Children’s surgeons in Krasnoyarsk have completed a multi-stage correction of a complex congenital heart defect

Radical correction of critical Ebstein’s anomaly with pulmonary atresia was successfully performed at the Krasnoyarsk FC. It took three years and three operations to completely correct the defect, the center’s press service reported.

A newborn girl weighing 2.7 kg was first admitted to the Krasnoyarsk Federal Sports Center at the beginning of 2018. Her condition was assessed as critical, and after a comprehensive examination a diagnosis was made: “critical Ebstein’s anomaly with pulmonary atresia.” A severe heart defect with an extremely high mortality rate. In this case, the tricuspid valve is so strongly displaced that it closes the exit from the right ventricle to the pulmonary artery, because of this, the left ventricle of the heart is compressed so much that it cannot function fully.

The child’s condition deteriorated sharply, and cardiac surgeons had to perform a palliative operation – remove the right ventricle from the bloodstream by closing the tricuspid valve with a patch, and bypass the blood flow from the aorta to the pulmonary artery using a prosthesis. Thus, only one ventricle of the child’s heart functioned, but the little patient’s life was saved.

Having assessed the situation at three months, cardiac surgeons performed the second stage of correction of the defect – the formation of a bidirectional cavapulmonary anastomosis, or Glenn operation. The child had her aorto-pulmonary prosthesis replaced, which had become too small for her, and the extra load was removed from her heart.

The third and final stage of the radical correction of the defect took place in April 2021.

“We conducted a full examination and came to the conclusion that it is possible to load the right ventricle with blood volume, and it should cope,” said the head of the pediatric cardiac surgery department of the Federal Center for Cardiac Surgery. Pavel Teplov. – It was a big risk, since calculations are one thing, reality is another. In fact, we had a more complex operation than Fontan, and the risks were much higher, but the result was not comparable: either one ventricle remained working, or, as expected, two. We took this risk together with our parents, and we succeeded. Three major surgeries are behind us, the hemodynamics of the heart are correct, we have restored the function of the valve, closed all the defects in the heart and formed the pulmonary artery. They discharged an already active child with rosy cheeks.”

As reported Chief Physician of the Krasnoyarsk Sports Center, Professor Valery Sakovich, Since the beginning of 2021, the center has operated on more than 30 newborns with critical heart defects. Among them were children weighing less than 2 kg; operations were performed, including under artificial circulation. “Over the 11 years of operation of our center, we have accumulated extensive experience in the treatment of congenital heart defects, some of which require interventions of the highest category of complexity,” he concluded.

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