eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology
Atrioventricular Septal Defect, Complete: Treatment & Medication
Updated: Oct 3, 2007
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
Although their effectiveness has been questioned, diuretics, digoxin, and angiotensin-converting enzyme (ACE) inhibitors have all been used to alleviate tachypnea and failure to thrive. In many medical centers, the surgical mortality rate at age 2-3 months is 5% or less. Therefore, unless symptoms are dramatically relived, medical treatment for children with symptoms of CHF is not pursued for more than a few weeks before definitive repair.
Surgical Care
Treatment for a complete AVC defect is surgical.
- Single-stage complete repair is currently preferred, but occasional cases of refractory CHF in a low-birth-weight infant may be palliated with the placement of a pulmonary artery band.
- Some patients with common AV canal (CAVC) have several additional muscular VSDs, banding of the pulmonary artery may alleviate their CHF for 6-12 months, during which time the VSDs may spontaneously close and thus simplify eventual CAVC surgery.
- The surgical mortality rate should be low. In the most recently published review of surgical outcomes of 363 patients with AVSDs who were treated between 1982 and 1995, the early mortality rate was 10.3%, and the 10-year survival rate was 83%.6
- A pulmonary-artery band, placed on the main pulmonary artery by means of a small lateral or anterior thoracotomy incision, obviates cardiopulmonary bypass in a premature neonate or small infant. However, it has the risks of distorting the origins of the branch pulmonary arteries if it migrates and of complicating eventual CAVC surgery if it erodes through the media and intima. Pulmonary-artery banding is best used, when deemed necessary, for only a few months in a patient who then will undergo complete intracardiac repair and pulmonary-artery band takedown.
- A major aspect of CAVC repair involves creating a competent mitral valve. A pericardial patch can be used for this augmentation and for tricuspid valve repair. Repair is occasionally done with 2 patches: a pericardial patch for the atrial septal defect and a polytetrafluoroethylene patch (Gore-Tex patch; W.L. Gore & Associates, Inc, Newark, DE) for the VSD with routine closure of the mitral valve cleft. The 2-patch technique with routine cleft closure and atrial septal incision may lower the incidence of residual mitral regurgitation.
- Ten Harkel et al (2005) recently reported intermediate follow-up results in patients who underwent surgical repair of AVSDs.7 During a mean follow-up of 66 months, 19% had severe mitral-valve regurgitation, and 9% required reoperation. Of note, 13% of patients with severe mitral-valve regurgitation in the immediate postoperative period had significantly improved mitral-valve function. For this reason, the authors cautioned against reoperation in the early postoperative period unless it is absolutely necessary.
- Additional aspects of complete repair of CAVC may include relief of associated LVOT obstruction, PDA ligation, removal of a previously placed pulmonary artery band, repair of stenosis of a pulmonary arterial branch, or relief of aortic-arch obstruction. Recent data suggest that children with AVSDs and Down syndrome have a prognosis better than that of children with the same cardiac lesion but not Down syndrome.
- Residual AV-valve insufficiency or stenosis is a major determinant of long-term outcome.
- Total circular annuloplasty is a simple procedure to help reduce AV-valve regurgitation, although most patients with severe AV-valve insufficiency or stenosis require more complex mitral valvuloplasty techniques. The need for mitral-valve replacement is not rare over the course of long-term follow-up, but it is ideally delayed until an adult-size prosthetic valve can be implanted.
- CAVC may be associated with other surgical conditions, including subaortic stenosis, coarctation of the aorta (CoA), TOF, and total anomalous pulmonary venous return. Each associated lesion may complicate complete repair and make it difficult to achieve a good hemodynamic result. In addition, these defects may add potential risk over follow-up (eg, recoarctation after CoA repair or pulmonary insufficiency after repair of TOF).
- Surgically induced AV block is a known complication of CAVC repair. Permanent pacing is required if AV conduction does not return postoperatively.
- For complex cardiac lesions involving an unbalanced CAVC, total cavopulmonary connection, otherwise known as a Fontan operation, may be indicated.
- In the presence of TOF, an aortic monocusp is used to compensate for deficient right AV-valve tissue. Right-dominant, unbalanced biventricular repair can be successfully completed in patients with mild LV hypoplasia. However, careful preoperative evaluation of the adequacy of the LV to support the systemic circulation is imperative.
- De Oliveira et al (2005) reported their experience with 2-ventricular repair of patients with unbalanced AVSDs and small RVs.8 Patients with a small RV had a high mortality rate, with an 87% 10-year survival, compared with a 100% survival rate in surgical patients with balanced AVSDs. Although a median sternotomy is the usual surgical approach, the thoracotomy approach was safely used for CAVC repair in some centers.
Consultations
Given the complexity of CAVC, a multidisciplinary team is usually required. This could include pediatricians, neonatologists, pediatric cardiologists, pediatric cardiothoracic surgeons, and pediatric intensivists, as well as a nurse coordinator and supportive ancillary staff. Additional consultants might include a geneticist for genetic counseling and a nutritionist.
Diet
A high-energy diet is needed because cardiac shunting results in high metabolic demands. Even at 125 kcal/kg/d, children still may not appropriately gain weight. Some children have such high metabolic demands that extraordinary energy intake, exceeding 150 kcal/kg/d, is necessary for growth. Pulmonary edema can lead to tachypnea that makes oral intake of nourishment too difficult. A nasogastric tube may be needed in severe cases of CHF with failure to thrive.
Activity
After the patient recovers from surgery, normal daily activities should be allowed.
Medication
Medical treatment is similar to treatment of any cardiac defect with volume overload. Digoxin is frequently used to decrease the heart rate and to increase inotropy, although little evidence (if any) suggests that it is effective in patients with CHF due to left-to-right shunt lesions. Diuretics may decrease preload and ACE inhibitors decrease afterload. Care must be taken when administering ACE inhibitors to reproductive-age females, given their teratogenic effects. More recent, but limited, data suggest that the use of beta blockers in patients with left-to-right shunts who have CHF improves symptoms.9
The daily dosage of digoxin is approximately 5-10 mcg/kg/d. The diuretic used most frequently in the author's institution is furosemide 1-2 mg/kg/d. In children with clinical signs of CHF, 58% improved with enalapril. The mean maximal dose was 0.3 mg/kg/d. The most significant adverse effect observed was renal failure, particularly in young infants with large left-to-right shunts. Most of the older patients in the author's institution who need ACE inhibitors are treated with lisinopril because of its lower cost and long half-life. The dose generally is 0.5 mg/kg/d, but is individualized for each patient. Data about the efficacy of beta-blockers in patients with large left-to-right shunts is sparse. In small studies, beta-blockers appear to decrease renin levels and heart rates in infants with CHF due to left-to-right shunts.
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. Current American Heart Association guidelines also recommend subacute bacterial endocarditis (SBE) prophylaxis for patients who have a complete repair and those who have a jet lesion aimed at a patch to impair the growth of endothelial cells on the patch.10 This situation may occur in patients with atrioventricular septal defects and can only be discovered by the use of imaging modalities such as echocardiography. For more information, see Antibiotic Prophylactic Regimens for Endocarditis.
Inotropic agents
The agents provide symptomatic improvement for CHF. Positive inotropic agents increase the force of contraction of the myocardium and are used to treat acute and chronic CHF. Positive or negative chronotropic agents may also increase or decrease the heart rate, provide vasodilatation, or improve myocardial relaxation. These additional properties influence the choice of drug for specific circumstances.
Digoxin (Lanoxicaps, Lanoxin)
Acts directly on cardiac muscle, increasing myocardial systolic contractions. Indirect actions increase carotid sinus nerve activity and enhance sympathetic withdrawal for any given increase in mean arterial pressure.
Adult
0.125-0.5 mg PO qd
Pediatric
Infants: 6-8 mcg/kg/d PO divided bid
2-5 years: 10-15 mcg/kg/d PO divided bid
5-10 years: 7-10 mcg/kg/d PO divided bid
>10 years: 3-5 mcg/kg PO qd
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 may increase levels
Aminoglutethimide, antihistamines, cholestyramine, neomycin, penicillamine, aminoglycosides, oral 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 may decrease levels
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; hypercalcemia predisposes patient to digitalis toxicity, and hypocalcemia can make digoxin ineffective until serum calcium levels are normal; to prevent digitalis toxicity, start magnesium replacement therapy in hypomagnesemia; incomplete AV block may progress to complete block; caution in hypothyroidism, hypoxia, and acute myocarditis
Diuretic agents
These agents provide symptomatic improvement for CHF and promote the excretion of water and electrolytes by the kidneys. They are indicated to treat heart failure or hepatic, renal, or pulmonary disease when sodium and water retention has resulted in edema or ascites.
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.
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 concentrations; 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; increased plasma lithium levels and toxicity possible when taken 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
Hypokalemia after long-term use
ACE inhibitors
These drugs are indicated for treatment of symptomatic CHF. 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.
Captopril (Capoten)
Short-acting ACE inhibitor. Predominant action is suppressing the renin-angiotensin aldosterone system. Prevents conversion of angiotensin I to angiotensin II (potent vasoconstrictor), increasing levels of plasma renin and reducing aldosterone secretion.
Adult
6.25-12.5 mg PO tid; not to exceed 150 mg tid
Pediatric
0.1-2 mg/kg/d PO divided tid/qid; increase dose as tolerated
Nonsteroidal anti-inflammatory drugs (NSAIDs) may reduce hypotensive effects; may increase digoxin, lithium, and allopurinol levels; rifampin decreases levels; probenecid may increase captopril levels; concurrent diuretics may enhance hypotensive effects
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
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Pregnancy category D in second and third trimester; caution in renal impairment, valvular stenosis, or severe CHF
Enalapril (Vasotec)
Competitive ACE inhibitor. Reduces angiotensin II levels, decreasing aldosterone secretion.
Adult
2.5-5 mg/d PO; increase prn
Dosing range: 10-40 mg/d PO qd or divided bid
Alternative: 1.25 mg/dose IV over 5 min q6h
Pediatric
0.1-0.3 mg/kg/d PO qd or divided bid
NSAIDs may reduce hypotensive effects; ACE inhibitors may increase digoxin, lithium, and allopurinol levels; rifampin decreases levels; probenecid may increase levels; concurrent diuretics may enhance hypotensive effects
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
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Pregnancy category D in second and third trimester; caution in renal impairment, valvular stenosis, or severe CHF
Lisinopril (Prinivil, Zestril)
Prevents conversion of angiotensin I to angiotensin II (potent vasoconstrictor), reducing aldosterone secretion.
Adult
10 mg/d PO; increase 5-10 mg/d q1-2wk; not to exceed 40 mg
Pediatric
Not established, limited data for 0.2 mg/kg PO qd initially; increase as BP and symptoms (eg, dizziness, light-headedness)
May increase digoxin, lithium, and allopurinol levels; probenecid may increase levels; coadministration with diuretics, increases hypotensive effects; concurrent diuretics and NSAIDs may enhance hypotensive effects
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
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Pregnancy category D in second and third trimester; caution in renal impairment, valvular stenosis, or severe CHF
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References
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Further Reading
Keywords
complete atrioventricular septal defect, AVSD, endocardial cushion defect, ECD, common AV canal defect, AVC defect, CAVC, ostium primum atrial septal defect, posterior ventricular septal defect, VSD, congestive heart failure, CHF, Eisenmenger syndrome, tachypnea, Down syndrome, Dandy-Walker malformation, Joubert syndrome, Ritscher-Schintal syndrome
