eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology
Atrioventricular Septal Defect, Partial and Intermediate: Treatment & Medication
Updated: Oct 3, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
Treatment for congestive heart failure (CHF) is occasionally required if mitral regurgitation (MR) cannot be adequately surgically reduced.
Surgical Care
Management of partial atrioventricular septal defect (AVSD) is primarily surgical, and repair includes patch closure of the atrial septal defect (ASD), mitral valve annuloplasty, or cleft closure. Other defects (eg, left ventricular outflow tract [LVOT] obstruction, patent ductus arteriosus [PDA]) may require repair during the same operation.
Repair is usually electively performed in children aged 2-5 years, unless significant mitral regurgitation (MR) is present, in which case earlier repair is indicated. However, in the current era, repair of AVSD can be successfully performed in patients who weigh less than 5 kg.2
- Surgical morbidity
- Severe MR develops in a significant number of patients after correction of ASD. In fact, MR is the most common indication for reoperation in patients after repair of both partial and complete AVSD.3
- LVOT obstruction may not be evident for years after the initial repair. LVOT obstruction is the second most common indication for reoperation in these patients.4
- Preoperative severe left-sided atrioventricular valve regurgitation and associated valve malformations are important risk factors for postoperative development of MR.5,3
- According to another study, predictors for reoperation include postoperative MR, presence of major associated cardiac malformations, associated left atrioventricular valve malformations, partial or absent left atrioventricular valve cleft closure, and a weight of less than 5 kg.2
- When the left-sided atrioventricular valve requires replacement because of unacceptable degrees of regurgitation, higher mortality and complete atrioventricular block are expected.6
- Spontaneous regression of left-sided atrioventricular valve regurgitation after the immediate postoperative period has been described, thus avoiding the need for reoperation.3
- Surgical mortality
- Depending on the surgical series, early postoperative mortality rate is less than 3% in patients with mostly uncomplicated partial AVSD.7,3
- However, poorer survival is seen in patients with major associated cardiac malformations and pulmonary hypertension, with an early postoperative mortality of 8%.2
Consultations
- Pediatric cardiologist
- Cardiovascular surgeon
- Geneticist if an abnormality is suspected (eg, Down syndrome)
Medication
Medical treatment is indicated in patients with congestive heart failure (CHF) usually before surgical repair. However, it may also be needed in patients in whom mitral regurgitation (MR) persists postoperatively. The treatment outlined below is usually indicated for outpatient management.
Angiotensin-converting enzyme inhibitors (ACE inhibitors)
These medications are used to decrease the afterload to the left ventricle (LV) produced by the MR. This effect is achieved by producing peripheral vasodilatation, which, in turn, reduces systemic blood pressure (ie, reduces afterload). Reduction in systemic blood pressure decreases the amount of blood pumped by the LV with each systolic contraction (ie, stroke volume) and also reduces the pressure at which the blood is ejected. This, in turn, diminishes the amount of blood regurgitated by the mitral valve from the LV into the left atrium (LA) during systole, which decreases pulmonary venous pressure and, thus, decreases pulmonary congestion. By decreasing the afterload to the LV, ACE inhibitors reduce the left-to-right shunt through the atrioventricular septal defect (AVSD) or the atrial septal defect (ASD) in the case of partial AVSD.
A recently published observational study by Cooper et al reported that babies whose mothers had taken an ACE inhibitor during the first 3 months of pregnancy had an increased risk of birth defects compared with babies whose mothers had not taken any drugs for high blood pressure.8 At this time, based on this one observational study, the US Food and Drug Administration (FDA) did not change the pregnancy categories for ACE inhibitors. The current pregnancy categories assigned to ACE inhibitors are C for the first trimester and D for the second and third trimesters.
Enalapril (Vasotec)
Prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in increased levels of plasma renin and a reduction in aldosterone secretion.
Helps control blood pressure and proteinuria. Decreases pulmonary-to-systemic flow ratio in the catheterization laboratory and increases systemic blood flow in patients with relatively low pulmonary vascular resistance. Has favorable clinical effect when administered over a long period. Helps prevent potassium loss in distal tubules. Body conserves potassium; thus, less oral potassium supplementation needed.
Patients who develop a cough, angioedema, bronchospasm, or other hypersensitivity reactions after starting ACE inhibitors should receive an angiotensin-receptor blocker.
Adult
2.5-5 mg/d PO; increase prn
Dosing range: 10-40 mg/d PO qd or divided bid
Alternatively, 1.25 mg/dose IV over 5 min q6h
Pediatric
0.1-0.5 mg/kg/d PO qd or divided bid. Doses as high as 1 mg/kg/d have been reported to be well tolerated.
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 given 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
Pregnancy category D in second and third trimesters; caution in renal impairment, valvular stenosis, or severe CHF
Captopril (Capoten)
Prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in lower aldosterone secretion. Rapidly absorbed, but bioavailability is significantly reduced with food intake. It achieves a peak concentration in an hour and has a short half-life. The drug is cleared by the kidney. Impaired renal function requires reduction of dosage. Absorbed well PO. Give at least 1 h before meals. If added to water, use within 15 min. Can be started at low dose and titrated upward as needed and as patient tolerates.
Adult
6.25-25 mg PO bid/tid; increase dose by 25 mg prn at 1- to 2-wk intervals; not to exceed 450 mg/d divided tid
ClCr 10-50 mL/min: give 75% of starting dose
ClCr <10 mL/min: give 50% of starting dose
Pediatric
Neonates: 0.05-0.1 mg/kg/dose PO q6-24h; titrate dose up to 0.5 mg/kg/dose prn
Infants: 0.15-0.3 mg/kg/dose PO q6-24h; titrate dose up; not to exceed 6 mg/kg/d in 2-4 divided doses prn
Children: 0.3-0.5 mg/kg/dose PO q6-24h; titrate dose up; not to exceed 6 mg/kg/d in 2-4 divided doses prn
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
Pregnancy category D in second and third trimesters; caution in renal impairment, valvular stenosis, or severe CHF
Lisinopril (Prinivil, Zestril)
Prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in lower aldosterone secretion.
Adult
10 mg/d PO; increase 5-10 mg/d at 1-2 wk intervals; not to exceed 40 mg
Pediatric
Not established, data limited; 0.2 mg/kg PO qd initially; increase as BP and symptoms (eg, dizziness, light-headedness) allow
NSAIDs may reduce hypotensive effects of lisinopril; ACE inhibitors may increase digoxin, lithium, and allopurinol levels; rifampin decreases lisinopril levels; probenecid may increase lisinopril levels; the hypotensive effects of ACE inhibitors may be enhanced when given 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
Pregnancy category D in second and third trimesters; caution in renal impairment, valvular stenosis, or severe CHF
Diuretics
These agents help decrease pulmonary congestion.
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 ascending limb of loop of Henle and distal renal tubule. Increases renal blood flow without increasing filtration rate. Onset of action is generally within 1 h. Increases potassium, sodium, calcium, and magnesium excretion.
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/dose increments until a satisfactory effect is achieved.
Diuretics have major clinical uses in managing disorders involving abnormal fluid retention (edema) or in treating hypertension, in which their diuretic action causes decreased blood volume. Chronic use of furosemide can lead to hypercalcemia with renal damage and electrolyte disturbances.
Adult
20-80 mg/d PO/IV/IM; titrate up to 600 mg/d for severe edematous states
Pediatric
1-2 mg/kg/dose PO; not to exceed 6 mg/kg/dose; do not administer more frequently than q6h
Alternatively, 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 with this medication
Documented hypersensitivity; hepatic coma, anuria, and 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, CO2, glucose, creatinine, uric acid, calcium, and BUN determinations during first few months of therapy and periodically thereafter
Spironolactone (Aldactone)
For management of edema resulting from excessive aldosterone excretion. Competes with aldosterone for receptor sites in distal renal tubules, increasing water excretion while retaining potassium and hydrogen ions. Therefore, it is generally used when concomitant chronic use of sodium-wasting diuretics such as furosemide is noted.
Adult
25-200 mg/d PO in 1-2 divided doses
Pediatric
Maintenance: 1 mg/kg/dose PO up to qid
May decrease effect of anticoagulants; potassium and potassium-sparing diuretics may increase toxicity of spironolactone
Documented hypersensitivity; anuria, renal failure or hyperkalemia
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 and hepatic impairment
Inotropic, Antiarrhythmic
It is used because of its direct inotropic effects in addition to indirect effects on the cardiovascular system.
Its indirect actions result in increased carotid sinus activity and enhanced sympathetic withdrawal for any given increase in mean arterial pressure. These effects help reduce the heart rate response to CHF, rendering a more effective stroke volume with each ventricular systole.
Digoxin (Lanoxin)
Enhances myocardial contractility by inhibition of Na+/K+ ATPase, a cell membrane enzyme that extrudes Na and brings K into the myocyte. Resulting increase in intracellular Na stimulates Na-Ca exchanger in the cell membrane, which extrudes Na and brings in Ca, leading to an increase in intracellular calcium in the sarcoplasmic reticulum of cardiac cells, therefore increasing contractility of myocyte (ie, positive inotropic effect). Has direct inotropic effects in addition to indirect effects on the cardiovascular system. Increases myocardial systolic contractions. It exerts vagomimetic action on sinus and AV nodes (slowing heart rate and conduction). Also, decreases degree of activation of sympathetic nervous system and renin-angiotensin system, which is referred to as the deactivating effect. May be given as a loading dose followed by a maintenance dose or simply as a maintenance regimen. Digitalis loading increases hazards of this drug. Therapeutic serum level range is 0.8-2 ng/mL.
Adult
0.125-0.5 mg PO qd
Pediatric
Premature infants: 0.005-0.0075 mg/kg if tablet; 0.004-0.006 mg/kg if capsule, IV, or IM divided q12h
Full-term infants: 0.006-0.010 mg/kg if tablet; 0.005-0.008 if capsule, IV, or IM divided q12h
1-24 months: 0.010-0.015 mg/kg if tablet; 0.0075-0.012 mg/kg if capsule, IV, or IM divided q12h
2-5 years: 0.0075-0.010 mg/kg if tablet; 0.006-0.009 mg/kg if capsule, IV, or IM divided q12h
5-10 years: 0.005-0.010 mg/kg if tablet; 0.004-0.008 mg/kg if capsule, IV, or IM divided q12h
>10 years: 0.0025-0.005 mg/kg if tablet; 0.002-0.003 if capsule, IV, or IM qd or divided q12h
IV calcium may produce arrhythmias in digitalized patients; 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, 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 effect of digitalis; 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 A-V block may progress to complete block when treated with digoxin; exercise caution in hypothyroidism, hypoxia, and acute myocarditis; adjust dose in renal impairment; highly toxic (overdoses can be fatal)
More on Atrioventricular Septal Defect, Partial and Intermediate |
| Overview: Atrioventricular Septal Defect, Partial and Intermediate |
| Differential Diagnoses & Workup: Atrioventricular Septal Defect, Partial and Intermediate |
 Treatment & Medication: Atrioventricular Septal Defect, Partial and Intermediate |
| Follow-up: Atrioventricular Septal Defect, Partial and Intermediate |
| Multimedia: Atrioventricular Septal Defect, Partial and Intermediate |
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References
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Further Reading
Keywords
atrioventricular septal defect, AVSD, partial AVSD, partial atrioventricular septal defect, atrioventricular canal defect, mitral cleft, ostium primum defect, partial atrioventricular septal defect, partial common atrioventricular canal, endocardial cushion defects, intermediate atrioventricular septal defect, transitional common atrioventricular canal, ventricular septal defect, right ventricular outflow tract, pulmonary stenosis, pulmonary vascular obstructive disease, congenital heart defect, Down syndrome, mitral regurgitation, MR, congestive heart failure, failure to thrive, heart murmur, atrial septal defect, patent ductus arteriosus, tricuspid stenosis, tricuspid atresia, perimembranous ventricular septal defect, VSD, hypoplastic left ventricle, hypoplastic LV, respiratory distress, exercise intolerance
