It is very rare for a child to be born with more than one malformation. And it is even more unusual for more than one malformation to be addressed in a single surgical intervention. In fact, it had never happened in Spain until the surgeons at Sant Joan de Déu repaired serious deficiencies in the heart and trachea of a baby in the same procedure.
The Barcelona hospital has released today the details of the unprecedented surgery, which took place on July 17, lasted five hours, and required the participation of twenty professionals. After a month in the ICU, the patient was discharged.
The baby had Tetralogy of Fallot, a serious congenital heart defect that impeded the flow of blood to the lungs. This malformation was detected by the healthcare professionals who were attending to the mother during her pregnancy through ultrasound scans.
The dilemma: whether to operate on the trachea first, start with the heart, or perform both procedures at the same time
A few days after birth, doctors detected that the child had a very narrow trachea along its entire length, causing respiratory failure and continuous stridor (abnormal respiratory sound), symptoms that would worsen over time.
The specialists in the cardiology and otolaryngology departments at Sant Joan de Déu decided to wait for the baby to gain weight while resolving a dilemma: should they operate on the heart first and then the trachea, start with the trachea, or do both at the same time?
Finally, the family was advised to repair both malformations in a single surgery to avoid the risk of the unoperated organ failing postoperatively and complicating the baby's situation. “If the tracheal part fails, it can compromise the cardiac part and vice versa,” explains Stefano Congiu, head of cardiac surgery.
“It is a very exceptional case. There are very few cases in the world. We have not found them described in the scientific literature. Normally, affected children present one malformation or another, but it is very rare for them to have both at the same time,” he continues.
The patient was eight months old when he underwent surgery
The baby was 8 months old when he entered the operating room. During the first hours, the surgeons repaired the heart malformation by closing the abnormal communication between the two ventricles with a pericardial patch. They then widened the outlet of the right ventricle towards the lungs in order to normalize blood circulation and oxygenation. A cardiogram was used to verify the complete correction of Tetralogy of Fallot.
So, with the patient still on extracorporeal circulation, the otorhinolaryngologists came into play to widen the tracheal duct, which improved breathing and stopped the stridor.
“The two individual interventions are not very complex or rare on their own, but by performing them simultaneously, the risks and complexities increase. If either part of the two interventions fails, the entire procedure fails. The margin for error is absolutely minimal,” stated the cardiothoracic surgeon.
If one part of the interventions fails, everything fails; the margin of error is minimal”
The specialists at Sant Joan de Déu have not found precedents of a similar case in the scientific literature. It's a unique case. “Now we will publish a scientific article mainly to say that both things can be operated at the same time and the patient can have a normal life.”
Indeed, the child has passed the first year of life, is growing normally, according to the regular check-ups at the hospital, and will not present any sequelae in the future.
