Introduction

As some of you know, in mid March Chris and I learned that our son Kai (now 3.5 years old), has a congenital heart defect known as "aortic coarctation." 1 in 10,000 children are born with this particular condition and it accounts for about 5% of the congenital heart defects, more common among boys than girls. Coarctation refers to the narrowing of the major artery that carries blood to the body. In Kai's case, the narrowing affects the blood flow to the lower part of his body so he sometimes complains of tired legs and his feet get cold ("cold feet, warm heart"). Most significantly, he has low blood pressure in his lower body and, subsequently, higher blood pressure in his upper body. To ensure that the lower body and organs get enough blood, the body compensates through elevated blood pressure in the upper body. Over time, this high blood pressure causes damage to the heart and, if uncorrected, puts Kai at risk for heart failure, strokes, aneurisms and the sort. Life expectancy for untreated coarctation is about 30 years.

In some cases, the coarctation is so severe and noticeable, that children are operated at birth or shortly thereafter. Others don't find out until their teens, during a routine physical. That our general pediatrician discovered it now is quite rare. We had gone in for a checkup to make sure Kai had recovered fully from his strep throat. Everything was fine until the pediatrician heard a heart murmur. 75% of children will have a heart murmur at some point, usually after illness with fever, and then it will pass. When we went to the hospital to get a chest x-ray, then an ekg and an hour-long echocardiogram, they discovered the coarctation, along with a bicuspid valve (two flaps instead of three). This news came well after midnight, on St Patrick's Day, in an Emergency Room so full that children were being treated in the hallway. Fortunately, Kai's heart hasn't suffered any damage yet and his heart muscle is strong. If the surgical removal of the tissue causing the narrowing is successful, he should be able to live a normal life without special precautions regarding physical activity. He might have to avoid weight-lifting, rowing, shoveling snow (too bad, Chris!) and roller-coasters, but aerobic activity such as soccer, baseball, tennis is fine. There is a 3-5% chance that the narrowing can reoccur over the course of Kai's life, but then it can be successfully and less invasively treated through angioplasty--and that is a growing field with innovative techniques. The long-term prognoses are a bit speculative, though, since the data only goes back to the 1990s. He will probably have yearly followups for the rest of his life, including a doppler/MRI test every 2-5 years.

Within the shock of this news, we've found hopeful aspects, most importantly, early discovery and the fact that the University of Michigan Congenital Heart Center is #4 in the nation. Our surgeon, Dr. Ohye, comes highly recommended, with over 15 years experience as a heart surgeon who has specialized on children since 1998. The moment we walked into our consultation meeting with him, we liked him. He exuded competence, efficiency and clarity, and is also a very personable, caring individual. Over the last month, he has patiently fielded my many questions via email. At a critical point, he said that I could continue to ask as many questions as I want, but that ultimately we have to take a "leap of faith" and trust the experts. Mentally getting to the point of acceptance and trust was not easy, but it is the only option. "You can't go over it, or under it, you've got to go through it"—as one of the books on Kai's bookshelf reminds us.

The surgery is now scheduled for May 24, 2011, barring any respiratory illnesses or other emergency operations (children get flown in from all over the country) that might intervene and would mean pushing back the date. Thus far, Kai doesn't know anything, although I'm sure he senses our anxiety. He's gotten so interested in medical paraphernalia and loves doctors, for now. The recommendation is to carry on with life as usual and start preparing Kai a few days in advance, including having him tour the hospital premises.