolemia and decrease in filling pressure; cardiac glycosides have additive effects on inamrinone
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
Discontinue therapy if symptoms of liver toxicity develop; correct hypokalemic states before administering therapy
Milrinone (Primacor)
Bi-pyridine positive inotrope and vasodilator with little chronotropic activity. Different in mode of action from both digitalis glycosides and catecholamines.
Adult
50 mcg/kg IV loading dose over 10 min followed by continuous infusion at 0.375-0.75 mcg/kg/min
Pediatric
Administer as in adults; although used as DOC in many pediatric intensive care units, safety and efficacy not well established
Milrinone precipitates in presence of furosemide
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
Monitor fluids, electrolyte changes, and renal function during therapy; excessive diuresis may increase potassium loss and predispose digitalized patients to arrhythmias; important to correct hypokalemia with potassium supplementation prior to treatment; patients showing excessive decreases in blood pressure should have infusion rates slowed or stopped; previous vigorous diuretic therapy has caused significant decreases in cardiac filling pressure; cautiously administer milrinone and monitor blood pressure, heart rate, and clinical symptomatology
Metoprolol (Lopressor)
Selective beta1-adrenergic receptor blocker that decreases automaticity of contractions.
Adult
100 mg/d PO or divided bid/tid initial; increase at 1-wk interval prn to a total of 450 mg/d if necessary
Pediatric
1-5 mg/kg/d PO 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; 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
D - Fetal risk shown in humans; use only 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; during an IV, carefully monitor BP, heart rate, and ECG
Atenolol (Tenormin)
Selectively blocks beta1 receptors with little or no effect on beta2 types.
Adult
50 mg/d PO; increase to 100 mg/d prn
Pediatric
1-2 mg/kg/dose PO qd
Coadministration with aluminum salts, barbiturates, calcium salts, cholestyramine, NSAIDs, penicillins, and rifampin may decrease effects; haloperidol, hydralazine, loop diuretics, and MAOIs may increase toxicity of atenolol
Documented hypersensitivity; congestive heart failure; pulmonary edema; cardiogenic shock; AV conduction abnormalities; heart block (without a pacemaker)
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Beta-adrenergic blockade may reduce symptoms of acute hypoglycemia and mask signs of hyperthyroidism; abrupt withdrawal may exacerbate symptoms of hyperthyroidism and cause thyroid storm; monitor patients closely and withdraw drug slowly; during IV administration, carefully monitor BP, heart rate, and ECG
Propranolol (Inderal, Betachron E-R)
Nonselective, beta-adrenergic receptor blocker with membrane-stabilizing activity that decreases automaticity of contractions. Allow time for drug to reach site of action (particularly if circulation is slow). Do not continue doses after desired alteration in rate or rhythm is achieved.
Adult
1-3 mg IV (under careful monitoring); not to exceed 1 mg/min to avoid lowering blood pressure and causing cardiac standstill; administer second dose after 2 min prn; thereafter, do not administer additional drug in <4 h; switch to PO ASAP; 10-30 mg PO tid/qid
Pediatric
2-4 mg/kg/d PO divided bid (ie, 1-2 mg/kg bid)
IV use is not recommended; however, for arrhythmias, 0.01-0.1 mg/kg slow push has been recommended; not to exceed 1 mg/dose; change to PO ASAP
Coadministration with aluminum salts, barbiturates, NSAIDs, penicillins, calcium salts, cholestyramine, and rifampin may decrease propranolol effects; calcium channel blockers, cimetidine, loop diuretics, and MAOIs may increase toxicity of propranolol; toxicity of hydralazine, haloperidol, benzodiazepines, and phenothiazines may increase with propranolol
Documented hypersensitivity; uncompensated congestive heart failure; bradycardia; cardiogenic shock; 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 decrease signs of acute hypoglycemia and hyperthyroidism; abrupt withdrawal may exacerbate symptoms of hyperthyroidism, including thyroid storm; withdraw drug slowly and monitor closely
Diuretics
Loop diuretics decrease plasma volume and edema by causing diuresis. The reduction in plasma volume and stroke volume associated with diuresis decreases cardiac output and, consequently, blood pressure. May improve pulmonary and systemic cardiovascular activity. Should be used cautiously because any drop in intravascular volume may cause a corresponding drop in cardiac output.
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 the previous dose, until desired diuresis occurs. When treating infants, titrate with 1-mg/kg per dose increments until a satisfactory effect is achieved.
Adult
20-80 mg/d PO/IV/IM; titrate to 600 mg/d for severe edematous states
Pediatric
1-2 mg/kg/dose PO; not to exceed 6 mg/kg per dose; not to administer >q6h
1 mg/kg IV/IM slowly under close supervision; not to exceed 6 mg/kg
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 taken concurrently with this medication; increased plasma lithium levels and toxicity are possible when taken concurrently
Documented hypersensitivity; hepatic coma; anuria; state of 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 mo of therapy and periodically thereafter
More on Isolated Coronary Artery Anomalies |
| Overview: Isolated Coronary Artery Anomalies |
| Differential Diagnoses & Workup: Isolated Coronary Artery Anomalies |
 Treatment & Medication: Isolated Coronary Artery Anomalies |
| Follow-up: Isolated Coronary Artery Anomalies |
| Multimedia: Isolated Coronary Artery Anomalies |
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References
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Further Reading
Keywords
isolated coronary artery anomalies, anomalous origin of epicardial coronary arteries, anomalous course of epicardial coronary arteries, anomalous termination of epicardial coronary arteries, congenital stenosis of the epicardial coronary arteries, atresia of the coronary arteries
