eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology
Double Outlet Right Ventricle, Normally Related Great Arteries: Treatment & Medication
Updated: Oct 6, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
- Initial evaluation and treatment are usually performed in an outpatient setting. Treatment varies depending on the anatomy of the lesion. Direct medical treatment of infants with double outlet right ventricle (DORV) at control of congestive heart failure (CHF). Hospitalize children who present with severe heart failure and treat them with fluid restriction and reduction of physical stress. Monitor children to ensure adequate weight gain because CHF can decrease oral intake and increase caloric expenditure. Other therapies include the following:
- Oxygen therapy may be required if pulmonary edema is present.
- Use oxygen only to relieve hypoxemia because it is a pulmonary vasodilator and can exacerbate left-to-right shunt and CHF.
- Promptly initiate diuretic therapy with furosemide.
- Glycoside therapy with digoxin can be initiated in a maintenance dose if severe CHF is not present.
- Systemic afterload reduction is important in treating infants with CHF. ACE inhibitors (ie, captopril, enalapril) are the most commonly used afterload-reduction agents.
Surgical Care
In 1957, Kirkland reported the first surgical repair of double outlet right ventricle using an intraventricular tunnel to establish left ventricular-aortic continuity via subaortic ventricular septal defect (VSD). Surgical repair usually requires cardiopulmonary bypass with moderate hypothermia. Many double outlet right ventricles have been repaired with a period of circulatory arrest.
Most transpositions are repaired using a biventricular approach with placement of an intraventricular baffle; this is more difficult without 2 well-developed ventricles or if the anatomy precludes a biventricular repair. An alternative repair is a Fontan procedure, which deteriorates with time.
In general, procedures depend on the location of the VSD and the size of the left ventricle. A significant number of patients undergo palliative procedures prior to definitive repair, especially when the patients have borderline or hypoplastic left ventricles. These procedures include pulmonary artery banding, Blalock-Taussig shunt, coarctation repair, or a stage I Norwood procedure.
- Double outlet right ventricle with subaortic VSD is repaired by VSD closure to baffle the left ventricular outflow to the aorta. It is typically repaired in patients younger than 6 months to prevent pulmonary vascular disease. If severe pulmonary stenosis is present, the condition and repair are similar to those of tetralogy of Fallot. Pulmonary stenosis often occurs with hypoplasia of the pulmonary arteries and coronary artery anomalies, making repair more difficult. Historically, this condition often was treated with initial shunting and definitive repair in patients aged 4-5 years.
- Double outlet right ventricle with subpulmonary VSD can be repaired using the following 3 methods:
- The first procedure involves construction of a left ventricle–to–subpulmonary outflow tract tunnel with a subsequent arterial switch. This is the preferred method when the aorta is malposed anteriorly. Coronary artery transfer is similar to that in transposition of the great arteries.
- The second method consists of construction of a long intraventricular tunnel to establish continuity between the left ventricle and the aorta and between the right ventricle and pulmonary artery.
- The third method involves closure of the VSD with baffling of the left ventricular outflow to the pulmonary artery with a subsequent atrial baffle (eg, Senning procedure, Mustard procedure). This method is associated with high operative and late mortality rates.
- Doubly committed or noncommitted VSDs often require a complex repair with a Fontan procedure and possibly reoperation for secondary subaortic stenosis. For example, a patient with double outlet right ventricle, complete atrioventricular septal defect (AVSD), and valvar pulmonary stenosis underwent repair involving patching the ventricular portion of the AVSD and translocating it into a subaortic position. A left ventricular–to–aortic tunnel was then created. Nine years after primary repair, the patient required right ventricle–to–pulmonary artery conduit replacement.
- One case series studied 50 children with double outlet right ventricle and adequate left ventricular size.10 Eleven patients in the study had double outlet right ventricle with transposition of the great vessels. Biventricular repair was performed in 48 of the children, and the overall mortality rate was 6%. Actual surgical mortality rate in patients with biventricular repair was 4.3%.
- In contrast, surgical and overall morbidity and mortality rates increase with more complex types of double outlet right ventricle. Takeuchi et al recently reported a case series of 96 patients with double outlet right ventricle and heterotaxy syndrome and/or complete atrioventricular canal defect.11 Only 8 patients had biventricular repair. Nine of the 17 neonatal patients survived. Of the 79 patients older than 30 days, 71 survived. The overall mortality rate was 17% in all patients.
Consultations
- Refer patients with heart murmurs and physical findings suggestive of double outlet right ventricle to a pediatric cardiologist.
- Consult a pediatric cardiac surgeon for possible repair following diagnosis of double outlet right ventricle.
- Consult pediatric critical care personnel. Following surgical repair, postoperative care normally occurs in the pediatric ICU.
- Involve a geneticist in the care of patients diagnosed with double outlet right ventricle who may have associated genetic syndromes, including velocardiofacial syndrome and DiGeorge syndrome.
Diet
- Children with CHF due to double outlet right ventricle often require increased caloric intake supplemented by the addition of medium-chain triglyceride or carbohydrate preparations to conventional infant formulas.
- Some children may require overnight, bolus, or continuous feeds by nasogastric tubes.
Activity
- Activity is not limited for infants initially diagnosed with double outlet right ventricle, unless they have CHF. For patients with CHF, reduce physical stress until the heart failure can be controlled.
- Advance the activity of patients in the postoperative period as tolerated, until a normal level of activity is achieved.
Medication
The overall goal of medical therapy in patients with double outlet right ventricle (DORV) is to prevent or control congestive heart failure (CHF).
Diuretic agents
These agents promote excretion of water and electrolytes by the kidneys. They are used to treat heart failure or hepatic, renal, or pulmonary disease when sodium and water retention has resulted in edema or ascites. They are also used to reduce plasma volume and, thus, improve CHF.
Furosemide (Lasix)
Titrate treatment dose to produce initial diuresis and subsequently to control symptoms.
Increases excretion of water by interfering with chloride-binding cotransport system, which, in turn, inhibits sodium and chloride reabsorption in ascending Henle loop and distal renal tubule.
Adult
20-80 mg/d PO/IV/IM in divided doses q6-12h; not to exceed 600 mg/d
Pediatric
1-6 mg/kg/d PO divided q6-12h
1-2 mg/kg/dose IV/IM q6-12h
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; varying degrees of hearing loss may develop; anticoagulant activity of warfarin may be enhanced when taken concurrently; increased plasma lithium levels and toxicity are possible
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
Hepatic cirrhosis (rapid alterations in fluid/electrolytes may precipitate coma)
Inotropic agents
Positive inotropic agents increase the force of myocardial contraction and are used to treat acute and chronic CHF. Some agents may also increase or decrease the heart rate (ie, positive or negative chronotropic agents), provide vasodilatation, or improve myocardial relaxation. These additional properties influence the choice of drug for specific circumstances. Agents used predominantly for their inotropic effects include cardiac glycosides and phosphodiesterase inhibitors.
Digoxin (Lanoxin)
Used to increase contractility of the left ventricle. Inhibits Na/K-ATPase, which causes intracellular calcium in the sarcoplasmic reticulum of cardiac cells to increase. This leads to a sustained but modest positive inotropic effect on the heart. Some question the inotropic effect of these medications on immature myocardium, while others have demonstrated improved left ventricular contractility without symptomatic improvement.
Adult
Total digitalizing dose (TDD):
0.75-1.5 mg PO
Divide TDD: Initially administer 50% and then administer the remaining two 25% portions at 6- to 12-h intervals (1/2, 1/4, 1/4)
Maintenance dose: 0.125-0.5 mg PO qd
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
Divide TDD: Initially administer 50% and then administer the remaining two 25% portions at 6- to 12-h intervals (1/2, 1/4, 1/4)Maintenance dose:
Preterm infant: 5-7.5 mcg/kg/d PO divided bid
Term infant: 6-10 mcg/kg/d PO divided bid
1 mo-2 years: 10-15 mcg/kg/d PO divided bid
2-5 years: 7.5-10 mcg/kg/d PO divided bid
5-10 years: 5-10 mcg/kg/d PO divided bid
>10 years: Administer as in adults
Medications that may increase digoxin levels include 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
Medications that may decrease serum digoxin levels include 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, 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 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 normal; 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
ACE inhibitors
These agents are used to reduce afterload and left-to-right shunting. ACE inhibitors are beneficial in all stages of chronic heart failure. Pharmacologic effects result in a decrease in systemic vascular resistance, reducing blood pressure, preload, and afterload. Dyspnea and exercise tolerance are improved.
Captopril (Capoten)
Prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in increased levels of plasma renin and a reduction in aldosterone secretion. Shown to increase systemic flow by reducing left-to-right shunting in patients with relatively low pulmonary vascular resistance.
Adult
12.5-25 mg/dose PO q8-12h, increase by 25 mg/dose; not to exceed 450 mg/d
Pediatric
Infants: 0.15-0.3 mg/kg/dose PO, titrate upward; not to exceed 6 mg/kg qd or divided qid
Children: 0.3-0.5 mg/kg/dose PO, titrate upward; not to exceed 6 mg/kg/d 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; the hypotensive effects of ACE inhibitors may be enhanced when given 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, or severe CHF
Enalapril (Vasotec)
Decreases pulmonary-to-systemic flow ratio in the catheterization laboratory and increases systemic blood flow in patients with relatively low pulmonary vascular resistance. It has a favorable clinical effect when administered over a long period.
Adult
2.5-5 mg/d PO; may gradually increase prn, not to exceed 40 mg/kg/d
Pediatric
Limited data available; suggested dose is 0.1 mg/kg PO qd or divided bid; increase prn over 2 wk; not to exceed 0.5 mg/kg/d
NSAIDs may reduce hypotensive effects of enalapril; ACE inhibitors may increase digoxin, lithium, and allopurinol levels; rifampin decreases enalapril levels; probenecid may increase enalapril levels; the hypotensive effects of ACE inhibitors may be enhanced when administered concurrently with diuretics
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
Use with caution and modify dosage with renal impairment (especially renal artery stenosis), hyponatremia, hypovolemia, severe CHF, or with coadministered diuretic therapy; severe hypotension may develop in patients who are sodium and/or volume depleted; initiate lower doses and monitor closely when starting therapy in these patients; experience in children is limited; use with caution in neonates
Phosphodiesterase Enzyme Inhibitor
This agent is used for short-term treatment of acute decompensated heart failure.
Milrinone (Primacor)
Positive inotropic agent and vasodilator. Selectively inhibits phosphodiesterase type III (PDE III) in cardiac and smooth vascular muscle, resulting in reduced afterload, reduced preload, and increased inotropy. Several studies that have compared milrinone with dobutamine demonstrated that milrinone showed greater improvements in preload and afterload and improvements in cardiac output, without significant increases in myocardial oxygen consumption.
Adult
50 mcg/kg IV loading dose over 10 min, followed by continuous infusion at 0.25-1 mcg/kg/min; titrate to maintain adequate systolic blood pressure and cardiac output
Pediatric
Data limited; 50-75 mcg/kg IV loading dose over 15 minutes followed by continuous infusion of 0.25-0.7 mcg/kg/min; titrate dose to effect
Milrinone precipitates in presence of furosemide
Documented hypersensitivity to milrinone, any component, or inamrinone
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 who show 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)
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| Differential Diagnoses & Workup: Double Outlet Right Ventricle, Normally Related Great Arteries |
 Treatment & Medication: Double Outlet Right Ventricle, Normally Related Great Arteries |
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References
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Further Reading
Keywords
double outlet right ventricle, normally related great arteries, DORV, both great arteries originating from the right ventricle, partial transposition complex of the great arteries, transposition of the aorta and levoposition of the pulmonary artery, congenital heart defect, CHD, ventricular septal defect, VSD, Taussig-Bing anomaly, tetralogy of Fallot, DiGeorge syndrome, DiGeorge's syndrome, neural crest, congestive heart failure, subaortic stenosis, arch obstruction, atrioventricular canal defect, mitral stenosis, coarctation of the aorta, interrupted aortic arch, mitral atresia, atrial septal defect, ASD, tricuspid regurgitation, Ebstein malformation, transposition of the great arteries, hypercyanotic spells, polycythemia, failure to thrive
