Morphological findings contributing to a failed Fontan procedure. Twelve-year experience

Area of Science:

• Pediatric Cardiology
• Congenital Heart Disease
• Cardiac Surgery

Background:

• The modified Fontan procedure is a palliative surgery for complex congenital heart disease.
• Early mortality after the Fontan procedure remains a concern.
• Understanding causes of death is crucial for improving outcomes.

Purpose of the Study:

• To determine the causes of death in patients who underwent the modified Fontan procedure.
• To identify risk factors associated with mortality after the procedure.

Main Methods:

• Retrospective review of 37 patients who died within 2 months of the modified Fontan procedure.
• Analysis of patient demographics, cardiac morphology, and previous interventions.
• Autopsy findings were correlated with clinical data.

Main Results:

• Myocardial issues, including hypertrophy and ischemic injury, were the primary cause of death.
• Subaortic stenosis was significantly more prevalent in non-survivors (40%) compared to survivors (15%).
• Pulmonary artery banding, especially when combined with subaortic stenosis, was associated with severe myocardial hypertrophy and death.

Conclusions:

• Myocardial pathology is the leading cause of death post-modified Fontan procedure.
• Advanced myocardial hypertrophy, exacerbated by pulmonary artery banding and subaortic stenosis, is a key factor.
• These findings highlight the need to address subaortic stenosis and optimize surgical strategies.