03/04/2013



The first such procedure performed in the Marion and John E. Anderson Pavilion, it is the fifth Hybrid Norwood Surgery by the hospital’s renowned Heart Institute.

When two-day-old Octavio Angel Hernandez Jr. arrived at Children’s Hospital Los Angeles last Christmas Eve, the 6.8 lbs. baby boy was fighting for his life. “He was in shock,” says Frank Ing, MD, a pediatric interventional cardiologist at Children’s Hospital Los Angeles. “He was dying because not enough blood was getting to his body.”

 

Earlier in the day, little Octavio Jr. had been the center of attention as the Hernandez family gathered at his parents Echo Park home to celebrate the holiday and their new family member’s arrival. But as the afternoon wore on, Octavio Jr.’s breathing became labored and his voice disappeared. “He had cold feet and hands, a bluish face, purplish lips,” recalls his father, Octavio Sr. “He was crying; but only his mouth was moving; there was no sound coming out. I turned to my wife and said, let’s put him in the car seat and go!”

 

By the time they arrived at the Emergency Department at Children’s Hospital Los Angeles, Octavio Jr. was in low output syndrome – his liver and kidneys were shutting down. The diagnosis: hypoplastic left heart syndrome, a congenital heart defect that shows up in about one in 2,500 births. The defect prevents the heart’s undersized left side – identified by underdeveloped mitral valve, left ventricle, aortic valve, and aorta – from pumping enough blood through the body. “Had we arrived 10 minutes later, we would have lost him,” his father says.

 

Fortunately, nature, science and expert emergency treatment combined to save Octavio Jr.’s life. Though Octavio Jr.’s left ventricle was under developed, blood flow to the body could be still be pumped by the right ventricle through the ductus arteriosus.  The problem is this blood vessel normally closes within a couple of days after birth as left heart takes over the function of pumping the oxygenated blood from the lungs to the body.  In Octavio’s case, he didn’t have a normal left heart to do the work and as the ductus closed, his body did not get adequate blood flow.  Doctors had to open the ductus up just to keep him alive. “We Caught him early enough in the process and put him on prostaglandins (PGE ), a medicine that relaxes the ductus and opened it back up,” explains Ing. “So now there’s better blood flow to the body -- but the body has taken a tremendous insult…and it doesn’t recover very easily.”

 

Octavio Jr. hadn’t suffered brain damage, but his kidneys and liver were damaged and his body needed three weeks of recover in the hospital’s cardiothoracic intensive care unit before Children’s Hospital Los Angeles Heart Institute surgeons could take the next step: open heart surgery.

 

His doctors – Ing and Cardiothoracic Surgeon Cynthia Herrington, MD, decided to perform the Hybrid Norwood, a less invasive surgery sometimes used in place of the Norwood surgery, a four-hour long procedure that requires the use of heart-lung bypass machine. “The insult to his body was severe,” explains Herrington. “His liver and kidneys came back but they were not back to normal, so for Octavio, the Hybrid Norwood was a better alternative.”

 

It would be the first Hybrid Norwood performed in Children’s Marion and John E. Anderson Pavilion, which opened in 2011, and fifth overall at the hospital. On Jan. 14, Octavio Jr. was brought to the hospital’s Catheterization Laboratory and the doctors went to work. Herrington opened up Octavio’s chest and began the delicate job of tying tiny bands, over Octavio Jr.’s left and right pulmonary artery branches to limit blood flow to over-circulated lungs.  The second step – Ing’s specialty – required the insertion of a stent – a tiny mesh tube –into the ductus that once in place, would keep the ductus open without medications and increase blood flow to Octavio Jr.’s body. Before Ing could implant the stent, Herrington inserted a sheath, a small hollow tube, in Octavio Jr.’s pulmonary artery. As Herrington steadied the sheath, Ing used a tiny guide wire with the aid of X-Ray to maneuver the stent through Octavio’s artery to the ductus arteriosis blood vessel. Expanding the stent, Ing secured the vessel, allowing blood to continue circulating through Octavio’s body. The surgery took less than an hour. “It went very smoothly,” says Herrington. “Octavio did very well.”

 

Octavio will need another second, more complex surgery later on, but he will be bigger and stronger to endure the procedure, Ing says. “We have the expertise here to handle the next stages,” he says. That second surgery, called the Glenn procedure, will take place in four to six months and will involve the complex reconstruction of the aorta arch. The third stage, the Fontan operation, takes place later.  

 

This procedure was also the first Hybrid Norwood performed by the team of Herrington and Ing, the ladder of whom was recruited to Children’s Hospital last September from Texas Children’s Hospital to collaborate with Heart Institute surgeons on such operations. In addition to working with the hospital’s renowned cardiothoracic surgeons on Hybrid Norwood operations, Ing also is working with the Heart Institute team to enhance the hospital’s Hybrid Catheterization Laboratory and a fetal cardiac intervention program, a joint effort between the hospital and the Departments of Pediatrics and Cardiac Surgery.

 

The hospital’s Heart Institute has a long tradition of innovation. The Children’s Hospital Cardiology and Heart Surgery program, which dates back to the late 1920s, performed the very first pediatric heart surgery – a Patent Ductus Arteriosus Ligation -- on the west coast in 1939. Seven decades later in 2009, Children’s Hospital performed 918 cardiothoracic surgeries, 682 catheterization procedures, 12,040 non-invasive procedures and 400 fetal procedures. In 2010, 7,331 echocardiograms were performed. In all, almost 8,000 children are treated at the hospital’s Heart Institute each year, and more than 900, like little Octavio Jr., undergo cardiac surgery.

 

In the tense moments leading up to their decision to rush their baby to the hospital, Octavio Sr. and his wife, Liliana Marquez, considered taking their son back to the hospital where he was born, but mother’s intuition intervened. “She felt it was very serious,” Octavio Sr. says. “Our daughter had her appendix out at Children’s so we felt confident they would know what to do.”

 

They were lucky to be nearby a world class children’s hospital. Back in November, a month prior to Octavio Jr.’s birth, the family decided to return to Los Angeles from Oklahoma, where Octavio Sr. was working in construction. “We came home to L.A. because I got a new job and it made sense for us to have our baby close to home. Plus our two daughters really missed their grandma!,” Octavio Sr. says. “Everything happens for a reason.”

 

After the Jan. 14 surgery, Octavio Jr. spent three more weeks in the hospital regaining his strength. His mom stayed by his side virtually the entire time, save for several days when she went home to see her daughters. Octavio Sr. came visited on nights and on weekends while extended family took turns watching their two girls, ages 4 and 6, who didn’t get to visit their baby brother because of the hospital’s restrictions during flu season. But on Feb. 7, Octavio Jr. went home. “He’s feeling good now,” says Octavio Sr. “He’s 9 pounds, his voice is back, he’s feeding from his bottle and he’s breathing great. The sound of him crying is like music to our ears.”