Related Experiment Video
Updated: Jun 7, 2025
Wireless Telemetry Device Implantation in a Fontan Ovine Model for Continuous and Long-Term Hemodynamic Monitoring
Published on: May 2, 2025
Defining the causes for Fontan circulatory failure in total cavopulmonary connection patients
Joeri Van Puyvelde1,2, Filip Rega1,2, Werner Budts2,3
1Department of Cardiac Surgery, University Hospitals Leuven, Leuven, Belgium.
Insights
Fontan failure, defined by mortality or severe symptoms, occurred in about 10% of patients within 15 years after total cavopulmonary connection. Right ventricular dominance led to systolic dysfunction, while left ventricular dominance resulted in restrictive pathophysiology or high pulmonary vascular resistance.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- The Fontan procedure is a palliative surgery for complex single-ventricle congenital heart defects.
- Long-term outcomes and causes of failure after total cavopulmonary connection (TCPC) require ongoing investigation.
Purpose of the Study:
- To identify and analyze the factors contributing to Fontan failure after total cavopulmonary connection.
- To differentiate failure mechanisms based on ventricular dominance.
Main Methods:
- A retrospective review of 217 patients who underwent TCPC between 1988 and 2023.
- Analysis of Fontan failure causes, including mortality, heart transplantation, and functional decline.
- Stratification of outcomes based on right versus left ventricular dominant morphology.
Main Results:
- Fontan failure occurred in 24 patients (11.1%), with freedom from failure rates of 77.2% at 20 years.
- Systolic ventricular dysfunction was the most common cause of failure (29%), particularly in right ventricular dominant patients.
- Restrictive pathophysiology and high pulmonary vascular resistance were common in left ventricular dominant patients.
Conclusions:
- Approximately 10% of patients experience Fontan failure within 15 years post-TCPC.
- Ventricular dominance dictates the primary mechanism of Fontan failure: systolic dysfunction for RV dominance, and restrictive pathophysiology/high PVR for LV dominance.
Objectives:
This study aims to identify the causes of failure in Fontan patients with a total cavopulmonary connection.
Methods:
We conducted a comprehensive review of all patients who underwent a total cavopulmonary connection procedure at our centre between 1988 and 2023, aiming to identify and analyse the factors contributing to Fontan failure (defined as mortality, heart transplantation, Fontan takedown, protein-losing enteropathy, plastic bronchitis or New York Heart Association Functional Classification class III or IV).
Results:
The study included 217 patients (median age at time of Fontan completion 3.7 years) with a median follow-up of 12.7 years (interquartile range 7.2-17.7). Systolic ventricular function decreased significantly over time in patients with right ventricular dominant morphology (P = 0.002), while systolic ventricular function remained stable in patients with left ventricular dominant morphology. Fontan failure occurred in 24 patients, with estimated freedom from Fontan failure rates of 97.7% [95% confidence interval (CI), 95-99] at 1 year, 93.9% (95% CI, 89-97) at 15 years and 77.2% (95% CI, 65-86) at 20 years of follow-up. Systolic ventricular dysfunction was the most common cause of failure (29%), followed by atrioventricular valve regurgitation (16.7%), a high pulmonary vascular resistance (16.7%), restrictive pathophysiology (16.7%) and obstruction (12.5%). Patients with right ventricular dominance developed most often systolic ventricular dysfunction, while patients with left ventricular dominant morphology developed most often restrictive pathophysiology or a high pulmonary vascular resistance.
Conclusions:
Approximately 10% of patients experienced Fontan failure within 15 years postoperatively. Patients with right ventricular dominance experienced progressive decline due to systolic dysfunction, while those with left ventricular dominance exhibited failure due to restrictive pathophysiology or high pulmonary vascular resistance.
Related Concept Videos
01:21Acute Respiratory Failure-II
The underlying physiological abnormalities that contribute to hypoxemic respiratory failure include:
01:30Acute Respiratory Failure-III
01:22Mitral Stenosis I: Introduction
01:26Imbalances in Cardiac Output
CHF can occur due to the failure of either side of the heart. Left-side failure leads to pulmonary congestion—the right side continues to send...
01:21Acute Respiratory Failure-I
Definition: It is defined by specific criteria based on blood gas measurements. Hypoxemia happens when the partial pressure of oxygen (PaO2) falls below 60 mmHg. At the same time,...
01:15Overview of Systemic and Pulmonary Circulation
The oxygenated blood is sent...