Welcome Billy! You made a pretty dramatic entrance into this world on April 21, Master William John Kimmel.
Billy is the newborn son of Molly McNearney and her husband, late night host Jimmy Kimmel.
If you were in a cave or a coma last week you might not know that he was born with a congenital (present at birth) heart defect that required an immediate life-saving operation, did well, and his story was shared by his dad on late-night TV.
Part of the monologue that touched on the controversial American Health Care Act that squeaked through the House last week caused quite a stir.
So what was wrong with little Billy? His heart formed abnormally as he grew as a fetus. His heart’s four issues comprise a problem called Tetralogy of Fallot, first described in 1671 but named after the French doctor Etienne-Louis Fallot who wrote of it in 1888.
This constellation of heart defects occurs in about 1 of every 2,000 live births and is seen in girls and boys equally.
The main abnormality is a narrowing or lack of development of the channel that leads from the pumping chamber on the right side of the heart to the lungs, that is, pulmonary stenosis or atresia. Along with this is a large defect in the wall that separates the right and left pumping chambers, a ventricular septal defect. Thirdly, the aorta, the large blood vessel that normally carries blood from only the left pumping chamber to the body, is positioned abnormally and sits over the hole in the septum and therefore carries mixed blood from both the pumping chambers at once. Finally, the normally thin-walled right pumping chamber thickens and gets “muscle-bound” because it’s facing a much higher pressure to work against than normal.
All these problems conspire to prevent enough blood from getting to the lungs to pick up oxygen, and the baby, especially when stressed, is quite literally blue, or in medical terms cyanotic.
Tetralogy is the most common of the complex cardiac defects and represents about 7 to 10 percent of inborn heart problems. It was one of the first ever to be successfully approached surgically. The story of how is all about Johns Hopkins Hospital, its famous surgeon, Alfred Blalock; a young female pediatrician, Helen Taussig; and a remarkable black lab technician, Vivien Thomas, working in Baltimore in the 1940s. I was blessed to know Taussig and Thomas, and honored to have trained and worked in the same department and operating rooms as Blalock.
Get the movie “Something the Lord Made.” Several years ago, this wonderful film told their story pretty accurately and culminated in the operation on November 29, 1944 in which Blalock, with Thomas advising him over his shoulder, created a “shunt,” envisioned by Taussig and turned “Blue Baby” Eileen Saxon, pink!
These operations showed that precise, delicate operations on tiny blood vessels were possible and opened the door to heart surgery. They bought time for many kids until the 1950s when operations that could completely correct the constellation of defects could be developed using the newly invented heart-lung machines.
Edward Park, the Chairman of Pediatrics at Hopkins, gave his young trainee, Taussig, a new-fangled “fluoroscope” and a stethoscope and told her to learn about children’s hearts and malformations. This amazing woman dedicated her life to the task, dissected all sorts of animal hearts, especially birds, examined countless children with minimal instrumentation and established pediatric cardiology as a specialty.
She approached Blalock with an idea of how to help “blue babies” and was rebuffed, but Vivien Thomas was intrigued and cajoled the professor to try.
Helen wrote a two-volume text on congenital heart defects and inscribed the last copy Harvard University Press had in stock to a young cardiac surgeon at Hopkins one week before she died in a car crash while headed to vote.
“Wishing Alfred Casale great satisfaction in medicine and thoracic surgery … Helen B. Taussig MD.”
Thanks Helen, from me and Billy.
Dr. Alfred Casale, a cardiothoracic surgeon, is Associate Chief Medical Officer for Geisinger Health and Chair of the Geisinger Cardiac Institute. Readers may write to him via [email protected]