I was born with a rare heart defect called Pulmonary Atresia with Intact Ventricular Septum. Say that fives times fast! 

Growing up I knew very little about my condition because my parents were great at handling everything and allowing me to be a kid! So, what exactly IS PA/IVS? I believe it is best represented by an illustration. Please see below...


















As you can see above, in the normal circulation, blood passes through the pulmonary artery to the lungs, to collect oxygen. In babies who have pulmonary atresia with intact ventricular septum, blood cannot pass to the lungs in this way. Instead, the blood passes from the aorta to the pulmonary artery through a connection called the ductus arteriosus – often called ‘the duct’. (See the illustration.) The duct is open while the baby is developing in the womb, but it usually closes shortly after birth. In a baby with pulmonary atresia with intact ventricular septum, it is vital that the duct stays open after birth if the baby is to survive. The very first stage of treatment is to keep the duct open. Without treatment, the duct would close and I would have died.

In my case, I had a Modified Fontan Procedure. In terms I can understand... they did some re-plumbing so that most of my blood bypassed my Pulmonary valve and went directly to the lungs so that the Valve did not have to work as hard. Once this procedure was complete, I was doing much better! 

I was then diagnosed with an arrythmia. Because of this, I needed a pacemaker. I have since had 4 pacemaker changes and I currently have a medtronic extended life pacemaker. I have had my current pacemaker since 2008 and it should last me until 2022 when I will get a new one.