How Well Are Patients Doing Up to 30 Years after a Mustard Operation?

Abstract

Presence of a VSD at the time of the Mustard operation defines a distinct subgroup with an increased risk for late death. Long-term survivors were in a good functional status but had to be reoperated frequently due to baffle complications that seemed to increase in adulthood.

Transverse strain predicts exercise capacity in systemic right ventricle patients

“…Late dysfunction of the systemic right ventricle (RV) in patients with complete transposition of the great arteries (TGA) after atrial switch (AS) repair is well documented [1][2][3]. Chronic pressure overload of the systemic RV and atrioventricular valve regurgitation frequently lead to contractile dysfunction and heart failure symptoms [1][2][3].…”

Mechanical circulatory support in patients with heart failure secondary to transposition of the great arteries

“…1 In a series of patients operated on between 1974 and 1982, 7 of 88 patients undergoing a Mustard procedure for TGA required reoperation for systemic ventricular failure. 7 Late systemic ventricular failure during the second and third decades of life in patients with anatomic repair procedures or in those with CCTGA poses few options. In patients with biventricular failure, transplantation is the treatment of choice, and data show that long-term survival is equivalent to non-congenital heart failure patients once the peri-operative risk (which may be as high as 60%) is excluded.…”

Pacemaker and Defibrillation Implantation in Patients with Transposition of the Great Arteries

“…By middle adulthood, it is estimated that over half of l-TGA patients, and nearly all d-TGA patients who underwent a Mustard or Senning procedure, will experience systolic dysfunction. 33,34 Furthermore, nonsustained VT is fairly common in the setting of right ventricle dysfunction. As such, careful consideration should be given prior to reflexively implanting ICDs in this population, as the risks associated with such complex and technically challenging procedures may outweigh the potential benefits.…”