In the Journals

AHA creates first scientific statement on Fontan circulation

Jack Rychik

As a greater number of patients who had Fontan operations reach adolescence and adulthood, more information on how to manage those patients is needed, according to a scientific statement published by the American Heart Association in Circulation.

The statement is expected to be the first in a series as the knowledge base on the condition grows, study chair Jack Rychik, MD, told Cardiology Today.

“The main reason why it was time for an AHA statement is that we’ve reached a threshold point where we now have a critical mass of human beings today who have survived with a Fontan circulation, individuals who have never before walked the face of the Earth,” Rychik, director of the Fetal Heart Program and the Fontan Rehabilitation, Wells, Activity and Resilience Development Program at Children’s Hospital of Philadelphia, said. “We’ve finally reached a population in the U.S. and around the world where it’s time to create some standards [and] consensus and to allow the cardiology community at large to be aware of this growing success, essentially a miracle of modern medicine. But yet [these patients] have numerous limitations.”

Organ system limitations

In recipients of the Fontan operation, there are CV limitations, but also other organ system limitations, Rychik said.

“The heart is still only half a heart, a single ventricle, there are cardiovascular [and] some physiological challenges that relate to that,” Rychik said. “Few organ systems are spared [as a result of Fontan circulation].”

Some of the physiological challenges include complications with growth and development, challenges and complications with liver scarring, and in older patients, challenges and complications with kidney function, Rychik said.

Complications with neurocognitive development have led to learning challenges in some patients in their childhood and teenage years, he said.

Patients with Fontan physiology face potential psychosocial challenges such as anxiety, stress and depression, Rychik said.

An additional complication of a Fontan circulation is lymphatic insufficiency, which leads to protein-losing enteropathy and plastic bronchitis, Rychik said.

“What it’s resulting in is about a 20-to-25-year survival of about 85%,” Rychik told Cardiology Today. “The glass is either 85% full or 15% empty, depending on your perspective. Again, these are people who would not be alive at all if they did not have this particular surgery.”

Rychik said while the patients have some degree of morbidity, most have a good quality of life.

Rychik said one of the goals of the statement is address the gaps in knowledge while reminding the cardiology and pediatric community to focus on more than the heart.

“Despite the fact that the primary problem is the heart condition, looking at things outside the heart will help improve many aspects of the condition. We hope that will be an important contribution,” he said.

To that end, the authors proposed a surveillance testing toolkit related to all affected organs of those with Fontan circulation.

Improving quality of life

Rychik said with a population of more than 60,000 worldwide with Fontan circulation, 40% of whom are older than 18 years of age, a better understanding of how to improve quality of life is essential.

“That number is only going to increase as we continue to be successful,” Rychik said. “You live more of your life over the age of 18 than you do under the age of 18, if we’re successful and therefore awareness on the part of the cardiology community to this unique situation is really, really important.” – by Earl Holland Jr.

Disclosures: Rychik reports no relevant financial disclosures. Please see the study for all other authors’ relevant financial disclosures.

Jack Rychik

As a greater number of patients who had Fontan operations reach adolescence and adulthood, more information on how to manage those patients is needed, according to a scientific statement published by the American Heart Association in Circulation.

The statement is expected to be the first in a series as the knowledge base on the condition grows, study chair Jack Rychik, MD, told Cardiology Today.

“The main reason why it was time for an AHA statement is that we’ve reached a threshold point where we now have a critical mass of human beings today who have survived with a Fontan circulation, individuals who have never before walked the face of the Earth,” Rychik, director of the Fetal Heart Program and the Fontan Rehabilitation, Wells, Activity and Resilience Development Program at Children’s Hospital of Philadelphia, said. “We’ve finally reached a population in the U.S. and around the world where it’s time to create some standards [and] consensus and to allow the cardiology community at large to be aware of this growing success, essentially a miracle of modern medicine. But yet [these patients] have numerous limitations.”

Organ system limitations

In recipients of the Fontan operation, there are CV limitations, but also other organ system limitations, Rychik said.

“The heart is still only half a heart, a single ventricle, there are cardiovascular [and] some physiological challenges that relate to that,” Rychik said. “Few organ systems are spared [as a result of Fontan circulation].”

Some of the physiological challenges include complications with growth and development, challenges and complications with liver scarring, and in older patients, challenges and complications with kidney function, Rychik said.

Complications with neurocognitive development have led to learning challenges in some patients in their childhood and teenage years, he said.

Patients with Fontan physiology face potential psychosocial challenges such as anxiety, stress and depression, Rychik said.

An additional complication of a Fontan circulation is lymphatic insufficiency, which leads to protein-losing enteropathy and plastic bronchitis, Rychik said.

“What it’s resulting in is about a 20-to-25-year survival of about 85%,” Rychik told Cardiology Today. “The glass is either 85% full or 15% empty, depending on your perspective. Again, these are people who would not be alive at all if they did not have this particular surgery.”

Rychik said while the patients have some degree of morbidity, most have a good quality of life.

Rychik said one of the goals of the statement is address the gaps in knowledge while reminding the cardiology and pediatric community to focus on more than the heart.

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“Despite the fact that the primary problem is the heart condition, looking at things outside the heart will help improve many aspects of the condition. We hope that will be an important contribution,” he said.

To that end, the authors proposed a surveillance testing toolkit related to all affected organs of those with Fontan circulation.

Improving quality of life

Rychik said with a population of more than 60,000 worldwide with Fontan circulation, 40% of whom are older than 18 years of age, a better understanding of how to improve quality of life is essential.

“That number is only going to increase as we continue to be successful,” Rychik said. “You live more of your life over the age of 18 than you do under the age of 18, if we’re successful and therefore awareness on the part of the cardiology community to this unique situation is really, really important.” – by Earl Holland Jr.

Disclosures: Rychik reports no relevant financial disclosures. Please see the study for all other authors’ relevant financial disclosures.