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100Level, Nigeria.
For people born with only one heart ventricle, the Fontan procedure is literally a life saver, but the condition often compromises later quality of living, new research shows.
A normal heart contains two ventricles to pump blood, one to the lungs and the other to the rest of the body. For children born with one ventricle, the Fontan procedure reroutes blood coming back from the veins to the main pulmonary artery connected to the lungs.
The statement, published Monday in Circulation, gives advice on how to deliver follow-up care for patients with Fontan circulation. This includes how to test for cardiovascular and other organ malfunction. The paper also highlights what knowledge gaps exist in the medical field of Fontan patients.
"We need more research into the basic biology of single ventricle hearts and whether the damage to other organ systems, such as kidney, liver and brain can be mitigated or reversed," Jack Rychik, a researcher at Children's Hospital of Philadelphia, said in a news release.
For Fontan patients, less blood pumps from the heart, leading pressure to build up in their veins. This poor efficiency can cause circulatory failure due to lack of oxygen circulating to cells.
"We are entering a new phase in the management of patients born with one ventricle. Provided that patients undergo regular follow-up with their healthcare provider, adopt a healthy lifestyle and are encouraged to participate in investigational clinical protocols and research, healthcare providers and patients can share an optimistic and hopeful view for a brighter future," Rychik said.
Fontan patients often suffer heart failure, heart rhythm disturbances, along with bone, kidney and liver dysfunction.
An American Heart Association estimate put the global number Fontan patients at 70,000, and about 40 percent were older than age 18. But many of those patients won't likely go on to live normal lives.
Fontan patients have lower life spans than people with normal hearts. A 2015 study showed the 30-year survival rate for Fortran patients was 43 percent, according to another study.
"Patients with Fontan circulation are going to consume an ever-increasing amount of resources as they grow in number and age into adult life. Healthcare providers, both pediatric and adult, will need to increase their understanding and knowledge of this unique cardiovascular condition in order to maintain and improve their quality of life," Rychik said.

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