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[Repair of complete atrioventricular canal before one year of age]
D Metras1, B Kreitmann, F Wernert
1Unité de chirurgie cardiaque, CHU de la Timone-Enfants, Marseille.
Insights
This study evaluated surgical outcomes for 22 infants with complete atrioventricular canal (CAVC) before age one. Early surgical intervention for CAVC in infants shows a significant mortality rate, highlighting the need for improved surgical techniques.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease Surgery
- Neonatal Intensive Care
Context:
- Complete atrioventricular canal (CAVC) is a complex congenital heart defect often requiring early surgical intervention.
- Infants with CAVC, particularly those with trisomy 21 or pulmonary arterial hypertension, face significant surgical risks.
- This study focuses on a cohort of 22 infants operated on before one year of age.
Purpose:
- To report the surgical outcomes and challenges in infants with complete atrioventricular canal (CAVC) operated on before one year of age.
- To analyze the characteristics of patients, surgical techniques, and early postoperative results.
- To identify factors contributing to mortality in this high-risk pediatric population.
Summary:
- Twenty-two infants with CAVC, mostly with trisomy 21 and resistant congestive heart failure, underwent surgery before one year old.
- All operations utilized deep hypothermia with circulatory arrest and the Rastelli technique with autologous pericardial patches.
- Mortality was 13.6% (3/22), with causes including low cardiac output, persistent pulmonary hypertension, and malignant hyperthermia.
Impact:
- The findings underscore the high mortality associated with early surgical repair of CAVC in complex pediatric cases.
- Results suggest potential areas for improvement in surgical management and postoperative care for CAVC patients.
- This study contributes to the understanding of risk factors and outcomes in neonatal and infant cardiac surgery for CAVC.
Abstract:
We report a series of 22 children with complete atrioventricular canal (CAVC) operated upon before the age of one year. The youngest patient was 1 month old and weighed 3 kg. The patients' mean age was 7 months and their mean weight was 5.4 kg. 15 patients had trisomy 21, and in one patient the CAVC was associated with tetralogy of Fallot. The remaining 21 patients had congestive heart failure resistant to medical treatment, with clinical evidence of pulmonary arterial hypertension (PAHT). At the time of surgery, 2 patients had been under artificial respiration for one month. All patients were explored by echocardiography and cardiac catheterization. The mean pulmonary pressure/aortic pressure ratio was 0.92; the mean pulmonary flow rate/systemic flow rate ratio (Qp/Qs) was 2.9/1 and the mean pulmonary resistance/systemic resistance ratio (Rp/Rs) was 0.22. All children were operated upon under deep hypothermia with circulatory arrest (mean 54 min); the patient with tetralogy of Fallot had an additional period of extracorporeal circulation. Fourteen patients had Rastelli's type A CAVC and 8 had type C CAVC. All were operated upon by the classical Rastelli technique, using a single autologous pericardial patch; in none of the patients was the septal "slit" or "commissure" entirely closed. Three patients died within 48 hours of the operation: the first one died of sudden low cardiac output 18 hours after surgery, the second one of persistent PAHT and the third one of malignant hyperthermia. The patient under artificial respiration before surgery could not be disconnected and died on the 30th post-operative day.(ABSTRACT TRUNCATED AT 250 WORDS)