Scimitar Syndrome

A few months ago, my sister, Karen, asked me to create a website that would allow her to write about congenital heart defects. Her son (my nephew), Nathan, was born with a rare heart condition called Scimitar Syndrome. One characteristic of this syndrome is dextrocardia, which means the heart is severely rotated backwards in the chest.

Other than being a little strange, this alone is not a problem; but it complicates the surgery to correct another problem, Partial Anomalous Pulmonary Venous Return. In PAPVR, an anomalous vein, shaped like a scimitar (a curved Turkish sword) carries oxygenated blood to the wrong chamber of the heart. Correcting this involves creating a baffle or tunnel to the correct chamber of the heart to redirect the blood flow. Finding a surgeon to perform this surgery on a backwards heart was challenging, but after a lot of research, Karen and her husband, Bryan, found just the right guy.

It was about three years ago that Karen and her family traveled to Michigan where a skilled pediatric cardiologist, Dr. Edward Bove, successfully repaired Nathan’s heart. The journey was rough, but Nathan is doing great these days. The website, NathansPrayer.com, is filled with helpful information about congenital heart defects and some stories about my wise-guy nephew. Check it out if you get the chance.