Last spring, a few months before his 2014 high school graduation, Ray Santa Cruz suddenly noticed a strange feeling in his chest.
Ray Santa Cruz of Salinas, Calif., seen here with mom Joanna, had a heart condition that could lead to sudden death. Lucile Packard Children’s Hospital Stanford leads the way in fixing a defect more common that most doctors realize. (Photo: Business Wire)
When he ran, he felt pain in his chest and noticed his breathing changed. Sometimes, if he stood up quickly, he felt faint and his legs weakened under him. Soon, the symptoms started interrupting his sleep. I couldnt breathe, he said. Whenever I would sleep, I would wake up to chest pain and my heart beating fast.
One night in early summer, his parentswho initially thought Ray was having anxiety attackstook him to a local emergency room because his symptoms became too troubling to ignore. His doctor told him he needed to be seen by a cardiologist.
After a battery of tests, the root of his problems was found: Ray had lived his whole life with a rare, congenital heart defect that affected his coronary arteries, the vessels that feed blood to the heart. By fall, his care had been transferred from Salinas, California, where he lives with his family, to a team led by Frank Hanley, MD, cardiothoracic surgeon at Lucile Packard Childrens Hospital Stanford and Stanford Childrens Health.
The name of Rays scary problem? Anomalous aortic origin of a coronary artery, a condition associated with a high incidence of sudden death. In early December, he underwent a complex surgery to correct the problem.
Thirty years ago, most patients with congenital abnormalities of the coronary arteries were only diagnosed after collapsing from a heart attack in their teens or 20s. But in the past dozen years, advances in imaging technologies have made it easier to see minute details of the coronary arteries, and more cases are being identified before these young patients suffer severe consequences.
While cardiologists are aware of acquired coronary artery problems in the elderly, many dont know that the condition can also affect younger patients, often causing them to be misdiagnosed.
Cardiologists treating younger patients will often prescribe a standard bypass operation, the type designed for older patients with the more typical, acquired blockages in the coronary arteries. Thats absolutely the wrong thing to do, said Hanley, the Lawrence Crowley, MD, Professor in Child Health and a professor of cardiothoracic surgery at Stanford University School of Medicine. Thats what you do for Grandpa.
In a coronary bypass procedure, a surgeon harvests blood vessels from a different part of the body, often the leg, and uses them to fashion a detour around a blocked artery. But for patients like Ray, that approach can backfire and the graft can stop functioning.
In Rays heart, his left coronary artery didnt branch off from the aorta in the correct area. Instead, it grew from the aorta on the opposite side, which meant it had to travel several extra inches through the wall of his heart. Every time his heart pumped, it squeezed the artery like it was in a vise, said Hanley. This decreased the blood flow to Rays heart and cut it off entirely for short periods when he exerted himself.
Defect more common than originally thought
In the 1980s, physicians only knew of about a dozen cases per year of AAOCA, usually found during autopsies. Doctors now know this defect is more common, but the exact number is hard to pin down since many cases are undiagnosed. In the 15 years since Stanford physicians developed surgical techniques to fix the defect, about 90 patients have been treated by the cardiothoracic surgery team at Lucile Packard Childrens Hospital. No other institution has treated as many cases in young people.
Hanley and his team have developed a series of delicate operations that directly address the abnormal arteries. Thats because AAOCA comes in several variations, and each requires a different surgical procedure for correction.
Most commonly, the procedure will involve unroofing a segment of the abnormal artery, or translocating, which re-routes the abnormal coronary artery if it is trapped between the aorta and pulmonary artery. Ray, unfortunately, had the rarest and most complex variation of AAOCA, and neither of these surgical approaches could be used. Rays coronary artery not only arose abnormally from the aorta, but it was also embedded in the muscle of the wall of his heart for several inches and travelled behind the pulmonary valve. Until recently, no effective surgical procedure had been developed to treat this form of the condition.
Over the last decade, the Heart Center team designed a novel procedure for Rays form of AAOCA. It involves removing the heart muscle overlying the portion of coronary artery buried in the muscle of the heart wall, and also moving the pulmonary artery trunk and valve forward on the heart to relieve compression of the underlying abnormal coronary artery.
It worked, and Rays repair was completed before his heart muscle incurred any lasting damage. But some cases are not diagnosed early enough to avoid permanent damage or sudden death. In rare cases, the damage has been bad enough that otherwise young, healthy patients have required heart transplants.
No one thinks young athletes will have heart problems like this, said Richard Mainwaring, MD, a cardiothoracic surgeon on Hanleys team and clinical professor of cardiothoracic surgery at the School of Medicine. The chest pains are mini-episodes of heart attacks. But coaches dont think youre having a heart attack, they think, you need to toughen up. But if you are 70, you would be rushed to hospital. Invariably, there is a delay in diagnosis.
The initial diagnosis was frightening and demoralizing for Ray and his family. Even just to hear the diagnosis, I was scared, he said.
Rays parents, Joanna Santa Cruz and Ray Olivares, credit the expertise and advanced techniques of the Stanford team for their sons health. He was up and walking quickly right after leaving the hospital, Joanna said.
Hanley and Mainwaring are confident Ray will make a full recovery. Hes still shaken by the long months he lived with his mysterious chest pains, but within weeks of his surgery in December, he said he already felt stronger.
This spring, hell leave for Sacramento to start automotive school, and hes looking forward to basking in his love of all things car-related.
Im happy its done and over with, said the affable Ray, no longer worried about those chest pains.It was a success, and now Im just taking things day by day.
About Stanford Childrens Health and Lucile Packard Childrens Hospital Stanford
Stanford Childrens Health, with Lucile Packard Childrens Hospital Stanford at its core, is an internationally recognized leader in world-class, nurturing care and extraordinary outcomes in every pediatric and obstetric specialty from the routine to rare, for every child and pregnant woman. Together with our Stanford Medicine physicians, nurses, and staff, we deliver this innovative care and research through partnerships, collaborations, outreach, specialty clinics and primary care practices at more than 200 locations in the U.S. western region. As a non-profit, we are committed to supporting our community from caring for uninsured or underinsured kids, homeless teens and pregnant moms, to helping re-establish school nurse positions in local schools. Learn more about our full range of preeminent programs and network of care at stanfordchildrens.org, and on our Healthier, Happy Lives blog. Join us on Facebook, Twitter, LinkedIn and YouTube.
Lucile Packard Childrens Hospital Stanford is the heart of Stanford Childrens Health, and is one of the nations top hospitals for the care of children and expectant mothers. For a decade, we have received the highest specialty rankings of any Northern California childrens hospital, according to U.S. News & World Reports 2014-15 Best Childrens Hospitals survey, and are the only hospital in Northern California to receive the national 2013 Leapfrog Group Top Childrens Hospital award for quality and patient care safety. Discover more at stanfordchildrens.org.