More love from Moira after pioneering heart fix in St. Luke’s

By KARL REYES, MD

Six years ago, Anthony and Marjorie Young thought that they would not see better days for their first- born child, Moira, who was diagnosed with multiple congenital cardiac defects. Now, their love is abloom at the heart of their charming six-year-old daughter whose full-size spirit has enabled her to successfully hurdle her medical condition with greater hope and joy.

Moira was born with Situs Inversus Totalis (SIT) and a severe form of Tetralogy of Fallot (TOF) which is a congenital heart defect condition involving at least four anatomical abnormalities. People with SIT have a complete right to left reversal (transposition) of the thoracic and abdominal organs, including the heart, that appear in mirror image when examined or visualized by tests such as x-ray. In other words, her heart is literally pointing to the right side of her chest instead of the left where it would normally point.

Making matters more complicated for Moira was her severe form of TOF. In a normal heart, there is a right and left ventricle, with a wall called the interventricular septum dividing the ventricles. There are also two upper chambers, the right and left atria at the upper part of the heart that connect the atria to the ventricles. These atria receive blood that goes into the heart, and this blood goes to the ventricles before exiting through the pulmonary valves that bring blood to the lungs and to the body respectively. The pulmonary artery carries oxygen-poor blood from the right ventricle to the lungs to pick up oxygen. Then, oxygen-rich blood is brought back into the heart, then into the aorta and to the rest of the body.

Moira did not have all these normal connections. She had a large hole in her heart ( ventricular septal defect) and there was no artery that connected the heart to her lungs. Instead, blood going to her lungs came from different arteries called aortopulmonary collaterals. Also, because of these defects, the muscle in the right side of her heart became very thick, as it needs to push blood through the hole and into the rest of her body.

The Young couple initially brought Moira to a tertiary care center where she underwent a life saving operation called a Blalock-Taussig Shunt. While not a permanent solution, this shunt was able to provide more blood flow to her lungs and allowed her condition to partially stabilize. The procedure would allow Moira to look and feel better, although occasional bouts of difficulty in breathing, easy fatigability, poor feeding and turning blue at times would persist. But she would deteriorate again as months pass by. This was mainly because these artificial shunts eventually become smaller or the patient outgrows the shunt. Thus, another shunt procedure will be needed, but will not last long as well.

She was brought to St. Luke’s Center for Congenital Heart Disease in Global City with Dr. Charles Cuaso, Head Pediatric Cardiologist and myself where a plan to restore the blood flow in Moira’s heart to how it should normally be was formulated.

First, a delicate open-heart operation to recreate her pulmonary arteries all the way to its branches and give it a new connection to the heart was done. Then, Moira’s pulmonary arteries were closely monitored and followed up with further rehabilitation. Moira was taken to the cardiac catheterization lab where a first was performed: special expandable branch pulmonary artery stents were deployed to relieve the narrowings in the left pulmonary artery without the need to undergo open heart surgery. After this was performed, Moira’s heart was again evaluated and she was deemed ready for her 2nd stage operation which would completely restore the normal flow of blood in her heart.

Thus, the Center’s highly skilled team performed the first cardiac hybrid (combined cardiac catheterization and surgery) operation for Tetralogy of Fallot in the Philippines. Through the use of cardiac catheterization techniques, a right branch pulmonary artery stent was deployed in the operating room directly into the heart with the heart still beating and with Moira connected to the heart lung machine.

This ensured the completely unobstructed flow of blood to Moira’s lungs without the need for extensive surgical reconstruction, which would have been the only option in the past when these techniques were not yet available. The procedure was capped with Ventricular Septal Defect (VSD) closure, sealing the hole in Moira’s heart.

Though it took all of 8 hours to perform the hybrid surgery, the Young couple and the surgical team were nonetheless happy with the successful outcome that is expected to be long lasting. Best of all, it will now allow Moira to play and have fun to her heart’s desire and to live a normal life like everybody else.

The St. Luke’s Center for Congenital Heart Disease, although in existence for just two years now, is fast becoming the pioneering hub of its specialty, as well as being the place to go to for the most difficult-to-treat congenital cases in the Philippines. It provides a state-of-the-art comprehensive approach in the diagnosis and treatment of congenital heart diseases, from fetal life to adulthood. Among the pioneering feats it recently accomplished was the hybrid single ventricle palliation for Hypoplastic Left Heart Syndrome and a hybrid trans-ventricular pulmonic valvotomy.

Dr. KARL G. REYES is a Thoracic and Cardiovascular Surgeon at St Luke’s Medical Center Quezon City and Global City. He is the current Head of Pediatric and Congenital Heart Disease Surgery, St. Luke’s Center for Congenital Heart Disease at Global City and Head of the Congenital Heart Disease Section at Quezon City. He returns to us from Children’s Hospital Boston - Harvard Medical School in Boston, Massachusetts. For inquiries please contact the St. Luke’s Center for Congenital Heart Disease in Global City at 7897700 ext 2014 or 2015.