My daughter, Brittani, is 19 years old and has just been diagnosed with Scimitar Syndrome.

She was diagnosed as a baby as having Dextrocardia and has had no health problems whatsoever. She became pregnant which ended in a miscarriage. During the D & C preop testing and chest xrays, they noticed the Scimitar. They said she needs surgery soon or she will die of heart failure by the age of 25. They also told her that the miscarriage was a blessing because her heart would not have been able to handle the stress.

She is going to see a specialist in St Louis, MO on Wednesday, 11/9/11 to get a referral to a surgeon. This has hit us really hard since we had no clue that anything was even wrong. She hasn’t had any symptoms at all.

Please keep her in your thoughts and prayers.

I have heard other examples of how an unrelated health event led to the discovery of serious health issues that may have gone undetected otherwise.  So sorry for Brittani’s loss, but grateful that her Scimitar Syndrome has been found and can be corrected. Prayers to both you and Brittani.

Nathan watches a Disney video during EKG testing at Children's Hospital in New Orleans.

Annual checkups are an important part of maintaining Nathan’s heart and back health.  Recently, Bryan, daughter Michelle, and I traveled with Nathan to Children’s Hospital in New Orleans to see how his heart looks four years after surgery to correct Partial Anomalous Pulmonary Venous Return (PAPVR), a problem associated with his scimitar syndrome.

The echocardiogram seems to be a challenge for the technician each year.  His heart is positioned strangely in his chest (dextrocardia), so it takes a little patience to capture the desired images.  It doesn’t help that Nathan is extremely ticklish, especially when the transducer is placed at his neck.

During Nathan’s heart surgery, a baffle (tunnel) was created to redirect oxygenated blood from the right atrium to the correct chamber, the left atrium.  The last time the technician was able to capture an image showing the baffle was a few days after his surgery in 2006.  So it was a pleasant surprise when the cardiologist spotted it and reported that it appears to be unobstructed and working fine!

Nathan poses by a whimsical seahorse painting while he waits for one of his doctors.

In addition to his congenital heart defect, Nathan was born with a hemi-vertebra which was diagnosed as congenital kyphosis three months after his heart surgery.  We visited Nathan’s pediatric orthopedic surgeon on the same day.   I wish I had snapped a photo of the radiographs Dr. King presented to us.  One showed his spinal column right after spinal fusion surgery; a piece of cadaver bone replaced the hemi-vertebra and was easily discerned from his natural vertebrae.  The latest x-ray showed the cadaver-bone vertebra and the two vertebrae directly above and below had transformed into one solid piece, just as it was supposed to do.  There was concern, however, that the fusion was curving about 40 degrees.  If it continues to curve, reaching 50 degrees or more, it may be necessary to place rods in his back.

I hope, I hope, I hope… it will hold.

We pushed that thought out of our minds and went to Copeland’s after our day at Children’s.  We had their fabulous Spinach and Artichoke dip with fried bow-tie noodles for an appetizer (Nathan only eats the noodles).  Bryan had barbeque with cornbread.  Michelle and I had a delicious pecan encrusted fish.  Nathan had his usual–chicken tenders with fries.  Dessert:  Cheesecake Napoleon with praline sauce for us big kids, Big Al’s Chocolate Fudge Cake for Nathan.

Sometimes, you have to look at life’s challenges with the mindset of that famous philosopher, Scarlett O’Hara:  “I’ll think about that tomorrow.”

Nathan Blakeney, EKG Pro, at Children's Hospital in New Orleans.

Last week, Bryan and I took Nathan to Children’s Hospital in New Orleans for his annual checkup.  His heart surgery to correct Partial Anomalous Pulmonary Venous Return, a problem connected to his Scimitar Syndrome, occurred about three years ago.  The first year after surgery, appointments were a few months apart, but he has done well, so he is seen once a year now.

I asked the nurse who hooked him up for the EKG (electrocardiogram) if his scimitar syndrome required a different placement of the leads.  She told me the leads are, indeed, placed differently on patients with dextrocardia.  To get a proper reading on a heart that is positioned on the wrong side, it appears that the leads are reversed.

Typical placement of EKG leads

I snapped a picture of Nathan while the nurse performed his EKG.  He has had several of these over the years, and although he was not crazy about being wired up as a baby, he now takes it in stride.  He  giggled  when Bryan told him to be glad he doesn’t have a hairy chest.

This interests me because Nathan has dextrocardia, too.  Hopefully, he will never need a heart transplant, but it certainly would pose a challenge if he ever did.  It was a relief to see a happy ending for the gentleman on GMA–his transplant was a success.

Heterochromia

Hands down, Nathan has the most unique anatomy in the Blakeney household. Besides the associated health issues, a component of Scimitar Syndrome is that his heart is twisted backwards in his chest.  Of course, this is, generally speaking, no laughing matter; we were tremendously blessed to have followed him through successful heart surgery.  But we do find our sense of humor about his dextrocardia sometimes.  We point out that he is the only member of the family who should pledge allegiance to the flag with his left hand over his right-sided heart.

Heterochromia: My daughter, Michelle, has a green eye and a blue eye.

So Nathan has the most unique anatomy in the family; but his sister, Michelle, has the most unique eyes in the family. Filling out forms that ask for eye color is straightforward for most of my kids.  Nathan, Adam, and Natalie, have green eyes.  Steven has blue eyes.  Michelle, however, is in a bit of a dilemma when asked to write down her eye color, because one eye is blue and one is green.  It’s a subtle difference, usually takes good lighting to recognize, but she has been grabbed by the face on occasion by the curious observer seeking a better look:  “Hey, what’s up with your eyes?”

David Bowie

Jane Seymour

A difference in the coloration of irises is called heterochromia.  There are two kinds of heterochromia, partial and complete.  In partial heterochromia, the iris has two different colored sections.  Complete heterochromia, which Michelle has, means the eyes are two different colors.

Dan Aykroyd

Kate Bosworth

Certain species of animals are more likely than humans to have this trait. A former boss of mine had a beautiful white manx with a tail that had a striking set of eyes, one blue and one gold, similar to the cat pictured above.  The imperfection had no negative effect at foo-foo kitty pageants; she frequently won awards, including Best of Show.  Other animals that may exhibit heterochromia include dogs (such as Siberian Huskies), horses, cattle, and water buffalo.

Gracie Allen

Even a few celebrities have heterochromia. Take a good look at the eyes of Jane Seymour, Kate Bosworth, David Bowie, and Dan Aykroyd.  The late Gracie Allen also sported two different-colored eyes.

Heterochromia is usually the result of heredity, although it can be caused by a disease or a syndrome (Neurofibromatosis or Waardenberg Syndrome, e.g.).  Occasionally, an eye may change color after an injury, which is the cause of David Bowie’s heterochromia.  Michelle?  She just has a case of funky eyes.

1. Normally, the right pulmonary vein enters the left atrium here. 2. In PAPVR, the right pulmonary vein abnormally enters the right atrium.

This is one of the best illustrations I have found for explaining PAPVR.

Partial Anomalous Pulmonary Venous Return (PAPVR) is a rare congenital heart defect in which blood flow from one or more of the pulmonary veins return to the right atrium instead of to the left atrium.  This is a problem because the right atrium shoots this already oxygenated blood right back to the lungs to be oxygenated again.

Of course, this is a very inefficient way for the heart to function. The heart must work extra hard to provide an adequate amount of oxygenated blood to the body; and over time, this causes a variety of consequences, including arrhythmias, pulmonary hypertension, and right-sided heart failure.

If 50% or more of the pulmonary veins anomalously return, this is considered clinically significant enough to warrant surgical correction. If the patient has an Atrial Septal Defect, the surgeon will close the defect with a patch and redirect blood from the anomalous pulmonary vein to the left atrium.  If there is no ASD, a baffle or tunnel will be created to redirect blood to the left atrium.

PAPVR associated with Scimitar Syndrome has the added complication of dextrocardia. The surgery will likely be performed on a heart that is rotated backwards.  The atrial septum is usually intact.

Our first look at Nathan after heart surgery

My own son, Nathan, had this surgery on July 25, 2006, at C.S. Mott Children’s Hospital at the University of Michigan Medical Center. Our experience included a full day of preliminary testing, surgery the following morning, and a stay in PICU.  After extubation, he was moved to a regular hospital room where he spent the first few days on strong pain medication.  Removal of the chest tubes occurred on about the third day–this was somewhat emotionally challenging but was successful, nonetheless.  By that evening, his personality began to return.  On the fourth day, nurses encouraged him to take a brief walk down the hall.  By the fifth day, he was well enough to be released!

Nathan takes his first walk down the hall after heart surgery to correct PAPVR, Scimitar Syndrome. His surgery was performed at C.S. Mott Children's Hospital, an excellent facility for the treatment of congenital heart defects.

We used a number of child-friendly books to prepare Nathan for surgery.  One of his favorites was Franklin Goes to the Hospital.

Other related posts on this website include:  Scimitar Syndrome, Dextrocardia, Nathan’s Prayer, Remembering C.S. Mott, and Dr. Edward Bove.

Also see my article in Parents magazine:  “Nathan’s Wonder Slide”

If your child has an upcoming heart surgery, consider adding him/her to our Prayer List.

Dr. Edward Bove is experienced in the treatment of complex congenital heart defects.

When faced with a complicated congenital heart defect, confidence in the skill and expertise of your child’s surgeon is of paramount importance.  When our son, Nathan, was diagnosed with Scimitar Syndrome, a rare condition characterized by dextrocardia, pulmonary hypoplasia, and Partial Anomalous Pulmonary Venous Return, we soon learned that our local surgeons had no personal experience performing the required repair.  After reviewing Nathan’s medical records, the closest doctor with a successful surgery under his belt was not willing to proceed due to the “severe rotation of the heart.”

There was tremendous anxiety in our household as we considered our options.  Do we allow a willing doctor to perform the surgical correction even though this would be his first attempt?  Or do we continue to search for a doctor skilled in correcting this rare CHD, possibly allowing Nathan’s heart to weaken in the passing months?

Thankfully, I came into possession of Michael Ruhlman’s book, Walk on Water:  Inside an Elite Pediatric Surgical Unit, which documents Dr. Roger Mee’s dynamic team at Cleveland Clinic in Ohio.  Mee, I discovered (to my initial chagrin), had retired, but the name of another “Walk on Water” surgeon caught my attention–Dr. Edward Bove.

I described our son’s condition to him via email, and was thrilled when he quickly and graciously wrote back that he “would be honored to evaluate” our son for surgery.  Bryan and I were extremely impressed with his credentials.  He had an 85% success rate with Hypoplastic Left Heart Syndrome, a complex condition requiring a series of three surgeries in order to transform half of a heart into a functional heart.  After reviewing Nathan’s medical records, he called one day to inform us that he would perform the surgery.  I took down the necessary appointment information, thanked him, hung up the phone…and shook for the next few minutes, in the grips of an odd combination of joy and terror.

In July of 2006, our family of seven boarded a plane in Hurricane Katrina-ravaged Gulfport, Mississippi, and flew to Ann Arbor, Michigan.  At the C.S. Mott Children’s Hospital, University of Michigan Medical Center, Dr. Edward Bove constructed a baffle out of Nathan’s own pericardium to tunnel through the right atrium to the left atrium, successfully rerouting oxygenated blood to the correct chamber.  That’s not even a Reader’s Digest Condensed version of the story, but suffice it to say, we will forever be grateful to Dr. Bove and his team at C.S. Mott.

My son, Nathan, now eight-years-old, has Scimitar Syndrome. His surgery, performed almost three years ago, was successful.

The abnormal pulmonary vein may be connected to the inferior vena cava or directly to the right atrium, sending already oxygen-rich blood into the chamber that sends blood to the lungs.  Because this is an inefficient way for the heart to work, the heart can become enlarged and weakened.  Untreated, this CHD can lead to dyspnea (breathlessness), atrial arrhythmias, pulmonary hypertension, and heart failure.

Other components of scimitar syndrome include dextrocardia and hypoplasia.  Dextrocardia, the severe rotation of the heart onto the right side of the chest or the mirror-image presentation of the heart, requires no surgical intervention.  Hypoplasia, however, can be associated with recurrent bouts of pneumonia, and in some cases, pneumonectomy or removal of the hypoplastic lung is necessary.

For some patients, conservative treatment is an option.  But if more than half of the pulmonary veins empty into the right atrium, it may be necessary to reroute blood flow into the left atrium.  This is achieved by surgically constructing a “baffle” or tunnel out of the patient’s own pericardium, creating a pathway through the right chamber and into the left.

A different approach may be warranted for patients with a large systemic collateral artery.  Coil occlusion, the placement of a coil to block blood flow, can improve symptoms of heart failure in infants with scimitar syndrome and can be a good surgical correction for this condition.

On a personal note, Scimitar Syndrome is the heart condition that my own child has battled.   See these related articles on this website:  Dr. Edward Bove, Nathan’s Prayer,  About, Partial Anomalous Pulmonary Venous Return, A Memory of Scimitar Syndrome, Three Year Anniversary.

Also see my article in Parents magazine:  “Nathan’s Wonder Slide”

Does your child have Scimitar Syndrome?  Is he/she facing surgery?  Consider adding your child to our prayer list.

In dextrocardia, the heart is abnormally positioned in the right chest instead of forming in the left.  This is frequently detected during prenatal ultrasound and may or may not indicate a heart problem.

Dextrocardia Situs Inversus (mirror image) is extremely rare and usually involves no related medical problems.  If the viseral organs are mirrored as well, this is referred to as Dextrocardia Situs Inversus Totalis, which carries a small risk (5-10%) of congenital heart disease, usually transposition of the great vessels.

Dextrocardia which presents as the severe rotation of the heart onto the right side is a more serious condition which will likely require surgical correction of a cardiac defect.  The patient may have a complex form of transposition of the arteries (levo-transposition) or he may have double outlet right ventricle, meaning both the aorta and the pulmonary artery arise from the right ventricle.  Different problems may be present such as a ventricular septal defect or perhaps, Scimitar Syndrome.*

Adaptations must be made for patients with dextrocardia in the event that use of a defibrillator is necessary.  ECG leads and pads should be placed in reverse.

[My son, Nathan, has Scimitar Syndrome, a rare congenital heart defect characterized by dextrocardia, pulmonary hypoplasia, and Partial Anomalous Pulmonary Venous Return.  See About for more information.]

- Article and illustration by Karen Blakeney