Children's Hearts Fund

The right atrium pumps the de-oxygenated blood into the right ventricle, which then pumps the blood through the branched pulmonary arteries into the lungs, where the blood exchanges Carbon Dioxide for Oxygen.  Once oxygenated, the blood passively flows through the pulmonary veins into the left atrium.  The left atrium, as in the case of the  right atrium simply collects blood.  It then pumps the oxygenated blood through a bicuspid valve into the left ventricle.  The left ventricle is the main pumping chamber of the heart, in that it is required to pump blood throughout the entire body.  The left ventricle then pumps the oxygenated blood through the body, initially, through the ascending aorta which then branches and twists and turns all over the body.  The oxygenated blood provides oxygen to all organs of the body and then returns to the right atrium (via the superior and inferior vena cava) to repeat the cycle.

As I indicated, Colin was born with only one full ventricle (his other ventricle was so small that it was not viewed as a pumping chamber)  The full ventricle was on the right side.  This would have presented a real problem in that the right ventricle was not built to pump blood throughout the body, but only to the lungs.  (Far less pumping force is required to pump blood to the lungs than throughout the entire body).  The left ventricle is therefore a much stronger pump.  Your right ventricle could not easily handle the task of pumping blood thru the body. Miracle number one solved that problem  Colin was born with another defect called transposition of the great vessels, which means that his ventricles were switched.  His remaining ventricle, therefore was a left ventricle on the right side of his heart; the ventricle that was built to pump blood through his body. (When he was first diagnosed with Single Ventricle Syndrome, the transposition was not detected).  We were concerned about the ability of what we thought was a right ventricle being the sole pumping chamber.

Another defect that was corrected, in part, by nature was his single inlet.  I mentioned previously that in a normal heart, blood passed from the lungs to the left atrium, then into the left ventricle to be pumped throughout the body.  The blood passes from the left atrium to the left ventricle through a bicuspid valve.  Without that valve, blood cannot pass from the left atrium into the left ventricle.  Unless, of course, it can somehow pass all the way around from the left atrium, into the right atrium, into the right ventricle and finally into the left atrium.  The problem was the wall between the right and left side of the heart called theseptum- Between the right and the left atrium is an atrial septum, and between the right and left ventricle is a wall called the ventricular septum.   Normally, these walls keep the oxygenated and de-oxygenated blood from mixing. 

In order, therefore to pass blood from the left atrium to the right atrium, then into the right ventricle and finally into the left ventricle, you would need a hole in the two septum.  God gave Colin just that .  He had an atrial septal defect and a ventricular septal defect.

With a heart like Colin's, it was necessary to turn a three chambered heart into a two chambered heart.  The reason is as follows: as I mentioned, the right side of the heart basically does one thing, it pumps de-oxygenated blood to the lungs.  At the same time, the left side of the heart basically does one thing, it pumps oxygenated blood through the body.  If you could somehow re-direct the de-oxygenated blood directly to the lungs from the veins (Superior Vena Cava and Inferior Vena Cava) you could eliminate the need for the right side of the heart.  You could then live with a two chambered heart.  

That is exactly what the pediatric cardiologist had done. In particular, three days from birth on July 10, 1996  Colin had his first heart surgery.  A procedure simply intended to increase the amount of blood flow to the lungs.  This enabled us to wait several months to begin the reconstruction.

In December of 1996, Colin was taken in for his first phase of the reconstruction, the Bi-directional Glenn Shunt.  This procedure essentially involved cutting the superior vena cava ( the vein that directs all de-oxygenated blood from the head and neck into the right atrium) and sewing it directly into the branched pulmonary arteries (the arteries through which the right ventricle normally pumps the de-oxygenated blood into the lungs.  This would essentially divert one-third of the bodies de-oxygenated blood directly into the lungs (in the mid to late 1980's both the superior and inferior vena cava's were diverted in one surgery to the pulmonary arteries, but the shock to the heart with such a sudden total change of the de-oxygenated blood flow was too shocking to the system.  The Fontan procedure was then split into two separate surgeries, the first to divert the top one-third of the de-oxygenated flow (The Glenn Shunt) and the second to divert the lower two-thirds of the de-oxygenated flow ( The completion Fontan))

During the December surgery, the doctor came out and told us that the atrial septal defect was closing and it had to be re-opened before they could do the Bi-directional Glenn Shunt.  In other words, the blood, which was flowing the long way around the from the left atrium, through the atrial septal defect into the right atrium and ultimately into the ventricles, was now being blocked by the closing atrial septal defect.  It had to be re-opened.  The doctor did an atrial septectomy and delayed the Bi-directional Glenn Shunt procedure.

In April of 1997, the time was right for the Glenn Shunt.  That procedure was performed in an uneventful manner. Colin was a home in his  walker within 72 hours after the surgery.  One third of his blood was diverted and his oxygen saturation slowly began to rise.

The Completion Fontan ( the procedure to divert the remaining de-oxygenated blood flow to the lungs ) was performed during  November 1997.  This was not uneventful.  He was kept in the hospital for several weeks with pleural effusion.  In other words, fluid was collecting in the pleural space of his lungs (a space between an inner and outer layer of the lungs.  Normally filled only with a slight lubricant) Moreover, a nerve associated with his right diaphragm had been injured during the surgery and was paralyzed.  The diaphragm is a muscle that is positioned beneath each lung and supports it as it expands and contracts (rises and falls ) Since it was paralyzed, Colins right lung was being compromised.  (The diaphragm was pushing up against his right lung and restricting the surface area for oxygen exchange). After a few weeks, a plication of the right diaphragm was performed to essentially reduce the force at which the right diaphragm was pushing on the right lung.

During the fall of 1998 Colin began to collect fluid in his abdomen, legs, face and neck and just about anywhere else fluid could collect.  Hew as soon diagnosed with Protein Losing Enteropathy. With this disease, Colin was losing protein in this stool.  As a result the protein levels in his blood were extremely low.  This disease is a killer. Specifically, over 50% of PLE kids die in the first five years after being diagnosed.  Furthermore, there is no known cause or cure.

Heparin is used as a lifetime commitment with some success in various facilities, other treatments were also used with some degree of success.  None, however, has proven to be a cure.