Partial atrioventricular canal with congestive heart failure in the first year of life: surgical options
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A Giamberti1, B Marino, D di Carlo
1Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Hospital, Rome, Italy.
Insights
Infants with partial atrioventricular canal and heart failure need early surgery. Mitral valve diameter is key for surgical success, with smaller diameters indicating higher mortality risk.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease Surgery
Background:
- Partial atrioventricular canal (pAVC) is a congenital heart defect.
- A subset of pAVC patients require early surgical intervention due to severe congestive heart failure within the first year of life.
Purpose of the Study:
- To analyze surgical outcomes in infants with pAVC requiring early intervention.
- To identify factors influencing prognosis, particularly the role of mitral valve size.
Main Methods:
- Retrospective analysis of 128 pAVC patients from 1982-1995, focusing on 35 who underwent surgery before age one.
- Surgical procedures included anatomic repair, aortic coarctectomy, and Norwood operation.
- Correlation of surgical results with echocardiographic mitral valve diameter.
Main Results:
- Mortality rates varied by procedure: 29% for anatomic repair, 22% for aortic coarctectomy, and 100% for Norwood operation.
- Infants with a mitral valve diameter < 2.5 x 10-2 m/m2 had a significantly higher mortality risk during repair.
- Long-term follow-up revealed need for secondary mitral valve procedures.
Conclusions:
- Early surgical intervention is critical for infants with pAVC and heart failure.
- Left-sided obstructive lesions and mitral valve anomalies significantly impact outcomes.
- Echocardiographic mitral valve diameter is a valuable tool for guiding surgical timing and strategy.
Background:
An important subgroup of patients with partial atrioventricular canal require an operation in the first year of life because of refractory congestive heart failure.
Methods:
From June 1982 to April 1995, of 128 patients with partial atrioventricular canal, 35 patients (27%) underwent surgical treatment at less than 1 year of life. Associated cardiac anomalies were present in 22 patients. Only 7 patients (20%) had Down's syndrome. Five patients with left ventricular hypoplasia underwent aortic coarctectomy (3 patients) or Norwood operation (2 patients). The other 30 patients underwent anatomic repair in 24 cases and aortic coarctectomy in 6. The surgical results of patients submitted for anatomic repair were retrospectively correlated with the echocardiographic mitral valve diameter.
Results:
There were 7 deaths (29%) after anatomic repair, 2 (22%) after aortic coarctectomy, and 2 (100%) after Norwood operation. Infants with a mitral valve diameter less than 2.5 x 10-2 m/m2 died at repair. In a mean follow-up of 73.5 months there were five secondary mitral valve plasties and three repairs after aortic coarctectomy.
Conclusions:
Among patients with partial atrioventricular canal, there is an important subgroup with clinical signs of heart failure in the first year of life. Left-sided obstructive lesions and complex mitral valve anomalies seem to play a fundamental role in the clinical evolution and prognosis of these patients. The echocardiographic mitral valve diameter may be useful for determining the correct surgical indication.