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A reconsideration of risk factors for the Fontan operation
J L Myers1, J A Waldhausen, H S Weber
1Department of Surgery, Milton S. Hershey Medical Center, Pennsylvania State University, Hershey 17033.
Insights
The modified Fontan operation is effective for complex congenital heart disease, with subaortic obstruction identified as a key risk factor for late death. Early intervention with specific surgical techniques can mitigate this risk.
Area of Science:
- Cardiology
- Pediatric Cardiac Surgery
- Congenital Heart Disease
Background:
- The Fontan operation is a palliative procedure for complex single-ventricle physiology.
- Previous surgical interventions are common in patients undergoing Fontan procedures.
- Variations in surgical techniques exist, including the original Fontan and modified approaches.
Purpose of the Study:
- To review the outcomes of the Fontan operation in a cohort of pediatric patients.
- To identify risk factors associated with early and late mortality after the Fontan procedure.
- To evaluate the effectiveness of surgical modifications in improving patient outcomes.
Main Methods:
- Retrospective review of 38 patients who underwent a Fontan operation.
- Comparison of outcomes between the original Fontan and modified Fontan procedures.
- Analysis of patient demographics, diagnoses, previous surgical history, and operative complications.
Main Results:
- Overall operative mortality was 10.5% (4 deaths).
- Subaortic obstruction was a significant risk factor for late death, particularly in patients with prior pulmonary artery banding.
- No deaths occurred in patients younger than 3 years old.
Conclusions:
- The modified Fontan operation is a viable option for complex congenital heart disease.
- Subaortic obstruction is a critical factor to address, necessitating specific surgical strategies like the Damus-Kaye-Stansel anastomosis.
- The modified Fontan operation can be safely performed in select patients as early as 6 months of age.
Abstract:
We reviewed our experience in 38 patients who underwent a Fontan operation. In the first five patients ages 7.5 to 23 years (mean, 15 years), a conduit was placed from the right atrium to the small right ventricle or the pulmonary artery (PA). The remaining 33 patients, ages 7 months to 14 years (mean, 4.8 years), had a modified Fontan operation with direct systemic venous or right atrial to PA anastomosis. The diagnoses were tricuspid atresia (n = 14), single ventricle (n = 10), hypoplastic right or left ventricle (n = 9), double-outlet right ventricle with inlet ventricular septal defect and pulmonary atresia or stenosis (n = 3), criss-cross ventricles and transposition of the great arteries (n = 1), and atrioventricular canal and anomalous pulmonary venous connection (n = 1). Thirty-two patients had previous surgery. Other procedures included PA banding (n = 7), systemic to PA shunts (n = 25), Norwood operation (n = 3), and a Damus-Kaye-Stansel anastomosis (n = 1), repair of total anomolous pulmonary venous connection (n = 1), a Blalock-Hanlon atrial septectomy (n = 1), and enlargement of a restrictive ventricular septal defect (n = 1). There were four operative deaths (10.5%), three from low cardiac output and one from subaortic obstruction. There were no deaths in patients younger than 3 years of age (n = 13). Subaortic obstruction developed in six of the seven patients who had pulmonary artery banding and resulted in three deaths. In our experience, diagnosis, previous surgery, type of previous operation, PA pressure, and younger age are not risk factors for early or late death. Subaortic obstruction is a major risk factor for late death. Accordingly we now perform a Damus-Kaye-Stansel anastomosis combined with a systemic to PA shunt in those children with excessive pulmonary blood flow who anatomically are likely to develop subaortic obstruction. A modified Fontan operation can be performed any time after 1 year of age and in some patients after 6 months of age, providing the anatomy and physiology of the patient are acceptable.