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Human Heart Surgery Saves Dog’s Life

Analysis by Dr. Karen Shaw Becker

arvc in boxer dogs

Story at-a-glance -

  • A nine-year-old dog named Sophie underwent a cardiac ablation procedure to treat her arrhythmogenic right ventricle cardiomyopathy (ARVC) in a surgery that was a first for a Philadelphia medical research lab
  • Sophie’s erratic heartbeat was similar to that experienced by humans withan arrhythmia, and a heart surgeon believed the same high tech but routine procedure would work for her as well as it did for humans
  • Boxers and American bulldogs are candidates for ARVC, which in humans strikes approximately one person in every thousand, but may not manifest itself until adulthood when heart muscle tissue begins deteriorating
  • Scientists discovered more than 10 years ago that ARVC is caused by a gene mutation, then found a genetic deletion in a gene producing striatin, an important binding protein that helps hold cardiac cells together
  • Patterns of Sophie’s arrhythmias were collected using a Reveal LINQ implanted just under her skin, which records and stores abnormal rhythm data up to three years and helped doctors target the spots in Sophie’s heart needing ablation

Doctors at the University of Pennsylvania’s (Penn) School of Veterinary Medicine in Philadelphia say it was a first: A nine-year-old dog named Sophie underwent a cardiac ablation procedure to treat her arrhythmogenic right ventricle cardiomyopathy (ARVC), commonly called an arrhythmia, and she came through with flying colors.

What put Sophie’s surgery on the map was that it was the first time a boxer or any other type of dog with heart distress had received such treatment. According to Anna Gelzer, the Penn School of Veterinary Medicine cardiologist who performed Sophie’s surgery, the treatment saved her life.

Because Sophie’s heart condition — an erratic heartbeat — was the same type of problem and showed very similar symptoms as those experienced by humans, Gelzer believed the same high tech but routine procedure would work for her as well as it did for humans, and she was right.

The events that preceded the diagnosis and surgery weren’t anticipated or even related; just two weeks prior, Sophie’s owner, Karen Cortellino, had taken Sophie to have a torn ligament repaired in her left knee at their local veterinarian’s office.

It was when they returned to have the stitches removed at a follow-up visit that Sophie dropped, unconscious. “Everything was great and literally we were just about walking out the door when Sophie collapsed,” Cortellino explained.

When Cortellino was told that Sophie had ARVC, she learned that humans as well as dogs, boxers in particular, are candidates for the disease that strikes approximately one person in every thousand. It may not manifest itself until adulthood when heart muscle tissue deteriorates, which leads to bouts of a rapidly beating heart.

Cortellino also learned about the possibility of cardiac ablation surgery for Sophie. Ablation involves a high energy catheter tip, which “burns tiny portions of damaged heart tissue to restore normal rhythms,”1 according to Penn Today.

The necessary equipment and medical personnel were just down the street, so aspects of the procedure were shared between Gelzer’s veterinary staff and expert physicians at Perelman School of Medicine’s Translational Cardiac Electrophysiology Laboratory. Gelzer explained:

“This collaboration and this close distance between our hospitals allows us to be able to utilize the tremendous access to all this knowledge … And from our experience with Sophie and other dogs to come, we may able to glean information that will be valuable to human medicine. It’s the best of both worlds.”2

What Is ARVC and What Can Be Done?

ARVC is an inherited disease that affects up to a quarter of the boxer breed, but can also strike American bulldogs. When scientists discovered the “breakthrough” more than 10 years ago that ARVC is caused by a gene mutation, a report submitted by the American Boxer Club observed, “Breeders were ecstatic. Finally, Boxers could be tested for the heart disease that oftentimes causes sudden death with no warning signs.”3 Notably:

“ARVC was first documented in Boxers in the early 1970s when sudden death began occurring even in young, healthy adult dogs. Some had fainting episodes during the months preceding their sudden death, while others had no signs anything was amiss … ”4

Prior to publication of the study in Human Genetics in 2010,5 lead investigator Kathyrn M. Meurs, director of the Veterinary Cardiac Genetics Laboratory at Washington State, studied the genetics responsible for the fatal heart disease in boxers for more than a decade. According to Meurs:

“We narrowed down candidate genes until we found a genetic deletion in a gene that produces striatin, a key binding protein that helps hold together cardiac cells … We believe in Boxers that ARVC has an autosomal dominant mode of inheritance with variable penetrance …

Some individuals with the mutation never develop the disease. In human beings, only about 50% of individuals with one of the known genetic mutations actually show the disease. Therefore, although some dogs will have a severe form of the disease and die, some will have a mild, manageable form, and some will never show evidence of the disease.”6

While drugs like sodium channel blockers and beta blockers may moderate the problem, the arrhythmia damage can be too great. Worse, a fatal arrhythmia could happen now or in years to come with little or no warning.

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After Heart Surgery, Sophie Is ‘Back to Her Perky Self’

Cortellino had adopted Sophie eight years earlier, just two weeks after the death of the family’s first dog, also a boxer. Cortellino said she felt more like Sophie rescued her, than the other way around, and that the term “Mommy’s baby” wouldn’t be an overstatement. Cortellino conceded, “She’s just a joy … It was love at first sight.”7

Cortellino, who had medical training in her background, began researching possible treatment options. She looked closely at an implantable cardiac defibrillator (ICD), but when she asked Gelzer about it, she found it to be possible for humans with a similar diagnosis, but not for dogs — not yet anyway. According to Gelzer:

“ICDs are designed to recognize human arrhythmias. But they’re not able to distinguish the normal variation in heart rate that a dog is capable of from a life-threatening arrhythmia.”8

Nervous about allowing her precious dog to undergo a surgery that had never been done on a dog, Cortellino’s son, Alex Peña, had advice that was, if nothing else, philosophical: “Look Mom, at the very least, Sophie is contributing to the possible welfare of other dogs.”9

Before the procedure, described as complex and several hours long, patterns of Sophie’s arrhythmias were collected using a Reveal LINQ implanted just under her skin. The device records a continuous ECG as a loop, and it can store abnormal rhythm data for up to three years. It helped the doctors focus on the exact spots in Sophie’s heart that needed the ablation.

Everything went smoothly and Sophie went home the following day. Among a rather large team of veterinary and medical experts present, both emotionally and physically involved in the procedure, there was relief and elation. As Cortellino was able to report later, “Sophie is back to her perky self.”10

Following her surgery in Philadelphia, for Sophie and dogs like her, there are now three places in the world where ablation surgery can be performed, including Italy and Ohio. It’s costly, but Sophie’s surgery, known as the pilot case study, was made possible due to grants allowing for cardiac mapping and ablation procedures for six more dogs.

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