In 2006, when our daughter Julia was about a year old, we had an incident in which she fell down a flight of stairs.  At the time, Renee and I were concerned that she may have dislocated her shoulder.  We took her to the emergency room, and by the time we saw the pediatrician on duty, she was running around like she was on an adventure.

Nonetheless, the doctor examined her and told us she heard a murmur, and we should mention it to her regular pediatrician the next time we saw him.  This turned out to be at her 15-month checkup.  He listened very intently and told us he did hear a murmur, and that he believed it to be a sticky valve that would resolve itself; but he referred us to a cardiologist at John Hopkins for a more thorough evaluation.

We saw Dr. Joel Brenner, who listened and did an EKG and concurred with our pediatrician, (probable bi-cuspid aortic valve with mild obstruction) but wanted to send us to Hopkins Hospital for an echocardiogram to double check.

After the echo, Renee and I sat and listened, completely stunned and overwhelmed, as Dr. Brenner explained to us that Julia had a defect called an Atrial Septal Defect.  This defect is a hole in the internal wall of the heart that divides the top chambers, the Atria.  He also explained that her particular type of ASD, called Sinus Venosus, almost always goes along with another defect called Partial Anomalous Pulmonary Venus Return.  This defect consists of the pulmonary vein from the right lung being connected to the wrong side of the heart.
 
We then were told we'd have to wait until Julia was 4 to have surgery.  Because she was asymptomatic and her condition wasn't life threatening, surgery at that stage was too much of a heroic measure, and it was better to wait until everything was larger.

In June '09, we finally had surgery.
 
When Julia's surgeon, a wonderful man named Dr. Luca Vricella at Johns Hopkins Children's Center, briefed us after surgery, he told us her heart, at 4 years old, was 30% larger than normal.    This was from the extra blood volume in the right ventricle caused by the PAPVR.  Her ASD was 15mm across, which is quite huge compared to the overall size of her heart.  She actually had a third defect as well, called a Patent Foramen Ovale.                

 AND SHE SHOWED NO SYMPTOMS WHAT-SO-EVER.

The experience has been trying.  Just a few weeks ago, we had a one-year echo check.  The doctors, her cardiologist, and her surgeon, reviewed her results and reported that there ever so slight of a leak around her patchwork and baffle.  They both said that it is minor, and it is no more significant than it was at one-month after surgery.  Her surgeon explained that the right atrium is a very low-pressure center, and he does not anticipate any additional complications or surgeries.  Nonetheless, we will go back in two years for our next check up.  I can't wait for the worrying to start over.
 
I guess I have come to the realization, like so many others, that this will never end, we will go from two-year checkup to two-year checkup, and each time we'll have to hold our breath waiting for results.

Thank you for allowing us to share,

Bob and Renee Hansen
Westminster, Maryland

An entire functional heart was created using a heart 'shell' and stem cells. This has been done in the rat & pig, with hopes of using a pig's heart 'shell' for people. Livers, kidneys, and pancreas (much harder) are in the works. They "injected the empty sac with heart cells from newborn rats. ...

For the first time, scientists have used light pulses to control a living animal's heartbeat, in a breakthrough that could lead to a greater understanding of congenital heart defects and even optical pacemakers.

Researchers led by Michael Jenkins at Case Western Reserve University in Ohio placed a laser fiber a millimeter away from a two-day-old quail embryo's heart. Using laser pulses, they were able to pace the heart's contractions, with no apparent damage to the developing tissue.

They think the light pulse creates a temperature gradient in heart tissue that opens ion channels in heart cells. This sparks an electrical impulse that makes the heart contract, according to a news release. The study is reported today in the early online edition of Nature Photonics.

The idea of controlling cells with light is not new, as Technology Review points out. Jenkins cites nearly a dozen previous studies that used laser pulses to pace heart cells in culture, as well as firing neurons with light. But this is the first time an entire heart in a living animal has been paced with light.

The method could be used to study and potentially prevent congenital heart defects, he says. Some researchers suspect that when the early embryonic heart beats slower or faster than normal, gene regulation changes, which alters development. Ensuring it beats at a normal pace could ensure healthy development of heart tissue, he says.

What's more, light pulses could be used to drive an optical pacemaker, which would be less invasive than today's implanted electrical devices. Electrode-based pacemakers and ventricular assist devices work well, but touching the heart with electronic contacts can weaken the muscle over time. Intrinsically modulating a heartbeat with light could be a better way.

The problem is that adult heart tissue is less transparent than those found in embryos, so it's harder for the light to penetrate. Jenkins and his colleagues are working on this now in adult quail hearts, Tech Review reports.

From Web Concepts, 16 August 2010.