Long-Term Outcome After Mustard Repair at Young Age: Longitudinal Follow-Up Into the Fifth Decade After Surgery

Area of Science:

• Cardiology
• Pediatric Cardiology
• Cardiac Surgery

Background:

• Transposition of the great arteries (TGA) repair using the Mustard procedure has raised concerns regarding long-term patient morbidity.
• Evaluating the long-term sequelae of this surgical approach is crucial for patient management.

Purpose of the Study:

• To assess the long-term clinical outcomes and quality of life in patients with TGA who underwent the Mustard repair.
• To provide insights into the decades-long trajectory of health in this patient cohort.

Main Methods:

• A single-center, longitudinal cohort study followed patients who had Mustard repair for TGA in their youth.
• Comprehensive cardiac evaluations and subjective quality of life assessments were conducted every ten years up to 48 years post-surgery.

Main Results:

• Cumulative survival reached 61% at 48 years, with a shift in mortality causes from sudden death to heart failure after 35 years.
• Event-free survival was only 14%, with common events including cardiac reintervention, symptomatic arrhythmias, and heart failure.
• Systemic right ventricular function declined significantly, with most survivors exhibiting reduced ejection fraction and VO2max.

Conclusions:

• The Mustard repair for TGA is associated with significant long-term mortality and morbidity.
• Patient conditions tend to deteriorate over decades, highlighting the need for early consideration of advanced therapeutic strategies.