Many people with congenital aortic heart valve disease may not be diagnosed or seemingly affected with symptoms of their condition. Congenital aortic valve disease presents in only about 0.5% of the  population as is often well tolerated. But in about 25% of people with the condition, complications can occur by middle age, causing the heart to pump inefficiently, increasing the risk of heart failure and, in severe cases, cardiac arrest.

Fortunately for Weston Sondeno of Big Timber, Montana, after he was born at St. Vincent Regional Hospital in Billings, a nurse in the newborn nursery heard a heart murmur which led to a heart ultrasound (echocardiogram) and his diagnosis with the heart condition.

The rate of aortic valve disease progression can be unpredictable. With the knowledge of Weston’s heart condition, his family scheduled an annual screening from the pediatric cardiology team at St. Vincent to monitor his health. Early on, his valve functioned well, and through his elementary years an intervention wasn’t needed, but his doctors felt that at some point heart surgery would likely be needed.

As an active, high-achieving teenager, Weston had his sights on big goals, including attending Montana State University and preparing for a career as a veterinarian. His annual heart check-ups had become routine, and the need for surgery seemed decades away.

This high school senior with a strong drive was stopped in his tracks last September. During his annual exam, Dr. Hugh Bigg, DO, pediatric cardiologist with Intermountain Health in Billings, noticed a change. While Weston had remained without symptoms and his heart’s function had remained normal by echocardiogram, there were signs of heart strain which indicated surgery would be required more urgently.

“My whole life I’d been told that maybe at age 50 or 60 I might have to have a valve replacement,” Weston said. “But at 17, to be told, ‘Hey you might have to have open heart surgery in the next year,’ I thought, whoa.”

Weston and his care team were now looking at options for a surgery that had gone from years to months away.

“Dr. Bigg and I talked about three or four common procedures for patients with my condition,” said Weston, “Because of my young age, we decided that the ROSS procedure would be best.”

The Ross procedure is a type of aortic valve replacement in which a cardiovascular surgeon removes the diseased aortic valve and replaces it with the patient’s own pulmonary valve (autograft); the pulmonary valve is then replaced with a pulmonary valve transplanted from an organ donor.

Patients who undergo the Ross procedure are able to live an active lifestyle and are less likely to experience heart failure. Other surgical options, such as a mechanical heart valve or a bioprosthetic valve would have required Weston to be on blood thinners. That, in turn, could have had a greater impact on the activities he could enjoy and, potentially, his future plans.

Weston was referred to the Intermountain Health Primary Children’s Hospital in Salt Lake City, which is a specialized, high-volume center with a highly experienced pediatric cardiac surgical team that regularly performs delicate, and complex heart surgeries in children, from newborns to young adults living with congenital heart disease.

“Telling a patient and their family that an open-heart surgery is going to be needed is never easy,” said Dr. Bigg. “Although these were some of my first consultations with Weston, how he processed this information was very impressive. I think it reflects the type of young man he is, how he was raised, and the Montana community he has grown up in.”

While it was difficult to come to terms with the fact that he would now be undergoing an open-heart procedure, Weston approached the challenge as he has most things in his life, with optimism.

“Weston was fully engaged in the process, choosing the surgery he felt would have the best long-term outcomes, and maintained his positive attitude throughout the experience,” shared Dr. Bigg. “Weston is so positive,” his mom Susan said. “A couple times, he’d say, ‘I don’t know what you guys are so worried about, I’m in great hands.”

Weston was walking the first day following the complicated eight-hour ROSS surgery and was discharged home after four days. In the end, Weston missed only nine days of school.  Weston went on to compete in the Montana State FFA Convention. There, he had four, top-five finishes, including first place, and won a scholarship that he’ll use at Montana State University beginning this fall.

“He’s intelligent, resilient, picks up on everything and remembers everything,” said his dad, Bryan. “Everything he learns he applies to real life. He’s a pretty amazing individual.”

“He graduated on time with a 4.0 and is now off to MSU. Not bad for a kid who had an 8-hour open heart surgery in the middle of his senior year. He doesn’t let much stand in his way.”

Expanding specialty care close to home

Weston and his family joined his caregivers for a recent ribbon cutting of the $1.4 million expansion of the Intermountain Health Fortin Pediatric Specialty Clinic in Billings. The renovation was made possible with generous donations from the Mary Alice Fortin Foundation, the Otto Bremer Trust and other individual donors. The renovation includes a child-friendly waiting and reception area, new physician and nurse workstations, a developmental pediatrics therapy room, echo and ultrasound room, and eight additional exam rooms.

In recent years, St. Vincent has seen more than a 35 percent growth in the number of children seen at the pediatric specialty clinic and we have grown our team of specialists from seven to 20 specialists in cardiology, pulmonology, endocrine and diabetes, developmental and neurologic pediatric care, and other areas.

“We are thankful for the generosity of the Mary Alice Fortin Foundation and the Otto Bremer Trust to allow us to continue our mission to meet the needs of the children and families in our communities,” shared Lee Boyles, President, St. Vincent Regional Hospital and Montana | Wyoming Market of Intermountain Health. “We understand the challenges that families face when they must travel to receive specialized care for their children when diagnosed with a serious or chronic condition. Our goal is always to keep as many kids, like Weston, as close to home as possible. With the renovation of the pediatric specialty clinic, we are taking the next step forward in providing cutting-edge, innovative pediatric care.”

The Mary Alice Fortin Foundation has long been one of the most significant supporters of growing pediatric care at St. Vincent and in Billings. In 2008, the foundation made a gift to establish Eastern Montana’s only pediatric intensive care unit at St. Vincent and the pediatric specialty clinic was opened in 2012 through a generous leadership gift. Through additional support, the foundation has expanded St. Vincent’s telemedicine capabilities, provided training for health care providers across our region in pediatric trauma and disease care, and launched the only pediatric surgery program in the region at St. Vincent.