The Words No Parent Wants to Hear

“There are some issues with Evan’s echocardiogram.”

Kendra took the initial phone call from the NICU nurse, called me, and relayed the message. There was something wrong with Evan’s heart, but they wouldn’t elaborate until I arrived at the hospital for his 8pm feeding. I hung up the phone and held back tears as I pushed my dessert plate away from me. My mom and I had gone out to Boston’s North End with two of her friends for restaurant night, and planned on heading to the hospital after dinner to visit with the twins.

Evan was born with a heart murmur, as was Kate. Many newborns have them, and generally they are not indicative of a problem with the heart. However, Evan’s murmur was loud – enough so that as we approached the date he would be discharged from the NICU, the neonatologist ordered an echocardiogram, “just in case.”

When I arrived at the hospital, the nurse practitioner briefly explained what the cardiologist from Boston’s Children’s Hospital had discovered – Evan had a small hole in his heart, and an extra flap of muscle inside his right ventricle. Both had formed in utero around 25-28 days gestation. She assured me it wasn’t life threatening, nor emergent. Somewhat relieved, I called Kendra and let her know the news.

On Thursday afternoon, I met Kendra at Addie’s preschool, and we drove into Boston to meet with Evan’s cardiologists. (I still can’t believe my ten-day old son has a team of cardiologists.) As we listened to them explain his heart defects in greater detail, we quickly ascertained that his condition was much more serious than we had understood. Evan has one of the most common congenital heart defects – a ventricular septal defect (VSD), coupled with one of the rarest – a double-chambered right ventricle (DCRV).

Image courtesy the American Heart Association

A VSD is a hole in the wall that separates the heart’s left and right ventricles. Many times these holes are so small that they either close up on their own, or cause no adverse effects. When they are larger, as is Evan’s, they allow blood to flow back and forth between the two lower chambers of the heart, which causes blood to flow backward into the lungs, making both the lungs and heart work harder. Symptoms include failure to grow, a blueish tint to the skin, easily tiring during physical activity, and rapid breathing.

Unlike VSDs, which are one of the most common congenital heart defects, Evan’s other defect – DCRV – makes up approximately 1.5% of all cases of congenital heart disease. Because of this defect, Evan has a random extra band of muscle around the interior lining of his right ventricle that effectively splits the chamber in two – hence the name, double-chambered right ventricle. Right now, this defect is working in his favor as it is obstructing some of the backflow of blood into his lungs. If it was not present, the size of his VSD would require more immediate surgery, but instead, it has bought him some time to see if the VSD gets smaller or closes.

If by some miracle the VSD closes, and the muscle band in his right ventricle regresses, he effectively will be cured. Obviously we are praying for miracles, but the more realistic possibilities end with open-heart surgery. The VSD could close, but the muscle band grow larger; the VSD could remain open and the muscle band grow larger; the VSD could remain open and the muscle band could regress. Only time will tell what the future holds for Evan.

Right now Evan is thriving and breathing easy. He has no outward symptoms of his heart defects. However, infants’ blood vessels take four to six weeks to fully open, so things may change in the next two months as the blood begins to flow faster through his body. We have an appointment with his cardiologist in two weeks to assess his progress.

Kendra and I are trying to stay positive. On the plus side, his particular defects can be fixed (albeit with open-heart surgery). The prognosis for a long, healthy, active and adventurous life is great. Evan has already proven to be a fighter, and we will support him as his grows older and conquers this hurdle.