Long-term surgical results of transposition of the great arteries with left ventricular outflow tract obstruction

Abstract

Objective The objective of this study was to evaluate the long-term surgical results of transposition of the great arteries with left ventricular outflow tract obstruction. Methods We conducted a retrospective study of patients with transposition of the great arteries or double outlet right ventricle with left ventricular outflow tract obstruction undergoing biventricular repair between 1980 and 2017. Results One hundred and eleven patients were enrolled and classified into five groups: atrial switch (n = 20), arterial switch (n = 12), Nikaidoh (n = 7), Rastelli (n = 48), and REV operation groups (n = 24). Early mortality was highest in Nikaidoh group (29%). Median follow-up was 18.2 years. Long-term survival was by far lowest in Nikaidoh group and comparable among the other 4 groups. Freedom from reoperation at 20 years was lowest in Rastelli group (32.1%) due to right ventricular outflow tract-related reoperations. While having no recurrence of left ventricular outflow tract obstruction, the arterial switch operation group had a high proportion of substantial neo-aortic regurgitation (29%). Conclusions The long-term survival was satisfactory regardless of the surgical technique except Nikaidoh group. The surgical option for transposition of the great arteries with left ventricular outflow tract obstruction should be selected based on the features of the respective procedures.

Accurate quantification of pulmonary perfusion ratio in children with congenital heart disease using partial volume corrected 4D flow cardiac magnetic resonance

“…The pulmonary vasculature increases in size and changes naturally over time due to aging, but in patients with TGA or TOF, the pulmonary vasculature is subjected to abnormal physiological changes that most likely affect the vasculature growth. As the result of the alterations in the pulmonary vasculature, the pulmonary perfusion may also undergo changes, and this can lead to true reductions in pulmonary blood flow and even to complications such as pulmonary hypertension and right ventricular dysfunction ( 3 , 5 ). Hence, these patient cohorts need close surveillance with repeated monitoring over the course of their lifetimes, starting in childhood.…”

“…TGA is a rare but serious heart defect, in which the two main arteries leaving the heart are transposed, resulting in two separate circulations that do not exchange oxygen efficiently. This abnormal morphology requires surgical intervention for correction, typically through the arterial switch procedure, e.g., the Rastelli procedure or the Nikaïdoh technique ( 5 ). The surgical correction could alter the pulmonary blood flow distribution and lead to complications such as pulmonary hypertension and right ventricular dysfunction.…”

“…Accurate quantification of pulmonary perfusion ratio in children with congenital heart disease using partial volume corrected 4D flow cardiac magnetic resonance Introduction Pulmonary blood flow is often reduced in patients with congenital heart defects (CHD). In patients with transposition of the great arteries (TGA) or tetralogy-of-Fallot (TOF), decreased pulmonary blood flow may affect the development of the lungs and the right heart, leading to poorer outcome (1)(2)(3)(4)(5). TGA is a rare but serious heart defect, in which the two main arteries leaving the heart are transposed, resulting in two separate circulations that do not exchange oxygen efficiently.…”

“…Many patients with TOF have residual right ventricle outflow obstruction that can lead to decreased pulmonary blood flow and increased resistance in the pulmonary circulation. The abnormal morphology can be surgically corrected with a transannular patch or pulmonary homograft (1)(2)(3)(4)(5). After surgery, pulmonary branch stenosis is not uncommon in both TOF and TGA.…”

Atrial Septal Defect vs Ventricular Septal Defect: Getting the Right Mix in Transposition of the Great Arteries

“…In this operation, an intracardiac baffle is created tunneling the left ventricle to the aorta and an external conduit connects the right ventricle to the pulmonary artery [8]. A retrospective study of 48 patients who underwent the Rastelli procedure between 1980 and 2017 showed an overall survival of 75.5% at 20 years [9]. A patient with this anatomy typically will require the initial palliative procedure of a Blalock-Taussig-Thomas (BTT) shunt to provide pulmonary blood flow.…”