Mt two latex-gloved fingers extended as far into Greta's vagina as they could through the tight rubbery of of her cervix, grazing the firm, smooth, slippery surface of her baby’s lowest part, but I could not feel the narrow gaps between the bones in the baby’s head. The “sutures” of the infant skull are what we use to confirm that the hard structure meeting the examiner’s fingertips is the baby’s head. I couldn’t be sure.
“We’ll need to do an ultrasound to make sure the baby’s head is down,” I told Greta as I extracted my fingers and snapped off the glove. She nodded, knowing from my previous explanations the importance of making sure that her baby was presenting “vertex” (head down) and not “breech” (butt or legs first). About 3 percent of babies are breech at the end of pregnancy; these babies have unacceptably high complication rates during vaginal delivery and therefore must enter the world via cesarean section. Earlier in the year, three consecutive first-time-pregnant patients of mine had breech babies. Many times we can turn these around via a procedure we call “version,” but the first we could not turn and the other two went into labor early, so all three had C-sections.
Two evenings later, on Wednesday, my afternoon off, my pager interrupted me during dinner with its rude vibration against my belt. I can’t seem to help getting annoyed when I’m paged on my days off. Often, these pages come to me by mistake. The answering service that connects patients to their physicians on call has trouble finding employees capable of reading a schedule and paging the right doctor.
This one wasn’t a mistake; it was Greta. “The doctor said the ultrasound showed a serious problem with the baby’s heart.” Her Swiss-German accent didn’t hide her concern.
Few things upset me more than patients calling me with questions about abnormal test results before I know about them. It should never happen. In this instance, the radiologist interpreting the ultrasound should have called me the moment he finished. The facility where the test was done was now closed. So here was Greta, full of anxiety and questions, calling her doctor, who could not help her. I apologized and promised I would figure things out first thing in the morning and call her back.
“She [the radiologist] said there was something in the heart,” Greta explained.
“Ultrasounds are not always that great for seeing things as they really are,” I offered in an attempt at reassurance. “I need to speak to the radiologist before I can give you advice that has any meaning. I’m really sorry I don’t know more right now.”
Worried and seething, I called my office, knowing that someone would still be there doing evening office hours. “I passed that message on to Sherri,” Bart, one of our better medical assistants, assured me, “and she told me to go ahead and arrange for Greta to see a pediatric cardiologist in two days.” Sherri is a great nurse practitioner, but sometimes she goes overboard to protect me. I’m the only provider in our practice who does obstetrics, so I have to know every time something important happens to a pregnant patient, and I have to decide who goes where and when.
I called the radiologist first thing the next day to get the news: the baby had a three-centimeter mass in the left ventricle. I don’t know what the average diameter of a newborn’s left ventricle is, but it’s not much more than three centimeters, so this mass was occupying most of the ventricle, which makes it difficult for the ventricle to serve its purpose: pumping oxygen-containing blood through the aorta into the entire body. This was, therefore, a disaster. It was also a surprise. Her ultrasound 20 weeks earlier had been normal. My wife Terry believes that all things happen for a purpose, and she may have been right to claim that the reason I had the had luck with three consecutive breech babies was to make sure I would order the ultrasound for this one so we could discover the heart problem before the baby’s birth. Masses in the heart are not common. They usually are rhabdomyomas, or lumps of smooth muscle, which grow because the person has an inherited disease. Greta’s baby appeared to have a single large mass in the heart, but none anywhere else, which gave the baby about a 50 percent chance of having tuberous sclerosis. TS causes these masses to form in many different organs, including the skin and the brain. Most people with TS have central nervous system involvement, which often causes seizures and leads to mental retardation in about a quarter of its victims. TS is passed on in families, but most people who have it acquire it through a spontaneous mutation and do not have any family history of the disorder. Of the other conditions associated with rhabdomyomas, most carry a similar or worse prognosis than TS.
So when I called Greta and Ken, her African-American software-engineer husband, whom I had met once during one of her prenatal visits, I told them to come right in to the office because I knew this would not be appropriate to handle over the phone. I didn’t have any appointments free that morning, but when something like this happens, other people have to wait. I had to tell them their son had a huge cardiac mass that would pose a formidable threat to his life within hours after birth. At birth, babies have a connection between the pulmonary artery and the aorta called the ductus arteriosus. The ductus allows blood coming from the right ventricle into the pulmonary artery to go straight into the aorta, avoiding the lungs and left side of the heart. Since this blood does not go to the lungs and exchange carbon dioxide for oxygen, the body cannot allow this to continue. The ductus almost always closes in the first 48 hours after birth, at which time the left ventricle must pump all the blood into the aorta. It seemed obvious that Greta’s baby’s left ventricle would not be able to do this unless a cardiac surgeon either redirected the circulation or removed some of the mass soon after birth.