Long-term results after surgical repair of incomplete endocardial cushion defects
E L Ceithaml1, F M Midgley, L W Perry
1Division of Cardiothoracic Surgery, University Hospital, Jacksonville, FL 32209.
Insights
Surgical repair of incomplete endocardial cushion defects in children shows low hospital mortality. Long-term outcomes reveal a need for reoperation in some, particularly for mitral valve issues.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease Surgery
- Cardiac Surgery Outcomes
Background:
- Incomplete endocardial cushion defects (IECDs) are congenital heart abnormalities requiring surgical intervention.
- Surgical outcomes and long-term management of pediatric IECD repair are critical for patient prognosis.
Purpose of the Study:
- To evaluate the long-term efficacy and safety of surgical repair for incomplete endocardial cushion defects in children.
- To identify risk factors for reoperation and long-term complications after IECD repair.
Main Methods:
- Retrospective analysis of 56 children undergoing IECD repair since 1964.
- Surgical techniques included patch closure of ostium primum defects and mitral valvuloplasty.
- Follow-up data analyzed for mortality, arrhythmias, need for reoperation, and functional status.
Main Results:
- Low hospital mortality (1.8%) with one early death.
- Late mortality was 5.7% (3 deaths) over 378 patient-years.
- 22.6% of patients required reoperation, primarily for mitral regurgitation, with a significant correlation to pre-repair severity.
- 88.5% of survivors are in NYHA class I functional status.
Conclusions:
- Surgical repair of incomplete endocardial cushion defects in children offers favorable short-term and long-term outcomes.
- Mitral valve function and regurgitation severity are key predictors of late reoperation needs.
- Long-term surveillance is essential for managing potential complications like mitral regurgitation and arrhythmias.
Abstract:
Since 1964, 56 children (mean age, 6.7 years) underwent repair of incomplete endocardial cushion defects. Forty patients had isolated ostium primum defects. Additional congenital defects were present in 17 patients (30%). All patients underwent patch closure of the ostium primum defect and 47 of 56 patients (84%) underwent mitral valvuloplasty. Hospital mortality was 1.8% (one death). Arrhythmias developed in 7 other patients in the early postoperative period, of which six were transient and resolved completely. One patient required early pacemaker placement for complete heart block. Cumulative follow-up was 378 patient-years. There were three late deaths (5.7%), and additional operations were required in 12 patients (22.6%). Seven of these 12 patients required mitral valve replacement for severe mitral regurgitation. The mean interval between initial repair and mitral valve replacement was 4.2 years, with only three valves needing replacement within 12 months. There was a significant correlation between the severity of mitral regurgitation before initial repair and subsequent need for mitral valve replacement. Late onset atrial arrhythmias have developed in 6 patients. Current functional status has been evaluated in 50 of 52 surviving patients and 88.5% are in NYHA class I, with the remainder in class II.
