Baby Bruce

Bruce Cheney, Jr.
Born: July 26, 1947
Died: October 14, 1947
Relation to author: Uncle

Bruce Cheney, Jr., was the first born child of Bruce Judson Cheney and Elizabeth Charlotte Paulus (married name Cheney). Very sadly, Bruce Cheney, Jr. died less than three months after he was born. The diagnosis was congenital heart disease, but more commonly he was what they called a "blue baby".

The term comes from the blue color of the baby's lips (also called cyanosis of the lips), a sign that the baby's body is not getting enough oxygen through the blood. 

We actually have a copy of the autopsy report, so with a little help from a friend (the explanation and diagram are confusing) we can learn about what was happening anatomically. I shared the autopsy (you can see it here and here) with my friend's father who is a pediatric cardiologist (special thank you to him!). He said that Baby Bruce had Total Anomalous Pulmonary Venous Return (TAPVR). 

For Baby Bruce, the pulmonary veins that should move the oxygenated blood from the lungs to the left atrium (which then would send the blood to the left ventricle and then to the aorta and out to the body) went instead to another vein up toward the upper chest and back to the right atrium, which takes deoxygenated blood from the body and sends it to the right ventricle and then to the lungs, via the pulmonary artery, to add oxygen. In other words, oxygenated blood from his lungs was not getting distributed to his body and was instead mixing with deoxygenated blood right at the heart in a continuous circle. You can see in the image below a normal heart on the right and TAPVR on the left. 

The autopsy also describes that the right atrium and ventricle were hypertrophied (enlarged) because they were receiving both deoxygenated blood from the body and oxygenated blood from the lungs. Meanwhile the left atrium and ventricles were thin-walled, almost paper thin because they received almost no blood. This is a common symptom of the venous circle. 

This condition happens, as best as I can understand and simplify it, because as the fetus develops, the pulmonary venous plexus - the precursor to the pulmonary veins - fails to link to the left atrium and instead retains connections to the primitive (early development) drainage systems and thereby retains a connection the right atrium.

Congenital heart problems for infants are no longer as fatal as they once were. Both early diagnosis (thanks to sonograms) and surgery are possible. In fact, my wife had a special sonogram to check our first baby's heart; during our 20 week anatomy scan, the doctors couldn't get a good view of the heart so they sent her for a special scan. The scan turned out fine, but I couldn't help think about how things would have been different if Bruce and Elizabeth had the technology we have access to today.

Surgery is the recommended solution for babies with this condition. You can see the full details I have here or a user-friendly guide here. They make an incision in the middle of the chest and separate the breastbone to reach the heart. Then they use "cardiopulmonary bypass with circulatory arrest" where the child is attached a heart-lung machine which acts as the child's heart and lungs and then they create a normal pulmonary venous pathway by connecting the pulmonary veins and the left atrium. The vertical vein, which is now unnecessary is usually ligated (tied-off). Basically, they create the connection between pulmonary veins and the left atrium that should exist. 

According to what I've read, the advent of surgery over the past 50 years or so means that most patients with this condition will survive. Early mortality - death within 30 days of surgical correction - is less than 10 percent, and late mortality - death 30 days after surgery - is about 5 percent. Long-term survival to adolescence is about 85 percent.

However, there are some lasting effects. Although the research is somewhat limited, one case series reported a poorer perception of health and school performance in survivors of TAPVC correction. And several case series noted an increased risk of arrhythmias, especially sinus node dysfunction, which, "may be due to disruption of the conduction system by the atrial incision used to repair TAPVC."

It begs the question though when this became treatable. Baby Bruce was born and died in 1947. Pediatric cardiology as a specialty was developing in the early 1950s. The first pediatric cardiac surgery was done in 1944 at Johns Hopkins, but surgeries that followed in the 1950s and 1960s were often unsuccessful. The development of "cardiopulmonary bypass in the mid 1950s and deep hypothermia with circulatory arrest in the early 1970s" allowed for pediatric cardiac surgery to advance. By the 1990s mortality had declined dramatically.  In other words, it would be a few decades before babies like Bruce, Jr., could be saved.