From neonates to senior citizens, people with life-threatening heart conditions face an increasingly bright future
by Bill Donahue

Although the heart is among the strongest muscles in the human body, it is also prone to profound weakness. In fact, heart disease remains the No. 1 cause of mortality in American men and women, according to the U.S. Centers for Disease Control and Prevention, accounting for approximately 600,000 deaths per year. In the Philadelphia area, however, medical institutions have made great strides to end the heartbreak, both literal and figurative.

In addition to prescriptions for moderate exercise and a heart-healthy diet, the region’s leading medical professionals have added a raft of tools to battle heart disease—namely, improvements in screening technology, pharmacology and minimally invasive surgical techniques. New therapies, or therapies currently in development, have emerged to help people of all ages overcome heart problems and experience an increasingly good quality of life in the process, according to Emil M. deGoma, M.D., medical director of the Preventive Cardiovascular Program for Penn Medicine.

“There is more evidence to suggest that coronary calcium scanning, which came about in the 1990s, is the single best way to refine the risk of heart attack,” he says. “It’s not an expensive test, and it’s a noninvasive test that does a rapid CT scan of the heart to see if there is plaque present.” It has proven particularly effective, he adds, for men ages 45 and older and women 55 and older who have risk factors and are not taking cholesterol medication.

He is also encouraged by the advent of genetic testing and innovative medications known as PCSK9 inhibitors, which have shown promise in significantly reducing “bad” cholesterol and lowering the risk of heart attack. Advances have also come on the surgical side. Open-heart surgery used to be the sole option for patients suffering from severe aortic stenosis—the calcification and narrowing of the aortic valve, which prevents normal blood flow and can cause chest pain, fatigue and shortness of breath. Now, with a procedure known as transcatheter aortic valve replacement (TAVR), high-risk patients can have the valve placed through either a catheter in the leg or the chest wall.

In Bucks County, the Richard A. Reif Heart Institute of Doylestown Hospital began doing TAVR procedures in November 2013, according to David G. Boland, M.D., interventional cardiologist with Doylestown Hospital. “Five years ago, the only thing to do was to take the valve out surgically and put a new valve in,” he says. “When people are in their late 70s, 80s and 90s, some of them likely have a multitude of medical problems, and that makes the surgery a higher-risk procedure. Some of them wouldn’t make it through the operation or they would have significant inability after the procedure.

“With percutaneous heart valves,” he continues, “meaning it goes in through the skin and not a surgical opening—either through the leg with a heart catheterization or an incision made in the ribs and the valve goes through the tip of the heart—the benefit is that people who were too high at risk can be good candidates. The length of stay is usually a little shorter, and in general people recover a little faster.”

Another paradigm shift has come in terms of the way medical centers determine specific treatment options. At Doylestown Hospital, for example, “heart valve teams” including cardiologists, interventional radiologists, cardiothoracic surgeons and a nurse practitioner assemble to discuss a patient’s most appropriate options and assess the risks of surgery.

“We’ll look at CT scans of the chest so we can see what the vessels look like, and we’ll look at what the kidneys’ function looks like,” says Dr. Boland. “Do they have major debilities that would keep them from rehabbing adequately or bad lung disease or bad kidneys that would make them inoperable? The cardiologist will know the [patient] the best and be able know if they get around with a walker or it they out chopping wood every day.”

Of course, TAVR is not without its challenges. In some ways, TAVR may require even more significant planning than open-heart surgery, according to Francis W. Grzywacz, M.D., a cardiologist and director of the echocardiography lab at Doylestown Hospital. With open-heart surgery, the surgeon can choose from differently sized valves and sew in the most appropriate fit. With TAVR, however, the team must perform multiple echocardiograms and other tests to ensure that the valve size is chosen perfectly.

“A lot of patients have been very pleased with the improvement in quality of life [after the TAVR procedure],” says Dr. Grzywacz. “You’ll hear people say, ‘I didn’t realize how short of breath I was.’ Through TAVR, they find they have improved functional capacity, even if it’s just going shopping, doing the cooking and cleaning around the house.

“Cardiology in general is going toward more noninvasive procedures,” he continues. “It’s in its infancy stage now, and it does provide elderly patients a way of improving their quality of life, with a much quicker recovery time. We now have more ways to fix things in a less invasive manner. People will feel better and recover quicker. That’s where the technology is going.”

Beginning Anew
Progress in cardiology has affected outcomes for not only the elderly but also those who are at the very beginning of their lives—or, in many cases, even before birth.

“The exciting thing is the timing of when [problems] can be picked up,” says Jack Rychik, M.D., director of the fetal heart program at the Children’s Hospital of Philadelphia. “We can now see structures in the heart as early as 12 weeks of gestation. … The heart is the size of an eraser tip on a pencil at 12 to 14 weeks, but we can identify within that the different chambers, the different vessels—things that are millimeters in diameter. The heart is done forming at eight weeks of gestation, and I would venture to say that the day we can use technology to [identify congenital heart defects] as early at eight weeks of gestation is only a few years away.

“One out of 100 people in a room will have some form of difference about the heart,” he continues. “The sooner we pick something up, the sooner we can manage it and improve the outcome.”

Dr. Rychik believes cardiology is “at the doorstep” of significant advancements in treating heart problems before birth. The shift has already begun, in fact. He recalls his role on a cardiothoracic surgical team that operated on a fetus through an incision in the mother’s uterus in order to remove a tumor from the fetus’ heart. With the tumor excised, the surgical team returned the fetus to the uterus and the mother carried it successfully to term.

“Over the course of 30 years, our focus has changed,” says Robert E. Shaddy, M.D., chief of the cardiology division at CHOP. “For so many years our goal was to get babies to survive surgery. … Now we’re doing so much better than we were 30 years ago or even 15 years ago. More and more we have focused our energies in areas that include the family and extended family.

“We do deliver babies here in a special delivery unit,” he continues. “If a [problem] is identified in utero and the baby is deemed high risk, it is ideal for when a patient needs something not only before birth but also immediately after birth. It has helped with babies who would otherwise have to be born elsewhere and then brought here later.”

Another example is CHOP’s Single Ventricle Survivorship Program, serving patients born with a condition in which one of the heart’s ventricles is too small or weak to pump blood effectively to the body and/or lungs—essentially, babies born with half a heart. Single-ventricle heart defects were once fatal, though, with early intervention, most children with single-ventricle defects survive. Through something known as the Fontan operation, these patients often undergo a series of surgeries in childhood to reconfigure the heart and circulatory system by establishing a normal direction of blood flow. The program, which Dr. Rychik directs, has attracted families from across the country.

“These are some of our most complex patients, and the treatments we offer them are not cures, and they’re not sufficient solutions for complete normalization,” he says. “Over time, [the Fontan] operation can come at a cost, ultimately causing damage to some other organs, and there may be some neurocognitive issues. This compelled us to put together a multidisciplinary program to focus on these issues, with specialists in a half-dozen areas. It’s a very unique type of patient. These are human beings that never walked the face of the earth before. We don’t know what lies ahead for them.”

Through advances that began as far back as the 1980s, infants today who are born with congenital heart problems have seen survival rates improve dramatically, according to Stephanie Fuller, M.D., cardiothoracic surgeon with CHOP and assistant director of surgery for the Perelman School of Medicine at the University of Pennsylvania. In fact, some patients who were born with congenital heart disease are now of child-bearing age and delivering babies—healthy babies—of their own.

“It’s a nice alternative, and it’s something truly tangible for a lot of women who never thought they would have children,” she says. “With many forms of congenital heart defects, we’ve seen survival rates go from the single digits to as high as 95 percent, which is extremely rewarding.”

Your Guide to Heart Health

A number of elite hospitals and medical centers devoted to cardiology care are based in Philadelphia and the surrounding suburbs. The following institutions have earned the designation as being among the best in the region.

Abington Memorial Hospital

Bryn Mawr Hospital
Bryn Mawr

Children’s Hospital of Philadelphia

Einstein Medical Center Philadelphia

Einstein Medical Center Montgomery

Lankenau Medical Center & Heart Pavilion

Lansdale Hospital

Nemours Cardiac Center at Alfred I. duPont Hospital for Children
Wilmington, Del.

Paoli Hospital

Penn Medicine

The Richard A. Reif Heart Institute of Doylestown Hospital

Riddle Hospital

Temple Heart and Vascular Institute