Heart diseases and defects come in myriad forms in children, some developing before birth, while some are part of genetic syndromes and some are acquired through other factors during childhood. But what all childhood heart diseases have in common is the need for more clinical solutions that can only be found through research. Children with heart diseases and defects experience some of the most extreme surgical, life-saving interventions and must overcome the challenges and obstacles that come along with those procedures and their complications and side effects. It is our hope that by sharing our children's stories, we will increase awareness of the impacts of pediatric heart disease and the need to move research forward.
Ritchie was born in 2005 with Tetrology of Fallot and an ASD. At 4 months old, he received full surgical repair: patch closure of a large VSD, pulmonary valvotomy, pericardial patch pulmonary arterioplasty, and primary closure of secundum atrial septal defect. Richie suffered in congestive heart failure for 3 months before his surgical repair. After surgery, he experienced seizures or stroke, most likely caused by being on the heart-lung bypass machine. By 7 months post-surgery, Richie was off all medications. At 3 years old, Ritchie was cleared to play contact sports and has rotated to every two-year follow up exams. His pulmonary valve is still slightly dysplastic. He may or may not need to have his pumonary valve replaced if it starts to fail in the future.
What research could do for Ritchie: Research has just now brought about the ability to replace pulmonary valves via catheter versus open-heart surgery. This technique needs to be enhanced and proven over time. Should Ritchie need a valve replaced, this technique will allow valve replacement without the risk and complications of open-heart surgery. Advancements in tissue engineering could also create living tissue valves that could be placed using catheters. These are a superior option because they would eliminate the need for taking anti-coagulation drugs. Also, research may elucidate the cause of higher incidence of sudden cardiac death among adults who received a repair of TOF as children. As the cause is unknown, research could lead to the prevention of sudden cardiac death.
Jane was born in China in 2003, with Tetrology of Fallot with Pulmonary Atresia. She has had 3 open heart surgeries and 5 heart catheterization procedures. Because she has parts in her heart that don't grow, she will need those parts replaced to match the demands of an adult heart. Those parts only last 10-20 years so will again need to be replaced during adulthood.
What research could do for Jane: Currently researchers have the ability to grow living vascular tissue using a patient's own cells to replace non-living tissue. This would mean one more surgery, versus numerous surgeries in her lifetime. She would avoid all the potential complications of those future surgeries. As open-heart surgery is one of the most expensive medical procedures, solutions using living tissue that won't need to be replaced will also pay for themselves by not taxing the health care system in the future.
Wren has Shone's Syndrome, a heart defect in which the left side of the heart has areas that are too small and obstructions which can progress over time. Wren was born in Seattle in 2006 and has had two heart surgeries and two cath procedures to help repair the area around his aortic valve and a coarctation in his aorta. His cardiologists are watching many areas of his heart including his aortic valve to see when he will need another open heart surgery to repair or replace this valve.
What research could do for Wren: Research that leads to better options for aortic valve replacment and living valve tissue, versus artificial valves, could reduce the number of surgeries Wren will need in his lifetime. It would also increase his chances of living an active life free from the shadow of reoccurent valve disease.
Click here to learn more about Wren: http://echobaby.blogspot.com.
Madison was prenatally diagnosed with the complex heart defect, pulmonary atresia with intact ventricular septum, Ebstein's Anomaly, and Hyplastic Right Ventricle. In short, the right side of her heart was severely underdeveloped and non-functional. She had three heart catheterizations and two open-heart surgeries. In 2-3 years, Maddie will undergo the last stage of her repair, called the Fontan Procedure. Upon completion of this open-heart surgery, her heart will function as a single ventricle, the left side of her heart doing all the work while blood passively flows to her lungs, bypassing the right side of the heart.
What research could do for Maddie: Having a single-ventricle heart, Maddie's future is uncertain. The oldest living patients with this condition are in their late 20s and early 30s. Her heart repair is simply palliative and she will most likely need a heart transplant in the future. Stem cell research is promising for single-ventricle patients like Maddie because of its potential. The re-growth of heart tissue and valves offers hope and options for a brighter future for kids with complex heart defects.
Click here to learn more about Maddie: http://allredbabygirl.blogspot.com
Evan was born in 2006 with Transposition of the Great Arteries (TGA, a congenital heart defect in which the two great arteries (pulmonary artery and aorta) are in reversed locations. Babies with TGA require open-heart surgery within days or weeks of birth to survive. Evan underwent the arterial switch operation to correct his TGA when he was 9 days old. He also has a single coronary artery and post-operative pulmonary stenosis (narrowing of the pulmonary artery).
What research could do for Evan: The arterial switch has been the surgery of choice to repair TGA for less than 20 years. While mid-term outcomes of the operation are largely successful, more must be discovered about a number of critical factors that may complicate heart function of this young patient population as they grow into adulthood. For example, the complexities of how the coronary arteries (major blood vessels supplying the heart) are functioning in these children are largely unresolved and will be of major impact in their future.
Keanon was diagnosed with Ventricular Septal Defect (VSD) at two weeks of age. Due to the location and size of the hole, it was determined that it could only be repaired with open-heart surgery. At 6 months old, his heart was repaired using a Dacron patch. Keanon is expected to live a normal and healthy life. He will be monitored closely as he grows up and though he has slight residual damage from his VSD, it is unlikely that he will need further surgical intervention.
What research could do for Keanon: More often, VSDs can be repaired using catheter procedures and devices. AS research, tools and techniques advance, even larger VSDs can be repaired without invasive open-heart surgery. Because heart repair procedures have advanced so quickly over the past few decades, doctors do not yet have solid outcomes for children who have had open-heart surgery, been on by-pass, or lived iwth patches for more than 20 years. Research will help refine materials used and gather data on children as they live longer lives, post repair. To read more about
Keanon's heart journey, visit: www.jimandtonneka.com
Caleb was diagnosed with Transposition of the Great Arteries at his 20 week ultrasound. He had the arterial switch procedure at 12 days old, and developed a narrowing of the pulmonary artery (pulmonary stenosis). Caleb has had two balloon angioplasty procedures to widen his pulmonary artery, and will need a stent in the future, and may need additional procedures to widen the pulmonary artery as he grows to adult size.
What research could do for Caleb: Because the arterial switch is a relatively new surgical procedure, no one has survived yet to middle age that has had the surgery. Prior research, led to the development of this technique, and additional innovations and techniques will need to be developed to address the unknown complications that will arise in their futures.
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Eli was born with coarctation of the aorta, large VSD and PDA. He had open heart surgery at 18 days old to repair the coarc and close the VSD with a ligation band. He suffered several complications and eventually had ballon angioplasty to widen the coarctation and a thoracic duct ligation to stop the chylothorax. Two years later he developed a subaortic stenosis and had a second open-heart surgery on 2/3/09 to repair this. He has since developed mitral valve leakage, and a bulging muscle above the main area where his heart's natural pace maker is located. He may require additional surgeries to address the subaortic stenosis as well as have the mitral valve and/or aortic valve replaced.
What research could do for Eli: Because the exact location of the heart's electrical system is invisible to surgeons, research could one day show how to locate this site so it is not impacted by surgery. Also, less-invasive techniques for replacing valves need to be perfected, as well as the creation of valves from living tissue, to reduce the need for multiple surgeries in the future.