Endocardial Cushion Defects Treatment & Management

Updated: Sep 15, 2014
  • Author: Mary C Mancini, MD, PhD, MMM; Chief Editor: Park W Willis IV, MD  more...
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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.

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Surgical Care

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  • 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 below). The right and left portions of the common AV valve are then resuspended from the patch. A 2-patch technique also may be used.
    Repair of the endocardial cushion defect. The patc Repair of the endocardial cushion defect. The patch is covering the ostium primum atrial septal defect.
  • Severe and irreversible pulmonary vascular disease is a contraindication to corrective surgery, and these children may be referred for cardiopulmonary transplantation. [10, 11, 12, 13]
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Diet

For infants in CHF, discretion with fluid intake and salt use is encouraged.

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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.

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