Why does Barth syndrome cause heart problems?

Barth syndrome is caused by changes in the tafazzin (TAZ) gene, which is also associated with a heart condition called isolated left ventricular noncompaction (LVNC). The tafazzin gene contains instructions for the tafazzin protein, which is located in mitochondria, the energy-producing cells in the body. Many of these cells are found in the heart. Tafazzin protein is involved in altering cardiolipin, which plays a critical role in the mitochondrial inner membrane. Cardiolipin is a lipid (a type of fat) that can only be found in the mitochondria.

Other Questions About Barth syndrome

• Why does Barth syndrome cause heart problems?
• Who else in my family should have testing for Barth syndrome?
• What specialists should I see for Barth syndrome?
• What should parents and siblings of people with Barth syndrome know?
• What should I know about the diet for children with Barth syndrome?