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Cardiac Function After Tetralogy of Fallot/Complete Atrioventricular Canal Repair
Elizabeth H Stephens1, Jennifer Tingo2, Marc Najjar1
11 Division of Cardiac, Thoracic, and Vascular Surgery, Columbia University Medical Center, New York, NY, USA.
Insights
Surgical repair of complete atrioventricular canal (CAVC) with tetralogy of Fallot (TOF) in infants shows low mortality. Long-term management of the right ventricular outflow tract (RVOT) remains a challenge after this complex congenital heart defect repair.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Cardiac Surgery Outcomes
Background:
- Complete atrioventricular canal (CAVC) and tetralogy of Fallot (TOF) repair is increasingly performed in infancy.
- Midterm outcomes, including valve function and residual obstruction, are less understood than perioperative results.
Purpose of the Study:
- To evaluate midterm outcomes after primary repair of CAVC with TOF.
- To assess valve function, ventricular function, and residual obstruction following CAVC/TOF repair.
Main Methods:
- Retrospective review of 20 patients undergoing CAVC/TOF repair between January 2005 and December 2014.
- Two-patch repair for CAVC; TOF repair involved transannular patch or valve-sparing techniques.
- Echocardiographic follow-up to assess cardiac function and obstruction.
Main Results:
- No in-hospital or late mortality; 20% reoperation rate.
- At midterm follow-up (mean 3.0 years), 15% had moderate atrioventricular valve regurgitation and 35% mild stenosis.
- One patient (5%) had moderate right ventricular outflow tract (RVOT) obstruction; moderate pulmonary insufficiency noted in 33% of valve-sparing patients.
Conclusions:
- Complete atrioventricular canal (CAVC) repair concomitant with tetralogy of Fallot (TOF) can be achieved with low mortality.
- Long-term management of the right ventricular outflow tract (RVOT) presents ongoing challenges after CAVC/TOF repair.
Background:
Repair of complete atrioventricular canal (CAVC) with tetralogy of Fallot (TOF) is a challenging operation increasingly being performed as a complete, primary repair in infancy. Previous studies have focused on perioperative outcomes; however, midterm valve function, ventricular function, and residual obstruction have received little attention.
Methods:
We retrospectively reviewed 20 patients who underwent CAVC/TOF repair (January 2005 to December 2014). A two-patch repair was used in all patients to correct the CAVC defect. Tetralogy of Fallot repair included transannular patch in 11 (65%) patients and valve-sparing in 6 (35%) patients.
Results:
The average age at surgery was 72 ± 122 weeks, 40% were male, and 80% had trisomy 21. Mean echo follow-up was 3.0 ± 3.0 years. There were no in-hospital or late mortalities. The rate of reoperation was 20%. At the latest follow-up, moderate left atrioventricular valve regurgitation was present in three (15%) patients and mild stenosis present in seven (35%) patients. One (5%) patient had moderate right ventricular outflow tract (RVOT) obstruction. The valve-sparing population was smaller at the time of surgery than the non-valve-sparing cohort (body surface area: 0.28 ± 0.04 vs 0.42 ± 0.11, P = .002) and less likely to have had a previous shunt (0% vs 64%, P = .01). Among the valve-sparing patients (six), at the latest follow-up, moderate pulmonary insufficiency was present in two (33%) patients.
Conclusion:
Repair of CAVC concomitant with TOF can be performed with low mortality and acceptable perioperative morbidity. Management of the RVOT remains a challenge for the long term.
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