Anatomic repair of congenitally corrected transposition of the great arteries (CCTGA), a rare congenital heart condition, has significant advantages over physiologic repair, shows a new, first-of-its-kind meta-analysis published in the World Journal for Pediatric and Congenital Heart Surgery. Led by NewYork-Presbyterian/Columbia physicians and researchers, the study, which includes 44 previous studies, sheds new light on a long-standing treatment controversy.
The study found that the two surgeries had similar rates of mortality during the procedure and in the hospital. However, compared to patients receiving a physiologic repair, anatomic repair patients had a significantly lower rate of dying after discharge, lower reoperation rates, and less postoperative ventricular dysfunction.
“This gives us very important information about the value of the anatomic repair that we can provide to patients,” says lead study author David M. Kalfa, MD, PhD, a pediatric cardiothoracic surgeon, Director of the Pediatric Heart Valve Center, and Surgical Director for the Initiative for Pediatric Cardiac Innovation at NewYork-Presbyterian/Columbia. “Although there are limitations to this kind of study, it pushes us towards anatomic repair.”
Dr. David Kalfa
This gives us very important information about the value of the anatomic repair that we can provide to patients. Although there are limitations to this kind of study, it pushes us towards anatomic repair.
— Dr. David Kalfa
The study also broke down anatomic repair patients by the type of operation patients received. Patients who got atrial and arterial switch procedures had lower in-hospital mortality and reoperation rates than those who had atrial switch with Rastelli.
A Challenging Condition
In CCTGA, which accounts for less than 1% of congenital heart diseases, the ventricles and their attached valves are reversed. The condition is typically associated with other abnormalities, included ventricular septal defect (VSD), pulmonary stenosis, and Ebstein’s anomaly, due to the stress placed on the ventricle from the anatomic mismatch.
Surgical repair of the condition falls into the two camps. Traditionally favored, the physiologic approach leaves the ventricles in the reversed position. It’s a simpler surgery, with just the associated defects repaired.
“For example, if the patient has a VSD (a communication between the two ventricles), you close the hole,” says Dr. Kalfa. “If the patient has pulmonary stenosis with some obstruction between the left ventricle and the pulmonary artery, you repair this obstruction.” Nevertheless, right ventricular dysfunction and heart failure can occur over time.
The anatomical repair repositions the ventricles to their proper place. It’s a more technically challenging surgery, but mitigates the long-term risks linked to physiologic repair, says Dr. Kalfa.
“We undertook this meta-analysis to try to better understand what the best option is for these patients,” he says. “The question was, ‘Should we go for higher risk anatomy repair right away or go for less risky physiologic repair that has complications down the road?’”
Although Dr. Kalfa and his team expected the anatomic version to have clear advantages, he says, “We were surprised by the fact that the anatomic repair was not associated with higher in-hospital mortality. We thought it might be a little riskier compared to a physiologic repair and that you may have more immediate post operative mortality, but that was not the case. All the findings are pushing us towards the anatomic repair.”
We were surprised by the fact that the anatomic repair was not associated with higher in-hospital mortality. We thought it might be a little riskier compared to a physiologic repair and that you may have more immediate post operative mortality, but that was not the case.
— Dr. David Kalfa
The Study Data
The research included 44 studies and 1857 patients with 535 in the physiologic group and 1322 in the anatomic group. Patients ranged from five days old to 71 years. Anatomic repair patients had significantly less post-discharge mortality (6.1% vs 9.7%; P = .006), lower reoperation rates (17.9% vs 20.6%; P < .001), and less postoperative ventricular dysfunction (16% vs 43%; P < .001). Patients who had atrial and arterial switch surgeries had significantly lower in-hospital mortality (4.3% vs 7.6%; P = .026) and reoperation rates (15.6% vs 25.9%; P < .001), compared to patients who had atrial switch with Rastelli.
“This data gives our community more information to justify an anatomic repair for patients with CCTGA,” says Dr. Kalfa. “It remains safe despite being quite complex and is associated down the road with better outcomes.”
This data gives our community more information to justify an anatomic repair for patients with CCTGA. It remains safe despite being quite complex and is associated down the road with better outcomes.
— Dr. David Kalfa
As a meta-analysis, the findings aren’t the last word on the question of which surgery is best for patients. But the small number of CCTGA patients makes a randomized controlled trial, the gold standard, impractical, Dr. Kalfa says.
Dr. Kalfa is working with the Congenital Heart Surgeons’ Society (CHSS) as principal investigator to establish a longitudinal registry with the goal of collecting prospective and retrospective data on patients with the disease. In addition he is involved with an international multicenter study to investigate the optimal management of CCTGA, which presented data at the AATS annual meeting in May 2023, CHSS Annual Meeting in October 2023, and was accepted for presentation at the next AATS meeting next year.
“There is a lot of momentum around this,” says Dr. Kalfa. “It’s pretty exciting and will likely bring a lot of visibility to the team here at NewYork-Presbyterian.”
