Max was born on October 4, 2010 with L Transposition of the Greater Arteries (also known as congenitally corrected transposition of the greater arteries) and Complete Heart Block.
Max's Journey with CHD
In August of 2011, Max had his first open heart surgery for PA Banding and Bi-ventricular pacing with Dr. Pedro del Nido at BCH.
After this surgery, we had several follow up appointments, at which time the doctors noted tricuspid valve regurgitation, aortic valve regurgitation and moderate right ventricle dysfunction. In January of 2013 it became clear that Max's right ventricle was not going to serve as the systemic ventricle for much longer.
On February 20, 2013, Dr. Pedro del Nido performed the Double Switch Surgery. Other than some minor post surgery complications (pneumothorax, thrush), everything went well and we were discharged on day seven. Max was only on lasix, aspirin and medicine to treat his thrush. He was not on medication to help with his heart function as both ventricles were working with no issues.
At all of our follow up appointments (local and a BCH), his LV and RV functions looked great.
Description of Max's Congenital Heart Defects
L Transposition of the Great Arteries
In a normal heart, the left-side pumping chamber (left ventricle) sends blood to the entire body, whereas the right-side ventricle pumps blood only a short distance, to the lungs. The left ventricle is built stronger than the right ventricle because it has to work harder. In congenitally corrected transposition of the great arteries (CCTGA), the heart twists abnormally during fetal development, and the ventricles are reversed. The stronger left ventricle pumps blood to the lungs and the weaker right ventricle has the harder chore of pumping blood to the entire body. CCTGA can cause problems, particularly for the right ventricle, which must work harder than it was meant to. For many patients, in the long run, the ability of the right ventricle to continue to function effectively will determine how well they are. There are several problems that co-exist in many patients with CCTGA and affect how well they do and what kind of treatment or surgical repair may be suitable. The most common associated lesions are:
A blockage on the way of the blood to the lungs (pulmonic stenosis)
CCTGA is a rare heart defect. Only .5 to 1 percent of all people with heart defects have CCTGA. This means there are about 5,000 to 10,000 people in the United States with this condition.
In CCTGA, both ventricles (pumping chambers) are reversed and the arteries coming off them are inverted as well. The end result is, paradoxically, a blood flow pathway which is normal. This led to the confusing term "corrected" for a complex malformation in which the heart is far from being normal.
Complete Heart Block
Heart block is an abnormal heart rhythm where the heart beats too slowly (bradycardia). In this condition, the electrical signals that tell the heart to contract are partially or totally blocked between the upper chambers (atria) and the lower chambers (ventricles). For this reason, it is also called atrioventricular block (AV block).
Complete heart block (also called Third-degree heart block) - With this condition, none of the electrical impulses can reach the ventricles. When the ventricles (lower chambers) do not receive electrical impulses from the atria (upper chambers), they may generate some impulses on their own, called functional or ventricular escape beats. Ventricular escape beats, natural backup signals, usually are very slow, however, and cannot generate the signals necessary to maintain full functioning of the heart muscle.
Max was implanted with a Bi-ventricular pacemaker in August 2011 to treat his complete heart block.
On the morning of August 3, 2013, things were not looking good. We were in discussions with the staff at Boston Children's Hospital about the possibility of having to go in the direction of a "Berlin Heart" to keep Max alive until transplant. His energy levels and appetite were severely diminished and his heart function was declining rapidly. The next day, August 4, 2013, Max received his new heart. We are extremely grateful to the family that gave Max a second chance at life. We cannot imagine the pain and anguish they have had to endure, and we pray that someday, in meeting Max, it will help them (if even just a little) with the grieving process.
On May 1, 2013, Dr. Bergersen performed a cardiac cath. They noted the structure of his heart looked good (no obstructions, stenosis, etc.), which confirmed that his poor LV function was the result of weak muscle tissue due to the increased work load after the Double Switch Operation. They continued with Milrinone at BCH and gradually added an oral heart failure med (Captopril), reducing the Milrinone until we were released on Wednesday, May 8th.
On Mother's Day, 5/12/13, we were coming back from Nico's soccer practice when Max started to scream, thrash and grab at his chest. We pulled the car over and called 911. As we were waiting for the ambulance, he began to vomit. He was re-admitted to the PICU at Baystate and put back on Milrinone (IV heart failure meds). We stayed there for a few days while they weaned him off the IV meds and started the oral meds again. We were released on Wednesday, 5/15/13, however, Max started to vomit again on 5/17/13.
We were admitted to Boston Children's Hospital again on 5/17/13 and placed back on an IV drip of Milrinone. Over the next two weeks, we met with doctors, nutritionists, social workers, infectious disease specialists, etc. Additionally, the heart failure team conducted a transplant evaluation to determine if Max was a good candidate. On May 30, 2013, the results of several blood tests showed that Max's LV function had gotten no better. His heart was severely dilated, which makes it more difficult for the LV to recover. Max was put on the heart transplant list on Friday, May 31 , 2013 as a level two (2) candidate. Shortly thereafter, he was upgraded to a level 1A candidate which is the highest level of priority.
Max's first eight (8) months (post-transplant) have gone very well. We have had some small bumps in the road (cell and antibody rejection), however, his most recent biopsy came back negative for all rejection. He is a happy, energetic three year old child, ready to start pre-school this coming Fall.