Only 40 years ago, a TGA was a death sentence. Today, thanks to medical progress, congenital transposition of the great arteries can be corrected well. This is also the case when TGA is associated with a ventricular septal defect (VSD) and a narrowing of the left ventricular outflow tract (LVOTO).

However, the presence of such a "complex TGA" poses special technical challenges for pediatric cardiac surgery. An initial study shows what correction using different procedures means in the long term for the risk of having to undergo reoperation or even dying from the congenital malformation. The good news: Given the severity of the heart defect, the risk of death is low, even with multiple repeat surgeries. At the same time, newer surgical techniques offer hope for improved long-term outcomes.

Diagnosis TGA-LVOTO

Approximately one in ten to one in twenty children born with a congenital heart defect have a TGA. Approximately one-third also have a ventricular septal defect (VSD), which helps to maintain blood flow. In about a quarter of these patients, including twice as many boys as girls, the outflow tract of the left ventricle is narrowed. This is known as LVOTO.

Since the late 1970s, five different surgical procedures have been developed to save the lives of those affected. The Mustard or Senning operation, also known as atrial reversal operation, which was common in the beginning, is now rarely performed. Today, depending on the type of TGA, VSD, and LVOTO, the most common procedures are the arterial switch operation, the Rastelli operation, the Nikaidoh operation, and the reparation à l'étage ventriculaire, or REV procedure.

The research team led by Fabian Kari, a cardiac surgeon specialized in congenital heart defects at the University Heart Center Freiburg, Germany, has now been able to investigate the long-term risks of the different surgical procedures for the first time in a multicenter study. The study was based on the long-term medical data of 139 TGA-VSD-LVOTO patients registered in the National Registry.

Good Research Base Enables Reliable Results

The researchers evaluated data on diagnoses, operations, and interventions from a follow-up period of approximately 1,700 patient-years. "This is unique in this form and only possible because the data could be collected continuously over a long period of time by the National Registry. These patients had been treated at 15 different German centers between 1968 and 2016. Without the reliable central collection of their data by the registry, such a study would not be feasible," says Fabian Kari.

High Long-Term Survival Rate

The results of the study clearly demonstrate the high quality of medical care in this field in Germany. Despite the complex nature of the heart defect, the thirty-year survival rate is around 80 percent, regardless of the surgical method. "Considering the severity of the diagnosis, this is encouraging," says Fabian Kari.

However, the researchers also found differences. "We found a trend towards a survival advantage after the newer surgical methods, Nikaidoh and especially REV procedure. The increasingly used REV procedure seems to be associated with the least number of reoperations as of today. In 64 percent of cases, no further surgery was required after a successful REV procedure.

Risk of Reoperation

After the initial life-saving procedure, which is performed in infancy in most cases and is successful in more than 90 percent of newborns, infants and toddlers, several follow-up surgeries are needed to get the heart working as it should. Repeat surgeries may be necessary later in life. The study results now provide more detailed information.

"The gold standard, the Rastelli procedure, led to more reoperations. This was to be expected because the animal material used does not last indefinitely," says cardiac surgeon Fabian Kari. In addition, the artificial connection from the left ventricle to the aorta created during the procedure sometimes needs to be replaced as the body grows. However, the study also shows an increase in "unexpected" reoperations. One reason for this was the recurrence of outflow tract obstructions.

Good Results With the REV Procedure

The conclusion of the researchers: "We have achieved good results with the REV procedure over the last five years. The exciting thing here is the activation of self-healing forces through the connection with the body's own materials. So far, we have seen that the body copes very well with this during growth," summarizes Fabian Kari.

The researchers hope to achieve even better results with the REV procedure in the long run. However, they will only know for sure when the results of the newer REV procedure, which is now being used more and more alongside the proven Rastelli procedure, are confirmed over a longer period of time. This will not be the last long-term study in the National Registry.