Mustard and Senning Correction for TGA in Sweden and Denmark

Study Questions:

What are the long-term survival and predictors of mortality after the Mustard and Senning procedure for transposition of the great arteries (TGA)?

Methods:

Surgical records were obtained from all centers that performed Mustard and Senning procedures in Sweden and Denmark. Information about deaths was obtained from the Danish and Swedish Centralized Civil Register.

Results:

A total of 468 atrial switch patients were identified. Perioperative 30-day mortality was 20%, and 60% were alive after 30 years of follow-up. Only pacemaker implantation is predictive of long-term outcome (hazard ratio [HR], 1.90; 85% confidence interval [CI], 1.05-3.46; p = 0.04). The risk of reoperation was correlated to the presence of associated defects and where the first Mustard/Senning operation was performed.

Conclusions:

The long-term survival of patients with Mustard and Senning correction for TGA appears to be primarily determined by factors in the right ventricle and tricuspid valve, and not the timing of or the type of surgery in childhood. The need for pacemaker implantation appears to be associated with increased mortality.

Perspective:

This study reports long-term survival after the Mustard and Senning procedure in Sweden and Denmark. Operative factors such as type of surgery (Mustard vs. Senning) did not impact long-term survival. Previous studies have shown patients do well for a period of time, with subsequent functional decline. The overall 30-year survival was 60%, although this includes significant perioperative mortality. It will be very helpful to understand the late natural history for this patient population to guide in decisions regarding medical management and need for cardiac transplantation. Interestingly, pacemaker placement was a risk factor for long-term mortality. It is unclear whether this pacemaker-associated mortality was related to underlying issues creating the need for a pacemaker, or complications of long-term pacing itself.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Cardiac Surgery and CHD & Pediatrics, Congenital Heart Disease, CHD & Pediatrics and Interventions, CHD & Pediatrics and Quality Improvement, Interventions and Structural Heart Disease

Keywords: Cardiac Surgical Procedures, Heart Defects, Congenital, Pacemaker, Artificial, Risk Factors, Survival, Transposition of Great Vessels, Tricuspid Valve


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