Barry Love, MD, a Pediatric Cardiologist and director of the Congenital Catheterization Lab and the Electrophysiology Service at the Mount Sinai Hospital talks about a condition called familial heart block and what options those families have.
Interview conducted by Ivanhoe Broadcast News in June 2018.
I wanted to ask you a little bit about congenital heart defects. Is there a change in thought in the way we treat pediatric patients now compared to over ten years ago, is there earlier intervention?
Dr. Love: There’s lots of ways that we treat congenital heart disease and certainly this has been an evolution. There was really a revolution of treatment starting in the forties, fifties and sixties. Since then, evolution in terms of the way that we treat children with heart disease. Certainly many of the things that we needed to offer before that could only be done surgically can now be done in the catheterization lab and so we’re able to offer a less invasive way of treating many forms of congenital heart disease that can now avoid major open heart surgery.
Could you tell me a little bit about the condition that Sam and his family have?
Dr. Love: Sam and his family have a condition called familial heart block. This first presented in his grandmother. When she was a young woman about age eighteen she fainted unexpectedly and was found to have a very slow heart rhythm where the upper chamber wasn’t talking to the lower chamber called complete heart block and she needed a pace maker at that time. This is many years ago and at that time the only way to put a pacemaker in was with surgery where the wires were put on the surface of the heart and the what’s called the pulse generator, the electronics that provide the stimulus to the heart to tell it to beat were connected in a box that was placed underneath the skin in the abdomen. She had a series of pacemakers that were placed that way but as a young woman the batteries didn’t last as long at that time, the wires were not as good and she needed a series of surgeries in order to replace those pacemakers over time. As technology advanced and as she got a bit older she had pacemakers put in from the vein where they thread a wire down to the heart. But again those pacemakers and leads were somewhat more primitive and they didn’t last for a while, she had repeated pacemakers placed and at a certain point as she got older all the veins were blocked up and she was really having difficulty from the fact that the veins were blocked and that she had a pacemaker for so long. Ultimately she ended up passing away just last year from complications from her pacemakers. Thankfully she was an older woman at that point but did have difficulties along the way with her pacemakers.
Now you fast forward to her grandsons.
Dr. Love: Fast forward to her grandchildren: Samuel is one of four children and three of the four have evidence that they will develop heart block at some point. They have an incomplete form that’s called right bundle branch block and we expect at some point they will develop complete heart block. And we’re concerned because Sam’s grandmother had two sisters that had more serious difficulties than just fainting and one of them had passed away at a young age presumably from complete heart block and so we’re concerned that if Samuel or his siblings who have the indicators that they will likely develop this that at some point they may suffer the same fate of fainting or more serious complications. We would like to provide them with a backup of pacing before God forbid that was to happen.
How important is it for people with these complicated heart conditions to get answers?
Dr. Love: It’s obviously very important to get specialized care and to get the right care and get the right answers. Sometimes the right answers are unknowable so we do our best with the information that we have. This family was actually seen by our cardiac genetic service and had extensive genetic testing. We were not able to identify the genetic link in this family and so are left with trying to figure it out based on other testing that we can offer them.
You were talking about how the grandmother had this big device, how much different is it now two generations later for people with heart conditions?
Dr. Love: Right. It’s much different and up until a year ago the pacemakers that we could offer still involve placing wires from the vein in to the heart and attaching it to an electronic box that’s placed underneath the skin. In fact Sam’s brother had that placed ten years ago.
Tell me about Sam’s brother.
Dr. Love: Sam’s brother had this pacemaker placed about ten years ago and came last week for a change of the pacemaker generator but he still has a wire in the heart and will be left with that wire likely for his lifetime and hopefully it will continue to work. But he’s left with the possibility that that wire may have a problem, it may need to be taken out which has its own risks associated with it or maybe to have that wire replaced.
What is the prognosis for Sam and his siblings?
Dr. Love: The prognosis should be very good given what we know now. The ability to provide them with backup pacing should and when it happens that they develop heart block potentially may be lifesaving. We’re trying to minimize things right now but have placed these as basically guardian angels for them in the event that they develop heart block as we expect that they will at some point in their lifetime.
No restrictions? This is a kid that loves baseball and likes to get out there.
Dr. Love: What’s different about Sam’s pacemaker as opposed to his brothers is that we placed this new pacemaker called the medtronic micra and it’s one that can be installed directly from the vein. It has no wires that go through the vein and no incision underneath the skin. And this pacemaker is entirely contained in the heart itself and because of that he’ll be able to participate in sports and activities with really minimal to no restrictions.
Is there one thing that you would want people to know that I didn’t ask you, is there a take away point for our viewers?
Dr. Love: One of the things I would say is that it’s very important to have consultation with experts when it comes to treating children with heart disease because they’re not just little adults and so pediatric specialists seeing these patients with these conditions are very important.
END OF INTERVIEW
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