Dr. Jay Junio

Case #3: The Ross Procedure

Ezra is a happy little kid who was born with a complicated heart. His aortic valve(AV), the valve from the heart that brings blood to the rest of the body is small and restricted. He initially underwent percutaneous aortic balloon valvotomy as a baby, which only opened up the valve for a while. As he grew, the opening became more and more restricted, thus limiting the flow of blood to vital organs. Now at 2 years old, he is having symptoms of heart failure and urgent surgery is needed as per his cardiologist, Dr. Kim Tolentino.

The plan was to use the pulmonic valve(PV), the valve from the heart that brings blood to the lungs to get oxygen, in place of his AV. This meant removing the entire PV from its right ventricle outflow position and placing it to the left ventricle outflow. This is also known as the Ross Procedure, a complex open heart surgery.

The procedure is very complicated and poses a lot of risks especially on a small patient. The last known Ross procedure performed at the Philippine Heart Center was more than 15 years ago by Dr. Serafin De Leon.

Ezra’s surgery was performed on the 1st week of May 2023. The most difficult part of his surgery was harvesting the pulmonic valve and placing it in the aortic position then reimplanting the coronaries (blood vessels supplying the heart). After completion, we were able to come off from the heart-lung bypass without any problems. His intraoperative echo showed good results and Ezra was then transferred to surgery ICU in stable condition. Four days after his surgery, his breathing tube was removed. A week after the surgery, he was back to his normal self and was able to go home.

The Ross is a technically challenging surgical procedure but it is the best procedure for children with severe aortic stenosis.
We are grateful to everyone, from Ezra’s cardiologists, anesthesiologists, intensivists and nurses, for making his surgical course a success.

Case #2: No U-Turn. Total Anomalous Pulmonary Venous Return (TAPVR)

One year ago, a 2-month-old baby was referred to me because of cyanosis and diaphoresis. By 2D echo, he was diagnosed to have Total Anomalous Pulmonary Venous Return (TAPVR). Because of his condition, the oxygenated blood from the lungs goes back to the right side of the heart instead of the left side, causing the entire heart to enlarge. A communication between the right and left side of the heart would be important for survival. When this communication is small or when there’s an obstruction in the pulmonary venous drainage, urgent surgery should be done irrespective of the baby’s age. If left uncorrected, they would have only few weeks or months to live.

Open-heart surgery was done to correct the problem which lasted 4 hours. Immediately after coming off bypass, the heart was smaller, and his oxygenation was significantly improved. He was transferred to surgical ICU in stable condition. While in the SICU, he developed ventilator-associated pneumonia which was treated by Dr. Bernadette Azcueta and her team with IV antibiotics and a lot of TLC. After staying for few weeks in ICU, he was finally brought to the ward and eventually discharged.

There is a dictum in congenital heart surgery that says, the smaller the patients are, the higher the risk. But there are heart conditions in which open heart surgery cannot be delayed any further.

The case above is an example that despite the complexity of the procedure, the young age of the patient and the complicated post-operative course, these babies have better survival when surgery is done at the right time.

Case 1: The Last Hope: A Case of an Old Dextro-Transposition of the Great Arteries (d-TGA)

An 8 year-old girl came to my clinic with bluish lips and nailbeds who was visibly catching her breath while talking. Hers was a case of CHD d-Transposition of the Great Arteries with Intact Interventricular Septum (TGA-IVS). Simply put, the aorta and pulmonary artery arose from the wrong sides of the heart.

She has undergone previous surgeries like BT Shunt, PA Banding and Atrial Septectomy when she was a baby to temporarily provide good oxygenation to her body. However, she has outgrown these procedures and without any intervention, she would only last for a few years. She desperately needed an Open Heart Surgery to correct the malformation. But her case was deemed too risky that she might not make it through surgery.

Her parents did not give up nor did we. Hence, we proceeded with Arterial Switch Operation which involved switching back the aorta and pulmonary arteries to their anatomical positions. This would have been a difficult surgery given the complexity, even more difficult given her age. The surgery lasted for 8 hours and proved to be a success. After a few days of intensive care management by Dr. Bernadette Azcueta, she was transferred out of the ICU with no post-operative complications. She was discharged after a week with pink lips and nailbeds, no longer catching her breath.

The case of Bea has proven one point - that there is always hope for every child. One must just be willing to take it.