Surgeons perform first heart procedure of its kind on young patient with scimitar syndrome
Heart surgeons at Kindai University Hospital in Japan used a “modified double-decker technique” to treat a two-year-old patient diagnosed with scimitar syndrome. It is believed to be the very first time scimitar syndrome has been treated with this particular technique.
The double-decker technique is more typically used to treat young patients presenting with partial anomalous pulmonary venous return to the superior vena cava. For this alternative approach, the surgeons reconstructed two blood flow pathways using the patient’s atrial wall—no artificial materials were required.
Genichi Sakaguchi, MD, a veteran cardiothoracic surgeon who specializes in structural heart disease treatments, led the procedure with assistance from several colleagues. The young patient was discharged 10 days later, and no postoperative complications have been reported.
“The patient was referred to our hospital for suspected congenital heart disease from another public hospital after developing a fever,” the group said in a statement. “The patient was diagnosed with scimitar syndrome by cardiac echocardiogram and contrast-enhanced computed tomography. After the surgery, there were no problems with the reconstructed blood flow pathways in the right pulmonary veins and inferior vena cava.”
The team added that this new-look approach “reduces the risk of obstruction” by creating wide, separate pathways for blood flow.
“The conventional intra-atrial tunneling divides the inferior vena cava into two pathways for the right pulmonary vein and the inferior vena cava,” the group added. “That is why the conventional technique is likely to create narrower pathways and develop obstructions. In addition, the surgical site is expected to grow following the patient’s somatic growth because these pathways were reconstructed by the pedicled atrial wall without any artificial material.”
What is scimitar syndrome?
Scimitar syndrome is a rare congenital heart defect seen in babies with an underdeveloped right lung and pulmonary artery. It earned its name due to the fact that the right pulmonary vein in patients with scimitar syndrome sometimes takes the shape of a scimitar sword.
According to the team at Kindai University Hospital, one to three out of every 100,000 children are born with this defect. They noted that delayed treatment can lead to pulmonary hypertension, right heart failure, respiratory failure and other significant complications.
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