heART

• Heart defects are the most common and deadly of all birth defects.
• Approximately 36,000 babies are born each year with a congenital heart defect.
• In 1950, a child born with a congenital heart defect had only a 20% chance of survival.  Today, survival rates have increased to 90%.
• Survivors of successful childhood intervention face life-long risks, including heart failure, stroke, and rhythmic disorders.
• Each year, nearly twice as many children die from CHD in the U.S. than from childhood cancer, yet funding for pediatric cancer research is five times higher than funding for congenital heart defects.
• For every dollar the government spends in medical funding, only a fraction of a penny goes toward congenital heart defects research.

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.

Senator Thad Cochran

This month, Senator Thad Cochran joined Senator Dick Durbin (D-IL) and Representatives Zack Space (D-OH) and Gus Bilirakis (R-FL) to introduce legislation to promote education and research for congenital heart defects.  His support of The Congenital Heart Futures Act is great news for 1.8 million people in America living with congenital heart disease.

“This legislation builds on recommendations by the National Heart, Lung, and Blood Institute to support and augment the research being done on congenital heart disease,” said Senator Cochran.  “The research will translate into better clinical treatments for Americans living with congenital heart disease.”

Quick Facts:

• Heart defects are the most common and deadly of all birth defects.
• Approximately 36,000 babies annually are born with a congenital heart defect.
• In 1950, a child born with a CHD had only a 20% chance of survival.  Today, survival rates have increased to 90%.
• Survivors of successful childhood intervention face life-long risks, including heart failure, stroke, and rhythmic disorders.
• Each year, nearly twice as many children die from CHD in the U.S. than from childhood cancer, yet funding for pediatric cancer patients is five times higher than funding for CHD.
• For every dollar the government spends in medical funding, only a fraction of a penny goes toward CHD research.

So, thank you, Senator Cochran, for your concern and support of children and adults with Congenital Heart Defects.

There’s a glossary in the back along with a list of resources and pertinent CHD books.  The Foreword by Sylvester Stallone and his wife, Jennifer, is also a nice touch.

If I have any reservations about the book, it is my discomfort with the question, “When do doctors recommend that you terminate a pregnancy?”  My own child would have been a candidate for termination by some standards, a supposition that ignores the intrinsic value of a life regardless of disabilities.