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Experience with repair of complete atrioventricular canal
Insights
Surgical repair of complete atrioventricular canal in 29 pediatric patients demonstrated good long-term hemodynamic status. While operative and late deaths occurred, survivors showed excellent outcomes, though high pulmonary vascular resistance requires further study.
Area of Science:
- Cardiovascular Surgery
- Pediatric Cardiology
Background:
- Complete atrioventricular canal (CAVC) is a complex congenital heart defect.
- Surgical repair is the standard treatment for CAVC.
- Long-term outcomes and risk factors require ongoing evaluation.
Purpose of the Study:
- To evaluate the long-term outcomes of surgical repair for complete atrioventricular canal.
- To identify factors influencing mortality and morbidity after CAVC repair.
Main Methods:
- Retrospective review of 29 patients undergoing CAVC repair since 1969.
- Analysis of pre- and post-operative catheterization data, including pulmonary hypertension and pulmonary vascular resistance (PVR).
- Assessment of patient demographics, surgical anatomy (Rastelli types A, C, and variants), and follow-up data.
Main Results:
- Operative mortality was 3 patients; late mortality was 5 patients (4 infectious).
- Pre-repair pulmonary hypertension was present in all patients.
- Twenty-one surviving patients showed excellent long-term hemodynamic status with follow-up up to 10 years 7 months.
Conclusions:
- Surgical repair of CAVC can lead to excellent long-term hemodynamic status in survivors.
- Infectious etiology contributed significantly to late mortality.
- Further long-term studies are necessary to fully understand the impact on patients with elevated pulmonary vascular resistance (PVR).
Abstract:
Twenty-nine patients at out institution have undergone repair of complete atrioventricular canal since 1969. There were 3 operative and 5 late deaths, 4 of which were of infectious etiology. Age at operation ranged from 2 months to 12 years (mean, 50 months). Weight ranged from 3.6 kg to 30 kg (mean, 12 kg). Before repair, catheterization studies revealed pulmonary hypertension in all patients with unobstructed pulmonary arteries. Pulmonary to systemic flow ratio ranged from 1.5 to 10.6 (mean, 3.5). Pulmonary vascular resistance (PVR) ranged from 0.7 to 21.7 (mean, 5.3) Wood units/m2. At repair, 14 patients had Rastelli type A anatomy, 14 had type C, and 1 patient had a variant with crossing chordae and double-outlet right ventricle (DORV). Postoperative catheterization has been done in 16 patients 2 months to 8 years (mean, 30 months) after repair. One patient had residual ventricular shunting and later underwent successful repair. A 2-year-old patient had severe mitral regurgitation and died following mitral valve replacement. One patient required a permanent pacemaker. The 21 surviving patients have been followed from 7 months to 10 years 7 months and have excellent hemodynamic status. Long-term studies are needed to assess the ultimate effect on patients with high PVR.