Some medical conditions are as common as a cold. Others are as rare as a blue diamond. Children’s Hospital Central California believes all kids deserve incredible care – no matter the complexity of their medical condition.
Since 1988, the Kids Day special edition of The Fresno Bee has featured stories of extraordinary children who received care at Children’s for all kinds of conditions. The 2014 Kids Day Ambassador is 12-year-old Mateo Escobar, and his heart may be as rare as a blue diamond.
During Mateo’s well-baby clinic visit at 4 months of age, his pediatrician, Dr. Leonard Thompson, could not find a pulse in Mateo’s lower extremities. The simple absence of a femoral pulse signaled a potentially life-threatening congenital heart defect.
“I’ll never forget,” said David Escobar, Mateo’s dad. “Dr. Thompson knew Mateo was born with a heart defect, but he said, ‘I’d prefer it if the staff at Children’s Hospital tell you.’”
The Escobars took Mateo to see Dr. James Prochazka, medical director of cardiology at Children’s, for an echocardiogram (an ultrasound study of the heart). This study showed a condition called coarctation of the aorta. The aorta is the blood vessel that supplies blood to the entire body, and Mateo’s had a narrowing at one point, much like an hourglass.
“Heart defects can present on the first day,” said Dr. Prochazka. “But typically babies with Mateo’s heart condition are born without symptoms and go home from the hospital.”
“If Mateo’s pediatrician hadn’t looked for a femoral pulse – and not all doctors do – we never would have known that something was wrong… until it would’ve been too late,” said David.
“With coarctation of the aorta the pulse is easy to find in the right arm, but it’s difficult to find in the legs,” said Dr. Prochazka. “The heart is working hard, like it’s trying to pump against a garden hose with a kink in it.” He referred the family to Dr. Edwin Petrossian, medical director of cardiac surgery at Children’s, for a routine repair.
“Prior to surgery, no one was concerned,” said Dr. Petrossian. “We thought it was a normal coarctation.”
However, a rare gem was hidden in Mateo’s chest. The aorta stems upward from the heart then makes a U-turn (called the aortic arch) and descends to carry blood to the lower parts of the body. Normally, the aortic arch curves to the left side of the trachea and esophagus (wind pipe and food pipe), which are always in the middle of the body in front of the spine. But Mateo’s arch curved to the right.
“It’s pretty rare to have a right-sided arch, and it’s extremely rare to have it without other heart problems that might have alerted us to the possibility of a right-sided arch,” said Dr. Petrossian. “This unusual malformation was not apparent on the preoperative echocardiogram. Today’s enhanced imaging capabilities might have shown this rare problem, but back then these anomalies were not always seen.”
Mateo had yet another unusual condition. Unborn babies have a blood vessel to bypass their lungs that normally closes soon after birth. The remnant of this blood vessel, known as the ligamentum arteriosum, together with a right-sided arch, formed a vascular ring encircling Mateo’s trachea and esophagus. “A vascular ring can cause symptoms with breathing or swallowing difficulties, but Mateo didn’t have these,” said Dr. Petrossian. “And vascular rings usually do not occur together with a coarctation of the aorta.”
Dr. Petrossian and his surgical team made an incision on the back of Mateo’s chest along his shoulder blade to repair what appeared to be a common congenital heart defect, but when they reached the heart, they discovered the unexpected challenge. “Coming in from the back it was very challenging to reach the narrowing,” said Dr. Petrossian. The routine surgery took nearly twice as long and involved repairing the vascular ring in addition to the coarctation.
“It was definitely not routine,” said Karen Escobar, Mateo’s mom. “They opened him up and said, ‘This we haven’t seen very often.’ Then Dr. Prochazka came to see us with his heart model to explain everything.”
Initially, Mateo did fine after surgery. However, regular follow-up appointments with Dr. Prochazka showed that as Mateo grew, the narrowing in his aortic arch was returning. The abnormal tissue at the site of the repair was not growing with him.
“It’s very rare to have recurrent coarctation,” said Dr. Petrossian. “Only about 1 to 5 percent of cases will have the narrowing return. You can’t leave a patient with narrowing.”
Mateo was scheduled for a heart catheterization procedure with Dr. Carl Owada, medical director of the cardiac catheterization laboratory at Children’s, who discovered that in addition to the narrowing of the arch at the original site of the repair, the blood vessel to the right arm was also narrow, which could result in a condition called subclavian steal syndrome. Subclavian steal happens when blood flow in the artery supplying blood to the head and brain reverses to supply blood to the arm, in essence “stealing” it from the head. Dr. Owada attempted a balloon angioplasty of the recurrent narrowing in the aorta and subclavian artery. The blockage, however, proved too rubbery to dilate with a balloon. Mateo would need further surgery.
Mateo’s first surgery had occurred at 5 months of age. The second took place five months before he turned 2. “I think the hardest part is right before surgery,” said David. “You’re holding your baby and you’ve got to hand him to the doctors.”
In surgery Dr. Petrossian fixed the residual coarctation, this time entering through the front of the chest to better reach the right-sided arch. But he could not risk taking the time needed to widen the artery to the right arm.
“During open heart surgery, you must place the patient on a machine to supply blood to the body and stop the heart and lungs while you perform the repair,” said Dr. Petrossian. “You don’t want a patient on cardiopulmonary bypass for too long. So we made the decision not to repair the narrowing to the right subclavian artery at that time. It was not worth the uncertain results.” Instead, Mateo’s expert medical team planned a separate procedure for a future date. “The repair to the artery could be done by Dr. Owada with a balloon and a stent,” he said. “Waiting for Mateo to grow older before placing the stent helps ensure it will be large enough to keep his artery from narrowing in adulthood.”
“After the surgery we stopped for dinner on our way home,” said David. “Mateo was asleep in the PICU and the staff recommended we go home and get some rest.”
“The phone rang while we were at the restaurant,” said Karen. “And we found out Mateo had internal bleeding and was being rushed into emergency surgery.”
David and Karen raced back to Children’s Hospital to wait while Mateo was once again entrusted into the skilled hands of Dr. Petrossian, who located the source of the bleeding and easily repaired it.
“It’s rare to have bleeding like that,” said Karen, who continued to be reminded that her son’s medical condition was “definitely not routine.”
“There were all these complicated things going on and it was very stressful,” said David. “Both my wife and I are decision-makers in our workplaces, but in this circumstance you are powerless. It was very difficult.” The medical team’s confidence that Mateo would make a full recovery greatly encouraged David and Karen. “Over my career I’ve attended a lot of retirement parties,” said David. “I see plaques handed out to recognize years of dedicated service, but I rarely see the level of dedication medical professionals have. I want to tell people they haven’t seen dedication until they see doctors like the ones at Children’s Hospital.”
“When we first learned of Mateo’s heart defect, some of our friends told us that we should get a second opinion,” said Karen. “But the cardiologists and surgeons at Children’s consult with different hospitals. They have round table discussions and share information, so we actually did get a second opinion and even more opinions than that.”
After Mateo turned 11, Dr. Prochazka discussed his case during the combined Department of Cardiology and Cardiothoracic Surgery Case Conference, and it was recommended that Mateo was now old enough to undergo a cardiac catheterization for balloon angioplasty and stenting of his right subclavian artery. Dr. Owada performed the procedure Jan. 3, 2013, restoring healthy blood flow to the right arm.
“Mateo runs around now and does great,” said David. “He’s in sports and scouts. The doctor hasn’t set any limits on his physical activities.”
Mateo makes annual visits to Children’s cardiology practice and Dr. Prochazka says all is well. “These cases are very rewarding because we can fix things,” he said.
“Now when friends have babies, I recommend they have the doctor check the pulse in the lower extremities,” said David. “I tell them to do it at the 4-month checkup because it saved Mateo’s life.”
For 27 years, Children’s has cosponsored Kids Day with The Fresno Bee and abc30 to benefit the Hospital known for its amazing people and incredible care. Among the hundreds of volunteers who plan to hit the streets before dawn on March 4 to sell the special edition newspapers, watch for our 2014 Kids Day Ambassador. After all, you can’t always buy a paper from someone whose heart may be as rare as a blue diamond.