Right Ventricular Dominance Is Associated With Inferior Outcomes After the Extracardiac Fontan

Abstract

Background: Investigations of ventricular dominance and outcomes after the Fontan procedure have shown conflicting results. This may be due to the inclusion of multiple modifications of the Fontan or the omission of recently identified complications of the procedure. We examined the association between right ventricular dominance (RVD) and morbidity/mortality in a contemporary cohort following the extracardiac (EC) Fontan. Methods: We studied all pediatric patients at our center who underwent a predominantly fenestrated EC Fontan from 2004 to 2016. Outcomes assessed were freedom from (1) Fontan failure (death, takedown, listing for transplantation) and (2) complication (arrhythmia requiring medication, postoperative pacemaker, or implantable cardioverter defibrillator requirement, stroke, thrombosis in the Fontan circuit, protein losing enteropathy, plastic bronchitis, New York Heart Association class >2). We defined the perioperative period as occurring before hospital discharge or within 30 days of the Fontan. Results: A total of 137 patients (median age: 34 months, 62% male, 60% RVD) underwent the EC Fontan. Median duration of follow-up was 5.8 years (interquartile range: 2.4-9.0). Freedom from any event was 82.5% (RVD = 77%, LVD = 91%, χ2(1) = 5.03, P = .025) and RVD was associated with reduced event-free survival (hazard ratio: 2.94, P = .02). No confounders were identified. In the perioperative period, RVD was associated with reduced complication-free survival ( P = .004). After this period, RVD was associated with reduced failure-free survival ( P = .003). Conclusions: In this contemporary, single-center cohort of EC Fontan patients, RVD was associated with inferior outcomes.

National Fontan Operation short‐term outcomes at or below 2‐years‐of‐age compared to older than 2‐years‐of‐age

“…7 A reason could be due to an earlier progression to FO in single ventricle lesions with right dominance (such as HLHS) to better preserve ventricular function as there is conflicting information suggesting poor long-term FO outcomes with right sided dominant lesions such as HLHS. 18 Interestingly, the patients in the Western region of the United States were more likely to have an LF (27% vs. 12%, p = −.003). It is difficult to ascertain the reason behind this observation.…”

The impact of dominant ventricle morphology and additional ventricular chamber size on clinical outcomes in patients with Fontan circulation

“…Several authors have previously reported worse outcomes for patients with an anatomical FSRV during the Fontan pathway 8, [27][28][29] . A FSRV resulted to have a lower EF and reduced myocardial functional reserve when compared to FSLV, possibly related to an unsatisfactory functional adaptation to pressure and volume overload 11 .…”

Ventricular function and dyssynchrony in children with a functional single right ventricle using real time three‐dimensional echocardiography after fontan operation

“…1 Systemic right ventricular morphology appears to be associated with an adverse outcome, reduced exercise capacity, and reduced ventricular function after the Fontan operation. 2 The synchronization of electrical conduction and mechanical activity of the heart is the premise of normal systolic function, and worsening RV mechanical dyssynchrony may be associated with an overall decline in RV function. 3 Therefore, quantitative assessment of ventricular function and dyssynchrony is important for predicting the outcome of the Fontan operation in children with an FSRV.…”