Long-Term Outcomes After the Fontan Procedure

Study Questions:

What are the long-term outcomes of patients who survive to age ≥16 years after a Fontan procedure?

Methods:

The Australian and New Zealand Fontan Registry (ANZFR) was used for this analysis. Mortality was confirmed using the National Death Index. Risk factors for morbidity and mortality were studied using multivariable analysis.

Results:

Six hundred and eighty three adult survivors of the Fontan procedure were identified in the ANZFR registry. Fontan type was atriopulmonary (AP) connection in 201 and total cavopulmonary connection (TCPC) in 482 patients. Of the TCPCs, 249 were lateral tunnels and 233 were extracardiac conduits. Survival for the entire cohort was 90% at 30 years and 80% at 40 years of life. Survival at 30 years of age was significantly worse for the AP patients (p = 0.03). From a symptomatic standpoint, 53% were New York Heart Association (NYHA) class I at most recent follow-up. At 40 years of age, only 41% of Fontan patients were free of serious events. Long-term morbidity included 20% of patients experiencing at least one new arrhythmia, 6% requiring a permanent pacemaker, 7% with a thromboembolic event, and 21% requiring surgical re-intervention.

Conclusions:

The authors concluded that morbid complications are common in adults after the Fontan procedure and that there is substantial premature mortality, particularly in patients after AP Fontan procedures.

Perspective:

This study made use of the comprehensive ANZFR to understand long-term outcomes for adults who had a Fontan procedure. Although the long-term survival is better than previous single reports in the very early surgical era, there remains significant morbidity and mortality for adults with Fontan circulation. A large majority of patients (65%) in this study had systemic left ventricular morphology, while systemic right ventricles were present in 24%. Ventricular morphology was indeterminate in 11% of patients. Additional risk factors beyond AP Fontan circulation for poor prognosis included male gender and moderate or severe atrioventricular valve regurgitation. This study will be helpful in educating patients and families regarding long-term concerns with the Fontan circulation.

Clinical Topics: Arrhythmias and Clinical EP, Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Cardiac Surgery and Arrhythmias, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and Heart Failure, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Interventions, CHD and Pediatrics and Quality Improvement, Acute Heart Failure, Interventions and Structural Heart Disease

Keywords: Arrhythmias, Cardiac, Cardiac Surgical Procedures, Fontan Procedure, Heart Defects, Congenital, Heart Failure, Heart Ventricles, Morbidity, Mortality, Premature, Outcome Assessment (Health Care), Pacemaker, Artificial, Risk Factors, Survivors, Thromboembolism


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