eMedicine Specialties > Cardiology > Congenital Heart Disease in the Adult
Endocardial Cushion Defects: Treatment & Medication
Updated: Oct 16, 2008
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Treatment
Medical Care
Medical treatment is designed to relieve the symptoms of CHF until operative correction is feasible. The objective of therapy is to avoid development of pulmonary vascular obstructive disease. When heart failure and associated pulmonary congestion are present, diuretics and digoxin are indicated.
Surgical Care
- Infants with partial AV septal defects that are symptomatic are referred for corrective surgery, which includes mitral valvuloplasty and closure of the atrial septal defect. Asymptomatic patients with an ostium primum defect are referred for elective repair after infancy.
- Patients with complete AV septal defects who do not have associated right ventricular outflow obstruction generally have pulmonary artery pressures near systemic levels. These patients will develop pulmonary vascular disease after the first year of life and usually are referred for corrective surgery in infancy.
- Historically, children were treated with pulmonary artery banding in infancy to protect the pulmonary vasculature from excessive blood flow and development of pulmonary vascular disease. Patients were referred for corrective surgery when older than 3-4 years.
- Corrective surgery can be performed even in early infancy, in several ways. A single Dacron patch can be used to close the atrial and ventricular septal defect (see Image 2). The right and left portions of the common AV valve are then resuspended from the patch. A 2-patch technique also may be used.
- Severe and irreversible pulmonary vascular disease is a contraindication to corrective surgery, and these children may be referred for cardiopulmonary transplantation.8,9,10,11
Diet
For infants in CHF, discretion with fluid intake and salt use is encouraged.
Activity
Rest during feeding is encouraged since one manifestation of dyspnea in these infants is the inability to feed. Generally, the child limits activity without encouragement.
Medication
Digitalis and diuretics are used to control the volume overload encountered in these patients until palliative or corrective surgery can be undertaken.
Diuretics
These agents are used to decrease volume overload.
Furosemide (Lasix)
Increases excretion of water by interfering with chloride-binding co-transport system, which in turn results inhibits sodium and chloride reabsorption in ascending loop of Henle and distal renal tubule.
Adult
20 mg/d PO/IV q8-24h, minimum; may increase to 600 mg/d
Pediatric
Neonates: 0.5-1 mg/kg PO/IV q8-24h; not to exceed 6 mg/kg PO or 2 mg/kg IV
Infants and children: 0.5-2 mg/kg PO/IV q6-12h; not to exceed 6 mg/kg
Continuous IV infusion: 0.05 mg/kg/h PO/IV; titrate to effect
Metformin decreases concentrations; interferes with hypoglycemic effect of antidiabetic agents and antagonizes muscle-relaxing effect of tubocurarine; aminoglycosides increase auditory toxicity—hearing loss of varying degrees may occur; may increase anticoagulant activity of warfarin; may increase plasma lithium levels and toxicity
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, CO2, glucose, creatinine, uric acid, calcium, and BUN determinations during first few months of therapy and periodically thereafter; use caution in hepatic disease; ototoxicity may occur in presence of renal disease, especially when used with aminoglycosides; may cause hypokalemia, alkalosis, dehydration, hyperuricemia, and increased calcium excretion; prolonged use in premature infants may cause nephrocalcinosis
Inotropic agents
These agents provide myocardial support in the perioperative period for patients with heart failure. The more restrictive the connection between the proximal and distal chambers, the more likely inotropic support will be required. A number of agents are available in this category.
Digoxin (Lanoxin)
Acts directly on cardiac muscle, increasing myocardial systolic contractions. Its indirect actions result in increased carotid sinus nerve activity and enhanced sympathetic withdrawal for any given increase in mean arterial pressure.
Adult
0.25 mg PO/IV q6h for 4 doses, then 0.25 mg PO/IV qd; adjust dose to blood levels
Pediatric
0.5 mg total dose PO/IV; then 0.25 total dose q8-18h for 2 doses
In premature infants, maximum dose is 20 mcg/kg PO/IV
In children, maintenance dose range is 8-12 mcg/kg/d PO/IV divided bid
Medications that may increase 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 levels include 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
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; IV calcium may produce arrhythmia 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; incomplete AV block may progress to complete block when treated with digoxin; exercise caution in hypothyroidism, hypoxia, acute myocarditis
More on Endocardial Cushion Defects |
| Overview: Endocardial Cushion Defects |
| Differential Diagnoses & Workup: Endocardial Cushion Defects |
 Treatment & Medication: Endocardial Cushion Defects |
| Follow-up: Endocardial Cushion Defects |
| Multimedia: Endocardial Cushion Defects |
| References |
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References
Person AD, Klewer SE, Runyan RB. Cell biology of cardiac cushion development. Int Rev Cytol. 2005;243:287-335. [Medline].
Cooper RS. Endocardial cushion defects: embryology, anatomy and pathophysiology. Adv Cardiol. 2004;41:118-26. [Medline].
Tardif JC, Schwartz SL, Vannan MA, et al. Clinical usefulness of multiplane transesophageal echocardiography: comparison to biplanar imaging. Am Heart J. Jul 1994;128(1):156-66. [Medline].
Weyman AE, Wann LS, Caldwell RL, et al. Negative contrast echocardiography: a new method for detecting left-to-right shunts. Circulation. Mar 1979;59(3):498-505. [Medline].
Williams RG, Rudd M. Echocardiographic features of endocardial cushion defects. Circulation. Mar 1974;49(3):418-22. [Medline].
Holmvang G, Palacios IF, Vlahakes GJ, et al. Imaging and sizing of atrial septal defects by magnetic resonance. Circulation. Dec 15 1995;92(12):3473-80. [Medline].
Jacobstein MD, Fletcher BD, Goldstein S, Riemenschneider TA. Evaluation of atrioventricular septal defect by magnetic resonance imaging. Am J Cardiol. Apr 15 1985;55(9):1158-61. [Medline].
Hanley FL, Fenton KN, Jonas RA, et al. Surgical repair of complete atrioventricular canal defects in infancy. Twenty-year trends. J Thorac Cardiovasc Surg. Sep 1993;106(3):387-94; discussion 394-7. [Medline].
Prêtre R, Dave H, Kadner A, Bettex D, Turina MI. Direct closure of the septum primum in atrioventricular canal defects. J Thorac Cardiovasc Surg. Jun 2004;127(6):1678-81. [Medline].
Silverman N, Levitsky S, Fisher E, et al. Efficacy of pulmonary artery banding in infants with complete atrioventricular canal. Circulation. Sep 1983;68(3 Pt 2):II148-53. [Medline].
Studer M, Blackstone EH, Kirklin JW, et al. Determinants of early and late results of repair of atrioventricular septal (canal) defects. J Thorac Cardiovasc Surg. Oct 1982;84(4):523-42. [Medline].
Carmi R, Boughman JA, Ferencz C. Endocardial cushion defect: further studies of "isolated" versus "syndromic" occurrence. Am J Med Genet. Jun 1 1992;43(3):569-75. [Medline].
Chin AJ, Keane JF, Norwood WI, Castaneda AR. Repair of complete common atrioventricular canal in infancy. J Thorac Cardiovasc Surg. Sep 1982;84(3):437-45. [Medline].
Cohen MS, Spray TL. Surgical management of unbalanced atrioventricular canal defect. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2005;135-44. [Medline].
Digilio MC, Giannotti A, Marino B, Dallapiccola B. Atrioventricular canal and 8p- syndrome. Am J Med Genet. Sep 1 1993;47(3):437-8. [Medline].
Ebels T, Ho SY, Anderson RH, et al. The surgical anatomy of the left ventricular outflow tract in atrioventricular septal defect. Ann Thorac Surg. May 1986;41(5):483-8. [Medline].
Fisher EA, Doshi M, DuBrow IW, et al. Effect of palliative and corrective surgery on ventricular volumes in complete atrioventricular canal. Pediatr Cardiol. Jul-Sep 1984;5(3):159-65. [Medline].
Korenberg JR, Kawashima H, Pulst SM, et al. Molecular definition of a region of chromosome 21 that causes features of the Down syndrome phenotype. Am J Hum Genet. Aug 1990;47(2):236-46. [Medline].
Neufeld EA, Sher M, Paul, MH. Pulmonary vascular disease in complete atrioventricular canal defect. Am J Cardiology. 1977;39:721-726.
Soto B, Bargeron LM, Pacifico AD, et al. Angiography of atrioventricular canal defects. Am J Cardiol. Sep 1981;48(3):492-9. [Medline].
Van Mierop LH, Alley Rd, Kausel HW, Stranahan A. The anatomy and embryology of endocardial cushion defects. J Thorac Cardiovasc Surg. Jan 1962;43:71-83. [Medline].
Vida VL, Barnoya J, Larrazabal LA, Gaitan G, de Maria Garcia F, Castañeda AR. Congenital cardiac disease in children with Down's syndrome in Guatemala. Cardiol Young. Jun 2005;15(3):286-90. [Medline].
Wakai CS, Edwards JE. Pathologic study of persistent common atrioventricular canal. Am Heart J. Nov 1958;56(5):779-94. [Medline].
Further Reading
Keywords
endocardial cushion defects, atrioventricular septal defects, atrioventricular canal defects, AV septal defects, canalis atrioventricularis communis, persistent atrioventricular ostium, abnormal development of endocardial cushions, heart failure, pulmonary vascular disease, congestive heart failure, CHF, ostium primum atrial septal defect, minimal insufficiency of the left AV valve, atrial arrhythmia, trisomy 21, Down syndrome
