Many are unaware of Leo’s long and complicated history in the Cardiac Intensive Care Unit (CICU) that began when he was just two weeks old. Prenatal echocardiograms were inconclusive in determining whether Leo had hypoplastic left heart syndrome (a syndrome where the left side of the heart is too small to support the body and the child must undergo three surgeries to create a single ventricle pumping chamber) or a simple coarctation of the aorta (an easy fix of dilating the aorta). The limiting factor for Leo would be whether his mitral valve would grow big enough to allow enough blood through. If so, then his other defects could be managed and he could get a mitral valve replacement when he was much older. This was the best case scenario for him.
After Leo’s birth, he remained in the gray area. Was his mitral valve sufficient, or should Leo have a single ventricle heart? After numerous echos and a CT scan, doctors determined that, although his mitral valve was abnormal, it did not appear to be causing any problems. The surgeon proceeded with an aortic arch repair for Leo’s hypoplastic aortic arch. This was more severe than a coarctation (narrowing) as his aorta essentially did not form and no blood could get through. Though deadly if untreated, it was not a particularly complicated surgery relatively speaking. It went smoothly and Leo recovered in the CICU and was expected to go home in about 2-3 weeks. All of the dread, all of the worst case scenario imaginings, the mention of a potential need for a heart transplant, all seemingly disappeared and his family’s nightmare appeared to be almost over.
The next morning was a different story. A new murmur was discovered and an echo revealed that Leo suddenly had pulmonary hypertension now that he had normal blood flowing through his heart with the closure of the PDA (a hole that is kept open so blood can still get to a baby’s body after birth until their surgery). The mitral valve was more of a problem than anyone expected. Blood was backing up into his left atrium and causing increased pressure in the pulmonary artery. What did this mean for Leo? The only answer was to try to remove as much fluid as possible, relax the pulmonary artery with medication, and see if he can get by.
Leo did get by for awhile. Gradually the fluid left his lungs, he weaned down to regular oxygen, and tolerated his feeds. There was even a going home checklist in his room with only a couple of boxes left.
On January 26th, Leo suddenly had an increase in his work of breathing. A chest x-ray revealed that the fluid in his lungs returned and an echo showed that his pulmonary hypertension was worsening. If he continued like this, it would cause permanent damage to his lungs. Leo would need a second surgery: a Norwood procedure, the first stage in converting the heart to a single ventricle, or an attempt at a mitral valve repair that was high-risk and only a temporary fix. Neither was a very good option in his case. The mitral valve surgery was risky and the Norwood procedure could only be done safely with low pulmonary artery pressures, which Leo did not have. It was an emotional week of unknowns but his family had complete faith in his surgeon. Knowing he was in such skilled hands made the walk to the pre-op area less excruciating for his mother.
Stay tuned for the next blog post to hear how Leo’s second surgery turned out and please consider donating to COTA on behalf of Leo! Thank you for reading!