A 7-year-old Texas girl received a new heart Monday, but her older sister is still on the transplant list.

The girls were diagnosed with restrictive cardiomyopathy, a rare heart disease in which the heart doesn’t relax as it should between pumps, so it doesn’t get the necessary amount of blood.

 

The two were put on the transplant list on April 3, 2008. Emily was a higher priority for transplant than her sister because she was in critical condition.

It took five hours for the transplant operation, but everything went well and now the girl has a new heart.

 

Shayde Smith is relieved now that her sister Emily has received a transplant, their mother told the Associated Press. The transplant was a relief for Emily’s father, who said they still had a long road ahead.

 

Therapy for this type of disease is limited. Blood flow is reduced and the amount of blood that would normally enter the heart is blocked in the circulatory system. In time, people who suffer from restrictive cardiomyopathy develop diastolic dysfunction that eventually leads to heart failure. The condition is rare, affecting less than one person in a million.

 

Without a transplant, the chance of survival is 40 to 50 percent one to two years after diagnosis, said Dr. Kristine Guleserian, the pediatric cardiothoracic surgeon who led the team operating on Emily at Children's Medical Center Dallas. Doctors say it is critical for a patient with this condition to receive a transplant.