Tiernan's Heart Drawings
Here is a normal heart. Now look at how Tiernan's heart grew.
Tiernan was born with:
Coarctation (Narrowing) of the Aorta
Complete Right Dominant Atrioventricular Canal (hole through all four chambers)
Mildly Hypoplastic LV/Aorta/Left side Valve
Left Valve Regurgitation
In his first surgery at Seattle Childrens Hospital, Dr. Cohen and Dr. Permut placed a band on the Pulmonary Artery (PA) to limit blood flow to the lungs and reconstructed the tiny aortic arch.
They also sutured off the PDA.
Tiernan's 3rd surgery was the Damus-Kaye-Stansel (DKS) and the Bidirectional Glenn (sometimes called the Hemi-Fontan). His Coarctation had returned so his heart had to pump against that and the PA band: a double obstruction. Because this happened, he developed severe Ventricular Hypertrophy, or the thickening of the ventricular walls, due to the hard work of pumping against the two obstructions.
In the DKS Dr. Woods and Dr. Wilke combined the PA with the ascending aorta so that all the blood from the heart gets pumped out to the body. Blood goes to the lungs passively as it returns from the head and down into the PA. As you can see in the drawings the Superior Vena Cava (SVC) and the Inferior Vena Cava (IVC) (the tube that runs along the left side of the picture) is separated and the SVC now drains directly into the PA. This causes Tiernan to live in a state of low blood oxygen saturation or Cyanosis. The oxygen in his blood measures around 85% on average. Heart healthy (non-smokers) have normal oxygen saturations of high 90's to 100%.
Eventually Tiernan will have the Fontan completion where they will connect the IVC to the PA and take the rest of the workload of pumping to the lungs off the heart. At this point his cyanosis will go away. This is likely to happen sometime in the next 4-5 years
