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Population-based perspective of long-term outcomes after surgical repair of partial atrioventricular septal defect
Karl F Welke1, Cynthia D Morris, Emily King
1Division of Cardiothoracic Surgery, Oregon Health and Science University, Portland, Oregon 97239-3098, USA. welkek@ohsu.edu
Insights
Surgical repair of partial atrioventricular septal defect (PAVSD) shows lower survival than the general population but offers good long-term functional health for most patients without Down syndrome. Reoperation for valve issues remains a significant concern.
Area of Science:
- Cardiology
- Pediatric Surgery
- Congenital Heart Defects
Background:
- Partial atrioventricular septal defect (PAVSD) is a congenital heart condition requiring surgical intervention.
- Long-term outcomes, including survival, reoperation rates, and functional status, are critical for assessing surgical repair effectiveness.
Purpose of the Study:
- To evaluate long-term survival rates after PAVSD surgical repair.
- To determine the incidence of reoperations, particularly for left atrioventricular valve pathology.
- To assess the functional health status of patients post-PAVSD repair.
Main Methods:
- A population-based cohort study analyzed patients under 19 who underwent PAVSD repair between 1958 and 2000 in Oregon.
- Data collection involved cumulative, prospective follow-up using questionnaires and medical record review.
- Patient-reported health status was assessed using the SF-12, specifically for patients without Down syndrome aged over 15.
Main Results:
- Survival rates were 95% at 30 days, 87% at 10 years, and 78% at 30 years post-repair.
- 11.3% of patients required reoperation for left atrioventricular valve pathology, with lower weight, absence of Down syndrome, and lack of mitral valve cleft repair being significant risk factors.
- Patient-reported SF-12 scores indicated physical and mental component means comparable to age-adjusted norms for those without Down syndrome.
Conclusions:
- Surgical repair of PAVSD is associated with a survival rate lower than the general population.
- A significant reoperation rate for left atrioventricular valve pathology necessitates ongoing monitoring.
- Patients without Down syndrome generally achieve normal functional health status post-PAVSD repair.
Background:
This investigation was designed to determine long-term survival, reoperation rates, and functional status after surgical repair of partial atrioventricular septal defect (PAVSD).
Methods:
This population-based cohort study with cumulative, prospective follow-up by questionnaire and medical record review included all patients aged younger than 19 years old in the state of Oregon who underwent surgical repair of a PAVSD from 1958 to 2000. The incidence of early death, late death, and reoperation for left atrioventricular valve pathology were determined. Patient-reported health status as measured by the Medical Outcomes Study Short Form 12 (SF-12) was obtained for patients without Down syndrome when they were aged older than 15 years.
Results:
Repair of PAVSD was done in 133 patients. Median follow-up was 8.7 years for a total of 1541 person-years. Mean age at the initial operation was 5.2 +/- 5.1 years (median, 3.4 years). Mean weight was 19.2 +/- 16.0 kg (median, 13.2 kg). Survival was 95% at 30 days, 87% at 10 years, and 78% at 30 years. Reoperation for left atrioventricular valve pathology was done 15 patients (11.3%). Lower weight, absence of Down syndrome, and lack of mitral valve cleft repair were significantly associated with undergoing reoperation. Patient-reported health status was obtained in 35 patients. For this group, the mean SF-12 summary scores for the physical component (52.8 +/- 9.0) and the mean mental component (50.3 +/- 11.0) were not significantly different from age-adjusted norms.
Conclusions:
The survival rate for this simple cardiac defect is lower than the general population. In addition, the reoperation rate is significant. Despite this, in general, patients without Down syndrome can expect normal functional health status.

