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Published on: November 18, 2018
Modified Fontan operation in patients with anomalies of systemic and pulmonary venous connection
M Ruzmetov1, M D Rodefeld, P Vijay
1Section of Cardiothoracic Surgery, James W. Riley Hospital for Children and Indiana University Medical Center, Indianapolis, IN 46202, USA. markruz@hotmail.com
Insights
The modified Fontan operation (FO) is safe for patients with single ventricle anomalies and abnormal venous connections. This procedure offers comparable survival rates to standard Fontan operations, even in complex cases like visceral heterotaxy syndrome.
Area of Science:
- Congenital Heart Surgery
- Pediatric Cardiology
- Cardiac Surgery
Background:
- Anomalies in systemic and pulmonary venous connections were previously considered contraindications for the Fontan operation (FO).
- Single ventricle anomalies often present with complex venous drainage issues.
- The modified FO aims to address these challenges in pediatric cardiac surgery.
Purpose of the Study:
- To detail technical considerations for modified Fontan operations in patients with anomalous venous connections.
- To identify mortality risk factors in this specific patient cohort.
- To evaluate the safety and efficacy of the modified FO in complex congenital heart disease.
Main Methods:
- Retrospective review of 63 patients with anomalous venous connections undergoing modified Fontan procedures (1989-2004).
- Analysis included patients with combined systemic and pulmonary venous anomalies, isolated systemic venous anomalies, and isolated pulmonary venous anomalies.
- Assessment of outcomes including early mortality, reoperations, and long-term survival, with a mean follow-up of 4.6 years.
Main Results:
- 51 patients (81%) had prior palliative surgery; 25 had visceral heterotaxy syndrome.
- Early outcomes included 1 early death and 2 Fontan takedowns; 2 reoperations for baffle revision.
- Late mortality was 8% (5 deaths), with 71% achieving Fontan completion. Actuarial survival was 92% at 1 year and 91% at 5-10 years.
Conclusions:
- The modified Fontan operation is a viable and safe option for single ventricle anomalies with anomalous systemic or pulmonary venous connections.
- Outcomes, including survival rates, are comparable to patients with normal venous connections.
- This approach extends the applicability of the Fontan procedure to a broader range of complex congenital heart defects.
Abstract:
The presence of anomalies of the systemic and pulmonary venous connection associated with single ventricle anomalies has been considered a contraindication for the Fontan operation (FO). The aim of this study is to outline the technical considerations associated with the presence of anomalies of systemic and/or pulmonary venous connections and to identify the risk factors for mortality in this group of patients undergoing the modified FO. Between 1989 and 2004, 63 patients (median age, 3.2 years) with anomalous systemic or pulmonary venous connection underwent a Fontan procedure at our institution. Nine patients had a combination of anomalous systemic and pulmonary venous connection, 49 patients had anomalous drainage only from the systemic circulation, and 5 patients had isolated anomalies of pulmonary venous return. Visceral heterotaxy syndrome was diagnosed in 25 patients. Previous palliative operations had been performed in 51 patients (81%). There was 1 early death, and 2 patients required take down of Fontan procedures. Two patients required reoperation for revision of the atrial baffle. At a mean follow-up of 4.6 +/- 3.4 years, there have been 5 late deaths (8%) and 45 patients (71%) have undergone Fontan completion. Actuarial survival was 92% at 1 year and 91% at 5 and 10 years-not significantly different from the overall survival of the Fontan patients. We conclude that the modified FO can be successfully performed in patients with anomalous systemic or pulmonary venous connections, including those with visceral heterotaxy syndrome, with morbidity and mortality rates that do not differ significantly from those achieved in all patients with normal connections.
